MASTER THE BOARDS

Internal Medicine
Board Review
Course Book 2013–2014

IM 4050 E

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 Master the Boards Internal Medicine Board Review Course Book
2013–2014

©2013 Kaplan, Inc.

All rights reserved. No part of this book may be reproduced or transmitted in any form
or by any means, electronic or mechanical, including photocopying, recording, or by
any information storage and retrieval system, without the written permission of the
Publisher, except where permitted by law.

Not for resale.

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 “I learned this, at least, by my experiment: That if one advances confidently
in the direction of his dreams, and endeavors to live the life which he
has imagined, he will meet with a success unexpected in common hours.
He will put some things behind, will pass an invisible boundary; new,
universal, and more liberal laws will begin to establish themselves around
and within him; or the old laws be expanded, and interpreted in his favor
in a more liberal sense, and he will live with the license of a higher order of
beings. In proportion as he simplifies his life, the laws of the universe will
appear less complex, and solitude will not be solitude, nor poverty poverty,
nor weakness weakness. If you have built castles in the air, your work
need not be lost. Now put the foundations under them.”

—Thoreau, Walden

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 We’d like to know what you think. Please share your
feedback with us at [email protected].

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 AUTHORS

Conrad Fischer, M.D.

Director of Educational Development
Jamaica Hospital Medical Center
Queens, New York

Associate Professor of Medicine, Physiology, and Pharmacology

Touro College of Medicine
New York, NY

Niket Sonpal, M.D.

Chief Resident
Lenox Hill Hospital, NSLIJ Health System

Assistant Clinical Professor

Touro College of Medicine
New York, NY

Scott Tenner, M.D., M.P.H.

Director, Medical Education and Research
Division of Gastroenterology
Maimonides Medical Center

Professor of Medicine,
State University of New York – Health Sciences Center
Brooklyn, New York

Chris Paras, D.O.

Endocrinology Faculty
Winthrop University Hospital

Assistant Professor of Medicine

Touro College of Osteopathic Medicine and Frank H. Netter School of Medicine
Quinnipiac University
New York, NY

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Internal_medicine.indb 6 5/2/13 1:51 PM
 Contents

Chapter 1. Cardiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1

Chapter 2. Gastroenterology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 65

Chapter 3. Neurology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 115

Chapter 4. Nephrology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 167

Chapter 5. Endocrinology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 215

Chapter 6. Hematology/Oncology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 271

Chapter 7. Pulmonology/Critical Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 337

Chapter 8. General Internal Medicine . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 387

Chapter 9. Dermatology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 411

Chapter 10. Rheumatology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 419

Chapter 11. Infectious Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 467

Chapter 12. Statistics/Preventive Medicine/Ethics . . . . . . . . . . . . . . . . . . . . . . 531

vii

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Internal_medicine.indb 8 5/2/13 1:51 PM
 Cardiology
1
ISCHEMIC HEART DISEASE

Case 1
44-year-old woman in office with intermittent substernal chest pain for last several
weeks. Sometimes with exertion, sometimes at rest, no fixed pattern. She also
complains of nausea. No past medical history.

1. Most common risk for coronary artery disease?

2. Worst risk factor for coronary disease?

3. What three features of chest pain on history or physical on the Boards tell you for sure
the pain is not ischemic in nature?

4. Her initial EKG is normal. Next step in management?

A. CKMB
B. Troponin
C. EKG
D. Stress
E. Holter

Answer:

5. What in the question will tell you to answer “a thallium (nuclear) stress test”?

6. When is a dipyridamole, adenosine, or dobutamine stress test the answer?

7. What is the most accurate means of determining if she has Prinzmetal angina?

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 IM Board Review Course Book

Summary: Case 1
The most common risk factor for coronary artery disease is hypertension. The other clear
risk factors are diabetes, hyperlipidemia, an HDL <40, tobacco smoking, and a family history
of premature coronary disease (age <55 in men, <65 in women). Although obesity is a gen-
eral reason for an increase in all cause mortality, it is not a distinct risk for coronary disease
by itself separate from the hypertension, diabetes, and hyperlipidemia that it causes. Many
causes of chest pain that are not cardiac in nature are dyspnea, diaphoresis, and anxiety. The
main ways to distinguish chest pain as definitely not being cardiac in nature on a board test
are: pain that changes with position and/or respiration or pain associated with chest wall
tenderness. When the patient cannot exercise, then you must use a pharmacologic method
of simulating exercise. The dipyridamole (or adenosine) stress test and the dobutamine echo
have the same sensitivity and specificity. The way to detect ischemia on a standard exercise
tolerance test is by looking for ST-segment depression as you achieve >80% of the maximum
heart rate. If the patient already has significant abnormalities of the ST segment on a baseline
EKG, then you cannot use standard exercise stress testing to make your diagnosis. When the
EKG has these abnormalities, your answer should be either a stress test using thallium or
sesta-MIBI or echocardiography. All of these methods have the same sensitivity and specific-
ity, and you cannot be asked to choose between them.

Prinzmetal angina occurs in younger patients who are often tobacco smokers who complain
of chest pain that has no fixed relationship to exercise. The pain often occurs at rest and
awakens the patient from sleep. Although these patients have ST-segment elevation, this find-
ing by itself is not specific enough to diagnose Prinzmetal. If you see a case with ST-segment
elevation, you must first treat the patient as if he had an acute infarction. The diagnosis
can be specifically made only by coronary angiography. Stimulation of the vasospasm with
acetylcholine helps confirm the diagnosis. Treatment of Prinzmetal is with nitroglycerin and
calcium channel blockers. Beta blockers should be avoided.

1. Which is a contraindication to dipyridamole-thallium testing?

A. Unilateral amputee
B. Asthma
C. Diabetes
D. Peripheral arterial disease
E. Obesity

Answer:

2. Patient with duodenal ulcer by scope two months ago. Been on PPI for 2 months. Now
with chest pain.
A. Aspirin yes
B. Aspirin no

Answer:

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 Cardiology

Case 2
44-year-old woman in office complaining of intermittent substernal chest pain
for several weeks. The pain happens sometimes with exertion and sometimes at
rest, no fixed pattern. Also complains of nausea. Stress test is abnormal. No past
medical history.

1. What is the next step in management?

A. Aspirin
B. Angiography
C. Echocardiogram
D. Nitrates
E. Metoprolol

Answer:

2. What will you do next for each of these? Note

Unstable Angina
LDL: 191 LDL: 161 LDL: 131 LDL: 101
• New pain
A. Diet and exercise • Rest pain
B. Statin • Worse pain
C. Niacin
D. Cholestyramine
E. Ezetimibe

Answer:

3. Coronary artery disease equivalents?

4. Most common adverse effect of statins?

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 IM Board Review Course Book

Summary: Case 2
This patient has an abnormal stress test, implying that the most important step is to start
therapy for ischemic heart disease. The most common error with a case like this is to answer
“Angiography” after the abnormal stress test. Although the angiogram is important in deter-
mining whether the stress test is a true positive, as well as determining whether coronary
bypass is necessary, this test is not as important as starting therapy. You should emphasize the
medications that will potentially impact mortality, such as aspirin and beta blockers. Nitrates
should be given to any patient who has ischemic chest pain. Calcium channel blockers should
be used for those who cannot tolerate beta blockers.

Note This patient should be started on a statin if the LDL is >100 mg/dL. These drugs are consid-
ered optional with an LDL between 70 and 100 mg/dL if there is CAD and continued smok-
Nuclear Isotopes ing or diabetes. If two or more risk factors are present, then your answer should be statins if
lifestyle modifications cannot get the LDL <130 mg/dL. The fact that this patient has no risk
• Thallium
factors for ischemic disease does not matter because she has an abnormal stress test. The same
• Sestamibi is true if she has diabetes, aortic disease, carotid disease, or peripheral arterial disease. These
• Teboroxime are all considered the equivalent of having coronary disease. If there is no coronary disease
or its equivalent, then you must look at the number of risk factors. If there is only one risk
factor (such as hypertension, HDL <40 mg/dL, tobacco smoking, premature family history
of disease, or age >45 in men, >55 in women) then you do not start statins until the LDL is
above 190 mg/dL. If two of these risk factors are present, then statins are started above an
LDL of 160 mg/dL. Although niacin, cholestyramine, ezetimibe, and fibric acid derivatives
such as gemfibrozil all lower LDL and raise HDL, they do not have as much of an effect on
reducing mortality as do the statins.

1. A 54-year-old man with a history of diabetes comes to the office for routine manage-
ment. He is found to have an LDL of 137. His stress test is normal. What is the goal of
therapy for his LDL?
A. <70
B. <100
C. <130
D. <160
E. <190

Answer:

2. Which has greatest risk of myositis when used in combination with a statin?
A. Cholestyramine
B. Niacin
C. Ezetimibe
D. Fibric acid derivatives

Answer:

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 Cardiology

Case 3
68-year-old man comes to emergency department (ED) of a small rural hospital
that has no cath lab with 1 hour of crushing substernal chest pain after shoveling
snow. The pain radiates to arm and jaw. He is sweating and nauseated. History of
hypertension. Smokes cigarettes. EKG: 2 mm ST elevation V2–V4.

1. Best initial step?

A. Oxygen
B. Nitrates and morphine
C. Aspirin
D. Thrombolytics
E. Metoprolol
F. Transfer for angioplasty

Answer:

2. What will you do if these treatments do not lead to resolution of the pain (i.e., if the
best initial therapy fails)?

3. When are calcium blockers the answer?

4. When is lidocaine or amiodarone the answer?

5. Most accurate diagnostic step at this time?

A. CK-MB
B. Troponin
C. Myoglobin
D. LDH

Answer:

6. What is the most common dysrhythmia to occur with reperfusion, and how will you
treat it?
A. Atrial fibrillation
B. Atrial flutter
C. Accelerated idioventricular rhythm
D. Ventricular fibrillation
E. Ventricular tachycardia
F. Asystole

Answer:
5

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 IM Board Review Course Book

7. When is coronary artery bypass grafting (CABG) the answer?

A. Left anterior descending lesion of >50%
B. Right coronary of >70%
C. Three vessels >70% stenotic
D. Right coronary >70% and circumflex >70%

Answer:

Summary: Case 3
The most important point to take into account on the examination is which measures will
lower mortality in an infarction. These are aspirin, angioplasty, thrombolytics, beta blockers,
ACE inhibitors if the ejection fraction is <40%, statins if the LDL is >100 mg/dL, and clopi-
dogrel. Although clopidogrel is effective, this drug is currently recommended as the standard
of care only if the patient cannot tolerate aspirin. Ventricular dysrhythmias developing within
48 hours of an acute infarction have no effect upon mortality. Calcium channel blockers
have no definite effect upon lowering mortality after a myocardial infarction. They should
be used only if the patient cannot tolerate beta blockers, such as in the case of severe asthma.
Prophylactic anti-arrhythmic medications such as lidocaine do not offer a survival advantage
as compared with simply monitoring the patient for serious ventricular arrhythmias and
treating them as they arise. This is probably because lidocaine is occasionally pro-arrhythmic.
Within the first 4 hours after the start of chest pain, the only enzyme that is routinely elevated
is the myoglobin. A normal myoglobin at 4 hours has enormous negative predictive value,
although it is nonspecific if it is elevated. The troponin offers greater specificity as compared
with CK-MB levels. The troponin will also remain elevated for up to 2 weeks after an infarc-
tion, particularly if there is renal insufficiency. The most common arrhythmia after the use of
thrombolytics is an accelerated idioventricular arrhythmia, but it does not need to be treated.
A coronary artery bypass is used in the management of an acute myocardial infarction only
if all the other modalities, including angioplasty, have been used and have been ineffective.

In general, angioplasty offers a greater survival advantage in the management of ST-segment

elevation infarctions. Thrombolytics are preferred when the patient presents within the first
3 hours of the onset of chest pain or if the question indicates that the hospital is a small one
that does not offer emergency angioplasty. If the patient presents after 3 hours, then transfer
to a hospital where angioplasty is possible is preferred as long as the procedure can be per-
formed within 2 hours.

1. Man comes with substernal chest pain and ST-segment elevation in II, III, aVF. He has
received aspirin and metoprolol, and has undergone angioplasty. Which is most likely
to lower his mortality?
A. Oxygen
B. Nitrates
C. ACE inhibitors
D. Statins
E. Morphine
F. Lidocaine

Answer:

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 Cardiology

Case 4
68-year-old man comes to ED with 1 hour of crushing substernal chest pain after
shoveling snow. The pain radiates to his arm and jaw. History of hypertension and
smokes. EKG shows 2 mm of ST elevation in V2–V4. Aspirin, metoprolol, and
atorvastatin have been started.

1. What will benefit the patient most?

A. Prasugrel
B. Spironolactone
C. Abciximab
D. Implantable defibrillator
E. Nitroglycerin

Answer:

Summary: Case 4
Absolute contraindications to the use of thrombolytics consist of a recent history (1 year)
of nonhemorrhagic stroke or head trauma, intracranial mass, intracranial bleeding at any
time in the past, active serious bleeding such as melena, or a risk of serious bleeding such as
an aortic dissection. Relative contraindications to the use of thrombolytics are diabetic reti-
nopathy (particularly with hemorrhages), pregnancy, a significantly elevated blood pressure
above 180/110 mm Hg, active peptic ulcer disease, or if the patient uses warfarin and has an
INR >2. An example of minor bleeding in which thrombolytics can still be used would be
menstruation or heme-positive brown stool.

Although primary angioplasty is superior to thrombolytics if it can be performed rapidly

(<90 minutes after the patient’s arrival in the emergency department), the standard of care in
the United States can still be considered thrombolytics if the patient presents within the first
3 hours after the onset of chest pain and there is no technically qualified angioplasty facility.
Only 20% of hospitals in the country can do primary angioplasty. The strongest indication
for the use of angioplasty with the initial management of a myocardial infarction is in those
patients with a contraindication to the use of thrombolytics and in those in whom thrombo-
lytics fail to result in reperfusion. Angioplasty is also superior to thrombolytics if the patient
arrives in the emergency department several hours after the start of pain.

All patients with acute myocardial infarction should have either clopidogrel or prasugrel
added to treatment. This is true even if angioplasty and stenting are not to be performed.
Prasugrel has a greater risk of bleeding compared with clopidogrel.

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 IM Board Review Course Book

1. 64-year-old man comes to ED 1 hour after he had 40 minutes of substernal chest pain.
He had a nonhemorrhagic stroke 6 weeks ago and currently has a blood pressure of
182/108 mm Hg. EKG shows ST elevation in V2–V4. He has received aspirin and oxy-
gen. Which is most appropriate in his management?
A. Thrombolytics now
B. Transfer for angioplasty
C. Nitrates
D. Coronary bypass surgery
E. Add metoprolol and ACE inhibitors only
F. Add metoprolol and ACE inhibitors, then give thrombolytics
Answer:

Case 5
68-year-old man comes with 1 hour of crushing substernal chest pain after
shoveling snow. He has a history of hypertension and smokes. His initial EKG
shows a new left bundle branch block. You are not able to determine if there is
ST-segment elevation.

1. What will you do next?

A. Thrombolytics
B. Abciximab
C. Tirofiban
D. Enoxaparin
E. Argatroban

Answer:

2. The patient undergoes reperfusion. Pain resolves. The next troponin several hours later
is higher. What to do?
A. Re-treat with thrombolytics
B. Catheterize/PCI
C. Nothing

Answer:

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 Cardiology

Summary: Case 5
Cocaine blocks the reuptake of norepinephrine at the synapse. This results in the accumula-
tion of norepinephrine with its powerful alpha-agonist activity. Cocaine-induced chest pain
should be managed with the use of benzodiazepines and nitrates. Calcium channel block-
ers are sometimes used. It is important to avoid the use of beta blockers. When you block
beta stimulation, you have an unopposed alpha activity, resulting in elevated blood pressure
and myocardial work. Thrombolytics would be used only if there was an angiographically
confirmed clot. The indication for the use of thrombolytics is the presence of at least 1 mm
of ST-segment elevation in at least 2 electrically contiguous leads on the EKG in a patient
presenting within 12 hours after the onset of chest pain. The other indication for the use
of thrombolytics is the presence of a left bundle branch block (LBBB) that is not definitely
known to be old.

1. Which decreases, the risk of atrial fibrillation most in coronary bypass surgery?
A. Propranolol
B. Digoxin
C. Verapamil
D. Diltiazem
E. Ramipril

Answer:

2. 24-year-old man comes with 30 minutes of chest pain after injecting cocaine. EKG is
normal. What would you order first?
A. Diltiazem
B. Metoprolol
C. Thrombolytics
D. Angioplasty
E. Angiography

Answer:

3. Which of the following will most likely experience a benefit in mortality from calcium
channel blockers?
A. Hypertensive
B. Asthmatic with coronary disease
C. Person with cocaine-induced chest pain
D. Person with Prinzmetal’s (variant) angina

Answer:

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 IM Board Review Course Book

Case 6
68-year-old man comes to ED with 1 hour of crushing substernal chest pain
radiating to his arm and jaw. He has never had chest pain before. EKG has 1 mm
of ST depression (or normal) in V2–V4. Aspirin and clopidogrel have been started.

1. Next step?
A. Ticlopidine
B. IV unfractionated heparin
C. Low molecular weight heparin
D. Argatroban

Answer:

2. His pain persists despite this. What will you do next?

A. Thrombolytics
B. Intra-aortic balloon pump
C. Angiography
D. Check troponin

Answer:

Summary: Case 6
Glycoprotein IIb/IIIa inhibitors, such as eptifibatide and tirofiban, are antiplatelet medications
that are best used in the management of severe cases of unstable angina. Their mortality ben-
efit is best in those patients with a high risk for progression, such as those with ST-segment
abnormalities, elevation in troponin levels, persistent pain; and in those with signs of conges-
tive failure such as rales, an S3 gallop, and a new or worsening murmur of mitral regurgitation.
Clopidogrel should be added to those patients with non–ST-segment elevation myocardial
infarctions who develop positive troponin levels.

10

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 Cardiology

Case 7
68-year-old man was admitted to your CCU earlier this evening with an inferior
wall myocardial infarction. He received tPA, beta blockers, aspirin, nitrates, oxygen,
morphine, and ACE inhibitors. You are now called because he has suddenly become
confused. Blood pressure 70/40 mm Hg, pulse 40/min, respirations 16/min, and
temperature 37.7°C (100°F) orally. His lungs are clear and he has canon “a” waves in
his neck. He has a 3/6 systolic murmur, his extremities are nonedematous, and he is
confused and disoriented on examination.

1. Most likely diagnosis?

2. Best initial therapy?

Summary: Case 7

When you see THIS Then THIS is the Answer THIS as the And THIS as the
in the case . . . diagnosis test treatment

11

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 IM Board Review Course Book

Canon a waves are the exaggerated neck vein impulse caused by the contraction of the atrium
against a closed tricuspid valve or against any form of increased right ventricular pressure
or obstruction to flow. Canon a waves are found in complete heart block, ventricular tachy-
cardia, ventricular pacemaker, tricuspid or pulmonic stenosis, pulmonary hypertension, and
with SVT or junctional rhythms.

Absent a waves: Atrial fibrillation

Prominent x descent: Cardiac tamponade or constrictive pericarditis
Absent x descent: Right ventricular infarction
Prominent v wave: Tricuspid regurgitation
Prominent y descent: Constrictive pericarditis

Right ventricular infarction complicates half of all cases of inferior wall (IW) myocardial
infarctions. The clue to the diagnosis is the development of sudden hypotension in someone
with IWMI. There will also be increased jugulovenous pressure (JVP) and clear lung fields.
Kussmaul’s sign (increasing JVP on inspiration) may also be present. The test is to do an EKG
with right ventricular leads showing ST elevation. With hemodynamic monitoring, the right
atrial pressure will be higher than the wedge pressure. The treatment is almost exclusively
with fluids in addition to the usual medications for the treatment of myocardial infarction.

All of the complications of an acute myocardial infarction can present with hypotension. Only
third-degree heart block will give you bradycardia, hypotension, and canon A waves in the
neck. The best initial management of third-degree or complete heart block is with atropine if
the patient is hypotensive. Although atropine may not help all patients, it is faster to administer
and far more comfortable than a transcutaneous pacemaker. Even a hemodynamically stable
patient with third-degree heart block must eventually be treated with a transvenous pacemaker.

Acute valve rupture can present with hypotension but should also result in tachycardia and
congestion on lung examination. If the patient is not hypotensive, then afterload reduction
with ACE inhibitors or nitroprusside should be used. If the patient has such severe disease
that these medications cannot be used or are ineffective, then the patient should have an
intra-aortic balloon pump placed in preparation for valve repair surgery.

1. 68-year-old man admitted with inferior wall myocardial infarction earlier this evening.
He develops sudden onset of hypotension, tachycardia, distended neck veins, and clear
lungs. No murmurs. BP unchanged during inspiration. He is given a bolus of normal
saline. Next step in this patient?
A. Right ventricular leads
B. Echocardiography
C. Swan-Ganz (right heart) catheterization
D. Pericardiocentesis
E. Surgical intervention

Answer:

12

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 Cardiology

2. 72-year-old man admitted to hospital last week because of anterior wall myocardial
infarction. He received aspirin, nitrates, metoprolol, atorvastatin, and trandolapril.
On his fifth hospital day he develops recurrent chest pain and a new murmur at his
left sternal border. Right heart catheterization shows an oxygen saturation in the right
atrium of 75% and a saturation in the pulmonary artery of 92%. What is the most
likely diagnosis?
A. Tricuspid valve rupture
B. Mitral valve rupture
C. Ventricular septal rupture
D. Atrial septal rupture
E. Aortic valve rupture

Answer:

3. Which of these increases with inspiration?

A. Mitral regurgitation
B. Mitral stenosis
C. Tricuspid regurgitation
D. Aortic stenosis
E. Aortic regurgitation

Answer:

Case 8
68-year-old man transferred from CCU 5 days ago after myocardial infarction
becomes part of your service. No further chest pain or discomfort for last several
days. Now ready for discharge.

1. What will you do prior to discharge?

A. Echocardiogram
B. Holter monitor
C. Electrophysiology studies
D. Exercise tolerance test

Answer:

2. Is angiography necessary?

3. When will you answer coronary artery bypass grafting?

A. Circumflex 99%, right coronary 85%
B. Left main 40%
C. Right coronary 90%, LAD 90% in a diabetic
D. Circumflex 70% and left main 30%

Answer:

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 IM Board Review Course Book

4. Which will benefit him most?

A. Tobacco cessation
B. Pneumococcal vaccine
C. Weight loss
D. Relaxation methods
Answer:

5. The patient’s wife asks you if the patient is capable of having sex. What do you tell her?

Summary: Case 8
Stress testing prior to discharge is generally indicated in all patients after a myocardial infarc-
tion. A submaximal stress test can be performed at 5 days post-myocardial infarction or a
maximal stress test at 2 weeks post-myocardial infarction. If ischemia is found, the patient
should be offered angiography to see if coronary bypass will potentially lower mortality. If a
patient continues to have ischemia postinfarction, there is no need to do the stress test and
he should go straight to angiography. Coronary artery bypass grafting is the definite answer
when the patient has left main coronary artery disease, or three-vessel disease. These are the
circumstances in which CABG has been most clearly shown to lower mortality. The other
indications for CABG are two-vessel disease with severely impaired left ventricular function
and diabetics with two-vessel disease or worse. In general, all patients postinfarction should
be discharged on aspirin, beta blockers, nitrates, and ACE inhibitors. The ACE inhibitor can
be stopped at 6 weeks if there is no evidence of left ventricular systolic dysfunction.

1. Patient is being discharged after myocardial infarction. What will you discharge him on?
A. Aspirin, clopidogrel, beta blockers, ACE inhibitors, statins
B. Beta blockers, ACE inhibitors, warfarin
C. Aspirin alone
D. Aspirin, beta blockers, warfarin, prasugrel

Answer:

2. What condition will cause orthodeoxia (desaturation while standing or sitting up)?
A. Hepatopulmonary syndrome
B. Ventricular septal defect
C. Diaphragmatic paralysis
D. Pulmonary embolism
E. ARDS

Answer:

3. Which of the following decreases level of digoxin in blood?

A. Amiodarone
B. Verapamil
C. Amoxicillin
D. Cholestyramine

Answer:
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 Cardiology

4. You are starting a patient on amiodarone for atrial fibrillation. The patient is already on
digoxin and warfarin. What would you recommend?
A. Increase digoxin dose and decrease warfarin dose
B. Increase digoxin dose and increase warfarin dose
C. Decrease both digoxin and warfarin dose
D. Decrease digoxin dose and increase warfarin dose

Answer:

Hepatopulmonary syndrome is a complication of cirrhosis characterized by hypoxia and

orthodeoxia. This is a right to left intrapulmonary shunt. It is lung disease and hypoxia on
the basis of liver disease. Amiodarone will inhibit the metabolism of both digoxin and war-
farin. When starting amiodarone, the doses of both digoxin and warfarin will likely need to
be decreased.

Case 9
68-year-old man with angina is about to undergo coronary angiography. He has
history of rash with use of dye in past.

1. What do you use to decrease the risk of an allergic reaction?

A. Steroids
B. Epinephrine
C. Diphenhydramine
D. Hydrocortisone and diphenhydramine
E. Loratadine

Answer:

2. A patient is about to undergo coronary artery bypass grafting. He will have a sternoto-
my and extra-corporeal cardiopulmonary bypass. What is the most common complica-
tion from this procedure?
A. Stroke
B. Sternotomy wound infection
C. Neurocognitive dysfunction
D. Myocardial infarction

Answer:

Summary: Case 9
The most effective way to prevent contrast induced hypersensitivity reactions is to administer
steroids and antihistamines. The most common complication of cardiopulmonary bypass,
which is necessary for coronary artery surgery, is neurocognitive dysfunction. This is a subtle
decrease in mental function that can be difficult to detect. The presumed mechanism of this
dysfunction is impaired cerebral perfusion. There is no abnormality found on imaging of the
brain because it is not a stroke.
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 IM Board Review Course Book

CONGESTIVE HEART FAILURE

Case 1
64-year-old woman with history of myocardial infarction comes to ED with
acute onset of shortness of breath over several hours. She can’t remember what
medications she is supposed to be using. She has a bag of potato chips in one
hand and a slice of pepperoni pizza in the other. She has rales to her apices, an
S3 gallop, pedal edema that extends to her areola, ascites, and jugulovenous
distension.

1. What will you do first for her?

Oxygen, diuretics, sit the patient upright; morphine, nitrates

2. She is still short of breath. What will you do now?

A. Digoxin
B. Diltiazem
C. Dobutamine
D. Metoprolol

3. What is the most accurate means of assessing her ejection fraction?

4. What will you do differently if labs show her creatinine is 2.3 (normal 0.4–1.1)?

5. When are angiotensin II receptor blockers the answer?

When the ACE inhibitors cannot be tolerated because of adverse effects, such as cough

6. What tests will make a difference in her acute management?

A. Chest xray
B. Echocardiogram
C. Nuclear ventriculogram
D. EKG
E. BNP

Two days later, the patient has completely improved. She is 10 lb lighter and
has no dyspnea.

7. What will you discharge her on?

A. ACE, beta blocker, spironolactone, diuretic
B. ACE, hydralazine, diuretic
C. ARB, spironolactone, digoxin
D. Digoxin, furosemide, ACE

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 Cardiology

Summary: Case 1
S3 gallop certainly indicates congestive failure. What should you think of as the answer to
“What is the most likely diagnosis?” in other circumstances? An S3 gallop can be considered
normal in a person under the age of 30. An S3 also occurs with mitral regurgitation and tri-
cuspid regurgitation.

Widely split S2 is found with mitral regurgitation, ventricular septal defects, right bundle
branch block, and right ventricular volume or pressure overload such as in left-to-right
shunts, pulmonic stenosis, and pulmonary hypertension.

The majority of patients with acute pulmonary edema or a decompensation of congestive

failure will respond to preload reduction and oxygen. Preload reduction is best accomplished
with intravenous diuretics, morphine, and nitrates. This therapy would be the same in sys-
tolic as well as diastolic dysfunction. A small number of patients with systolic dysfunction
will need acute therapy with intravenous dobutamine as an inotrope. Long-term therapy
with the positive inotrope milrinone will only increase mortality and should not be used.
This management as well as the use of ACE inhibitors does not differ in the setting of renal
insufficiency. A creatinine as low as 2.3 in particular is not a contraindication to any of these
medications. The shortness of breath and hypoxia are far more dangerous to patients than a
theoretical risk of worsening renal insufficiency. Angiotensin receptor blockers such as losar-
tan, valsartan, irbesartan, candesartan, or telmisartan are specifically the best answer when
patients cannot tolerate the long-term use of ACE inhibitors because of adverse effects, such
as cough.

Systolic dysfunction is best treated with ACE inhibitors and beta blockers because these are
the two medications with the greatest mortality benefit. Spironolactone also lowers mortality.
Diuretics are used in any patient with fluid overload, be it secondary to systolic or diastolic
dysfunction. For those who are still symptomatic from systolic dysfunction despite these
therapies, digoxin is used in order to decrease the rate of hospitalization and frequency of
acute exacerbations and decompensation.

Diastolic dysfunction is best treated with beta blockers. Digoxin has no benefit. ACE inhibi-
tors are of less certain benefit.

1. Which is associated with widely split S2?

A. Left bundle branch block
B. Aortic stenosis
C. Right bundle branch block
D. Eisenmenger phenomenon
E. Hypertrophic obstructive cardiomyopathy
Answer:

2. An 18-year-old patient is seen for routine examination before playing basketball. There
is paradoxical splitting of S2 heard at the base and no murmur. What is the diagnosis?
A. Left bundle branch block
B. Right bundle branch block
C. Pulmonic stenosis
D. Mitral regurgitation
Answer:
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 IM Board Review Course Book

Case 2
36-year-old multiparous woman comes to office a few weeks after the delivery of
her last child. She has developed progressive shortness of breath with rales and
an S3 gallop. Chest x-ray shows pulmonary vascular congestion with an enlarged
heart. Echo: dilated heart, diminished systolic function throughout. No history of
coronary artery disease, hypertension, alcohol use, or valvular heart disease.

1. Most likely diagnosis?

2. Best initial therapy?

A. Lisinopril
B. Furosemide, lisinopril
C. Spironolactone, lisinopril, carvedilol, furosemide
D. Carvedilol, furosemide

Answer:

3. What is the best long-term therapy for this patient, and what is her prognosis?
A. Beta blocker, spironolactone
B. Furosemide, biventricular pacemaker
C. If ejection fraction worsens, proceed to transplantation
D. Hydralazine

Answer:

Summary: Case 2
Peripartum cardiomyopathy is diagnosed in any patient presenting with congestive failure
in her third trimester of pregnancy or within the first 6 months postpartum after excluding
other causes of congestive failure. This is exclusively a systolic dysfunction with a dilated
cardiomyopathy. The management is the same as systolic dysfunction in general, except that
ACE inhibitors cannot be given during pregnancy because of potential teratogenicity. If the
heart returns to normal size, as it often does eventually, then the prognosis is very good. If the
heart remains dilated, then only a transplantation will eventually solve the problem.

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 Cardiology

1. 44-year-old man comes with congestive failure from idiopathic dilated cardiomyopathy.
He has a left bundle branch block pattern with QRS duration of 140 m/sec. Echo shows
left ventricular enlargement with an ejection fraction of 22%. He is already on an ACE Note
inhibitor, spironolactone, and carvedilol. What will benefit him most?
Nesiritide does not lower
A. Add hydralazine and isosorbide dinitrate mortality.
B. Biventricular pacemaker
C. Implanted cardioverter
D. Both biventricular pacemaker and implanted cardioverter

Answer:

2. 63-year-old woman confused and disoriented. Pulse 110, BP 80/60 mm Hg, and a tem-
perature 37.2°C (99°F) The hematocrit is 27%, WBC 12,000, BUN 40, creatinine 1.8,
with normal chest x-ray. The Swan-Ganz catheter shows: right atrium 4 (nl 2–8), right
ventricle 20/4 (nl 20–25/6–12), wedge 6 (nl 6–12), cardiac output 6 L/min (nl 3.6–5.2),
and peripheral resistance 325 (nl 700–1,600). What is the most likely diagnosis?
A. Massive pulmonary embolism
B. Cardiac tamponade
C. Septic shock
D. Hypovolemic shock

Answer:

3. BNP is elevated in:

A. Diastolic dysfunction
B. Systolic dysfunction
C. Right heart failure from COPD
D. Pulmonary emboli
E. All of the above

Answer:

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 IM Board Review Course Book

VALVULAR HEART DISEASE

Introduction to Auscultation

AV valves: Holosystolic murmur

S S

V valves: Mid-systolic murmur

AV valves: Late systolic murmur

AV valves: Mid-diastolic murmur

S1 S2

AV valves: Late-diastolic murmur

V valves: Early diastolic murmur

Continuous murmur

Case 1
27-year-old woman comes to office for mild dyspnea. She emigrated from South
America several years ago. Respiratory rate 22/min, jugulovenous distention, and
bilateral basilar rales. Cardiac examination reveals a loud S1 and a high-pitched
sound in diastole immediately followed by a diastolic murmur at the apex.

1. Most likely diagnosis?

2. What is the best initial test? What is the most accurate diagnostic test?
Echocardiogram is always first. Cardiac angiography is more accurate and can give direct
pressure measurements.

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 Cardiology

3. What is the best initial therapy?

Several months later the woman returns because of worsening dyspnea despite
therapy. She is now 6 months pregnant.

4. What feature on cardiac exam would be indicative of worsening disease?

A. Loud S1
B. Systolic murmur
C. Opening snap earlier
D. Opening snap later

Answer:

5. What is the best therapy for her now?

A. Termination of pregnancy
B. Mitral valve replacement
C. Mitral balloon valvotomy now
D. Mitral balloon valvotomy after delivery

Answer:

6. What happens to blood return to heart with Valsalva maneuver?

A. Increased thoracic pressure increases return
B. Increased thoracic pressure decreases return
C. Decreased thoracic pressure increases return

Answer:

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 IM Board Review Course Book

Summary: Case 1
Loud S1 is caused by mitral stenosis, a short PR interval such as from WPW, tachycardia, and
thyrotoxicosis. Anything that results in a premature (early) closure of the mitral valve makes
the S1 loud.

All forms of valvular heart disease present with symptoms of congestive failure when they
finally become symptomatic. Mitral stenosis (MS) is especially characterized by the compli-
cations of having an enlarged atrium. These complications are atrial fibrillation, recurrent
emboli, dysphagia from the pressure of the enlarged atrium against the esophagus, hoarse-
ness from pressure on the recurrent laryngeal nerve, and jugulovenous distension. The echo-
cardiogram is the best initial test for all forms of valvular heart disease. The cardiac angio-
gram is the most accurate test. The catheterization will allow the most accurate assessment
of the exact valve diameter as well as a direct measurement of the pressure gradient across
the valve. As MS worsens, the opening snap moves closer to S2. The best initial therapy for
MS is a balloon valvuloplasty.

1. Which is associated with fixed splitting of S2?

A. ASD
B. VSD
C. Mitral stenosis
D. Aortic stenosis
E. Mitral regurgitation

Answer:

2. Which is most dangerous to a pregnant woman?

A. Hypertrophic cardiomyopathy
B. Mitral stenosis
C. Mitral regurgitation
D. Atrial septal defect without pulmonary hypertension

Answer:

3. WPW has which of the following?

A. Loud S1
B. Soft S1
C. Wide splitting of S2
D. Narrow splitting of S2

Answer:

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 Cardiology

Case 2
34-year-old woman in the office because of palpitations and chest pain. There
is no syncope or lightheadedness with the palpitations. She cannot pinpoint
any particular event that brings on the pain. She has a high-pitched sound in
midsystole followed by a murmur. No rales and no jugulovenous distension.

1. Most likely diagnosis?

2. What would you do to confirm the diagnosis?

Echocardiography

3. How will maneuvers such as Valsalva, squatting, and hand grip affect the murmur?

4. What therapy needed, if any?

A. No treatment
B. Verapamil
C. Hydralazine
D. Metoprolol
E. Enalapril

Answer:

5. Does this patient need endocarditis prophylaxis if she needs a dental filling or colonoscopy?
A. Clindamycin
B. Amoxicillin
C. Nothing
D. Ampicillin and gentamicin
E. Antibiotic only if a biopsy is done

Answer:

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 IM Board Review Course Book

Summary: Case 2
Mitral valve prolapse (MVP) most commonly presents with atypical chest pain and palpita-
tions. The best initial therapy is with beta blockers. Antibiotic prophylaxis is not needed even
if the prolapse is severe enough to result in mitral regurgitation or a murmur. Dental fillings
and colonoscopy, even with a biopsy, do not need prophylaxis. The murmur of MVP worsens
whenever the heart decreases in size, and improves or becomes softer when the heart increases
in size. Valsalva and standing decrease venous return to the heart. This will decrease cardiac
size, and the degree of prolapse will increase or worsen. This leads to an earlier click and a
greater intensity or loudness of the murmur. Amyl nitrate decreases afterload. The effect of
amyl nitrate during auscultation is to increase the intensity of the murmurs of aortic stenosis,
(IHSS) HOCM, and MVP. Amyl nitrate will decrease the murmurs of MR, AR, and VSD. Amyl
nitrate is like giving an ACE inhibitor. ACE inhibitors help the treatment of MR, AR, and VSD,
and therefore, amyl nitrate will help lessen the intensity of these murmurs. This will empty the
heart more, and the MVP will again worsen. Hand grip or arterial cuff inflation increases after-
load. The effect of hand grip is the opposite of everything described for amyl nitrate. Hand
grip increases pressure on the brachial artery and increases afterload. Hand grip will worsen
MR, AR, and VSD and improve AS, IHSS, and MVP. This will decrease cardiac emptying and
increase cardiac size, which will decrease the intensity of the murmur of MVP.

1. Which of the following murmurs becomes worse with hand grip?

A. Aortic stenosis
B. Hypertrophic obstructive cardiomyopathy
C. Mitral valve prolapse
D. Aortic regurgitation

Answer:

2. Which of the following will increase with phenylephrine?

A. Aortic stenosis
B. Hypertrophic obstructive cardiomyopathy
C. Mitral valve prolapse
D. Aortic regurgitation

Answer:

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 Cardiology

Case 3
77-year-old man comes because of chest pain on exertion. Exam reveals 3/6
murmur at second right intercostal space radiating to carotid arteries. There is a
paradoxically split S2, S4 gallop, and slow upstroke of the carotid arteries. EKG has
an S wave in V2 and an R wave in V5 of 20 mm each. Cardiac enzymes are normal,
and the patient is ready to leave the hospital the following day.

1. Most likely diagnosis?

2. Most accurate diagnostic test?

3. What will be the effect of hand grip, Valsalva, and leg raise on the murmur?

4. Worst prognostic factor for this patient?

A. Angina
B. Syncope
C. Palpitations
D. Congestive failure
E. Atrial fibrillation

Answer:

5. What treatment?

6. How would you manage this patient if he were asymptomatic and just had the murmur?

7. Most dangerous medical therapy?

A. ACE inhibitor
B. Spironolactone
C. Beta blockers
D. Amiodarone
E. Digoxin

Answer:

8. What is the utility of balloon valvuloplasty?

It is only for those who refuse or cannot tolerate surgery.

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 IM Board Review Course Book

Summary: Case 3
Aortic stenosis (AS) is most commonly caused by calcification of the valve as the patient
ages. AS presents most commonly with angina. Syncope is not as common a presentation as
angina. Angina occurs because there is an obstruction to the flow of blood into the coronary
ostia. In addition, there is an increased oxygen demand because of left ventricular hypertro-
phy causing increased wall tension and more myocardial oxygen consumption. At least half of
the patients with AS have concomitant coronary artery disease. The worst prognostic factor is
the presence of congestive failure. Once the heart starts to dilate there is nothing that can be
done to restore the heart to normal size. The most accurate diagnostic test is the angiogram.
Only angiography allows an exact assessment of the pressure gradient across the valve.

Aortic stenosis is a disease that is managed almost exclusively by surgery. Preload reduction
with diuretics can be used in small amounts in those with congestive failure but is difficult
to manage because it can decrease cardiac output. ACE inhibitors can actually be dangerous
because these patients do not have the ability to simply increase cardiac output in response to
a decrease in afterload. ACE inhibitors can cause syncope. If symptoms of angina, syncope, or
congestive failure develop from aortic stenosis, then valve replacement is needed. Balloon val-
vuloplasty is not effective and is only rarely used as a bridge to surgery or in patients too ill to
undergo surgery. Asymptomatic patients should be followed with annual echocardiography.

Valsalva maneuver and sudden standing lead to a decrease in the intensity, or loudness, of the
murmur, because there is less blood to go across the stenotic valve. This decreases the pres-
sure gradient and decreases the intensity of the murmur. The opposite is true of leg raise and
squatting. Hand grip is a maneuver that increases afterload by compressing the peripheral
arteries. It will decrease the pressure gradient across the valve, and the murmur will decrease
in intensity.

A narrow or paradoxically split S2 is found whenever there is delayed closure of the aortic
valve. This occurs in aortic stenosis, severe hypertension, IHSS (HOCM), and left bundle
branch block.

Fixed splitting of the S2 is found predominantly in atrial septal defects (ASD).

1. Which of the following patients is the most appropriate to receive sildenafil (Viagra®)?
A. A patient with angina who is on isosorbide dinitrate and atenolol
B. A patient with mitral regurgitation and a normal left ventricle
C. An elderly man with aortic stenosis and left ventricular hypertrophy
D. A patient with hypertrophic cardiomyopathy

Answer:

2. Which of the following is associated with paradoxical splitting of S2?

A. Right bundle branch block
B. Wolf-Parkinson-White
C. First-degree AV block
D. Second-degree AV block
E. Left bundle branch block

Answer:

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 Cardiology

3. 72-year-old man with syncope. Echocardiogram shows calcified aorta with an area of
0.5 cm2. Peak systolic gradient is 90 mm Hg. Next best step?
A. ACE inhibitor
B. Nifedipine
C. Venous compression trousers
D. Coronary angiography
E. Warfarin

Answer:

4. 20-year-old male on college basketball team comes for routine exam. Heart rate 50/min,
BP 110/70 mm Hg, soft S3 gallop, and a grade 1 systolic ejection murmur over left ster-
nal border. Murmur decreases in intensity with standing and Valsalva maneuver, and
increases after exercise. EKG shows mild left ventricular hypertrophy and sinus pauses
up to 1 second. Echo shows ejection fraction 64% and a minimal increase in wall thick-
ness. What do you recommend to this patient?
A. Do not participate in sports any longer
B. Propranolol
C. Pacemaker placement
D. Implantable defibrillator
E. No specific treatment

Answer:

5. Old man with aortic stenosis has syncope leading to hip fracture. What’s first?
A. Fix hip
B. Fix heart

Answer:

Case 4
26-year-old medical student short of breath playing basketball. He had syncopal Note
episode during one of the games. He has S4 gallop and 3/6 systolic murmur at left
sternal border that improves with hand grip and worsens with standing. An S4 gallop is a sign of
a noncompliant stiff left
ventricle. Fourth heart sounds
1. Most likely diagnosis?
are found when there is left
ventricular hypertrophy, such
2. Most common presentation of this disorder? as in hypertension, AS, or
A. Dyspnea HOCM, or with ischemia.
B. Syncope
C. Sudden death
Answer:

3. Best initial therapy, and what to do if medical therapy fails?

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 IM Board Review Course Book

INSPIRATION EXPIRATION

NORMAL SPLITTING
S1 A2 P2 S1 A2 P2

WIDE SPLITTING
(Pulmonic stenosis, RBBB, S1 A2 P2 S1 A2 P2
Mitral regurgitation, VSD, PDA)

PARADOXICAL SPLITTING
(Aortic stenosis, hypertrophic S1 P2 A2 S1 P2 A2
cardiomyopathy, LBBB)

FIXED SPLITTING
(Atrial septal defect) S1 A2 P2 S1 A2 P2

Summary: Case 4
HOCM most commonly presents with shortness of breath secondary to decreased diastolic
function of the heart. Although syncope and sudden death can occur, these are not as com-
mon as simple shortness of breath. The EKG should show signs of left ventricular hypertro-
phy. Overall, the most effective lifesaving therapy for HOCM is an implantable defibrillator in
patients with a history of syncope and a family history of sudden death. The best initial medi-
cal therapy for HOCM is with negatively inotropic agents such as beta blockers or calcium
channel blockers. These agents will decrease systolic emptying of the heart and will decrease
the degree of obstruction of the left ventricular outflow tract. The beta blockers are particu-
larly effective because of their effect on heart rate. As the heart rate slows, there is increased
diastolic filling. This decreases the degree of obstruction. If these agents are not effective, then
a catheter can be placed into the septal perforator arteries and small amounts of absolute
alcohol can be infused, causing small infarctions. These small infarctions will decrease the
outflow tract obstruction. If medical and catheter-directed therapy are not effective, surgical
myomectomy is a final resort. Surgery for HOCM is only used if all else fails.

1. Which decreases with amyl nitrate?

A. Mitral regurgitation
B. Aortic stenosis
C. Hypertrophic obstructive cardiomyopathy
D. Mitral valve prolapse

Answer:

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 Cardiology

2. A 32-year-old man is admitted to the telemetry unit after a syncopal episode. He has
dyspnea on exertion for 10 years. Uncle died at age 50 of sudden death. Echo shows
hypertrophic obstructive cardiomyopathy. After 24 hours of continuous cardiac moni-
toring, no rhythm disturbance is revealed. What is the most beneficial therapy?
A. Implantable cardioverter defibrillator
B. Propranolol
C. Verapamil
D. Disopyramide
E. Metoprolol

Answer:

3. Which will increase with Valsalva maneuver and decrease with squatting?
A. Mitral regurgitation
B. Aortic regurgitation
C. Hypertrophic obstructive cardiomyopathy
D. Aortic stenosis

Answer:

Case 5
A 36-year-old man comes to the hospital for a TIA. He has progressive dyspnea
on exertion for the last few months. Temperature 38ºC (100.4ºF) orally. He has
a diastolic murmur but no opening snap. The murmur changes dramatically with
body position. He has elevated sedimentation rate.

1. Most likely diagnosis?

2. What test will you perform?

3. What therapy?

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 IM Board Review Course Book

Summary: Case 5
A mid-diastolic murmur in a patient with shortness of breath and signs of embolic phenom-
ena can be from mitral stenosis. Clots in the atrium can cause fever and an elevated sedimen-
tation rate as well. The main way to distinguish between mitral stenosis and an atrial myxoma
such as the patient described here is that there is no opening snap with an atrial myxoma. In
addition, the murmur of a “tumor plop” changes prominently with a change in body position
as the tumor flops around in the atrium. The only therapy of any significant benefit for an
atrial myxoma is surgical removal of the lesion.

CONGENITAL CARDIAC ANOMALIES

Case 1
1. Which is true about congenital bicuspid aortic valve?
A. Rate of aortic stenosis is same as normal valve
B. Can lead to aortic regurgitation
C. Endocarditis prophylaxis is needed
D. Transthoracic echo will miss it
E. No chest pain, syncope or dizziness occur

Answer:

2. Which is most commonly associated with bicuspid aortic valve?

A. Coarctation
B. Mitral valve prolapse
C. VSD

Answer:

Summary: Congenital Cardiac Anomalies

Bicuspid aortic valve gives largely the same symptoms and is assessed with the same diagnos-
tic tests as aortic stenosis. Patients develop chest pain, syncope, lightheadedness and signs of
CHF. Aortic root dilation can occur leading to aortic regurgitation. Endocarditis prophylaxis
is not needed. This is not a cyanotic congenital heart disease. Neither form of aortic disease
has an effective medical therapy.

The major difference with bicuspid aortic valve is a much greater use of balloon valvuloplasty
in younger patients. The only cure is valve replacement, but in younger patients catheter
procedure can be effective before the valve calcifies.

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 Cardiology

1. 41-year-old patient, who is asymptomatic, comes for evaluation of a murmur. The

patient has mitral valve prolapse with mitral regurgitation. The ejection fraction is
50%; and the left ventricular end systolic diameter is 52 mm. There is no coronary
artery disease. What is the best management for this patient?
A. Repeat the echo in 6 months
B. Diuretics and ACE inhibitors
C. Digoxin
D. Nifedipine
E. Refer for valve replacement

Answer:

Note

Mitral regurgitation (MR) and aortic regurgitation (AR) are medically treated, predominantly
with vasodilator therapy such as ACE inhibitors or nifedipine. These drugs decrease the rate of
progression of left ventricular dilation. Asymptomatic patients are managed by observing them
with annual echocardiography. Surgical repair or replacement is performed for any symptomatic
patient. It is also performed in asymptomatic patients if there is echocardiographic evidence of the
development of left ventricular dysfunction.
• F or MR, surgical criteria is a left ventricular (LV) end systolic diameter (ESD) of >40 mm or an
ejection fraction (EF) <60%.
• For AR, it is an LVESD of >55 mm or an EF <55%.

2. 63-year-old man with 3/6 systolic murmur at apex that radiates to the axilla. No
symptoms. Echo shows mitral regurgitation with an ejection fraction of 52% and a left
ventricular end-systolic diameter of 48 mm. What would you recommend?
A. Valve replacement
B. Lifelong warfarin
C. Aspirin
D. Digoxin
E. No therapy necessary

Answer:

3. Which is most dangerous to a pregnant woman?

A. Eisenmenger syndrome
B. Aortic regurgitation
C. Aortic stenosis
D. Mitral valve prolapse
E. Previous peripartum cardiomyopathy with persistent left ventricular dysfunction

Answer:

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 IM Board Review Course Book

4. Patient with atrial septal defect comes for evaluation. She is asymptomatic and has a
pulmonary/systemic flow ratio of 1.3:1. What would you recommend?
A. Never become pregnant
B. Okay to become pregnant
C. Have surgical closure, then become pregnant

Answer:

5. Patient on long-term total parenteral nutrition develops a cardiomyopathy. Which is

the most likely etiology?
A. Magnesium deficiency
B. Copper
C. Selenium
D. Zinc

Answer:

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 Cardiology

PERICARDIAL DISEASE

Case 1
Man comes to ED with 1 day of chest pain. Pain worsens when he takes a deep
breath and when he goes from a sitting position to lying down. Temperature of
38.6ºC (101.2ºF) orally.

1. Most likely diagnosis?

2. Most common causes of this problem?

3. Most specific finding on EKG?

A. Sinus tachycardia
B. ST-segment elevation
C. PR depression
D. T-wave flattening

Answer:

4. What is the best initial therapy, and what will you do if he returns after several days
because this has not been effective?

5. A patient comes with recurrent episodes of pericarditis. He has been treated with
NSAIDs and steroids several times. What to do to prevent next one?
A. Prednisone continuously
B. Colchicine
C. Cyclosporine

Answer:

Summary: Case 1
Pericarditis is most commonly caused by a viral infection of the pericardium. When there
is no other significant past medical history in the case, then the most likely cause is a virus.
Any infection can cause pericarditis. Any inflammatory disorder such as uremia, radiation
therapy, or connective tissue disorder can cause pericarditis as well, although SLE is prob-
ably the single most common cause. The best initial method of establishing a diagnosis is by
observing diffuse ST-segment elevation in virtually all leads on the EKG. The most specific
EKG finding for pericarditis is PR-segment depression. Therapy consists of correcting the
underlying cause if one is found, such as tuberculosis. For viral pericarditis, the initial therapy
is with NSAIDs. If NSAIDs and colchicine are a choice, then that should be the right answer.
Colchicine definitely helps in addition to an NSAID. If these are not effective, then steroids
should be used.

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 IM Board Review Course Book

Note

Pericardial tamponade is caused by any form of pericarditis that leads to the accumulation of
sufficient effusion fluid to compress the right ventricle or right atrium in diastole. The most specific
clinical feature for tamponade is the development of a pulsus paradoxus. This is a >10 mm Hg
drop in systolic blood pressure on inspiration. The EKG finding in tamponade is electrical alternans.
Treatment is with pericardiocentesis and pericardial window placement.
Constrictive pericarditis can be caused by any chronic infection, inflammation, or cancer that
involves the pericardium. Constrictive pericarditis is characterized by the signs found in right
ventricular failure, such as edema, ascites, and hepatosplenomegaly. The most characteristic physical
findings are the presence of Kussmaul’s sign (increased JVP on inspiration) and a pericardial knock.
The best initial test for constrictive pericarditis is a chest x-ray looking for calcifications and fibrosis.
The most accurate diagnostic test is an MRI or a CT scan. There is no good medical therapy for
constrictive pericarditis. The treatment is pericardial stripping.

1. Man who emigrated from Vietnam 25 years ago comes to office for progressively wors-
ening edema, ascites, and shortness of breath. You find jugulovenous distention, edema,
hepatosplenomegaly, and an extra heart sound in diastole. EKG shows low voltage.
Chest x-ray shows calcifications over heart shadow. CT scan shows pericardial thicken-
ing. Which of is most likely in this patient?
A. S3
B. S4
C. Pulsus paradoxus
D. Kussmaul’s sign
E. Paradoxical split of S2

Answer:

2. Most effective therapy?

A. Diuretics
B. Thrombolytics
C. Pericardiocentesis
D. Surgery

Answer:

3. Which of the following will show equalization of diastolic pressures on right heart
catheterization?
A. Constrictive pericarditis
B. Right ventricular infarction
C. Tricuspid valve rupture
D. Ventricular septal rupture
E. Atrial septal rupture

Answer:

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 Cardiology

Case 2
Patient, in a motor vehicle accident, with shortness of breath, jugulovenous
distention, hypotension, and tachycardia.

1. Most likely diagnosis?

2. Most specific physical finding?

A. Hypotension
B. S4 gallop
C. S3 gallop
D. Decrease in blood pressure of 10 mm Hg on inhalation
E. Increase in jugulovenous pressure on inhalation

Answer:

3. Most specific EKG finding?

A. Sinus tachycardia
B. Low voltage
C. Electrical alternans
D. ST-segment elevation
E. PR-segment depression

Answer:

4. Initial therapy?
A. Diuretics
B. Pericardial window
C. Pericardiocentesis
D. ACE inhibitor

Answer:

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 IM Board Review Course Book

Summary: Case 2
Tachycardia, hypotension, and jugulovenous distention are the triad consistent with pericar-
dial tamponade. The motor vehicle accident is sufficient reason for tamponade. The most
specific physical finding is pulsus paradoxus, which is a decrease is systolic blood pressure
>10 mm Hg on inhalation. Kussmaul’s sign is a rise in jugulovenous pressure on inhalation.
This is more often found in constrictive pericarditis. The EKG finding most specific for
pericardial tamponade is electrical alternans. This is the alternation between big and small
QRS complexes based on how far the heart is from the anterior chest wall. Sinus tachycardia
and low voltage can occur, but they are not specific for pericardial tamponade. PR-segment
depression is the most specific EKG finding for pericarditis. Pericardiocentesis is the most
important first therapy. A pericardial window is used later. Diuretics can be potentially fatal
in pericardial tamponade.

1. Which of the following Swan-Ganz catheter values is most likely to be found in pericar-
dial tamponade?
A. Elevated wedge pressure, decreased cardiac output, increased systemic resistance
B. Decreased wedge pressure, decreased cardiac output, increased systemic resistance
C. Step up on oxygen saturation from the right atrium to the right ventricle
D. Decreased wedge pressure, increased cardiac output, decreased systemic resistance
E. Equalization of all diastolic pressures (right atrium, right ventricle, pulmonary
artery, wedge)

Answer:

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 Cardiology

RHYTHM DISTURBANCES

Case 1
71-year-old man brought to ED with light-headedness and chest discomfort. As you
are examining him, he loses consciousness and his pulse. CPR is initiated and an
EKG is performed which shows ventricular fibrillation. Electrical shocks with 200,
300, and 360 joules are administered without effect.

Ventricular Fibrillation

Heart Rhythm P Wave PR Interval QRS

Rate (in seconds) (in seconds)
Extremely Fibrillatory
300-600 irregular Absent N/A baseline

1. Next step in the management of this patient?

2. When will your answer be to use intracardiac medications?

Never

3. When will your answer be thoracotomy?

Never

4. What will you do if this initial therapy is not effective?

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 IM Board Review Course Book

5. Patient’s EKG becomes normal. CK-MB and troponin drawn before beginning of
ventricular fibrillation are elevated. Echocardiogram reveals ejection fraction 54%. He
remains in sinus rhythm.

Which of the following would you use upon discharge to manage this patient’s rhythm
disturbance?
A. Amiodarone
B. Procainamide
C. Pacemaker
D. Automatic implantable cardioverter defibrillator (AICD)
E. Beta blockers

Answer:

Summary: Case 1
The use of prophylactic anti-arrhythmic medications in those with acute coronary syn-
dromes is not effective. Pacemakers will not help with ventricular fibrillation. The AICD is
specifically not useful in serious dysrhythmias associated with acute ischemia. AICDs are
for those with hemodynamically significant VT or VF not effectively treated with medical
therapy, or for those in whom it can be induced on electrophysiological studies despite the
use of medications. Beta blockers are the best suppressive medication for ischemia-related
rhythm disturbances after the patient has been converted back to sinus rhythm.

Ventricular fibrillation is always treated initially with electrical defibrillation. If three

attempts at defibrillation are not effective, then either epinephrine or vasopressin should be
used prior to the next attempt at defibrillation. If this is not effective, then amiodarone or
lidocaine should be used next. If lidocaine and amiodarone are both in the answer choices,
you should choose amiodarone. Bretylium, intracardiac medications, and thoracotomy are
not effective and are always wrong answers when they are in a question.

1. 63-year-old woman sitting in ER has stopped breathing. EKG is found to be in ven-

tricular fibrillation. No spontaneous respirations and is not breathing. What would you
do first?
A. Intubation
B. Synchronized cardioversion
C. Unsynchronized cardioversion
D. Precordial thump

Answer:

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 Cardiology

Case 2
63-year-old man brought to ED because of syncope. His wife initiated CPR. In the
field, he was found to be in ventricular fibrillation and was successfully defibrillated
with a single shock at 200 joules. In ED, he is alert and hemodynamically stable.
He is started on both lidocaine and amiodarone. A repeat EKG, cardiac enzymes,
and a thallium stress test the following day are all normal.

1. What would you do next?

A. Electrophysiology study
B. AICD
C. Angiography
D. AICD, if beta blockers fail

Answer:

2. What is the most accurate and specific test to determine this patient’s therapy for his
rhythm disturbance?
Electrophysiological testing to find inducible VT/VF

3. What event in hospital would signify the worst prognosis for this patient?
Recurrence of the dysrhythmia while on medications

4. What finding on echocardiogram would indicate the worst prognosis?

A. Apical hypokinesis
B. Septal dyskinesis
C. Decreased ejection fraction under 35%
D. Mitral regurgitation
E. Patent foramen ovale

Answer:

5. Best therapy for this patient?

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 IM Board Review Course Book

Summary: Case 2
If ventricular fibrillation or ventricular tachycardia occur in the first 48 hours after an infarc-
tion, no long-term anti-arrhythmic therapy is necessary beyond beta blockers. The point of
the case described here is that if these rhythm disorders occur outside the setting of acute
ischemia or a correctable cause, then they need an implantable defibrillator. Although an
electrophysiology study is the most accurate diagnostic test, it is unnecessary for a patient like
this one who has a clear case of unprovoked ventricular fibrillation. The same is true if the
rhythm found were a hemodynamically unstable ventricular tachycardia. The worst prognos-
tic factor is if the arrhythmia recurs while the patient is on anti-arrhythmic drugs. Because
his arrhythmia has not recurred, the worst prognostic factor is the presence of myocardial
disease with a low ejection fraction. In a case like this, however, he would need an implant-
able defibrillator (AICD) even if the ejection fraction is normal because he had unprovoked
sudden death from ventricular fibrillation.

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 Cardiology

VENTRICULAR TACHYCARDIA

Case 1
A 74-year-old man has a syncopal episode in his apartment. He awakens
spontaneously and is brought to the emergency department (ED). He has a
history of hypertension and is on beta blockers. In the emergency department, he
has another episode of sustained ventricular tachycardia but no symptoms beyond
palpitations. His blood pressure (BP) is 114/78 mm Hg, respirations are 16/min,
chest is clear, and mental status is normal.

I aVr V1 V4

II aV1 V2 V5

III aVf V3 V6

Rhythm Strip: II

1. What are the indications for immediate cardioversion in this man?

2. Best initial therapy?

A. Synchronized cardioversion
B. Unsynchronized cardioversion
C. Amiodarone
D. Sotalol

Answer:

3. When will your answer be magnesium?

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 IM Board Review Course Book

Summary: Case 1
The indications for electrical cardioversion in a patient with ventricular tachycardia are
hypotension with a systolic blood pressure <90 mm Hg, congestive heart failure, altered
mental status, and chest pain. When patients are hemodynamically stable and have sustained
ventricular tachycardia, then they should be treated with either amiodarone or lidocaine.
Magnesium is given to those with torsades de pointes or those who are identifiably hypo-
magnesemic. If all of these are in the answer, then choose amiodarone. An implantable defi-
brillator (AICD) should be placed in any patient with a hemodynamically significant case of
ventricular tachycardia or in those who have sustained monomorphic ventricular tachycardia
induced in the electrophysiology laboratory. An AICD is particularly beneficial for those
patients who also have left ventricular dysfunction with a low ejection fraction and in those
whose arrhythmia can still be induced despite the use of anti-arrhythmic medications.

Case 2
74-year-old man is converted to sinus rhythm from ventricular tachycardia
with amiodarone. Echocardiogram shows an ejection fraction of 34% and left
ventricular dysmotility. Cardiac enzymes and a coronary angiogram are normal.

1. What is the best therapy for this patient?

A. Electrophysiology study
B. TEE
C. AICD
D. Nuclear ventriculogram
E Amiodarone alone

Answer:

Summary: Case 2
Prolonged QT syndromes lead to an increased risk of torsades, syncope, and sudden death.
A number of medications can prolong the QT, such as quinidine, dofetilide, ibutilide, and
tricyclic antidepressants. When there is no specific toxic effect causing the prolonged QT, you
should suspect a congenital prolonged QT syndrome. The treatment of choice in this case
is beta blockers. Beta blockers and calcium channel blockers are the only anti-arrhythmic
medications that have no pro-arrhythmic effect.

1. A 64-year-old woman is admitted for her third episode of syncope in the last 6 months.
EKG, telemetry monitoring, echocardiogram, and examination are all normal.
Electrophysiology studies are able to induce ventricular tachycardia that is sustained.
What is the best therapy?
A. Amiodarone
B. Sotalol
C. Dofetilide
D. Implantable defibrillator

Answer:

2. An 18-year-old, Asian man is awakened by an alarm clock and has syncope two minutes
later. There is a right bundle branch block pattern on his EKG. What is the diagnosis?

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ATRIAL ARRHYTHMIAS

Case 1
29-year-old senior medical resident comes to the emergency room with
palpitations. He has been studying for the boards and has had 5 cups of
coffee, 4 beers, 3 cheeseburgers, 2 “power drinks,” and 1 Viagra. EKG shows
supraventricular tachycardia at a rate of 160. BP 124/80 mm Hg.

1. What would you do first?

A. Carotid sinus massage
B. Intravenous heparin
C. Adenosine
D. Quinidine
E. Disopyramide
F. Cardioversion

Answer:

2. What is next if this is unsuccessful?

3. What other medications are acceptable to control the rate?

Calcium channel blockers, beta blockers, and digoxin

4. If the SVT continues to be recurrent, what is the best long-term therapy?

5. If the case were changed to atrial flutter, how would the above answers differ?

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 IM Board Review Course Book

Supraventricular Tachycardia

Loss of P-waves

P-waves

HR 145

Summary: Case 1
There is a lot of overlap in the management of all forms of atrial arrhythmias. Any hemody-
namically unstable patient should undergo synchronized cardioversion. You do not need to
anticoagulate prior to cardioverting an unstable patient. If the patient is hemodynamically
stable and has supraventricular tachycardia (SVT), an attempt should be made to perform
vagal stimulation maneuvers such as carotid sinus massage. If these are unsuccessful, then
the best next step in management is to use adenosine. Adenosine is a medication that is used
exclusively in those with SVT. It is not useful for atrial fibrillation or flutter. If adenosine
is ineffective, then AV nodal blocking agents should be used. This is the same for all forms
of atrial tachycardias. Beta blockers, calcium channel blockers, and digoxin are all effective.
Digoxin has the disadvantage of being the slowest in onset, the most toxic, and less effective
with tachycardias that occur with exertion. The long-term management of SVT includes
attempting to eradicate the pathway of AV nodal re-entry with catheter ablation.

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 Cardiology

Case 2
32-year-old woman with recurrent episodes of atrial dysrhythmias. On last visit
to emergency department an intravenous infusion of diltiazem worsened her
condition into hemodynamically unstable ventricular tachycardia that required
cardioversion. Current EKG shows sinus rhythm with normal ST segments and PR
interval of 0.08 seconds.

10mm/mV 22 mm/s Filter ON 10 mm/mV

I - II - III aVR-aVL-aVF V1 - V2 - V3 V4 - V5 - V6

Rhythm (V1) 10 mm/mV

1. Most likely diagnosis?

2. What would you expect to find on auscultation?

3. What is the best medical therapy if this patient pops into SVT or hemodynamically
stable ventricular tachycardia again?

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 IM Board Review Course Book

4. Best long-term therapy?

5. Which drugs are contraindicated?

Summary: Case 2
Note Pre-excitation syndromes present with a short PR segment. The most common is Wolf-
Parkinson-White. WPW can lead to either SVT or ventricular tachycardia. Medications such
WPW by itself is not as digoxin or calcium channel blockers can block conduction down the normal AV nodal
dangerous with exercise. pathway and increase conduction down the aberrant tract. This drives the patient into SVT
or ventricular tachycardia. In a hemodynamically unstable patient the treatment is cardiover-
sion. When the patient is hemodynamically stable, the best therapy is procainamide. This is
because procainamide is effective in SVT and atrial fibrillation as well as ventricular tachycar-
dia. Other agents that are effective are amiodarone, propafenone, sotalol, and flecainide. If all
of these appear in the answer for WPW treatment, then the answer is procainamide. After the
initial episode has resolved, long-term therapy involves ablation of the accessory tract with a
catheter using radio-frequency waves.

Note

Multifocal atrial tachycardia is an atrial dysrhythmia that occurs almost exclusively secondary to
chronic lung disease such as COPD. It is a narrow-complex tachycardia similar to wandering atrial
pacemaker, except that the rate is rapid. You typically find at least 3 different P-wave morphologies.
In the past it was commonly found with theophylline toxicity. The management is to treat the
underlying pulmonary disease. The dysrhythmia is controlled acutely with verapamil, diltiazem, or
beta blockers.

Case 3
76-year-old woman with hypertension and diabetes comes for a routine visit.
She is maintained on a diuretic with good control of blood pressure. Heart rate is
118 and irregular. She firmly denies symptoms and had no idea that her rate was
elevated or that her rhythm was irregular or for how long. EKG: atrial fibrillation
at 118.

1. Best initial step in management?

Control the rate with beta blockers or calcium blockers. Because of her history of hyperten-
sion, beta blockers would be preferable.

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 Cardiology

2. After this is accomplished, what would you do next?

Anticoagulation with dabigatran, warfarin, or rivaroxaban

3. Which cardioverting medications have the highest risk of torsades de pointes?

Atrial fibrillation

Summary: Case 3
The initial management of hemodynamically stable patients with rapid atrial fibrillation
involves controlling the rate with beta blockers, calcium blockers, or digoxin. If all of these
are given as answer choices, then you must look at the case for other disorders or comorbidi-
ties that are either an indication or a contraindication for these medications. For example,
if there is asthma in the history, then the answer is not beta blockers. After the rate has been
controlled, patients should either receive anticoagulation for 3 weeks prior to an elective
cardioversion or undergo a transesophageal echocardiogram to exclude an atrial thrombus.

Rate control and long-term anticoagulation is equal or superior to control of the rhythm.

If cardioversion is performed, patients should receive anticoagulation with warfarin for

4 weeks to prevent a clot from forming as atrial function is restored. If the patient cannot suc-
cessfully achieve or maintain a sinus rhythm, then long-term anticoagulation with warfarin
is indicated to prevent emboli. Anticoagulation is not necessary prior to cardioversion if the
atrial fibrillation has been present for less than 48 hours. Dofetilide and ibutilide are highly
effective but also have the highest risk of developing torsades de pointes. The most effective
medication for maintaining a patient in sinus rhythm after cardioversion is amiodarone.
Routine rhythm conversion is not done.

CHADS = CHF, hypertension, age >75, diabetes, stroke

If the CHADS score is ≥2, use dabigatran, warfarin, or rivaroxaban.

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 IM Board Review Course Book

Case 4
47-year-old gastroenterologist has been having palpitations. EKG shows atrial
fibrillation with rate of 80. No past medical history, no diabetes, and no
hypertension. Echo normal. When you discuss cardioversion by electricity or
medications, he refuses and instructs you to do something to yourself that is
anatomically impossible.

1. Best therapy for this patient?

A. Electrical cardioversion
B. Amiodarone
C. Aspirin
D. Heparin, then warfarin
E. Warfarin

Answer:

Summary: Case 4
Note Warfarin is not necessary in those with “lone” atrial fibrillation. This is defined as occur-
ring in those who are <75 with no history of stroke, transient ischemic attack, hypertension,
A. Fib for <48 hours needs no diabetes, or structural heart disease on echocardiogram. These patients can be treated with
anticoagulation. aspirin alone. This patient has a CHADS score of 0.

CHADS of 0-1: aspirin only

Dabigatran, rivaroxaban, and warfarin are used when CHADS score is ≥2. Dabigatran and
rivaroxaban are as good or better than warfarin for non-valvular atrial fibrillation.

What is this?

A. Normal Sinus Rhythm

B. Supraventricular tachycardia (SVT)
C. Junctional rhythm

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 Cardiology

Junctional Rhythm

Answer:

Note

Junctional tachycardia is a narrow-complex tachyarrhythmia generally at a rate of 70 to 150. You

recognize the disorder by finding inverted P wave in leads II, III, and aVF. These indicate retrograde
conduction of the impulse up from the AV node backward toward the SA node. The most common
cause is digoxin toxicity. Other causes are inferior wall infarctions, valve surgery, and myocarditis.
Acute treatment is with beta blockers, and long-term management is to treat the underlying cause.

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 IM Board Review Course Book

BRADYCARDIA

Case 1
54-year-old man comes to you in the office to discuss smoking cessation. You
notice his pulse is 52. He says that you shouldn’t worry about this because he runs
marathons and climbs mountains. EKG shows Mobitz II second-degree heart block.

1. What is the most appropriate action?

A. Nothing
B. Atropine
C. Pacemaker

Answer:

Summary: Case 1
If a patient has a slow heart rate and signs of hypoperfusion, the best initial step is always to
administer atropine. If there is hypotension, syncope, near-syncope, or light-headedness, and
there is bradycardia from any cause, atropine is the first thing to do because this is the fastest
way to raise the heart rate. If atropine is not effective, then an external pacemaker is used. If
the patient is asymptomatic and hemodynamically stable, you only need to treat third-degree
heart block and Mobitz II second-degree heart block. Even if a patient is asymptomatic, a
patient with a Mobitz II should have a permanent transvenous pacemaker placed anyway
because of the high risk of syncope. The same is true of third-degree (complete) heart block.

Case 2
How would you manage a patient with the EKG shown below?

1st degree AV block (PR = 280 ms)

Long PR Longer Longer!! Dropped beat

1. What is the diagnosis?

2. Treatment?

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 Cardiology

Case 3
Lead VI

1. What is seen in rhythm strip A, and what is the management?

2. What is seen in rhythm strip B?

Case 4
Asian man, 38, had syncope. Shocked out of V. fib. Father died at 45. EKG below.

What is it?
Treatment?

R'
R'
R R

V1 V2 V3

Case 5
Treatment of symptomatic tachy-brady syndrome?

A. Pacemaker
B. AICD
C. Beta blockers and pacemaker
D. Ablation

Answer:
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 IM Board Review Course Book

ENDOCARDITIS

Case 1
Injection-drug user in ED with fever. 2/6 systolic murmur. There are no Janeway
lesions, Osler nodes, Roth spots, or splinter hemorrhages.

1. What is the best initial step in management?

2. Most likely organism?

3. Best empiric therapy?

A. Ceftriaxone
B. Ampicillin and gentamicin
C. Vancomycin
D. Vancomycin and gentamicin
E. Oxacillin

Answer:

4. Strongest indication for valve replacement?

A. CHF
B. Prosthetic
C. Fungal
D. Abscess
E. Emboli

Answer:

Summary: Case 1
Anyone who has a fever and a new murmur or a change in the character of a murmur should
be suspected of having endocarditis. If the patient is an intravenous-drug user with a fever,
you should suspect endocarditis even in the absence of a murmur. These patients develop
acute endocarditis from Staphylococcus aureus, which is extremely damaging to the endothe-
lial lining of the heart. Blood cultures are always the best initial step. The echocardiogram
should never be done first. The transthoracic echocardiogram has only a 65% sensitivity at
best for endocarditis. The best empiric therapy for endocarditis in patients who are injected-
drug users is vancomycin and gentamicin because of the high frequency of methicillin-
resistant S. aureus. If the organism is eventually found to be sensitive to oxacillin, then van-
comycin should be switched to either oxacillin or nafcillin.

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Case 2
72-year-old woman with fever of a few weeks’ duration. You find murmur of mitral
regurgitation that was clearly not there 2 months ago.

1. What will you treat her with if she grows Strep. viridans with MIC <0.1 (very low)?

2. What will you treat her with if she grows Strep. viridans that is less sensitive with an
MIC between 0.1 and 0.5?

3. What will you do if she grows Enterococcus?

4. What will you do if she grows Strep. bovi or Clostridium septicum?

5. What will you treat her with if she has a prosthetic valve?

6. Most common cause of culture-negative endocarditis?

A. HACEK
B. Coxiella
C. Streptococcus viridans
D. Staphylococcus

Answer:

7. Injection drug user with endocarditis of tricuspid valve on nafcillin and gentamicin for
two weeks for a sensitive staphylococcus, continues to have fever and positive blood
cultures. Shortness of breath develops. What is the next step?
A. Switch to vancomycin
B. Add rifampin
C. Treat for two more weeks then replace the valve
D. Replace the valve immediately

Answer:

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 IM Board Review Course Book

Note Summary: Case 2

The overall point in the therapy of endocarditis is that the first step is to decide what type
Clostridium septicum is of organism is present: Strep. versus sensitive Staph. versus resistant Staph. This guides us in
associated with colon cancer. knowing whether to base the regimen on penicillin, as in the case of Strep. viridans or Strep.
bovis, or to base the regimen on oxacillin (or nafcillin) for a sensitive Staph. or vancomycin
for a resistant Staph. In terms of streptococci, the more resistant the organism, the longer
you need to give additional synergistic therapy with an aminoglycoside such as gentamicin.
Sensitive Strep. viridans or bovis can be treated with penicillin or ampicillin as a single agent
for 4 weeks. As the level of resistance rises to an MIC between 0.1 and 0.5, you add gentami-
cin for the first 2 weeks. As the MIC rises above 0.5 or if you have an enterococcus, then you
extend the entire length of therapy to 6 weeks and use gentamicin the entire time. The best
therapy for a sensitive Staph. aureus is always nafcillin or oxacillin. If the organism is sensi-
tive, these drugs have a greater efficacy compared with vancomycin. Gentamicin is needed for
only the first 5 days. If the patient has a resistant Staph. aureus or has Staph. epidermidis, then
vancomycin is mandatory and gentamicin is not used for MRSA. Rifampin is added to the
therapy of prosthetic valve endocarditis for greater tissue penetration into the vegetations.
Sicker patients need more synergy.

Case 3
58-year-old woman is with fever and murmur is found to have endocarditis from
viridans group Streptococcus in 3 of 3 blood cultures drawn. Patient placed on
penicillin, 2 million units intravenously every 4 hours. Three days later patient
feels better. White cell count dropped from 14,000 to 12,000. Fever lower, and 1 of
3 blood cultures grows the same Streptococcus.

1. What should you do?

A. Switch to vancomycin
B. Perform valve replacement surgery
C. Repeat echocardiogram
D. Add rifampin
E. Continue the present therapy and repeat the culture in 3 to 4 days if the patient is
still febrile

Answer:

Summary: Case 3
A patient with endocarditis can remain febrile for as long as a week in as many as 75% of
cases even after the start of effective therapy. The persistent fever after 3 days is not a reason
for concern. This is why endocarditis is treated for 4 to 6 weeks. Endocarditis is a persistent
infection, and the blood cultures can remain positive for a week as well. If the patient contin-
ues to have positive blood cultures after a week, you should consider repeating the echocar-
diogram to see if an abscess has formed.

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 Cardiology

Case 4
62-year-old man admitted for fever and endocarditis from viridans group
Streptococcus with a mechanical prosthetic valve. He is maintained on warfarin
with an INR of 2.8.

1. What would you do about the anticoagulation?

Summary: Case 4
The use of anticoagulation in patients with endocarditis is an area of great anxiety
and concern because of the possibility of causing emboli. The only clear case
in which warfarin should be continued is in the patient with the highest risk of
thrombosis, such as a patient who has a mechanical heart valve.

Case 5
Patient with history of injection drug use with fever, cough, and hemoptysis. 2/6
murmur at lower left sternal border. Increases with inspiration. Chest x-ray shows
nodules.

What is diagnosis?

Treatment?

Case 6
What are the appropriate prophylactic regimens for each of the following patients?

1. 45-year-old woman with mid-systolic click followed by murmur about to have several
dental extractions.

2. 64-year-old man with prosthetic aortic valve about to have several dental fillings.

3. 72-year-old man with pacemaker and implanted defibrillator undergoing

hemicolectomy.

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 IM Board Review Course Book

4. A 33-year-old man with previous endocarditis secondary to injection-drug use who is

about to have a skin biopsy of infected skin.

5. A 65-year-old man with hypertrophic obstructive cardiomyopathy who will have a

prostate biopsy, cystoscopy, and urethral dilation with cystitis present.

6. A 72-year-old man who presents with previous quadruple bypass, an isolated click
from mitral valve prolapse, and a secundum atrial septal defect, and who will now have
a caesarian section.

Summary: Cases 5–6

Give prophylaxis if the patient both has a hemodynamically significant lesion and will undergo
a procedure that produces bacteremia. Both elements are necessary. High-risk cardiac defects
are prosthetic valves, previous endocarditis, cardiac transplant recipients, and complex cyanotic
heart disease that has not been repaired. The following are no longer indicators for prophylaxis:
• aortic stenosis or regurgitation
• mitral stenosis or regurgitation
Note • mitral valve prolapse that produces regurgitation or a murmur
• IHSS (HOCM)
Pen allergic? Use clindamycin. • ventricular septal defects, and
• noncyanotic congenital heart disease that has been surgically corrected. The secun-
dum type of atrial septal defect does not need to be prophylaxed either.

Bacteremia-causing procedures are: dental work that causes bleeding (not fillings) or respi-
ratory tract surgery. Prostate manipulation such as a biopsy, TURP, urinary stricture dila-
tion or urinary catheterization, sclerotherapy of varices, esophageal stricture dilation, or
endoscopy are no longer indicators for prophylaxis. Surgery of the respiratory and bowel
mucosa both need prophylaxis. Flexible endoscopy (even with biopsy), transesophageal
echocardiography, and caesarian section do not need prophylaxis against endocarditis. For
dental work, the best prophylaxis is still amoxicillin. In a patient allergic to penicillin, you can
use clindamycin, azithromycin, or clarithromycin as well as first-generation cephalosporins.
Bowel and urinary procedures in the presence of infection are an indication for ampicillin
and gentamicin with high-risk lesions. If the patient is penicillin-allergic, then use vanco-
mycin. Gentamicin is added for GI or urinary procedures and cardiac lesions at high risk of
endocarditis, such as prosthetic valves.

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 Cardiology

Lesions that DO NOT need prophylaxis are:

• secundum ASD
• tricuspid regurgitation
• MVP with only a click
• repaired congenital disease
• previous coronary bypass
• pacemakers, and
• defibrillators

Procedures that DO NOT need prophylaxis are:

• fillings
• suture removal
• flexible endoscopy
• transesophageal echocardiography
• cardiac catheterization
• angioplasty, and
• female genitourinary procedures.

The bottom-line is that the vast majority of cardiac lesions no longer need prophylaxis. The
only lesions needing prophylaxis are prosthetic valves, unrepaired complex cyanotic heart
disease, and those with previous endocarditis. Aortic stenosis, aortic regurgitation, mitral
stenosis, mitral regurgitation, hypertrophic cardiomyopathy, and mitral valve prolapse even
with regurgitation no longer need prophylaxis.

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 IM Board Review Course Book

PERIPHERAL ARTERIAL DISEASE (PAD)

Case 1
67-year-old man comes to office for mild soreness in his calves. The discomfort has
been going on for several months moderate in intensity. Although it rarely makes him
have to stop walking, the discomfort never occurs at rest. Examination normal. Pulse
normal. History of tobacco smoking and diabetes. LDL 145. On an ACE inhibitor.

1. Initial test?

2. Most accurate diagnostic test?

Angiography

3. What therapy will you start him on at this time?

Aspirin, stop smoking, statins, cilostazol, and exercise

4. Single most effective therapy for PAD?

5. Best antiplatelet medication?

6. A patient has PAD and an MI. Which medication will benefit the patient?
A. Diltiazem
B. Metoprolol
C. Nitroglycerin
D. Furosemide

Answer:

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Summary: Case 1
Most patients with peripheral vascular (or arterial) disease have normal physical examina-
tions. Although cool, pale skin, bruits, and the absence of pulses are highly specific, they are
infrequently present. Pentoxifylline has limited therapeutic effect and is less effective than
cilostazol. The best initial test is the ankle/brachial ratio. This should normally be greater
than or equal to 1. Although Doppler has some utility, angiography is still the most accurate
test. Therapy for peripheral arterial disease includes aspirin, exercise, comfortable footwear,
smoking cessation, cilostazol, and occasionally pentoxifylline. Pentoxifylline has a rather
minimal benefit, which is not always found in studies. Cilostazol is an antiplatelet medica-
tion that also has an antispasmodic effect. Cilostazol also stops the proliferation of vascular
endothelial cells. Clopidogrel has a greater efficacy than pentoxifylline. The combination of
aspirin and clopidogrel is more effective than either drug alone but has more adverse effects,
such as bleeding. If medical therapy is ineffective, then bypass surgery is needed. Beta block-
ers are not contraindicated in patients with PAD who need them for coronary disease or CHF.

1. Greatest risk for peripheral arterial disease?

A. Smoking
B. Diabetes
C. Hypertension
D. Lack of exercise
E. Hyperlipidemia
F. Age

Answer:

2. 28-year-old Asian woman with fever, myalgia, and diminished peripheral pulse. Most
accurate diagnostic test?
A. ESR
B. Anti-endothelin antibodies
C. Angiography
D. Biopsy
E. CT scan

Answer:

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 IM Board Review Course Book

AORTIC DISEASE

Case 1
64-year-old man comes to emergency department (ED) with sudden onset of
substernal chest pain radiating to back. BP is 160/103 in right arm and 128/85 in
left arm. Diastolic murmur at lower left sternal border. EKG is normal.

1. Best initial test?

2. Most accurate test?

3. Initial treatment?

4. Most effective therapy?

Surgical repair of the defect, if necessary

Abdominal ultrasound shows a 3.5-cm pulsatile mass consistent with an aneurysm.

5. How will you best manage this patient?

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 Cardiology

Summary: Case 1
Acute dissection of an aortic aneurysm presents with chest pain. If the test wants you to be
able to answer a diagnostic question, it has to give you a clue, such as radiation of the pain
to the back, particularly in between the scapulae, or a widened mediastinum on chest x-ray.
Overall, the most accurate test is an angiogram. Prior to the angiogram, there are several less
invasive tests that can be performed, such as MRI, transesophageal echocardiogram, or CT
angiogram. All of them have about the same sensitivity and specificity; and it is difficult to
choose one over the other. Surgical repair is the most effective therapy. Initial therapy con-
sists of beta blockers and nitroprusside. Beta blockers are particularly effective because they
decrease pulse pressure and may retard progression of the dissection.

Abdominal aortic aneurysms are managed with surgical repair if they are wider than 5 cm.
For aneurysms smaller than 5 cm, repeat ultrasonography should be performed once a year.
You then operate when the aneurysm becomes larger than 5 cm. The rate of spontaneous
rupture is very low in aneurysms under 5 cm.

Side Effects of Certain Drugs Used in Cardiology

Procainamide: ANA positive lupus-like syndrome, wide QRS and torsades

Quinidine: Hepatitis, thrombocytopenia, hemolytic anemia, torsades, decreased

digoxin excretion

Phenytoin: Heart block, ataxia, nystagmus, vertigo, seizures, rash, pseudolym-

phoma, megaloblastic anemia, peripheral neuropathy

Amiodarone: Bradycardia, increased heart block, increased digoxin concentration,

ventricular arrhythmias, corneal micro-crystallization, hypo- and
hyperthydroidism, pulmonary fibrosis, hepatitis, and blue tint of
exposed skin

Captopril: Loss of taste, leucopenia, cough, urticaria

Sotalol: Torsades, decreased LVF, bradycardia, and other side effects associated
with beta blockers

Mexiletine: Leukopenia, CNS and GI side effects

Plavix (Clopidogrel): TTP-like syndrome

Nicotinic acid: Hyperglycemia, hyperuricemia, liver dysfunctions, flushing

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 IM Board Review Course Book

PREOPERATIVE EVALUATION

Case 1
68-year-old man being evaluated for hip replacement. Asymptomatic able to play
golf twice a week. He had a myocardial infarction many years ago. Stress test
1 year ago was normal.

1. What would you recommend?

A. Exercise tolerance test
B. Stress thallium
C. Dobutamine echocardiography
D. Angiography
E. No further evaluation necessary prior to surgery

Answer:

Case 2
58-year-old man for elective cholecystectomy. History of diabetes and hypertension.

1. Which is most likely to benefit during perioperative period?

A. Nitroglycerin
B. Aspirin
C. Atenolol
D. ACE inhibitors
E. Angioplasty

Answer:

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 Cardiology

Case 3
Healthy man evaluated for elective hernia repair. Plays tennis regularly. EKG: left
bundle branch block.

1. What would you recommend?

A. Exercise tolerance test
B. Stress thallium
C. Dobutamine echocardiography
D. Angiography
E. No further evaluation necessary prior to surgery

Answer:

Case 4
74-year-old man to undergo carotid endarterectomy. Had coronary bypass 3 years
ago. No chest pain since that time.

1. What would you recommend?

A. Exercise tolerance test
B. Stress thallium
C. Dobutamine echocardiography
D. Angiography
E. No further evaluation necessary prior to surgery

Answer:

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 IM Board Review Course Book

Case 5
88-year-old woman is admitted to with sigmoid volvulus. She is demented and
cannot offer a history. After initial reduction by endoscopy, the volvulus recurs and
colon is remarkably dilated.

1. What would you recommend?

A. Exercise tolerance test
B. Stress thallium
C. Dobutamine echocardiography
D. Angiography
E. No further evaluation necessary prior to surgery

Answer:

Perioperative Question
1. Which is the most important prognostic factor prior to surgery?
A. Diabetes
B. Hyperlipidemia
C. S3 gallop
D. Tobacco use
E. Myocardial infarction 5 years ago
F. Ectopic beats
G. Hypertension

Answer:

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 Gastroenterology
2
DISEASES OF THE ESOPHAGUS
1. Dysphagia

• Motility Disorders → Achalasia

→ Scleroderma
→ Diffuse Esophageal Spasm

• Obstructive Disorder → Esophageal Carcinoma

→ Peptic Stricture
→ Esophageal Ring

56-year-old man presents with complaints of heartburn 3 × per week, awakening

him from sleep. There is no dysphagia, odynophagia, or weight loss. He has been
taking Tums “by the gallon.” Despite taking over-the-counter famotidine (Pepcid®),
he continues to have heartburn. He smokes one pack of cigarettes per day. The
symptoms have been occurring for 5 years.

1. What is the Next best step in management?

A. Barium swallow
B. Upper endoscopy
C. 24-hour pH
D. Empiric PPI (proton pump inhibitor)

Answer:

2. 56-year-old man undergoes upper endoscopy that shows erosive esophagitis and
Barrett’s esophagus. What is the risk of developing esophageal cancer over his lifetime?
A. 1%
B. 10%
C. 20%
D. 50%

Answer:

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 IM Board Review Course Book

3. What characteristic(s) of Barretts’s esophagus affect the risk of developing esophageal

adenocarcinoma?
A. Frequency of heartburn
B. Length of Barrett mucosa
C. Lifespan of the patient
D. Results of pathology
E. All of the above

Answer:

4. Can treatment with PPI reverse the epithelial change in Barretts?

A. Yes
B. No

Which can it reverse?

A. Dysplasia → metaplasia
B. Metaplasia → normal mucosa

Answer:

5. Barrett’s screening
How often do you screen patients who have Barretts?
EGD + Barretts + →
1 year later no (−) dysplasia + metaplasia →
Low grade dysplasia → if still low grade again →
Dysplasia → metaplasia
High-grade dysplasia →

6. When is life expectancy normal in Barretts?

7. What is the best initial treatment?

A. PPI twice a day
B. PPI daily
C. H-2 blocker, clarithromycin, and amoxicillin
D. PPI daily and upper endoscopy every year indefinitely
E. PPI daily and upper endoscopy in 2 to 3 years

Answer:

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 Gastroenterology

8. After initiating therapy with omeprazole in a patient with “heartburn,” the patient
continues to have symptoms. What should you do to treat or to evaluate the heartburn?
A. Barium swallow
B. Upper endoscopy
C. 24-hour pH
D. Double the dose of the PPI

Answer:

9. 38-year-old man now has dysphagia that is associated with severe substernal pain. The
pain occurs regardless of eating. Diagnosis and treatment?
A. Achalasia, do dilation
B. Diffuse esophageal spasm, give nifedipine
C. Scleroderma, use PPIs
D. Esophageal carcinoma, perform surgery

Answer:

10. Most likely etiology if dysphagia begins after 10 years of heartburn?

A. Peptic (Schatzki) ring
B. Scleroderma
C. Squamous cell esophageal cancer
D. Chagas esophagus

Answer:

11. A patient with dysphagia undergoes a barium swallow which shows a tubular esopha-
gus with a tight lower esophageal sphincter. Next test?
A. Barium swallow
B. Upper endoscopy
C. 24-hour pH
D. Manometry/motility study

Answer:

12. Best test to evaluate dysphagia in a 43-year-old woman with a negative endoscopy and
weight loss?
A. Barium swallow
B. Esophageal manometry/motility
C. 24-hour esophageal pH
D. Serum gastrin
E. CT (computed tomography) scan of chest

Answer:

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 IM Board Review Course Book

13. Heartburn and regurgitation for the last 5 years in a 60-year-old man lead to which of
the following?
A. Adenocarcinoma of the esophagus
B. Barrett esophagus
C. Esophageal stricture
D. Gastric outlet obstruction
E. Esophageal ulcer

Answer:

14. HIV+ with oral thrush presents with painful swallowing.

Most likely diagnosis? →

What is there is no improvement?

Next Best Step? →

Dysphagia with
HIV CD4 < 100

Yes

Empirically start
fluconazole

Yes Improvement No

Continue therapy Perform upper

and HAART endoscopy with
biopsy

CMV – large HSV – small

ulcerations. Tx: ulcerations. Tx:
ganciclovir or acyclovir
foscarnet

Answer:

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 Gastroenterology

15. What is the best diagnostic test to evaluate a 33-year-old man who is awakening every
night with wheezing? There is no heartburn or history of asthma.
A. Upper endoscopy
B. Barium swallow
C. 24-hour ambulatory pH
D. Esophageal manometry
E. Nuclear scan

Answer:

16. 60-year-old woman with daily heartburn for 3 months failed to respond to a trial of
proton pump inhibitor therapy. Upper endoscopy, reveals esophageal mucosa that is
normal. The most appropriate diagnostic procedure is which of the following?
A. Endoscopic biopsy
B. Esophageal motility
C. 24-hour ambulatory esophageal pH
D. Upper GI (gastrointestinal) series (esophagography)

Answer:

17. 45-year-old man has had dysphagia of increasing severity over the past year. He has
recently lost 5 lb. The upper endoscopy shows distal erythema of the esophageal mucosa
and resistance to the passage of the endoscope at the esophagogastric junction. No
anatomic lesion is seen. Esophageal motility shows lack of peristalsis in the body of the
esophagus and a high-pressure lower esophageal sphincter with incomplete relaxation
with swallowing. Which one of the following treatments would NOT be appropriate
for this patient?
A. Pneumatic dilatation
B. Botulinum toxin injection
C. Surgical myotomy
D. Anticholinergic agents
E. Calcium channel blockers

Answer:

18. 57-year-old male smoker and drinker has had dysphagia to solids worsening over 1
month, during which time he has lost 10 lb. The most appropriate initial diagnostic
procedure would be which of the following?
A. Barium swallow
B. Endoscopy
C. Esophageal motility
D. 24-hour esophageal pH (ambulatory)

Answer:

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 IM Board Review Course Book

19. 45-year-old woman has a long history of dysphagia found to be due to esophageal
“webs.” She has a chronic anemia of unclear etiology. Over the last 6 months, she has
had increased dysphagia and has lost 10 lb. An endoscopy is performed and a friable
mass is seen. What is the most likely diagnosis?
A. Squamous cell carcinoma
B. Adenocarcinoma
C. Benign stricture
D. Reflux-associated ulcer

Answer:

20. Patient with intermittent dysphagia and chest pain to solids and liquids presents to the
office. He also states the difficulty swallowing is associated with chest pain.
Most likely diagnosis? →
Next best step? →
What will manometry show? →
Next step in management? →
If no response →

21. Young patient states he had a lump of steak get stuck 3 times in the last 6 months. He
states it always occurs with the first bite. Followed by regurgitation, followed by eating
the rest of the meal normally.
Most likely diagnosis? →
Next best step? →

22. Patient with dysphagia has an EGD that shows no change in the anatomy of the lower
esophagus.
Most likely diagnosis? →

23. Young female presents with multiple food impactions over several months. EGD shows
a furrowed appearance or concentric rings. Patient has multiple allergies.
Most likely diagnosis? →
Next best step? →
Best management for eosinophilic esophagitis →

24. An:
Older man with osteoporosis…
Teenager on tetracycline…
Old woman with gastroparesis…
…Presents with complaints of dysphagia

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 Gastroenterology

Most likely diagnosis? →

Best counseling step →
Best step in management →

Medication-induced esophageal injury heal without intervention within a few days

If the patient presents with difficulty swallowing again…

Most likely diagnosis? →

Stricture formation after scarring from pill esophagitis

Next best step? →

Pneumatic dilation

Summary: Case 1
Upper endoscopy should be performed in all persons over age of 45–55 who complain of Note
long-standing heartburn, especially if there are warning signs such as weight loss and dyspha-
gia. There is an increased relative risk of Barrett esophagus and esophageal cancer in persons Meds that cause pill esophagitis:
who have heartburn more than 3 times per week for over 5 years. Barrett esophagus poses a
ASA
real risk for the development of adenocarcinoma of the distal esophagus; however, the risk is
probably less than 0.5% per year. The risk is dependent on age, gender, race, length of mucosa Bisphosphonates
involved, tobacco use, treatment, and histology (presence of dysplasia). The risk of squamous Tetracyclines
cell carcinoma is related to gender, alcohol use, tobacco use, history of caustic ingestion, acha-
KCl
lasia, esophageal webs (Plummer-Vinson syndrome), and tylosis. The best test for confirming
the diagnosis of GERD is a 24-hour pH. Only this test can verify the presence of acid reflux Quinidine
disease. Endoscopy will be normal in almost 50% of patients with heartburn secondary to
GERD. Dysphagia to both solids and liquids in young persons is typically a motility disorder,
such as scleroderma, multiple sclerosis, or Chagas esophagus. IBS, globus hystericus, and Note
anxiety disorder should also be considered. In older persons, CNS events (stroke) are more
common. Peptic rings, inflamed Schatzki rings, and complications of GERD should also be Situations that have higher pill
considered in persons with a long-standing history of heartburn. Achalasia should be con- retention rates
sidered in patients with dysphagia. The characteristic finding of (1) loss of normal contrac-
tility of the esophagus and (2) persistent tightening of the LES (lower esophageal sphincter) • L ack of an adequate liquid
establishes the diagnosis. The gold standard for the evaluation of achalasia is the esophageal bolus and a long period in
motility test. Treatment options include balloon dilatation, botulinum toxin, calcium chan- the recumbent position
nel blockers, and Heller myotomy. • Ingestion of a pill
immediately prior to sleep
GERD Breakdown
• A ge > 70 years and
Symptoms:
decreased peristaltic
Chest pain amplitudes
Heartburn • P atients with cardiac
Nocturnal cough disease, particularly
Asthma following thoracotomy
Hoarseness
Loss of dental enamel

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 IM Board Review Course Book

Frequent throat clearing

GERD → Barretts → Adenocarcinoma

Treatment:

Weight loss
Head of bed elevation
Stop smoking
Dinner 3 hours before bedtime
PPIs superior to H2 blockers
Fundoplication surgery if no response to above

When do you do endoscopy in GERD?

GERD symptoms that don’t improve with PPIs:

Odynophagia and weight loss

Symptoms >5 years
Age >50, male
Confirmed history of Barretts

1. Patient improves after 3 months of being on a PPI. Treat for life?

Next best step?

2. 55-year-old male with hyperlipidemia and 25-year smoking history presents with
2 hours of chest pain that does not relieve with rest. Troponin negative × 3. Echo nega-
tive. Cath negative. No dysphagia.
Most likely diagnosis?
Next best step?

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DISEASES OF THE STOMACH

Case 1
A 66-year-old man presents with vague epigastric “gnawing” discomfort that
occasionally awakens him from sleep. There has been no melena, hematochezia,
fever, chills, or weight loss. Past medical history includes asthma, a TIA, and peptic
ulcer disease many years prior. Medications are aspirin and beclomethasone
inhaler. Physical examination reveals a soft abdomen, mildly tender in the
epigastrium. Abdominal ultrasound is normal.

1. What is the best initial test in the evaluation of this patient?

A. Upper gastrointestinal series
B. Upper endoscopy
C. MRI (magnetic resonance imaging) of the abdomen
D. CT scan with intravenous contrast
E. Serology for Helicobacter pylori

Answer:

Upper endoscopy shows a 1-cm ulcer in the gastric antrum.

2. How does the management of this endoscopic finding differ from a duodenal ulcer?
A. Biopsies of the ulcer are needed.
B. Helicobacter pylori evaluation is necessary.
C. Repeat upper endoscopy will be needed after 6 weeks of treatment.
D. Both A and C are true.

Answer:

3. If the upper endoscopy showed multiple esophageal ulcers and duodenal ulcers in the
second and third portions, what additional tests should be performed?
A. Enteroscopy
B. Serum gastrin level
C. Small bowel series
D. Serum salicylate level
E. Capsule endoscopy

Answer:

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 IM Board Review Course Book

4. If biopsies show mucosa-associated lymphoid tissue (MALT) lymphoma, what is the

best initial treatment?
A. 5-FU/cisplatin
B. Surgical resection
C. Lansoprazole, amoxicillin, clarithromycin
D. External beam radiation

Answer:

5. If serum gastrin level is 498 pg/mL (normal 40–200 pg/mL), what is the best next step?
A. Secretin stimulation test
B. MRI of abdomen
C. EUS (endoscopic ultrasound) of pancreas
D. CT scan of abdomen

Answer:

6. If serum gastrin level is 1,800 pg/mL (normal 40–200 pg/mL) and CT scan of abdomen
with contrast is negative, what is the best next step?
A. EUS and octreoscan (nuclear somatostatin receptor scintigraphy)
B. Octreoscan
C. MRI of abdomen
D. CT scan without IV (intravenous) contrast
E. EUS

Answer:

7. For which one of the following conditions is therapy for Helicobacter pylori a proven benefit?
A. NSAID-induced PUD
B. Duodenal ulcers
C. Non-ulcer dyspepsia
D. Gastric cancer
E. Gastroesophageal reflux disease

Answer:

8. Which one of the following conditions is not a risk factor for developing a complication
of NSAID-induced ulceration (bleeding, perforation, gastric outlet obstruction)?
A. History of PUD
B. Age over 75
C. Concomitant corticosteroid use
D. Cardiovascular disease
E. Helicobacter pylori infection

Answer:

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 Gastroenterology

9. In a patient in the medical intensive care unit, which one of the following disorders
would be associated with a significant risk for hemorrhage from stress-related mucosal
disease?
A. MI with congestive cardiac failure
B. Exacerbation of asthma not requiring mechanical ventilation
C. Respiratory failure requiring mechanical ventilation
D. Diabetic keotacidosis
E. Acute renal failure

Answer:

10. Which one of the following tests is the most objective means of documenting
gastroparesis?
A. Endoscopy
B. Upper GI series
C. Ultrasonography
D. Nuclear medicine scintigraphy

Answer:
Note
H. Pylori disease breakdown
Treatment Regimens for
Gram negative, urease producing
H. Pylori:
Increased in 3rd world
PAC for 14 days
Causes PUD (duodenal) PPI
Gastric Malignancy → Maltoma Amoxicillin
Clarithromycin
H. Pylori testing
MOC for 14 days – PCN allergy
Metronidazole
Omeprazole
Clarithromycin
Non Endoscopic Endoscopic Treatment Failure
• Antibody test for • To culture for
Tetracycline
diagnosis, not for follow up resistance for culture
• Urea breath test for diagnosis • Urease testing Metronidazole
and for eradication testing • Gold standard Bismuth salicylate
• Fecal antigen test for PPI
diagnosis and follow up

Note
1. True or False – Erosive gastritis is caused by:
Alarm symptoms:
NSAIDS T F
Weight loss
ETOH T F Heme-positive stool
Smoking T F Odynophagia/Dysphagia
Stress T F

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 IM Board Review Course Book

2. In which of the following situations is a PPI indicated?

A. Major Surgery
B. Burns
C. Ventilator
D. Head trauma
E. All of the above

Answer:

3. 42 year old male immigrant with peptic ulcer disease. What are the 3 most commonly
tested causes?
1. H. pylori
2. NSAIDs
3. Zollinger–Ellison Syndrome

What 3 lifestyle habits prevent healing in PUD?

1. Alcohol
2. Diet
3. Smoking
4. Stress

4. 33 year old female has MALT lymphoma on biopsy.

Most likely diagnosis? →
Best step in management →
After 2-3 months next best step →
If still positive →

Test for translocation (11:18)

Followed by → radiation or resection

5. Non-Ulcer Dyspepsia
56-year-old male with upper abdominal pain without weight loss, diarrhea, or change
in bowel habits. Exam normal. EGD and bx negative for HP. No ulcers seen.
Most likely diagnosis? →
Best step in management →
<50 and no alarm symptoms
>50 or alarm symptoms

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Summary: Case 1
Gastric carcinoma may appear as an ulcer and respond initially to treatment. This can result
in a delay in the diagnosis, which profoundly influences care. Upper endoscopy should be
performed in all persons over the age of 45 who complain of epigastric pain, especially if
there are warning signs, such as weight loss, anemia, and early satiety. The risks for the devel-
opment of peptic ulcer disease include H. pylori, aspirin (acetylsalicylic acid), NSAID (non-
steroidal anti-inflammatory drug) use, CNS (central nervous system) trauma/mass, burns,
and severe stress (ventilators).

ZE (Zollinger-Ellison) syndrome is seen in patients with ulcer disease, typically involving

the duodenum and esophagus. The secretin stimulation test is the best initial test in the
evaluation of patients suspected of having ZE syndrome. Intravenous secretin will result in a
persistent elevation, or even rise, in the gastrin level. The most common ulcer in ZE is a large
single duodenal ulcer. However, ZE should be considered in all patients with severe acid–
peptic disease and distal duodenal ulcers. A serum gastrin greater than 1,000 in the setting of
the appropriate clinical symptoms is diagnostic of ZE. The workup then includes staging and
identification of the lesion. The nuclear scan with octreotide can detect disease. Because the
most common lesion is in the pancreas or duodenal wall, endoscopic ultrasound is a standard
in identification of the gastrinoma. Aside from PUD (peptic ulcer disease), H. pylori is associ-
ated with gastric lymphoma and gastric cancer. Superficial MALT lymphoma can be treated
initially with antibiotics directed at H. pylori. H. pylori treatment should include a PPI and
two antibiotics (know metronidazole resistance for your area). The two antibiotics should
include clarithromycin, amoxicillin, tetracycline, and/or metronidazole.

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DIARRHEA

Enterotoxigenic Invasive Parasitic

• E.coli • C. jejuni • Giardiasis
• Vibrio cholerae • Salmonella • Amebiasis
• C. perfringens • Shigella • Cryptosporidium
• B. cereus • O157:H7 • Isospora
• S. aureus • C. difficile
• Vibrio parahemolyticus
• Vibrio vulnificus

1. A 44-year-old woman presents with diarrhea. What is the most likely cause of her
symptoms (include best test and treatment) under each of the following conditions? The
symptoms began on a cruise ship 24 hours ago. There is associated nausea and vomiting.
A. Rotavirus
B. Norovirus
C. E. coli
D. Campylobacter
E. Staphylococcus

Answer:
Treatment:

2. The woman works in a nursery school.

A. Rotavirus
B. Norovirus
C. Enteroadherant (pathogenic) E. coli
D. Campylobacter
E. Staphylococcus

Answer:
Treatment:

3. The diarrhea is associated with left lower quadrant pain, blood, and fever.
A. Rotavirus
B. Norovirus
C. E. coli
D. Campylobacter jejuni
E. Staphylococcus toxin

Answer:
Treatment:

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4. The patient consumed potato salad at a picnic. The symptoms began with vomiting
6 hours after eating.
A. Rotavirus
B. Norovirus
C. E. coli
D. Campylobacter jejuni
E. Staphylococcus toxin

Answer:
Treatment:

5. Sushi was consumed 2 days ago and blisters are forming on the patient’s legs.
A. Rotavirus
B. Vibrio vulnificus
C. E. coli
D. Campylobacter jejuni
E. Staphylococcus toxin

Answer:
Treatment:

6. The patient has recently returned from Mexico.

A. Vibrio cholera
B. Shigella
C. Enterotoxigenic E. coli
D. Campylobacter jejuni
E. Staphylococcus toxin

Answer:
Treatment:

7. The patient has bloody bowel movements after consuming some raw (or undercooked)
chopped meat.
A. Vibrio cholera
B. Shigella
C. E. coli 0157:H7
D. Campylobacter jejuni
E. Staphylococcus toxin

Answer:
Treatment:

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8. The patient consumed fried rice.

A. Bacillus cereus
B. Listeria monocytogenes
C. E. coli 0157:H7
D. Campylobacter jejuni
E. Staphylococcus toxin

Answer:
Treatment:

9. The patient consumed old creamy pastries and now has pain in the RLQ (right lower
quadrant).
A. Malafia bubu
B. Bacillus cereus
C. Enterotoxigenic E. coli
D. Campylobacter jejuni
E. Yersinia entercolitica

Answer:
Treatment:

10. The patient also has diabetes, chronic bloating, and weight loss. She has two or three
watery bowel movements per day that are often foul-smelling. The symptoms have been
going on for over a year. She has recently been found to have an iron deficiency anemia.
A. Celiac disease
B. Giardia lamblia
C. Trophyerma whipplii
D. All of the above

Answer:
Treatment:

11. The patient has profuse watery diarrhea and a mass in her pancreas.
A. VIPoma
B. Glucagonoma
C. Zollinger-Ellison syndrome
D. A and C are consistent with this diagnosis.
E. All of the above

Answer:
Treatment:

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12. The patient has diabetes, hypertension, and family history of CAD (coronary artery
disease). She is a smoker; and the diarrhea began 6 hours after crampy left upper quad-
rant pain. There is now scant red blood in the stool.
A. Chronic intestinal ischemia
B. Ischemic colitis
C. Acute mesenteric ischemia

Answer:
Treatment:

13. A 50-year-old man presents to the emergency room with a 24-hour history of 8–10
episodes of liquid stools without blood. There is diffuse abdominal cramping that is
relieved by each bowel movement. The patient denies tenesmus. His pulse is 91/min
and blood pressure is 120/87 mm Hg. He is afebrile and not orthostatic. The best initial
approach to this patient would be which of the following?
A. Admit for observation
B. IV hydration with lactated Ringer
C. Empiric antibiotics with ciprofloxacin
D. Reassurance with oral fluids
E. Flexible sigmoidoscopy with biopsy of the rectosigmoid region

Answer:
Treatment:

14. Which of the following produces an inflammatory diarrhea (fecal leukocytes)?

A. Giardia lamblia
B. Norwalk agent
C. Staphylococcus aureus
D. Clostridium difficile
E. Vibrio cholerae

Answer:
Treatment:

15. 67-year-old woman presents with diarrhea. She has a history of coronary artery dis-
ease. Last night, severe left-sided pain awoke her from sleep, and the diarrhea began
4 hours later. There is scant blood. Currently, there is no pain, fever, or chills. A colo-
noscopy last year was normal. Physical examination is unremarkable. The best next step
would be which of the following?
A. Angiogram
B. MRA (magnetic resonance angiography)
C. CT scan of the abdomen
D. Clear liquids and observation

Answer:
Treatment:
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Note 16. Diarrhea and abdominal pain after exposure to antibiotics.

Most likely diagnosis →
Strain of C. difficile that causes
toxic megacolon is BINAP1 Next best step →
strain. Best initial diagnostic step →
Most accurate diagnostic step →

Note Stool is + for C. difficile toxin; treated with metronidazole for 10 days and symptoms
improve. 2 weeks after the treatment develops diarrhea and abdominal pain again.
PPI raises the risk of Next best step →
C. difficile.
Stool is + for C. difficile toxin; treated with metronidazole for 10 days and symptoms
improve. 2 weeks after the treatment develops diarrhea and abdominal pain again.
Next best step →

Stool is + for C. difficile toxin; patient started on antibiotics but a day later patient has
increased distension, tenderness, and lethargy. BP 88/50. HR 120. Lactate 16 mEq/L.
X-ray shows ileus.
Most likely diagnosis?

Next best step? →

17. Best therapy to prevent C. difficile in a patient getting antibiotics for sinusitis or any
disease?

18. After returning from a vacation in Colombia, a patient presents with non-bloody
diarrhea, epigastric pain, iron-deficiency anemia, and eosinophilia.
A. E. coli
B. Giardia
C. Yersinia
D. Strongyloides
E. Cholera

Answer:
Treatment:

19. Patient with AIDS has large volume non-bloody diarrhea. Organisms are seen on modi-
fied acid fast stain.
A. Cryptosporidium
B. Herpes
C. CMV
D. E. coli
E. Yersinia

Answer:
Treatment:

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20. Patient is found to have a mass in his liver.

A. Cryptosporidium
B. Ascariasis
C. E. Histolytica
D. Inflammatory bowel disease
E. E. coli

Answer:
Treatment:

21. Patient with diarrhea, food intolerance, nausea and vomiting, and abdominal
discomfort. Steatorrhea is present O&P workup is negative. Labs reveal peripheral
eosinophilia+. What is the most likely diagnosis?

22. Young patient with abdominal pain described as crampy relieved by the passage of
small amounts of stool and mucus. Occasionally she notes constipation. No nausea,
vomiting, melena, hematochezia, or weight loss. Lab exams are normal.
Most likely diagnosis? →
Best treatment if:
Diarrhea predominant →
Constipation predominant →

23. Nursing student presents with chronic diarrhea. Stool workup reveals osmolality
of 300. Stool Na is 50 and K is 40. NaOH turns the sample red. What is the most likely
diagnosis?
Most likely diagnosis →
Next best step →

24. Diarrhea with RLQ pain, rash, and fecal leukocytes are positive.
Most likely diagnosis →
Treatment →

25. Patient with steatorrhea and laboratory shows hemoglobin of 9.0 and MCV of 104.
A fecal occult blood test is negative. Abdominal CT scan which shows small bowel
diverticula. What is the most likely diagnosis?
Most likely diagnosis → Bacterial overgrowth

Next best step →

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26. Patient with iron deficiency anemia (low serum iron, high TIBC, low ferritin and
microcytic indices). No alarm symptoms. EGD and colonoscopy are normal. Which of
the following would help in diagnosis?
Most likely diagnosis →
Next best step →

Which is the most specific antibody?

27. Patient with celiac disease responds well to this diet for the next 12 years. Now presents
with diarrhea, abdominal pain and weight loss. What is the most likely diagnosis?
Most likely diagnosis →
Next best step →

Patient with celiac disease now on gluten free diet for 3 months. Still with pain and
weight loss. What is the most likely diagnosis?

Celiac Breakdown

• Bulky, greasy stool (steatorrhea) and weight loss, abdominal distention, presenting
only with anemia
• Usually iron deficiency anemia, occasionally elevated PTT and/or transaminases
• Associated with auto-immune diseases: (Dermatitis herpetiformis, DM I,
Hashimoto’s thyroiditis and seizure disorders)
• Diagnostics: anti-tissue transglutaminase (most specific), + anti-endomysial, anti-
gliadin or antibodies, villous atrophy on small bowel biopsy
• Treatment: Gluten-free diet (no wheat, barley, or rye)
• Long-term complications of malignancy (small bowel T-cell lymphoma), esophageal
carcinoma

28. Recent travel to the 3rd world now has abdominal pain and diarrhea. Wheezing over the
past year, which has been resistant to inhaled beta-agonists and steroids. + peripheral
eosinophilia.
Most likely diagnosis →
Next best step →

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29. A patient with weight loss, diarrhea, abdominal pain, and multiple joint pain that
returned from India 5 months ago and lost 15 lbs over this time. Also has generalized
lymphadenopathy. Small bowel biopsy shows dilated lymphatics and foamy macro-
phages with PAS+ granules. What is the most likely diagnosis?
Most likely diagnosis →
Next best step →

30. Whipple’s Disease Breakdown

• Caused by bacillus Tropheryma whippelii
• Fever, diarrhea, weight loss, arthritis and occasionally neurological deficits
• Exam: lymphadenopathy and arthritis; occasionally macular skin rash, various
neurologic deficits and murmurs
• Anemia, hypoalbuminemia, hypocarotenemia
• Small bowel biopsy shows foamy mononuclear cells filled with periodic acid-Schiff
staining material (PAS +), PCR for Tropheryma whippelii
• Treatment: Penicillin and streptomycin; ceftriaxone and streptomycin for CNS
disease) for 10-14 days then Bactrim for at least 1 year (cefixime or doxycycline if
sulfonamide allergic)

Summary: Case 1
The most common causes of infectious diarrhea are noroviruses. Cultures are typically nega-
tive, and symptoms resolve relatively quickly. Bacterial pathogens can be divided between
invasive and noninvasive. The noninvasive pathogens have classic characteristics, including
nausea, vomiting, creamy foods (Staphyloccocus); watery diarrhea (enterotoxigenic E. coli);
diarrhea and pain in the RLQ (Yersinia entercolitica); and consumption of fried rice
(B. cerius). The most common cause of invasive diarrhea in the United States is Campylobacter
jejuni. Salmonella is less common. E. coli 1057:H7 presents as bloody diarrhea, typically
associated with consumption of meat. Stool for culture and fecal leukocytes (Wright stain)
establishes the diagnosis. Malabsorption can be documented by fecal fat and serum beta
carotene. Chronic causes of diarrhea include small bowel disease (Giardia, celiac disease,
abetalipoproteinemia, amyloid, Whipple disease), pancreatic disease (chronic pancreatitis),
and inflammatory bowel disease. Small bowel biopsy in general establishes the diagnosis.
Celiac disease, sprue, and gluten enteropathy refer to a common disease that presents with
small-bowel malabsorption. Iron deficiency anemia is often found (and may be a presenting
sign). Selective IgA deficiency and diabetes are commonly associated. Antiendomyseal and
antigliadin antibodies are often positive (sensitivity and specificity over 90%). Small-bowel
biopsies typically have flattened villi that grow to normal after institution of a gluten-free
diet. Small-bowel lymphoma is associated with this disease. Ischemic colitis presents in
patients at risk for CAD (C. difficile–associated diarrhea) with pain followed by diarrhea.
Flexible sigmoidoscopy establishes the diagnosis by antimesenteric involvement of the colon.

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Disorder Diagnosis Clues or Risk Factors

Microscopic colitis Biopsies of colonic mucosa Secretory diarrhea pattern; includes collagenous colitis,
lymphocytic colitis
Ischemia Colon or small bowel imaging and Vascular disease, history of hematochezia, pain
biopsies
Pancreatic insufficiency Tests for excess fecal fat and Chronic pancreatitis, hyperglycemia, or history of
pancreatic calcifications pancreatic resection
Eosinophilic enteritis Small bowel biopsy (full-thickness Eosinophilia, hypoalbuminemia
biopsy)
Enteral feedings History Classic osmotic diarrhea
Bile acid malabsorption Diagnosis of exclusion, empiric History of resection of <100 cm of distal small bowel
response to cholestyramine
Bile acid deficiency Diagnosis of exclusion, excess Cholestasis, resection of >100 cm of small bowel
fecal fat
Radiation exposure History, small bowel and/or colon May begin years after exposure, strictures or hypervascu-
imaging lar mucosa with characteristic biopsy findings
Dumping syndrome History previous gastrectomy or Postprandial flushing, tachycardia, diaphoresis
gastric bypass surgery
Self-induced diarrhea Tests for stool pH, sodium, potas- Melanosis coli, somatization or other psychiatric
sium, and magnesium, history of syndromes
excess laxative use
Medications History Acarbose, abx (especially PCN, macrolides), antineoplas-
tic agents, colchicine, laxatives, magnesium-based
antacids, cathartics, PPI, etc.

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SCREENING FOR COLON CANCER

Case 1
1. What is the best method of screening a 55-year-old woman with no symptoms?
A. Colonoscopy and repeat examination if no polyps in 5 years
B. Colonoscopy and repeat examination if no polyps in 10 years
C. MRI of abdomen
D. Stool occult cards, colonoscopy if positive
E. Virtual colonoscopy

Answer:

2. What method(s) is/are acceptable for screening for colon cancer?

A. Colonoscopy
B. Flexible sigmoidoscopy
C. Stool occult cards
D. Barium enema
E. All are acceptable.

Answer:

3. What other method is equally as effective as colonoscopy in the evaluation of an occult-

positive stool?
A. Virtual colonoscopy
B. Flexible sigmoidoscopy and barium enema
C. Flexible sigmoidoscopy
D. Barium enema
E. Nothing is equally as effective as colonoscopy in the evaluation of occult-positive
stool.

Answer:

4. If a 1-cm tubular adenoma is found on colonoscopy, when should the next colonoscopy
be performed?
A. 6 months
B. 1 year
C. 3–5 years
D. 10 years

Answer:

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5. What is the best screening strategy for a 34-year-old woman whose father died of colon
cancer at age 55?
A. Colonoscopy every 5 years, beginning at age 40
B. Colonoscopy every 10 years, beginning at age 50
C. Colonoscopy every 5 years, beginning at age 45
D. Colonoscopy every 2 years, beginning at age 40

Answer:

6. What is the best screening strategy for a person with ulcerative colitis who has had
disease present for 14 years?
A. Colonoscopy with biopsies in 4 quadrants every 10 cm every 1 to 2 years
B. Flexible sigmoidoscopy every year
C. Colonoscopy every 5 years
D. Colonoscopy every 10 years
E. Annual barium enema

Answer:

7. What is the best screening strategy for a 38-year-old man whose mother died of colon
cancer at age 49, brother had a giant dysplastic polyp, and grandfather had colon can-
cer? His sister had uterine cancer at age 52.
A. Colonoscopy every 5 years, beginning at age 39
B. Flexible sigmoidoscopy annually, beginning at age 12
C. Colonoscopy every 1 to 2 years, beginning at age 25 (therefore, now)
D. Barium enema annually
E. Colonoscopy every 3 to 5 years

Answer:

8. What is the best screening strategy for the 12-year-old son of a 38-year-old woman who
had colon cancer at age 20? The boy’s 19-year-old brother, on a recent examination, was
found to have multiple polyps.
A. Colonoscopy every 1 to 2 years, beginning at age 25
B. Flexible sigmoidoscopy every year, beginning at age 12 (now)
C. Colonoscopy every 5 years, beginning at age 25
D. Barium enema every 5 years
E. Flexible sigmoidoscopy every year, beginning at age 30

Answer:

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Summary: Case 1
Colon cancer is preventable. All persons over the age of 50 should undergo a colonoscopy for
screening for colon cancer every 10 years (best strategy). Fecal occult tests, barium enema,
and flexible sigmoidoscopy are inferior tests but are considered acceptable screening tests. If
there is a family history of colon cancer, every family member, regardless of age, should have
a colonoscopy either at the age of 40 or at an age 10 years younger than the age at which the
family member developed the disease, whichever is earlier. In these patients, the colonoscopy
is repeated every 5 years. If a polyp is found, a surveillance colonoscopy should be performed
after 3 to 5 years. All persons with ulcerative colitis/Crohn colitis should undergo annual
colonoscopy with biopsy after 10 years of disease. Patients with Lynch syndrome (hereditary
nonpolyposis colorectal cancer) should be screened every 2 years, beginning at age 25. Familial
adenomatous polyposis should be considered in persons with family histories of colon cancer
in their early 20s and 30s. Screening is with flexible sigmoidoscopy, beginning at age 12.

Colonic Polyps
• Types
• Hyperplastic – thickened mucosa not pre-malignant
• Tubular adenoma and villous adenoma – premalignant
• Risk of Ca in situ increases: Size > 2 cm, more villous, more sessile
• At Risk
• 5-10% of people older than 40 have colonic polyps
• 1st degree relatives of polyp patients have a 5x increased risk of having polyps
• Clinical
• Usually asymptomatic, hemoccult +, rarely gross bleed or obstruction
• Diagnosis
• Colonoscopy with removal

Type Number; Size When to do Colonoscopy

Low risk – Tubular Single < 1 cm 3-5 years
High risk – Villous Multiple > 1 cm 1 year

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 IM Board Review Course Book

INFLAMMATORY BOWEL DISEASE

Case 1
A 44-year-old woman presents with diarrhea, weight loss, fatigue, and RLQ pain
over the last 2 weeks. She has 3 to 8 watery bowel movements per day with
mucus, no melena, and no hematochezia. P.E. reveals low-grade fever, injected
sclera, multiple aphthous ulcers, decreased bowel sounds, and tenderness in the
RLQ. There are multiple erythematous lesions on her shins. Rectal examination
reveals a small perirectal fistula.

1. Which finding is exclusively associated with Crohn disease?

A. Low-grade fever
B. RLQ pain
C. Erythema nodosum
D. Perirectal fistula
E. Episcleritis

Answer:

2. What is the best single test to determine the presence of Crohn disease (highest diag-
nostic yield)?
A. Small-bowel series with air contrast (enteroclysis)
B. Colonoscopy
C. Flexible sigmoidoscopy
D. MRI

Answer:

3. Which dermatologic or ocular manifestation of Crohn disease does not correlate with
activity of disease?
A. Erythema nodosum
B. Pyoderma gangrenosum
C. Episcleritis
D. Maculpapulosis

Answer:

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4. What medication should be initiated in this patient who has mild/moderate inflamma-
tory bowel disease?
A. Sulfasalazine
B. Mesalamine
C. Prednisolone
D. Ciprofloxacin

Answer:

5. If the patient does well on mesalamine and then relapses, what medication should be
added?
A. Sulfasalazine
B. Mesalamine
C. Prednisolone
D. Ciprofloxacin

Answer:

6. On tapering the prednisone, she has relapses. What medication should be initiated in
this patient to avoid persistent use of steroids and to prevent relapses?
A. Rifaxamin
B. 6-Mercaptopurine/azathioprine
C. Methotrexate
D. Ciprofloxacin

Answer:

7. If the alkaline phosphatase is found to be elevated, what do you suspect?

A. Primary biliary cirrhosis
B. Sclerosing cholangitis
C. Biliary sludge
D. Common bile duct stone
E. Bone disease

Answer:

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8. Which extraintestinal manifestation of ulcerative colitis is not affected by early col-

ectomy?
A. Sclerosing cholangitis
B. Metastatic colon cancer
C. Small-joint arthritis
D. Episcleritis
E. Uveitis

Answer:

9. Persons with Crohn disease involving the ileum are at risk for developing which of the
following?
A. Calcium oxalate kidney stones
B. Cholesterol gallstones
C. B12 deficiency
D. Bile salt diarrhea
E. All of the above

Answer:

Note 10. After being treated with mesalamine and azathioprine, a fistula to the perianal region
develops. What medication(s) should be added to assist in healing the fistula?
Before starting anti-TNF must
A. Infliximab, ciprofloxacin, and metronidazole
check PPD and hepatitis B
B. Methotrexate
serology
C. Metronidazole
D. 6-mercaptopurine and sulfasalazine
E. Prednisolone

Answer:

11. Which of the following statements about colorectal carcinoma associated with Crohn
disease is true?
A. It usually occurs in women.
B. The frequency of carcinoma is similar in patients with extensive, long-standing,
unresected Crohn colitis to those patients with extensive, long-standing ulcerative
colitis.
C. The right colon is involved in over 70% of cases.
D. The mean age of patients with colorectal carcinoma is 35.
E. The occurrence of the carcinoma is unrelated to the duration of the Crohn disease.

Answer:

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12. Which of the following does not occur after extensive ileal resection for Crohn disease?
A. Nephrolithiasis
B. Iron-deficiency anemia
C. Cholelithiasis
D. Megaloblastic anemia
E. Bile salt diarrhea

Answer:

13. Which medication is effective for inducing remission but is not effective in maintaining
remission?
A. Mesalamine
B. Sulfasalazine
C. Corticosteroids
D. 6-Mercaptopurine
E. Azathioprine

Answer:

14. Which of the following is the most likely cause of a relapse of ulcerative colitis?
A. Spicy food
B. Raw vegetables
C. NSAIDs
D. Smoking

Answer:

15. Reducing the risk of colorectal cancer in inflammatory bowel disease is related to which
of the following?
A. Surveillance colonoscopy with biopsy
B. Vitamins
C. Long-term steroids
D. Annual barium enema

Answer:

CD UC
ASCA Positive Negative
ANCA Negative Positive

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Summary: Case 1
Inflammatory bowel disease (IBD) can present with chronic symptoms or acute symptoms
(bowel obstruction, sudden onset of diarrhea). Erythema nodosum, aphthous ulcers, pyo-
derma gangrenosum, seronegative arthritis, episcleritis, and uveitis may all manifest. Because
in most patients with ulcerative colitis the rectum is involved, a flexible sigmoidoscopy can
assist in the evaluation. Although endoscopic appearance can be deceiving, architectural dis-
tortion of the crypts is a hallmark feature on biopsy. As the ileum is most often involved in
patients with Crohn disease, a small-bowel series is preferred. Colonoscopy can evaluate the
rectum and distal ileum (in most patients). Treatment should begin with mesalamine (5-ASA)
in patients with mild to moderate disease. Steroids are added if there is no response, or if
disease is severe. The drug 6-MP and its parent, azathioprine, are steroid-sparing medications
used in patients who relapse. Efficacy is not reached for 2 to 3 months after initiation of treat-
ment. Due to involvement of the terminal ileum, B12 deficiency, calcium oxalate stones, and
gallstones can occur. Although surgery is curative in patients with ulcerative colitis, 50% of
patients with Crohn disease will have a relapse within 5 years of surgery. Sclerosing cholangitis
complicates IBD. Progression to cirrhosis and cholangiocarcinoma cannot be prevented by
treatment of the underlying IBD (e.g., surgery). Metronidazole has been shown to be effective
in perianal Crohn disease. Infliximab (Remicaide®) is effective in fistulizing Crohn disease.

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UPPER GASTROINTESTINAL BLEEDING/

END-STAGE LIVER DISEASE

Case 1
74-year-old man presents with hematemesis and 3 watery, black bowel movements
over one day. There has been 2 weeks of increasing fatigue and jaundice. He takes
naproxen for headaches. Temperature 39°C (102.2°F), BP 100/60 mm Hg, pulse
120/min. Sclerae are yellow with decreased bowel sounds, tense ascites, and trace
lower extremity edema. Lab results are:

WBC: 14,000 PT: 22 (INR 3) (elevated)

AST: 145 Platelets: 77,000 (low)
Albumin: 2.2 (low) Bilirubin: 12.7 (markedly elevated)
HCT: 28 Alk phosphatase: 144
ALT: 66

1. Which of the following is true?

A. Octreotide should not be given until an endoscopy is peformed, verifying the pres-
ence of varices.
B. The true hematocrit is likely around 20, and 2 units of blood should be given.
C. Fresh frozen plasma should be given only if the bleeding continues.
D. Antibiotics should not be given prophylactically.

Answer:

2. After stabilization, paracentesis reveals WBC of 200 (80% PMNs), and albumin of 0.5.
What does this indicate?
A. Spontaneous bacterial peritonitis (SBP) is present.
B. Portal hypertension is present.
C. Hepatoma is likely present.
D. Lifelong antibiotics to prevent SBP are necessary.
E. B and D are correct.

Answer:

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3. Upper endoscopy reveals varices, which are treated by band ligation. A clean-based
ulcer is identified. Which of the following is true?
A. The risk of rebleeding from a clean-based ulcer is low (less than 5%).
B. Metoprolol should be given on discharge to prevent a recurrent bleed from the varices.
C. Sclerotherapy is superior to band ligation in the treatment of varices.
D. Transjugular intrahepatic portal systemic shunting (TIPS) should be performed
on discharge to prevent a recurrent bleed from the varices.

Answer:

4. Three days after admission, the patient continues to have fever and jaundice. What
medication should be started?
A. Spironolactone
B. Lactulose
C. Metronidazole
D. Prednisone

Answer:

5. 45-year-old man with end-stage liver disease presents to your office after being hospi-
talized for bleeding varices. He was discharged 1 month ago. After band ligation was
performed, pantoprazole was prescribed. His last endoscopy was at the hospital at the
time of the last band ligation. He feels well, with no further episodes of bleeding. There
is obvious ascites and lower extremity edema. Which of the following is recommended?
A. Propranolol
B. Spironolactone
C. Furosemide
D. Upper endoscopy with possible band ligation
E. All of the above

Answer:

6. 35-year-old accountant, otherwise healthy, presents after having 2 episodes of melena.

In the emergency department, he is tachycardic. After 2 L of saline is given, an upper
endoscopy is performed. The upper endoscopy reveals a clean-based, 1-cm ulcer in the
antrum. The hematocrit is 35 after hydration, and vitals are normal. Rectal examination
reveals dark brown stool, occult-positive. The next appropriate step in management
would be which of the following?
A. Admit to the intensive care unit
B. Admit to a monitored floor
C. Admit to a regular floor and repeat endoscopy the next morning
D. Discharge the patient, to follow up as an outpatient, prescribe omeprazole, and
avoid aspirin and nonsteroidals.

Answer:

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Summary: Case 1
Hemodynamic resuscitation is the most important aspect in the management of patients
with gastrointestinal bleeding, and it must precede endoscopy. Correction of coagulopathy
is also preferred. IV fluids, blood, and FFP (fresh frozen plasma) should be given initially.
Octreotide is indicated in all patients with suspected variceal bleed. Intravenous PPI has
clearly been shown to be effective in bleeding peptic ulcer disease. Due to obvious hemostatic
effects, PPIs will likely be effective in patients with variceal bleeding. The endoscopic appear-
ance of the ulcer guides treatment and determines prognosis:

Appearance Risk of Rebleeding Treatment

Clean base 5% Oral meds/D/C
Flat spot 20% Heater probe
Clot 20% Remove clot
Visible vessel 50% Heater probe
Spurting vessel 50% Heater probe/injection

The serum-ascites albumin gradient (SAAG) differentiates ascites from malignancy and TB
from portal HTN (hypertension). A SAAG greater than 1.1 is associated with portal HTN.
SBP (spontaneous bacterial peritonitis) is defined by a PMN (polymorphonuclear neutro-
philic leukocyte) count of greater than 250. Prophylaxis of recurrent SBP is recommended
in patients with (1) prior SBP, (2) current variceal bleed, and/or (3) ascites protein less than
1 mg/dL. Prednisone is effective in treating severe alcoholic hepatitis (there are several for-
mulas, however; focus on the patients with classic findings: a bilirubin >15 and PT [INR >2]
that is significantly elevated). A low-salt (not low-protein) diet is indicated in patients with
ESLD (end-stage liver disease) complicated by ascites. A nonselective beta blocker prevents
first and recurrent bleeding in patients with varices (use of propranolol or nadolol in patients
with known varices is a standard of care!).

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 IM Board Review Course Book

PANCREAS

Case 1
44-year-old woman presents to the hospital with epigastric pain radiating to the
back associated with nausea and vomiting. The pain began suddenly 3 hours ago.
She has no fever, chills, or diarrhea.

BP 130/70, P 88, R 12 AST: 255 (elevated)

Abd: No bowel sounds, marked Bilirubin: 1.1
epigastric tenderness
Amylase: 2,311 (elevated)
WBC: 15,500
Alkaline phosphatase: 122
BUN, Creatinine: Normal
ALT: 244 (elevated)

1. Which of the following is/are true regarding the diagnosis?

A. A serum lipase is needed to confirm the diagnosis.
B. A CT is needed to confirm the diagnosis.
C. Laboratory testing alone demonstrates pancreatitis.
D. MRCP is needed to rule out a common bile duct stone.

Answer:

2. Which of the following is true regarding severity in this patient?

A. The patient has mild disease.
B. The patient currently has mild disease but must be observed closely during the first
48 hours.
C. The patient has severe disease.
D. Necrotizing pancreatitis is definitely not present.

Answer:

3. What does a purple discoloration of the skin on the left flank indicate?
A. An intraperitoneal hemorrhage
B. A retroperitoneal hemorrhage
C. Necrotizing pancreatitis
D. Interstitial pancreatitis

Answer:

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4. If the bilirubin is elevated, when should an ERCP (endoscopic retrograde cholangio- Note
pancreatography) be performed?
80% of pancreatitis caused by
A. If the alkaline phosphatase is found to elevated
alcohol and gallstones.
B. If the pancreatitis becomes severe
C. If the patient develops signs and symptoms of biliary sepsis Gallstones are the single MCC
of acute pancreatitis.
D. If the patient develops a significant amount of pancreatic necrosis
Alcohol is the single MCC of
Answer: chronic pancreatitis.

Ultrasound shows numerous gallstones in the gallbladder, and the biliary tree is
not dilated. After 3 days, the pain resolves, and amylase and LFTs (liver function
tests) return to normal.

5. Which of the following recommendations is necessary?

A. A cholecystectomy
B. Avoidance of alcohol for life
C. MRCP
D. ERCP

Answer:

6. Which medication(s) is/are likely to induce acute pancreatitis?

A. Azathioprine
B. DDI (didanosine)
C. DDI and azathioprine
D. Metformin

Answer:

7. The most likely cause of acute idiopathic recurrent pancreatitis is which of the following?
A. Sphincter of Oddi dysfunction
B. Microlithiasis, biliary sludge
C. Pancreas divisum
D. Autoimmune pancreatitis
E. Viral etiologies

Answer:

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 IM Board Review Course Book

8. The best approach to a 38-year-old woman who has no history of pancreatitis or alco-
hol use and is found to have a 3.7-cm septated cyst in the tail of the pancreas on CT
scan and endoscopic ultrasound is which of the following?
A. Biopsy
B. Distal pancreatectomy
C. Internal drainage
D. CA 19-9 level
E. MRI of the pancreas with MRCP

Answer:

9. The diagnosis of infected necrosis during an attack of acute pancreatitis is best made by
which of the following?
A. Clinical deterioration
B. MRI
C. Early surgical exploration
D. Image-guided fine-needle aspiration of necrotic areas or fluid collections
E. Contrast-enhanced CT scan

Answer:

10. While riding a bicycle, a 22-year-old woman is thrown forward when she stops sud-
denly. She hits her abdomen. The next day she presents to the ER with severe pain. She
has marked abdominal tenderness and an amylase of 2,120. The most likely diagnosis is
which of the following?
A. Ruptured spleen
B. Traumatic pancreatitis
C. Ruptured viscus
D. Gastric ulcer

Answer:

11. 55-year-old man with jaundice is found to have a 2-cm mass in the head of the pan-
creas, documented by MRI. EUS confirms that the lesion does not penetrate the portal
vein, and there are no gallstones or common bile duct stones. There is no adenopathy,
and the patient is otherwise healthy. He has no history of alcohol use and no other
medical problems. What is the most appropriate management?
A. ERCP/brushings and stent placement
B. Pancreaticoduodenectomy (Whipple procedure)
C. Repeat MRI in 3 months
D. Repeat MRI in 6 months
E. CT scan guided biopsy

Answer:

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 Gastroenterology

12. 45-year-old woman presents with abdominal pain, amylase of 3,200, AST of 150.
Bilirubin is normal. Alkaline phosphatase is slightly elevated. She denies alcohol use.
Ultrasound of abdomen is normal. There are no dilated bile ducts. Her serum triglycer-
ide level is 450. The most likely cause of the acute pancreatitis is which of the following?
A. Hypertriglyceridemia
B. A passed gallstone
C. A medication
D. A mass in the pancreas
E. Alcohol

Answer:

Risks for acute pancreatitis:

• I - Idiopathic
• G - Gallstones • S - Scorpion venom
• E - Ethanol (alcohol) • H - Hyperlipidemia,
• T - Trauma hypothermia, hypercalcemia
• S - Steroids • E - ERCP and emboli
• M - Mumps • D - Drugs
• A - Autoimmune
(Polyarteritis nodosa)

CD UC
ASCA Positive Negative
ANCA Negative Positive

13. Elderly alcoholic 10 days after onset of pancreatitis with persistent fevers and high WBC
count.
Diagnosis:
Treatment:

14. Elderly alcoholic presents 2 months after bout of pancreatitis. Has abdominal fullness.
Palpable cystic mass on exam.
Diagnosis:
Treatment:

If > 6 cm and > 6 weeks: Perform percutaneous drainage or endoscopic drainage

IF PAINFUL

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 IM Board Review Course Book

Case 2
34-year-old man presents for a narcotic prescription due to chronic pain, which he
claims is chronic pancreatitis. He describes epigastric pain that is dull and radiates
to the back. The pain began several years ago after a 10-year history of binge
drinking. He has had no diarrhea or weight loss with no prior hospitalizations or
surgeries. He takes pancrelipase. The abdomen reveals normal bowel sounds and
is soft but tender in the epigastrium.

CBC: Normal Electrolytes: Normal

BUN, Creatinine: Normal LFTs: Normal

Albumin: Normal Amylase: Normal

1. What is the most sensitive and specific test in the diagnosis of chronic pancreatitis?
A. ERCP
B. MRCP
C. Endoscopic ultrasound (EUS)
D. Secretin stimulation test
E. X-ray

Answer:

2. If chronic pancreatitis is present, what is the best method of treating chronic pain in the
absence of diarrhea or dilated pancreatic ducts?
A. EUS celiac axis block
B. Pancreatic enzyme supplementation
C. Antioxidants
D. Narcotics

Answer:

3. Which patient with pain from chronic pancreatitis would benefit most from surgery?
A. The patient with an inflammatory mass in the head of the pancreas
B. The patient with a dilated dorsal (Santorini) duct
C. The patient with steatorrhea
D. The patient with diabetes

Answer:

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 Gastroenterology

4. A patient is diagnosed with pancreatitis secondary to hypertriglyceridemia. He devel-

ops hypotension, renal failure, ARDS, and DIC. He has received imipenem, is on a
mechanical ventilator in the ICU, and has no evidence of gallstones or biliary obstruction.
Diagnosis:
Treatment:

5. A young patient presents with nausea, vomiting, and severe epigastric pain radiating
to the back for the past couple hours. Intestinal perforation and infarction are ruled out
and you suspect acute pancreatitis. Labs reveal amylase of 190 U/dL. What condition
could falsely depress the amylase levels?

→ Hypertriglyceridemia

Summary: Cases 1 and 2

Acute pancreatitis is typically self-limited. However, one-fourth of patients develop severe
disease. The amylase is not predictive of severity. Severity is defined by the presence of a
Ranson score greater than 3 (after 48 hours) or the obvious development of organ failure.
The most common causes of acute pancreatitis are alcohol, gallstones, triglycerides over
1,000, and medications. Gallstones are by far the most common cause. A cholecytectomy is
recommended in all patients with acute pancreatitis and an ultrasound demonstrating gall-
stones. The cholecystectomy should typically occur prior to discharge due to the high risk of
recurrence. ERCP is reserved for patients with dilated ducts and/or persistently elevated LFTs
after an attack of pancreatitis. Noncontrast CT scan is helpful in diagnosis (if needed). IV
CT scan is needed for the assessment of necrosis. Necrosis can be sterile or infected. Infected
necrosis is a diagnosis made by fine-needle aspiration of pancreatic necrosis. Infected necro-
sis requires surgical debridement.

Chronic pancreatitis is often a difficult diagnosis to make early in the course of the disease.
Pancreatic calcifications are only seen in one-third of patients on KUB (kidney, ureter, and
bladder) and slightly more on CT scan. ERCP, EUS, and the secretin stimulation test are the
best tests (sensitivity over 95%). Pancreatic enzymes have a limited role in treating pain but
are effective in treating diarrhea (malabsorption). Surgical (Puestow lateral pancreatic jejunos-
tomy) and endoscopic (stent placement) therapy are helpful in patients with dilated pancreatic
ducts.

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 IM Board Review Course Book

HEPATITIS

Case 1: Chronic Hepatitis

A 42-year-old woman is found to have abnormal liver function tests. She has
mild fatigue. She denies a history of hepatitis. She received a blood transfusion
in 1993 after undergoing a complicated hysterectomy. She takes no medications
and consumes 1 or 2 beers per day. She denies IV drug abuse, tattoos, or sexual
promiscuity.

Physical examination reveals a 155-lb (66-kg) woman who is 5 feet, 1 inch tall.
BP is 130/70 mm Hg, pulse 88/min, and respirations 12. Physical exam is normal
except for an enlarged liver. No jaundice.

CBC: Normal Electrolytes: Normal

BUN, Creatinine: Normal AST: 124 (elevated)

ALT: 99 (elevated) Alkaline phosphatase: 122

Bilirubin: 1.1 Albumin: Normal

1. What tests should be submitted for analysis? What clinical characteristics suggest
hemochromotosis, Wilson?

2. All tests are negative except for the hepatitis C antibody, which is positive. What is the
best next test to confirm the diagnosis of chronic hepatitic C?
A. ELISA (enzyme-linked immunosorbent assay) anti-hepatitis C antibody
B. RIBA immunoblot
C. Hepatitis C RNA branched chain assay (qualitative)
D. Hepatitis C RNA PCR (quantitative)

Answer:

3. Which factor poses the greatest risk for obtaining hepatitis C?

A. Blood transfusion in 1993
B. Sexual contact on 5 occasions with a person with hepatitis C
C. Intravenous drug use in the 1980s
D. Shellfish consumption

Answer:

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 Gastroenterology

4. The patient’s husband is hepatitis C-negative. They have been having unprotected
intercourse for the last 10 years. What do you recommend?
A. No sexual contact should occur until he is vaccinated.
B. A barrier must be used, such as a condom.
C. He should be tested monthly.
D. No barrier is necessary, as the risk of transmission is less than 5% over his lifetime.

Answer:

5. What is the best test to determine the degree of severity or stage of the disease?
A. Genotype
B. Liver biopsy
C. Viral load
D. Albumin

Answer:

6. What test(s) can best predict the patient’s response to treatment?

A. Genotype
B. Liver biopsy
C. Viral load
D. Liver function tests

Answer:

7. What is the best treatment for chronic hepatitis C?

A. Lamivudine
B. Pegylated interferon and ribavirin
C. Pegylated interferon
D. Adefovir
E. Boceprevir, interferon, and ribavirin

Answer:

8. If the hepatitis B surface antigen is positive, what confirmatory test(s) is/are needed?
A. Hepatitis B e antigen (HbeAg) and hepatitis B DNA
B. Liver biopsy
C. MRI
D. Hepatitis B IgM
E. Surface antibody and core antibody

Answer:

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9. If the hepatitis B e-antigen (HbeAg) is negative but the hepatitis B DNA is elevated to
2 million, why are the LFTs elevated?
A. Steatohepatitis
B. Pre-core mutant
C. Hepatitis delta suprainfection
D. Hepatitis E infection
E. Hepatitis D infection

Answer:

10. If the hepatitis B e-antigen (HbeAg) is positive, what are the treatment options?
A. Lamivudine
B. Interferon
C. Adefovir
D. Entecavir
E. All are acceptable.

Answer:

11. Who is most at risk of developing hepatocellular carcinoma?

A. Person with hemochromotosis
B. Person with hepatitis B
C. Person with hepatitis C
D. Person with alcoholism

Answer:

12. If a person with chronic liver disease has a positive ANA, which of the following is needed?
A. Anti-smooth muscle antibody
B. Anti-LKM (anti–liver-kidney microsomal antibody)
C. Ro and La antibodies
D. Antimitochondrial antibodies

Answer:

13. What is the best method of treatment if the ANA is positive?

A. Prednisone
B. Ursodeoxycholic acid
C. Ribavirin
D. Interferon

Answer:

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14. During the evaluation of an elevated ALT, a 45-year-old alcoholic man is found to have
a serum iron concentration of 245 mg/dL (elevated) and a total iron saturation of 80%.
The serum ferritin is 2,120 ng/mL. The physical examination shows no evidence of
chronic liver disease. The best initial test would be which of the following?
A. Liver biopsy with Prussian blue staining
B. Test for HFE (hemochromatosis) gene mutation (C282Y and/or H63D)
C. MRI for evaluation of iron overload
D. Phlebotomy

Answer:

15. In the evaluation of ascites, which of the following is true?

A. LDH (lactate dehydrogenase) plays a role in determining exudates versus transudate.
B. Albumin level in the ascites is important in determining whether portal hyperten-
sion is present.
C. A neutrophil count over 150 is diagnostic for spontaneous bacterial peritonitis.
D. If the PT is 20 (INR 2.8), paracentesis should be deferred until FFP is given.

Answer:

16. Which of the following is true regarding chronic hepatitis C?

A. The genotype of the virus should not be tested; it is experimental.
B. The virus is not likely to be present in blood transfused in 1988.
C. More than 25% of persons infected will manifest cirrhosis within 20 years after
infection.
D. Pneumococcal vaccine should be given; hepatitis A vaccine should be avoided.
E. Interferon will decrease progression to end-stage liver disease and hepatocellular
carcinoma.

Answer:

17. Which of the following medications results in toxic hepatitis complicated by early
fibrosis?
A. Amiodarone
B. Nitrofurantoin
C. Cephalexin
D. Isoniazide
E. Ciprofloxacin

Answer:

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 IM Board Review Course Book

Summary: Case 1
Screening for hepatitis C occurs initially with an ELISA antibody. Approximately 80% of per-
sons with a positive antibody have hepatitis C. This is confirmed by the presence of an HCV
RNA. Qualitative tests are more sensitive than quantitative tests. Risk factors for transmission
include blood transfusion, IV drug abuse, and sex. However, sexual transmission is difficult.
In a couple who have had sex for years, the CDC does not recommend barrier precautions
due to the low risk of transmission. However, patients are encouraged not to share razors or
toothbrushes. The risk of progression is dependent on age, alcohol use, and liver biopsy find-
ings. Only a liver biopsy (not the height of the enzymes) can determine the degree of activity
of the virus and the stage of the disease. Treatment is most effective in patients who have a
genotype other than 1. Unfortunately, most people in the United States have genotype 1. The
standard of care is to use combined pegylated interferon with ribavirin. Cure rates (long-term
eradication of virus) are seen in almost 50% of patients with genotype 1. In patients with
chronic hepatitis B, treatment is needed only if the e antigen is positive, which is associated
with high DNA levels. However, there is a precore mutant where the e antigen assay is falsely
negative. These patients have elevated LFTs and an elevated HBV DNA. Treatment with either
interferon, lamivudine, or adefovir is acceptable (no combination therapy with interferon
in hepatitis B!). If the LFTs are normal, the e antigen is negative, and the HBV DNA is normal
or minimally elevated, do not treat hepatitis B! Patients with an HbeAg that is negative, with
a positive surface antigen, are considered carriers. These patients are infectious but no treat-
ment is needed. Progression to liver cancer and cirrhosis in these patients is low.

Hepatitis B

Surface Core Surface E Antigen LFTs HBV DNA

Antibody Antibody Antigen
Vaccine + − − − − −
Immune + + − − − −
Carrier − − + − − +/−
Mutant − − + − + +
Active − − + + + +
*Only active disease and pre-core mutants are treated. The active disease, pre-core mutants, and carriers
are to be considered infectious.

Hepatitis C
Acute hepatitis C is the only form of acute hepatitis to receive treatment. Use interferon,
ribavirin, and either telaprevir or boceprevir. Either telaprevir or boceprevir is added to
treatment. The treatment is the same for chronic hepatitis C.

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Case 2: Acute Hepatitis

You are asked by the orthopedic surgeon to see a 66-year-old man who underwent
a right hip replacement and now has profoundly elevated LFTs. The patient was
brought in after suffering a syncopal episode and a fall leading to a right hip
fracture. Two days ago, surgery was performed. The patient feels well. There is no
history of liver disease. Findings are:

Medications: Amlodipine, metformin Bilirubin: 1.3

Abd: Normal bowel sounds; abdomen is AST: 1,211 (elevated)

soft and nontender
Alkaline phosphatase: 243 (elevated)
ALT: 877 (elevated)
Albumin: Normal

1. These LFTs are consistent with which of the following?

A. Ischemic hepatitis
B. Acute hepatitis B
C. Acetaminophen overdose today
D. Acute hepatitis A

Answer:

2. What is the best therapy for ischemic hepatitis?

A. Steroids
B. Supportive
C. Interferon
D. Intravenous immunoglobulin

Answer:

3. If a patient is found to have acute hepatitis B, what should you recommend?

A. Supportive care
B. Lamuvidine
C. Interferon
D. Steroids
E. Interferon and ribavirin

Answer:

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 IM Board Review Course Book

4. If a patient has acute hepatitis C, what is the treatment?

A. Supportive care
B. Lamuvidine
C. Interferon
D. Steroids
E. Interferon and ribavirin

Answer:

Summary: Case 2

Treatment for acute hepatitis is largely supportive. The differential diagnosis includes shock
(postoperative), toxin, hepatitis A, B, D on B, C, E. In patients with acute viral hepatitis, treat-
ment is recommended only in patients with acute hepatitis C. In patients with acute hepatitis C,
especially if there is an elevated bilirubin, interferon early (within the first month) significantly
decreases the risk of chronic infection (80% to less than 5%). In patients with acute hepatitis A,
B, D, and E, treatment is supportive. Acetaminophen overdose is treated with early institution
of acetyl-cysteine to replenish the glutathione in the liver, which helps in detoxification.

1. An 18-year-old man has had lifelong constipation, requiring repeated enemas. His
abdomen is distended and there is a tubular mass in the left lower quadrant. There is
no stool in the rectum. Barium enema reveals a dilated colon above the rectum. The
rectum is not dilated. The most likely diagnosis is which of the following?
A. Irritable bowel syndrome
B. Laxative abuse
C. Chronic intestinal pseudo-obstruction
D. Adult Hirschsprung disease
E. Crohn disease

Answer:

2. 60-year-old woman has had constipation for many years. She has a continuous feeling
of needing to defecate and a sensation of incomplete evacuation. The stool is brown,
hard, and negative for occult blood. Flexible sigmoidoscopy reveals dark mucosa. The
most likely diagnosis is which of the following?
A. Laxative abuse
B. Crohn disease
C. Chronic intestinal pseudo-obstruction
D. Irritable bowel syndrome

Answer:

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3. A 28-year-old woman with Down syndrome has had chronic constipation since her
early teens. She has had multiple admissions for abdominal pain. Radiographic stud-
ies when asymptomatic have shown air–fluid levels, but obstruction has never been
demonstrated. The stool is brown, soft, and negative for occult blood. The most likely
diagnosis is which of the following?
A. Crohn disease
B. Adult Hirschsprung disease
C. Laxative abuse
D. Chronic intestinal pseudo-obstruction

Answer:

4. Which of the following regarding non-alcoholic steatohepatitis (NASH) is true?

A. It is commonly seen in emaciated alcoholic patients.
B. Diabetes mellitus is a common predisposing factor.
C. The biopsy findings are different from those seen in alcoholic liver disease.
D. An ERCP will confirm the diagnosis.
E. Corticosteroids remain the mainstay of treatment.

Answer:

5. The best approach to a 30-year-old patient who has cholelithiasis and complaints of
abdominal bloating, gas pain, and normal LFTs is which of the following?
A. Dissolution therapy with ursodeoxcholic acid
B. ERCP with stone extraction
C. Open cholecystectomy
D. Laproscopic cholecystectomy
E. No medical therapy at this time

Answer:

6. 23-year-old presents with a change in behavior and abnormal LFTs. The AST is 122,
ALT 154, alkaline phosphatase 199, and bilirubin normal. The psychiatrist wants to use
clozapine. You should do which of the following?
A. Repeat LFTs prior to agreeing to the use of clozapine
B. Send off a hepatitis B surface antibody
C. Send off a serum ceruloplasmin
D. Obtain an ERCP
E. Perform a contrast-enhanced CT scan of the patient’s head

Answer:

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 IM Board Review Course Book

7. 45-year-old woman presents with pruritis and abnormal LFTs. She was well until last
year, when her AST, ALT, and alkaline phosphatase were found to be 2 times normal.
Now the alkaline phosphatase is 4 times normal; and bilirubin is slightly elevated. There
is no abdominal pain, fever, chills, or weight loss. Abdominal ultrasound is negative.
What is the best diagnostic test to confirm your suspicion?
A. Antinuclear antibody
B. Antimitochondrial antibody
C. ERCP with possible endoscopic sphincterotomy
D. Surgical exploration
E. Hepatitis C antibody

Answer:

8. What treatment(s) should be initiated?

A. Ursodeoxycholic acid
B. Ursodeoxycholic acid and cholestyramine
C. Ursodeoxycholic acid and prednisone
D. Cholecystectomy
E. Radiology-guided percutaneous placement of biliary stent

Answer:

9. 45-year-old woman in the ICU was admitted 2 days ago with sepsis and found to have
abnormal LFTs and dilated bile ducts on ultrasound and CT. She was placed on met-
ronidazole/cefepime and is continuing to do poorly. What is the best approach to this
patient?
A. Repeat the ultrasound
B. Add vancomycin
C. ERCP with possible endoscopic sphincterotomy
D. Surgical exploration
E. Radiology-guided percutaneous placement of biliary stent

Answer:

11. 44-year-old man with chronic diarrhea and a vesicular rash on the extensor surfaces of
the hands is found to have flatten villi on small bowel biopsy. Which of the following is
true?
A. He is at an increased risk of small bowel adenocarcinoma.
B. A gluten-free diet will not be helpful.
C. Dapsone has never been shown to help these patients.
D. Even on a gluten-free diet, the anti-endomyseal antibodies will remain elevated.
E. The xylose test will be positive. (e.g., low xylose in the urine)

Answer:

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 Gastroenterology

12. 77-year-old woman has diarrhea for 6 months’ duration. Upper endoscopy and colo-
noscopy, including biopsies, are negative. Stool analysis is negative. Small bowel series
reveal several large jejunal diverticuli. What is the next best step in treatment?
A. Repeat stool analysis
B. Biopsy the ileum
C. Mesalamine
D. Ciprofloxacin and metronidazole
E. Budesonide

Answer:

13. 39-year-old male with jaundice confusion and malaise. + RUQ pain. Normal fever,
HR 95, BP normal.
Exam: Icterus, ascites, and hepatomegaly.
AST 300
ALT 15
ETOH level 500
Platelets 95000
Paracentesis 200 WBCs with 50 PMN

Most likely diagnosis

Best management?
→ Discriminant Function = 4.6 * (Patient’s PT – Control PT) + TBili

When to start steroids?

>32 points indicates poor prognosis and patient may benefit from glucocorticoid
therapy.

14. 31-year-old woman with jaundice.

ANA, negative
ASMA, negative
AMA, positive
Liver biopsy – lymphocytic destruction of bile ducts

Diagnosis:
Treatment:

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15. 31-year-old woman with jaundice.

ANA, positive
AMA, negative
Liver biopsy – piecemeal necrosis of hepatocytes.

Diagnosis:
Treatment:

16. 31-year-old woman with jaundice.

ANA, negative
ASMA, negative
AMA, negative
Liver biopsy – mild inflammation with concentric fibrosis around bile ducts.

Diagnosis:
Treatment:

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 Neurology
3
Case 1
65-year-old woman comes to ED with sudden onset of right-arm weakness that
began while she was watching television. Her daughter states her mother was alert
but unable to talk clearly. Exam shows a right hemiplegia with brisk right-sided
reflexes and up-stroking Babinski. Patient has slurred speech.

1. Diagnosis?

2. First step in management?

3. What is an appropriate diagnostic evaluation?

A. Echo, Holter, carotid Doppler
B. Echo, factor V mutation
C. Holter, lupus anticoagulant, ESR
D. Carotid Doppler, EEG
E. ESR, CRP, Echo

Answer:

4. What are the indications for the following interventions in acute stroke?

Heparin:

Aspirin:

Clopidogrel:

Ticlopidine:

Warfarin:

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TPA:

Carotid endarterectomy:

Dipyridamole:

Summary: Case 1
Stroke should be considered in any patient who presents with acute onset of a focal neuro-
logic deficit.

The initial test of choice will always be a noncontrast CT scan of the head. This test is done
to distinguish between hemorrhagic and ischemic stroke. Noncontrast CT scan is the most
sensitive test for detecting blood in the brain. CT scans are often negative for ischemia within
the first 48 hours after symptom onset. Diffusion-weighted MRI (magnetic resonance imag-
ing) is the most accurate test for detecting cerebral ischemia.

Note The diagnostic workup of patients with acute ischemic stroke involves searching for embolic
sources (echocardiogram, carotid duplex, and 24-hour Holter monitor). Also consider a
Restart aspirin 24 hours after workup for inherited hypercoagulability (thrombophilia).
tPA for stroke
There are numerous medications used to treat acute ischemic stroke. Giving heparin in acute
ischemic stroke is incorrect because of adverse events associated with treatment. For every
stroke prevented, one intracranial hemorrhage is caused. Therefore, treatment with heparin
in acute ischemic stroke is limited. Antiplatelet therapy is most useful in secondary preven-
tion of ischemic stroke. Aspirin is considered first-line treatment for secondary prevention of
ischemic stroke. When patients have a known allergy to aspirin or continue to have recurrent
cerebrovascular events on aspirin alone, dipyridamole may be added or switch to clopidogrel
to enhance antiplatelet therapy. Ticlopidine is no longer used because the rates of thrombotic
thrombocytopenic purpura and leukopenia are unacceptably high. Warfarin is used if there
is chronic atrial fibrillation. Tissue plasminogen activator is given if the patient presents
within 3 hours of symptom onset. Contraindications to the use of tissue plasminogen activa-
tor include stroke or serious head trauma within 3 months, hemorrhage (gastrointestinal or
genitourinary) within 21 days, surgery within 14 days, history of intracranial hemorrhage,
BP greater than 185/110 mm Hg, current use of anticoagulants, platelets less than 100,000, or
coagulopathy (PT >15 s). Patients who receive tissue plasminogen activator in an appropriate
manner have better neurologic function 3 months post-CVA than do patients who did not
receive tissue plasminogen activator. Carotid endarterectomy is recommended when occlu-
sion exceeds 70% of the arterial lumen; and the lesion is symptomatic.

5. 71-year-old woman with hypertension and right carotid bruit is having a stroke. Her BP
230/130. Intravenous nitroprusside is started; and the pressure drops to 135/96 mm Hg.
She then develops right arm and leg weakness. What is the next step in management?
A. Add aspirin
B. Thrombolysis
C. Emergency head CT scan
D. Discontinue nitroprusside
E. Carotid stenting and angioplasty

Answer:

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Case 2
Man comes with sudden vision loss that he describes as “a curtain falling in front
of my eye.” Denies any current focal weakness but does attest to an earlier episode
of right-arm weakness that resolved spontaneously on its own. Exam is nonfocal.
Symptoms resolve before his arrival in the emergency department.

1. Most likely diagnosis?

2. What medication would you start?

Note
A. Aspirin
B. Ticlopidine No tPA waking up with stroke
timing unknown.
C. Thrombolytics
D. Dipyridamole

Answer:

3. If already taking aspirin, what medication would you add?

4. When is surgery indicated in treatment of asymptomatic carotid bruit?

5. 58-year-old man with a history of diabetes and hypertension comes with sudden
onset of urinary incontinence, personality changes, and weakness of half of his body.
Weakness is worse in the lower extremity. Most likely area of the defect?
A. Anterior cerebral artery
B. Middle cerebral artery
C. Posterior cerebral artery
D. Posterior inferior cerebellar artery

Answer:

6. 65 with loss of consciousness. Awakens dizzy with face numb on right, body weak on
left. Most accurate test?
A. MRI
B. CT

Answer:

7. Carotid 85% stenosis. Treatment:

A. Carotid endarterectomy
B. Carotid angioplasty
C. Clopidogrel
D. Aspirin and clopidogrel

Answer:
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Summary: Case 2
In a patient who presents with sudden onset of a focal neurologic deficit that resolves spon-
Note taneously within minutes of onset, the most likely diagnosis is a transient ischemic attack
(TIA). The blood supply of the brain is divided into 2 major systems of blood supply: the
Vertebrobasilar carotid or anterior circulation, and the vertebrobasilar (posterior) circulation. The major
Dizzy blood vessels comprising the anterior circulation include the anterior cerebral artery (ACA)
and middle cerebral artery (MCA).
Diplopia
Dysarthria The best initial therapy for any TIA after verifying the absence of blood on a noncontrast CT
scan of the head is aspirin. If the patient presents with a TIA while on aspirin, then the next
step would be to switch to clopidogrel. The indications for endarterectomy for asymptomatic
carotid atherosclerotic disease are not clearly defined. You should rarely consider surgery in
these cases and only with a high-grade stenosis in a patient who is a good operative candidate.

Occlusion of the ACA presents with contralateral weakness and sensory loss in the leg more
than the upper extremity. Urinary incontinence, confusion, and behavioral disturbances are
common.

Occlusion of the MCA presents with contralateral hemiplegia, hemisensory loss, and hom-
onymous hemianopia with eyes deviated toward the cortical lesion. Dominant hemisphere
involvement results in aphasia.

The posterior circulation provides blood supply to the cerebellum, brain stem, occipital lobe
of the cortex, and pons. The major blood vessels that comprise the posterior circulation are
the posterior cerebral artery (PCA), basilar artery (BA), and vertebral arteries. Occlusion of
the PCA presents with contralateral homonymous hemianopia, visual hallucinations, and
agnosias. Occlusion of the penetrating branches of this vessel can result in CN III palsy with
contralateral hemiplegia (Weber syndrome) or CN III palsy with contralateral ataxia or ath-
etosis (Benedikt syndrome). Specific syndromes associated with occlusion of BA branches
include the “locked-in syndrome” (paramedian branches) presenting as quadriparesis with
intact vertical eye movements; and Wallenberg syndrome (posterior inferior cerebellar
artery), which presents as ipsilateral facial sensory loss, contralateral body sensory loss, ver-
tigo, ataxia, dysarthria, dysphagia, and Horner syndrome.

Occlusion of the major cerebellar arteries produces vertigo, vomiting, nystagmus, and ipsi-
lateral limb ataxia. It is important to make this distinction because a carotid Doppler ultra-
sound is appropriate in the workup of a TIA only when the symptoms are consistent with
anterior circulation involvement.

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1. 29-year-old, healthy male resident called to present at Grand Rounds by his chairman.
A few minutes after beginning his presentation, he suddenly develops a cold sweat,
sits down in a chair, and slumps to the left. Five seconds later his arms and legs jerk
several times. Thirty seconds later, he is clearly conscious, realizes what has happened,
and begins to apologize in embarrassment. What is the most likely diagnosis?
A. Neuro-cardiogenic syncope
B. Seizure disorder
C. Cardiac dysrhythmia
D. Hypoglycemia

Answer:

2. Which should be added routinely to all NON-hemorrhagic strokes?

A. Statin
B. Metformin
C. Prasugrel
D. ACE

Answer:

3. Which is the major difference in posterior circulation stroke compared to middle and
anterior?
A. Use of aspirin or clopidogrel
B. Use of heparin and ticlopidine
C. Use of carotid Doppler and MRI
D. Use of echo

Answer:

Case 3
27-year-old woman with lancing, knifelike pain in left cheek. Pain began suddenly
and is truly debilitating. Exam significant for bilateral lower-extremity hyperreflexia
with increased muscle tone and stabbing, knifelike pain elicited by gentle
percussion of cheek. Funduscopic exam shows right-sided optical nerve pallor. She
had a transient episode of visual disturbance last year.

1. Diagnosis?

2. Treat the facial pain?

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3. Best initial test to diagnose the underlying disease?

4. Most accurate test?

5. Acute treatment?
A. Intravenous immunoglobulins
B. Plasma exchange
C. Steroids
D. Interferon
E. Glatiramer

Answer:

6. What medications arrest progression of disease?

A. Glatiramer and interferon
B. Steroids
C. Plasmapheresis
D. Methotrexate
E. Cyclophosphamide
F. Immunoglobulins

Answer:

7. What are the major complications of this disease? How should they be managed?

8. 34-year-old woman comes with blurry vision, especially when looking to the right.
You note horizontal nystagmus. On conjugate gaze to right, you note that the left eye is
unable to cross the midline. Diagnosis?
A. Lacunar infarction
B. Amyotrophic lateral sclerosis
C. Myasthenia gravis
D. Multiple sclerosis
E. Guillain-Barré syndrome

Answer:

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9. 28-year-old woman with weakness of the lower extremities and visual disturbance.
MRI is equivocal for multiple sclerosis. Which is most appropriate in determining the
diagnosis?
A. Visual evoked potentials
B. CT scan of the head
C. PET scan
D. Cerebrospinal white cell count
E. Cerebrospinal fluid, oligoclonal band

Answer:

Summary: Case 3
Commonly, patients with multiple sclerosis (MS) will present complaining of weakness,
numbness, tingling, or unsteadiness of a limb. Urinary urgency or retention, blurry vision,
and double vision are all common initial manifestations of the disease. Symptoms may per-
sist for several weeks or may resolve spontaneously over a few days.

There are several forms of the disease that may change the management of the disease and
are therefore important to recognize. After their first exacerbation, most patients will have
a disease-free period that lasts months to years. In relapsing, remitting disease progression
is characterized by relapses of active disease with incomplete recovery during the periods of
remission. In some patients the progression of disease becomes more aggressive, so that a
consistent worsening of function occurs. This form of disease is called secondary progressive
disease. In the least common form, the patient’s symptoms are progressive from the onset of
disease with the early onset of disability. This is called primary progressive disease.

It is important to understand when the diagnosis of multiple sclerosis should be suspected.

Classically, the diagnosis is made clinically when a young patient (usually less than 55 years
of age) presents with a history of multiple neurologic complaints that cannot be explained
by the presence of one CNS lesion. In other words, you should suspect the diagnosis when a
patient presents with multiple neurologic deficits separated by time and space (i.e., anatomy).

A number of triggers are known to exacerbate the disease. Infections or trauma may acutely
worsen disease. Pregnancy, especially 2–3 months after birth, may also exacerbate a patient’s
symptoms.

1. A woman develops what she describes as a thick tongue and blurry vision after taking
a hot shower. What is the most likely diagnosis?
2. Which is associated with progressive multifocal leukoencephalopathy (PML)?
A. Glatiramer
B. Mitoxantrone
C. Natalizumab
D. Beta interferon

Answer:

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MRI of the brain is the most accurate test to diagnose MS, reaching a sensitivity of 85 to
95% in symptomatic persons. Increased T2 density and decreased T1 intensity represent the
increased water content of demyelinated plaques. Enhancement of lesions with gadolinium
indicates active MS lesions that may enhance for up to 2 to 6 weeks after an exacerbation. MS
is an unusual disease in that the best initial test for the diagnosis is also the most sensitive
one, namely MRI of the brain and spine.

Evoked response potentials detect slow or abnormal conduction in response to visual, audi-
tory, or somatosensory stimuli. The test is not specific for the diagnosis of MS and is rarely,
if ever, used to make the diagnosis.

Cerebrospinal fluid (CSF) analysis usually reveals a mild pleocytosis (usually less than 50
cells/uL) and a total protein that is mildly elevated. A protein level exceeding 100 mg/dL is
unusual and should be considered as evidence against the diagnosis of MS. An elevated IgG
index (oligoclonal bands) is found in 70 to 90% of patients with MS. The finding is nonspe-
cific and, as a result, CSF for oligoclonal banding is recommended only when the MRI is not
confirmatory but clinical suspicion for MS remains high.

The treatment of multiple sclerosis can be divided into disease-modifying therapy, treatment of
complications, and treatment for symptomatic relief during an acute exacerbation.

In relapsing-remitting disease there are three disease-modifying agents—IFN-β1a, IFN-β1b,

and glatiramer acetate—that have been shown to reduce the number of clinical exacerbations
and the number of MRI lesions. More importantly, these medications seem to delay the onset
of significant disability.

In secondary progressive disease, IFN-β1b and mitoxantrone have been shown to reduce the
number of exacerbations, reduce MRI activity, and delay the onset of disability. In patients
who receive mitoxantrone, dose-related cardiotoxicity is a concern; this drug should be given
only to patients with normal ejection fractions. In patients who have relapsing-remitting
disease or secondary progressive disease and cannot tolerate treatment with IFN-β1b, IFN-
β1a, or glatiramer acetate, you can consider treatment with methotrexate, cyclophosphamide,
intravenous immunoglobulin, or azathioprine.

No approved disease-modifying therapy exists at this time for primary progressive disease.

The length and intensity of an acute exacerbation is shortened by the administration of gluco-
corticoids. Typically, an acute exacerbation is treated with 3 days of intense IV steroids, followed
by a course of oral medication tapered over 4 weeks. In patients with severe disease who are
unresponsive to steroid therapy, plasma exchange can be used as an alternative treatment.

For patients with spasticity, baclofen is the most effective medication. Tizanidine and diaz-
epam are useful for nocturnal spasticity but are limited in their use for daytime symptoms
because they cause intense somnolence. Pain secondary to trigeminal neuralgia and dysthe-
sias responds well to carbamazepine, gabapentin, phenytoin, or tricyclic antidepressants.
Bladder hyperactivity is treated with oxybutynin, whereas urinary retention is treated with
bethanecol. Fatigue may be treated with amantadine or fluoxetine. Erectile dysfunction can
be treated with sildenafil acetate.

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Case 4
32-year-old woman comes to emergency department with difficulty walking that
began 2 weeks ago. She has a sensation of “pins and needles” affecting the
lower extremities. Symptoms worsening over last 2 weeks. For 2 days she has
some difficulty breathing when walking down the block. 1 month ago she had
an episode of diarrhea and low-grade fever that lasted 3 days and resolved on its
own. Exam significant for bilateral lower extremity weakness with hyporeflexia, and
decreased sensation in both hands.

1. Diagnosis?

2. Next step in management?

A. Plasmapheresis
B. Electromyography (EMG)
C. CSF protein levels
D. CSF cell count
E. Forced vital capacity

Answer:

3. Initial treatment?
A. Intravenous immunoglobulin
B. Steroids
C. Cyclophosphamide
D. Azathioprine

Answer:

4. Most effective treatment?

A. Intravenous immunoglobulin
B. Intravenous immunoglobulin combined with plasmapheresis
C. Intravenous immunoglobulin combined with steroids
D. Plasmapheresis combined with steroids

Answer:

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5. Most accurate diagnostic test?

A. Muscle biopsy
B. Pulmonary function tests
C. Nerve conduction velocity
D. Acetylcholine esterase antibodies
E. Serum protein electrophoresis

Answer:

6. When are steroids indicated?

7. 73-year-old woman with weakness and paresthesia that began in distal hands and feet
3 days ago. History significant for myocardial infarction 3 years ago. You note absent
patellar reflexes bilaterally. Next step in management?
A. Lumbar puncture
B. Electromyography/nerve conduction
C. Intravenous immunoglobulins
D. Plasmapheresis

Answer:

8. 48-year-old woman with weakness of legs worsening over last 2 weeks. Exam reveals
loss of deep tendon reflexes. Most likely organism to have caused this?
A. E. coli 0157:H7
B. Scombroid
C. Campylobacter
D. Salmonella
E. Shigella

Answer:

Summary: Case 4
Most patients with Guillain-Barré syndrome (GBS) will present with rapidly developing
weakness that typically begins in the lower extremities and moves upward. On physical
examination, the patient is noted to lack reflexes in the affected muscle groups. The progres-
sion of the symptoms will develop over hours to days. The legs are usually more affected than
the arms and face. Fever, constitutional symptoms, or bladder dysfunction are rare associated
symptoms and should raise the possibilities of alternate diagnoses.

In addition to the motor weakness, patients with GBS typically will complain of sensory
disturbances that can take the form of pain or tingling dysthesias. Sensory changes are due
to the loss of large sensory fibers producing loss of reflexes and proprioception. Autonomic
instability (profuse sweating, postural hypotension, labile blood pressure, cardiac dysrhyth-
mias) occurs in severe GBS, requiring treatment in an intensive care unit.

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Approximately 75% of patients who present with GBS will have a history of an infection pre-
ceding the onset of symptoms by 1 to 3 weeks. The infection is typically of the respiratory or
gastrointestinal systems (Campylobacter jejuni), though GBS may be preceded by infections with
human herpes virus, cytomegalovirus, and Epstein-Barr virus. The only association between
immunizations and GBS occurred in 1976 with the swine influenza vaccine. More recent formu-
lations of influenza vaccine are associated with one additional case of GBS per million patients
immunized. GBS occurs more frequently in patients with HIV, SLE, and lymphoma.

The diagnosis of GBS lies principally in recognizing the typical pattern of weakness with
fever, constitutional symptoms, and the absence of reflexes. The characteristic finding is an
elevated protein without an associated rise in the cell count. These changes in the CSF do not
occur until 48 hours after the onset of symptoms. The most accurate test for the diagnosis
is electromyography (EMG). The EMG is used to detect evidence of demyelination of the
peripheral nerves.

Treatment should be initiated as quickly as possible because available therapy becomes inef-
fective approximately 2 weeks after the onset of symptoms.

Intravenous immunoglobulin and plasmapheresis are equally effective in treatment. There

is no benefit to combination therapy. Glucocorticoids are not effective in the treatment of
acute GBS. It is extremely important to monitor the vital capacity in patients with GBS and
to initiate early respiratory support to prevent death from respiratory failure.

Case 5
39-year-old man comes to office complaining of seeing double. Patient describes
seeing two of everything. Problem began several weeks ago and has been
worsening over last few days. Also noticed several episodes of choking while
eating over the last week. Symptoms are worse later in the day. Exam shows ptosis
greater on right than left, and diffuse weakness that worsens after repetitive action.
Pupillary reaction to light is normal. Sensory exam is normal.

1. Diagnosis?
A. Botulism
B. Guillain-Barré syndrome
C. Polio
D. Myasthenia gravis
E. Parkinson disease

Answer:

2. Initial diagnostic test?

A. Edrophonium (Tensilon) testing
B. Muscle biopsy
C. Acetylcholine receptor antibodies
D. Chest x-ray
E. EMG

Answer:
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3. Most accurate test?

4. Imaging test of choice?

A. Head CT
B. Head MRI
C. Chest CT
D. Spine MRI
E. Gallium scan

Answer:

5. When will you treat with the following?

Anticholinesterases:
Prednisone:
Thymectomy:
Plasmapheresis:
Intravenous immunoglobulin:

The same patient is admitted to the hospital with an unrelated episode of S. aureus
endocarditis. He is treated with vancomycin and gentamicin for his infection. You
are called to the bedside to evaluate the patient after he is found unresponsive
and in respiratory distress.

6. What caused the respiratory failure?

7. 54-year-old smoker with proximal muscle weakness, fatigue, and tiredness that improves
with repetition of exercise. What is most likely found?
A. Thymoma
B. Small cell lung cancer
C. Myasthenia gravis
D. Colon cancer

Answer:

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Summary: Case 5
The major features on history to make the diagnosis of myasthenia gravis (MG) are muscle
weakness and fatigability. Initially, patients will complain of diplopia, ptosis, and difficulty swal-
lowing. Speech may have a “mushy” quality or a nasal quality with facial weakness manifesting
as a “snarling” appearance when smiling. As the disease progresses, weakness may become gen-
eralized, involving proximal muscles in an asymmetric pattern. Deep tendon reflexes are intact.

Eaton-Lambert myasthenic syndrome is characterized by increasing muscle strength on repeti-

tive contraction. This syndrome is seen in association with malignancy, especially small cell
carcinoma of the lung.

Botulism may cause a myasthenic-like illness; but the pupils are usually dilated and repetitive
nerve stimulation (electromyography, EMG) shows an incremental increase in muscular fiber
contraction (opposite of myasthenia gravis).

The best initial test for the diagnosis of MG is the acetylcholine receptor antibody test. In
generalized MG, 80% of patients will have a positive test. In the presence of fatigable muscle
weakness, a positive antibody test is specific and virtually diagnostic of MG.

The edrophonium (Tensilon) test is sensitive but is not specific for the diagnosis. Additionally,
patients may experience nausea, diarrhea, fasciculations, syncope (rare), or bradycardia dur-
ing the test.

The most accurate test for the diagnosis of MG is single-fiber EMG. The characteristic finding
is a decremental decrease in muscle fiber contraction on repetitive nerve stimulation.

Anticholinesterase (usually pyridostigmine) medications are useful for the symptomatic treat-
ment of MG. If treatment with anticholinesterase medications is unsuccessful in providing
symptomatic relief, you should consider immunosuppressive therapy.

There are numerous medications used for immunosuppressive therapy. These interventions
differ primarily in the onset of therapeutic benefit. Glucocorticoids are effective in improv-
ing weakness, but will take 1 to 3 months before you observe a clinical benefit. Steroids are
the initial immunosuppressive of choice. If patients fail steroid therapy, azathioprine is the
most widely used medication used in combination with steroids. The benefits of azathioprine
therapy may take more than 3 to 6 months to peak.

In patients who are postpubertal and age <55 with generalized myasthenia gravis, thymectomy
is indicated before initiation of immunosuppressive therapy. Thymectomies are also performed
when a thymoma is present to prevent the spread of malignant thymic disease.

Plasmapheresis and intravenous immunoglobulin are immunosuppressive therapies noted

for their ability to rapidly improve weakness in MG. They are therefore reserved for patients
in acute myasthenic crisis.

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Case 6: Vertigo
73-year-old man in clinic complaining of dizziness. Patient describes his dizziness
as a sensation of the “earth rolling under his feet” as he walks. Also describes
“room is spinning around him.” Started suddenly and with nausea and vomiting.
Does attest to hearing a “roaring sound in his ears” and difficulty hearing your
questions, forcing you to repeat yourself a number of times.

1. Diagnosis?

2. When will central vertigo be the most likely diagnosis?

The neurologic examination is significant for nystagmus, which is horizontal and is

suppressed by fixation. Additionally, you note that the nystagmus is unidirectional
and has a rotational component. Finger-to-nose is normal bilaterally.

3. Next step in the management?

4. When will the answer be MRI of the brain?

5. When will the most likely diagnosis be each of the following?

Ménière disease:
Benign positional vertigo:
Labyrinthitis:
Vertebrobasilar TIA:
Perilymphatic fistula:

6. 76-year-old man comes to your office complaining of bilateral hearing loss. Weber test
does not localize to either ear; but the Rinne test shows air conduction greater than
bone conduction. Most likely diagnosis?
A. Labyrinthitis
B. Vestibular schwannoma
C. Presbycusis
D. Perilymphatic fistula
E. Multiple sclerosis

Answer:

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Summary: Case 6
There are several points on history and physical examination that will distinguish central
from peripheral vertigo. Patients with central vertigo tend to have chronic onset to their
symptoms. The vertigo is not associated with hearing loss or tinnitus. On physical examina-
tion, the presence of neighborhood signs—i.e., a focal CNS examination—argues strongly
for the presence of a central vertigo. The nystagmus in central vertigo is multidirectional,
pure, and vertical, and does not suppress with fixation. Patients with peripheral vertigo will
have a sudden onset of their symptoms. Their vertigo is associated with hearing loss and tin-
nitus. The CNS examination will be nonfocal and the nystagmus is usually horizontal, mixed,
and unidirectional, and suppresses with fixation.

Once you have determined that the patient has peripheral vertigo, there is a wide differential
diagnosis that should be considered.

Ménière disease is characterized by tinnitus, hearing loss, and episodic vertigo. Each episode
lasts 1 to 8 hours. The symptoms wax and wane as the endolymphatic pressure rises and falls.
The two most common causes of Ménière disease are syphilis and head trauma. Ménière
disease is treated with a low-salt diet and diuretics. In patients who fail medical therapy, you
can consider surgical decompression.

Benign paroxysmal positional vertigo is a cause of peripheral vertigo that is characteristically

exacerbated by head movement or change in head position. Typically, episodes will occur
in clusters that persist for several days. There will be a latency of several seconds after head
movement and before the onset of vertigo. The vertigo usually lasts 10 to 60 seconds. Benign
paroxysmal positional vertigo is treated with positional maneuvers that attempt to move the
otolith out of the circular canals.

Labyrinthitis presents with sudden onset of severe vertigo that lasts for several days with
hearing loss and tinnitus. The disease frequently follows an upper respiratory tract infection.
Vertigo secondary to labyrinthitis is treated symptomatically with meclizine and diazepam.

Central vertigo is caused by any cerebellar or brain stem tumor, bleed, or ischemia. Drug tox-
icity and overdoses are important causes of central vertigo. Also consider multiple sclerosis
in the young patient with unexplained central vertigo. Symptomatic treatment for peripheral
vertigo includes meclizine or, in severe cases, diazepam.

Perilymphatic fistula is a form of peripheral vertigo related temporally to head trauma (blunt
trauma to the ear, e.g., a hand slap to the ear) or extreme barotraumas during air flight, scuba
diving, or vigorous Valsalva maneuver.

The Weber test is heard best in the normal ear when the hearing loss is sensorineural, and is
heard best in the abnormal ear when the hearing loss is conductive.

With the Rinne test, air conduction is normally greater than bone conduction.

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Case 7
43-year-old woman comes to ED with sudden onset of severe headache.
Denies history of headaches. She was nauseous and vomited once while being
transported to hospital.

1. Most likely diagnosis?

If a patient presents with headache and one of the following,

seriously consider CT scan or MRI of the brain:

• “Thunderclap” headache
• “This is the worst headache of my life”
• Progressively worsening headache
• Onset at age >40 years
• Headache precipitated by coughing, sneezing, bending, or straining
• Fever, weight loss, malaise, jaw claudication
• Focal neurologic examination

2. Stat head CT scan without contrast finds no evidence of intracranial bleed. Next step in
management?
A. CT scan with contrast
B. MRI
C. Lumbar puncture
D. Angiogram
E. Neurology consultation

Answer:

3. When is angiogram appropriate in subarachnoid bleed?

4. Lumbar puncture is performed. CSF finds no evidence of xanthochromia.

What diagnosis is most likely?

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5. Next step in management?

6. 28-year-old football player brought to hospital for a brief loss of consciousness from
head trauma sustained during a game. No symptoms at present. Normal neurologic
examination. CT scan of head done for the trauma shows 4-mm aneurysm in circle of
Willis. What should you do about the aneurysm?
A. Nothing
B. Angiogram
C. Surgical repair
D. Catheter embolization
E. Nimodipine

Answer:

7. Person with sudden onset headache, stiff neck. CT shows subarachnoid hemorrhage.
What is important step?
A. Shunt placement
B. Angiogram
C. Surgical repair

Answer:

8. Person with subarachnoid has angiogram locating site of aneurysm. Active bleeding has
stopped. What to do?
A. Surgical repair
B. Beta blockers
C. Catheter embolization with platinum wire

Answer:

9. Which of the following is more likely to decrease risk of stroke?

A. Nimodipine
B. Diltiazem
C. Aspirin
D. Nothing proven to stop stroke

Answer:

Summary: Case 7
In any patient who presents with a sudden onset of first-time headache, especially when
the patient describes it as the “worst headache of my life,” you must suspect a subarachnoid
hemorrhage (SAH) as the most likely diagnosis. The best initial test to identify any intracra-
nial bleeding is the noncontrast CT scan of the head. If after performing a CT scan of the
head you still have a high clinical suspicion of a subarachnoid hemorrhage, the next step

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is to perform a lumbar puncture (LP). The purpose of the LP is to identify the presence of
xanthochromia, which is consistent with RBC (red blood cell) breakdown in the CSF. If after
the LP is performed there is no xanthochromia, the next step is to perform an MRV (mag-
netic resonance veinography) to rule out an acute cerebral vein thrombosis as the cause of
this patient’s headache. Management of any subarachnoid hemorrhage centers around early
aneurysm repair because any further bleeding carries a 60% mortality. Angiography is the
diagnostic test of choice preoperatively. The most common cause of mortality and morbid-
ity after repair is vasospasm. The best initial therapy for vasospasm associated with SAH is
volume expansion resulting in “triple H” therapy (hemodilution, hypertension, and hyper-
volemia). Treatment of any cerebral vein thrombosis is IV heparin.

Asymptomatic AV (atrioventricular) malformations should be removed in the presence of a

history of aneurysmal bleeding or if they are larger than 10 mm in diameter.

Case 8
53-year-old woman in ED with pain that began over the weekend. While lifting
heavy boxes. The pain is lower back without radiation. The last time she urinated
was over 24 hours ago. She has bilateral lower extremity weakness associated with
bilateral hyperreflexia. She has undergone mastectomy and has just completed her
fifth year of treatment with tamoxifen.

1. Next step in management?

A. CT scan with contrast
B. CT myelogram
C. MRI
D. Steroids
E. Radiation
F. X-ray of the spine

Answer:

2. When is an immediate imaging study indicated in the evaluation of back pain?

• Fever
• History of malignancy
• Major neurologic deficit involving more than 1 nerve root
• Pain that persists for more than 4 to 6 weeks

Nerve Root Motor Reflex Sensory

L4 Foot dorsiflexion Knee jerk Medial calf
L5 Big-toe dorsiflexion None Medial foot
S1 Foot eversion Ankle jerk Lateral foot

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3. Initial test?
A. CT scan with contrast
B. CT myelogram
C. MRI
D. X-ray of the spine

Answer:

4. Most accurate test?

A. CT scan with contrast
B. CT myelogram
C. MRI
D. X-ray of the spine

Answer:

5. 65-year-old woman with wide-based gait and confusion. Patient’s daughter denies the
patient has any history of alcoholism. Diagnosis?
A. Frontal lobe tumor
B. Parkinson disease with dementia
C. Vitamin B12 deficiency
D. Old cerebellar infarct

Answer:

6. Bilateral upper extremity weakness, fasciculations, and atrophy with bilateral loss of
pain and temperature. Diagnosis?
A. Brown-Sequard syndrome
B. Amyotrophic lateral sclerosis
C. Syringomyelia
D. Guillain-Barré syndrome
E. Syphilis

Answer:

7. Loss of pain and temperature sensation on left side with loss of proprioception on right.
Diagnosis?
A. Brown-Sequard syndrome
B. Amyotrophic lateral sclerosis
C. Syringomyelia
D. Guillain-Barré syndrome
E. Syphilis

Answer:

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Summary: Case 8
Patients with spinal cord compression will commonly present with insidious onset of mild
sensory disturbance, lower extremity weakness, and sphincter or sexual dysfunction. Pain is
the earliest symptom in the majority of patients (96%). Pain may be intensified by actions that
increase intrathoracic and thus CSF pressure. The diagnosis of acute spinal cord compression
has to be suspected based on the history and neurologic examination. The importance of hav-
ing a high index of suspicion for the diagnosis is essential to instituting appropriate therapy
early in the course of the disease. Histories of cancer, fever, or bowel or bladder incontinence/
retention are all points in the clinical history that strongly suggest the possibility of acute spi-
nal cord compression. On neurologic examination, a dermatomal sensory level with bilateral
lower extremity weakness, increased lower extremity muscle tone, and upper motor neuron
signs below the level of compression are all consistent with the diagnosis of acute cord com-
pression. Thoracic cord compression is the most common site of compression (70%), because
the spinal cord is narrowest at that point. Symptoms may progress quickly.

Plain x-rays are abnormal in 84 to 94% of all cases. The diagnostic test of choice is MRI of the
spine. When a spinal MRI is contraindicated, CT myelogram is the diagnostic test of choice.

High-dose dexamethasone should be started immediately once the diagnosis is suspected.

After the specific etiology is delineated more clearly by MRI, specific therapy may be initi-
ated. For radiosensitive tumors such as lymphoma or multiple myeloma, radiation therapy
should be started as soon as possible. Surgical decompression is the treatment of choice for a
herniated disk, epidural abscess, or hematoma. The prognosis depends mainly on the func-
tional status of the patient at the time of presentation. Up to 80% of patients who are able to
ambulate initially retain that ability after treatment. Only 5% of patients without antigravity
leg strength are able to ambulate after treatment.

Syringomyelia is defined as cavitation of the spinal cord. It occurs as either communicating

(with the CSF pathways) or noncommunicating. Communicating syringomyelia is usually
associated with the Arnold-Chiari malformation, whereas the noncommunicating syringo-
myelia is typically secondary to trauma of the spinal cord. Typically, there is sensory disso-
ciation with impaired pain and temperature and intact sensation to light touch. There may
be lower motor neuron manifestations at the level of the lesion, with upper motor neuron
signs below the lesion. Cavitation most commonly occurs at the level of the cervical cord.
Treatment is surgical.

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Case 9
37-year-old woman comes with “creepy, crawly” feeling with itching of her legs that
gets worse at night. She feels an urge to move her legs, and the movement seems
to alleviate her symptoms. Exam is negative.

1. Diagnosis?
A. Multiple sclerosis
B. Parkinson disease
C. Restless leg syndrome
D. Anxiety

Answer:

2. Initial therapy?
A. Fluoxetine
B. Pramipexole or ropinirole
C. Amitriptyline
D. Citalopram
E. Venlafaxine
F. Haloperidol

Answer:

3. What to replace?
A. B12
B. Pyridoxine (B6)
C. Iron

Answer:

4. Other medications you consider are:

• Levodopa/carbidopa
• Gabapentin
• Opioids
• Benzodiazepines

Answer:

Summary: Case 9
Restless leg syndrome is characterized by the presence of unpleasant creeping sensations that
arise from deep within the legs and arms. The symptoms occur especially when the patient
is attempting to relax. The etiology is unknown. Restless leg syndrome is common during
pregnancy and can accompany uremic or diabetic neuropathy, amyloidosis, or underlying
malignancy. It can also be associated with iron deficiency anemia. Treatment is with a dopa-
mine agonist, benzodiazepines, or opioid analgesics.

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Case 10: Tremor

25-year-old man comes to clinic with “shaking of his hand” that seems to
worsen when he moves. Also notices mild shaking of his head. He has become
progressively more self-conscious of his problem since he started dating his
girlfriend exclusively. Problem has gotten so bad that he drinks 3 beers every night
to help control the tremor. Exam shows normal gait and finger-to-nose.

1. Diagnosis?
A. Parkinson disease
B. Alcohol dependence
C. Anxiety
D. Essential tremor

Answer:

2. What would you stop in this patient?

A. Caffeine and stimulants
B. Bronchodilators
C. Steroids
D. All of the above

Answer:

3. Best initial therapy?

A. Propranolol
B. Primidone
C. Alprazolam
D. Clozapine
E. Mirtazapine
F. Topiramate

Answer:

4. When is thalamotomy indicated?

Summary: Case 10
In a patient who presents with tremor, the immediate question is to differentiate tremor of
cerebellar origin from Parkinson disease and benign essential tremor. In the case presented
here, the most likely diagnosis is essential tremor because of the paucity of abnormalities on
examination and the worsening tremor with movement (unlike Parkinson disease). Caffeine,
beta agonists, and steroids may worsen this condition, and treatment consists of propranolol
or alprazolam. Alcohol in small amounts also brings some relief to essential tremor.

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Case 11: Altered Mental Status

1. Man brought to emergency department after being found unresponsive in street. He

has no identification per ambulance personnel. Best initial management?
A. Urine toxicology screen
B. Flumazenil
C. Naloxone, thiamine, dextrose
D. Dextrose
E. Bicarbonate

Answer:

After about 10 minutes of furious activity, the patient’s airway is protected by

emergent intubation, and he is hemodynamically stable.

2. When is opioid overdose the diagnosis?

3. When is uncal herniation the diagnosis?

4. When is atropine overdose the diagnosis?

5. When is barbiturate overdose the diagnosis?

6. 54-year-old woman is brought to ED with “blurry vision” that began 1 week ago. When
you attempt an interview, she states that she would like some lemonade because it is “so
very hot.” When asked where she is, she responds “Why, at the county fair.” Exam: wide-
based gait, nystagmus, lateral rectus palsy of right eye. Next step in the management?
A. Administer thiamine and then glucose
B. Administer glucose and then thiamine
C. CT scan of head
D. Serum toxicology for ethanol and barbiturates

Answer:

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Summary: Case 11
With any patient who presents in an unconscious state, the next step is to secure the patient’s
airway and ensure adequate breathing and circulation. The initial medications given to any
patient who presents with coma of unknown etiology include naloxone, thiamine, and glucose.

When you are presented with a patient in a coma, the next step is to try to determine the
etiology of the loss of consciousness. Any coma can have various infectious, metabolic, or
structural causes. The presence of nuchal rigidity and fever argues strongly for the presence
of CNS infection as a cause of the coma. Focal neurologic examination in response to painful
stimuli argues for structural cause of the coma. When conjugate gaze deviation is present,
it suggests either an ipsilateral cortical lesion or a contralateral pontine lesion. The loss of
pupil reactivity implies midbrain involvement, and loss of ocular movement suggests pontine
involvement. Opioid overdose should be suspected when coma is accompanied by pinpoint
pupils. Uncal herniation is the most likely diagnosis when ipsilateral pupillary dilatation is
present with loss of consensual pupillary response. Atropine overdose should be suspected
in patients in a coma who have dilated and fixed pupils. Barbiturate overdose is associated
with bullous skin lesions. Brain death is considered possible when all brain-stem reflexes have
been absent for >6 hours. The presence of seizure activity or decerebrate or decortical postur-
ing is not consistent with brain death. Brain death cannot be established when a patient has
received sedatives or neuromuscular blockers. The apnea test is consistent with brain death
when there are no spontaneous breaths for a period >6 seconds in the presence of a PCO2
>60 mm Hg.

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Case 12
HIV-positive man with diabetes comes to clinic with “pins and needles” in hands and
feet. Problem began several months ago and has been progressively worsening.
More recently, he has noticed weakness in his hands and some difficulty walking.
He has hypothyroidism for 5 years, treated with levothyroxine. Neuro exam is
significant for loss of position and vibration sense in all 4 extremities, and gait
disturbance. Reflexes are symmetrically hyporesponsive in all extremities.

1. Diagnosis?
A. Vitamin B12 deficiency
B. Diabetes
C. Pyridoxine deficiency
D. Didanosine use
E. Hypothyroidism
F. Thyrotoxicosis

Answer:

Blood smear and CBC are normal. B12 level in lower range of normal.

2. Next step in diagnosis?

A. Hemoglobin A1c
B. Vitamin B6 level
C. T4/TSH level
D. Stop all antiretroviral medications
E. Nerve conduction studies
F. Methylmalonic acid levels
G. Schilling test
H. Anti-intrinsic factor antibodies

Answer:

Summary: Case 12
Subacute combined degeneration occurs with vitamin B12 deficiency. Patients will complain
of distal paresthesias and weakness of the extremities, followed by spastic paresis and ataxia.
On examination, there is a combined deficit of vibration and proprioception with pyramidal
signs (plantar extension and hyperreflexia). Diagnosis is made by low serum vitamin B12, and
treatment is with vitamin B12 replacement.

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Case 13
48-year-old woman comes with difficulty walking that started gradually and
progressively worsened over last few months. She has some difficulty swallowing
that started 1 month ago. Exam: right foot drop with hyperreflexia and increased
muscle tone, although the head droops. You also note atrophy of thenar and calf
muscles bilaterally. Wasting of tongue and resting fasciculations.

1. Diagnosis?
A. Lead poisoning
B. Parkinson disease
C. Amyotrophic lateral sclerosis (ALS)
D. Myeloma
E. Vitamin B12 deficiency
F. Hypocalcemia

Answer:

2. Treatment?
A. Plasmapheresis
B. IVIg
C. Steroids
D. Spinal cord growth factor
E. Riluzole

Answer:

3. After several months, patient returns to clinic with exertional shortness of breath and
worsening somnolence during the day. What would you recommend?
A. Tracheostomy
B. Endotracheal intubation
C. Lung transplant
D. Continuous positive airway pressure (CPAP)
E. No therapy is effective.

Answer:

Summary: Case 13
Amyotrophic lateral sclerosis should be the most likely diagnosis in any patient who presents
with a combination of upper and lower motor neuron findings on neurologic examination.
The treatment for ALS consists of riluzole, which acts to reduce the presynaptic release of
glutamate.

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Case 14: Parkinson Disease

65-year-old man brought by wife, who states her husband has been taking a
very long time getting dressed. He has fallen a number of times. Handwriting has
become smaller. Exam is significant for small-stepped, shuffling gait, resting tremor
of right hand, and cogwheel rigidity of right arm and wrist.

1. When is amantadine indicated?

Note
MAO-inhibitor:
Selegiline
2. When are anticholinergics (benztropine, trihexyphenidyl) indicated? Rasagiline
Place in therapy, not clear

3. When is carbidopa/levodopa indicated?

4. When are COMT inhibitors (tolcapone, entacapone) indicated?

5. When are dopaminergic agonists (bromocriptine, pramipexole, ropinirole) indicated?

6. When is deep brain stimulation indicated?

7. When is surgery indicated?

8. 55-year-old man with Parkinson disease and severe functional impairment was recently
started on carbidopa/levodopa for significant bradykinesia. His wife brings him to your
office, stating that he is “seeing bugs crawling on the floor” and claiming to be the first
man who has landed on Mars. Next step in management?
A. Initiate therapy with ropinirole
B. Discontinue the carbidopa/levodopa
C. Initiate therapy with quetiapine
D. Suggest a low-protein diet

Answer:

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9. • Frequent falls/postural instability • HYPEREXTENSION of neck/body • Cognitive

impairment/dementia • Vertical gaze impairment so it is hard to look down and walk
down stairs
Diagnosis?

Treatment?

Summary: Case 14
The cardinal manifestations of Parkinson disease are bradykinesia (manifested by slow move-
ments, mask facies, reduction of automatic movements), cogwheel rigidity, postural instabil-
ity, and resting tremor.

Several other diseases can imitate Parkinsonism. Severe depression can cause a paucity of
spontaneous movement that can mimic Parkinsonism. Essential tremor can be mistaken for
the tremor of Parkinson disease, but essential tremor can be distinguished by a lack of other
neurologic symptoms, a family history of tremor, and amelioration with alcohol. Normal
pressure hydrocephalus can present with ataxia and gait disturbances that can be mistaken
for Parkinson disease. The presence of dementia and urinary incontinence with dilated ven-
tricles on CT scan of the head help identify this disorder. Huntington disease can present
with akinesia and chorea. The positive family history usually suggests the correct diagnosis.

The diagnosis of Parkinson disease is a clinical one. It is important to identify any secondary
causes of a patient’s Parkinsonism that are potentially reversible. There is no diagnostic test
of choice that can identify patients with Parkinson disease.

There are many medications available for the treatment of Parkinson disease. The underlying
pathophysiology that causes Parkinson disease is the imbalance of dopaminergic (too little)
and cholinergic (too much) tone on the basal ganglia. Thus, medical treatment revolves
around increasing dopaminergic tone or decreasing cholinergic tone on the basal ganglia.

Not surprisingly, the medications available for the medical treatment of Parkinson disease
either directly stimulate dopamine receptors (carbidopa/levodopa, dopamine agonists), indi-
rectly increase the amount of dopamine available (COMT inhibitors, selegiline, amantadine),
or block acetylcholine stimulation of the basal ganglia (benztropine, trihexyphenidyl).

The first step when considering what medication to start in a patient with Parkinson disease
is to evaluate the patient’s functional status. Patients with intact functional status are man-
aged differently from patients with compromised functional status.

Patients with intact functional status (less bradykinesia) are not generally given carbidopa/
levodopa as initial therapy. If they are younger than 60 years, such patients are started on
anticholinergic medication. If the patient is older than 60 years, the treatment of choice
is amantadine. The reason why anticholinergics are relatively contraindicated in elderly
patients is that their side effects (dry mouth, urinary retention, constipation, confusion/hal-
lucinations) occur more frequently and severely in the elderly patient.

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For patients with compromised functional status (more significant bradykinesia), the best
initial therapy is going to be carbidopa/levodopa. Carbidopa inhibits extracerebral dopa
decarboxylase, allowing more of the levodopa to reach the CNS where it is needed. Levodopa
is the precursor to dopamine. Carbidopa protects the levodopa from breakdown in the
periphery, ensuring its secure delivery to the CNS. There are several late complications to
carbidopa/levodopa therapy. Dyskinesia (abnormal movements), akathisia (restlessness),
and “on-off ” phenomena are all disconcerting to the patient. All of these late side effects are
termed “response fluctuations” and can be managed by using a sustained release form of car-
bidopa/levodopa, adding a dopamine agonist, adding selegiline, adding a COMT inhibitor,
or restricting the main protein meal to the night.

Surgery should be considered only for patients who cannot tolerate medical therapy or who
are not responding adequately to medical therapy. The procedures usually done include
pallidotomy or thalamotomy.

Parkinsonian Syndromes
When the history is . . . The most likely diagnosis is . . .
Parkinson + orthostatic hypotension Shy Drager syndrome
Parkinson + ataxia Olivopontocerebellar atrophy
Parkinson + vertical gaze palsy, “wide-eyed stare” Supranuclear palsy
Acute-onset Parkinson Vascular disease
Urinary incontinence + dementia + “magnetic gait” Normal pressure hydrocephalus

Case 15: Seizures

34-year-old woman brought after roommate finds her unconscious on floor.
Roommate describes seeing jerking and twitching repeatedly, frothing at mouth,
and defecating on herself. Patient currently unresponsive. She suddenly begins to
jerk violently in a generalized fashion.

1. Initial treatment?

2. If seizures continued, what medication would you give?

3. If seizures still continued, what would you give?

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4. If seizures still continued, what would you give?

5. When is it reasonable to stop epileptic medications in a patient with epilepsy?

6. 27-year-old woman brought after suddenly losing consciousness while playing basket-
ball. Her mother tells you that before the woman passed out, her left foot began twitch-
ing, at which time she began to froth at the mouth. At that point, all of her limbs began
shaking violently. The woman is lethargic, but you cannot appreciate any neurologic
deficit. What is the next step in the management?
A. Begin therapy with phenytoin
B. Observation; no therapy indicated at this time
C. Begin therapy with lamotrigine
D. Begin therapy with pregabalin
E. Begin therapy with gabapentin

Answer:

7. Patient has a complex partial seizure with motionless stare, chewing, lip smacking, and
confusion. Where is the lesion?
A. Frontal lobe
B. Parietal lobe
C. Temporal lobe
D. Occipital lobe

Answer:

8. Man from Mexico comes for seizures. CT scan of brain shows multiple cystic lesions
with calcification. Next test?
A. Serology for cysticercosis
B. Serology for toxoplasmosis
C Stool for ova and parasite examination
D. Blood culture
E. HIV testing

Answer:

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Summary: Case 15
Seizures are caused by “VITAMINS”:

Vascular (stroke, bleed, arteriovenous malformation)

Infection (meningitis, abscess, encephalitis)

Trauma (especially penetrating)

Autoimmune (CNS vasculitis)

Metabolic (hyponatremia, hypocalcemia, hypomagnesemia, hypoglycemia, hypoxia, drug


overdose/withdrawal)

Idiopathic

Neoplasm

PSychiatric

A seizure is an essentially paroxysmal, involuntary event (associated with abnormal move-

ment or change of consciousness or both). Characteristically, seizures are sudden in onset,
with or without an aura. Patients often complain of disorientation, sleepiness, and muscle
aching for minutes to hours after the event. Patients may also experience incontinence,
tongue-biting, and headache as a result of the seizure. It may be difficult at times to differen-
tiate a seizure from syncope, and it is important to obtain a complete history from any indi-
vidual who witnessed the event. Generally, patients with syncope will not complain of sig-
nificant post-ictal symptoms. They will recover consciousness within several minutes of the
event and, on physical examination, will not have evidence of incontinence or tongue-biting.

It is important to classify seizures according to their clinical features because this will deter-
mine what medications will be used for treatment. Seizures can be classified as partial versus
generalized, and complex versus simple.

Partial seizures occur within discrete portions of the brain. The patient will often com-
plain of involuntary jerking of a finger or hand. When consciousness is maintained for the
duration of the seizure, it is termed a simple partial seizure. When there is a change in con-
sciousness during the seizure, it is termed a complex partial seizure. When a partial seizure
progresses to a generalized seizure, it is called a partial seizure with secondary generalization.
Typically, the seizure will begin focally and become generalized as the seizure activity involves
both cerebral hemispheres.

Generalized seizures arise from both cerebral hemispheres spontaneously without any
detectable focal onset. Generalized tonic-clonic (grand mal) seizures are characterized by
tonic contraction of muscles throughout the body, followed by intermittent relaxation of
various muscle groups (clonic phase). Absence seizures (petit mal) are characterized by sud-
den, brief loss of consciousness without loss of postural tone. Characteristically, the electro-
encephalogram (EEG) will show a generalized, symmetric 3-Hz spike-and-wave discharge
pattern. Atonic seizures are characterized by sudden loss of postural tone lasting 1 or 2 sec-
onds. Myoclonic seizures are characterized by sudden brief muscle contraction.

Status epilepticus is defined as recurrent or continuous seizures.

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EEG is the test of choice for the diagnosis of epilepsy. The diagnosis of idiopathic seizures
is made only after secondary precipitating factors have been ruled out. Always check serum
electrolytes, glucose, toxicology, and an arterial blood gas to rule out hypoxia as a cause of a
patient’s seizure. A CT scan or MRI of the head is usually indicated to rule out a structural
lesion as the cause of seizure. It is important to think of any seizure as a symptom—much like
shortness of breath or chest pain—that has an extensive differential diagnosis. The evaluation
of any seizing patient is to rule out reversible causes of seizure.

The treatment of seizures can be divided into the acute management of the acutely seizing
patient (status epilepticus) and the management of the epileptic patient.

The first step in the treatment of any acutely seizing patient is to secure the airway, breathing,
and circulation. Once adequate airway, breathing is ensured and the patient is hemodynami-
cally stable, the next step is to simultaneously evaluate and treat any precipitating causes of
seizure. If a reversible cause is identified, it should be treated aggressively.

If the patient continues to seize, the following strategy is appropriate: The initial drug of
choice is lorazepam or diazepam, both of which are benzodiazepines. These medications
work by potentiating GABA receptor function. If the patient continues to seize, the next
medication to add is either phenytoin or fosphenytoin. These medications work by inhibiting
sodium-dependent action potentials. CNS side effects of phenytoin include diplopia, dizzi-
ness, and ataxia. Systemic side effects include gum hyperplasia, lymphadenopathy, hirsutism,
and rash. If the patient continues to seize, the next medication to add is phenobarbital. Side
effects include sedation, ataxia, and rash. If, despite all of the above therapy, the patient con-
tinues to seize, then you can add either midazolam or propofol.

In patients with first-time seizures, anticonvulsant therapy should be started only if the
patient has an abnormal neurologic examination, has presented with status epilepticus, has a
strong family history of seizure, or has an abnormal EEG. Otherwise, first-time seizures are
generally not treated.

For primary generalized tonic-clonic seizures, valproic acid is considered first-line treatment.
(If valproic acid is not among the answer choices, you may select lamotrigine.) Valproic acid
works by increasing the availability of GABA. Side effects include ataxia, tremor, liver toxicity,
low platelets, gastrointestinal irritation, and hyponatremia. Lamotrigine works by decreasing
glutamate release. Side effects include diplopia, ataxia, rash, and Stevens-Johnson syndrome.
Absence seizures are treated with ethosuximide as first-line therapy. (If ethosuximide is not
among the answer choices, valproic acid is an acceptable option.) For myoclonic and atonic
seizures, valproic acid is the treatment of choice.

Partial seizures, whether they are complex or simple and whether or not they progress to
secondary generalized seizures, are all treated the same. Carbamazepine and phenytoin
are considered first-line therapy. Valproic acid and lamotrigine are considered acceptable
alternatives.

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Case 16: Dementia Syndromes

74-year-old man is brought by daughter for father’s memory. He has been
forgetting the places of simple things. Exam normal. Mini-Mental Status Exam is
significant memory impairment.

1. Diagnosis?

When the history is . . . The most likely diagnosis is . . .

Personality changes before memory impairment

Stepwise progression of dementia with temporal

association to vascular event
Dementia associated with anhedonia, insomnia,
sadness, feelings of worthlessness and anxiety
Gradual onset of impairment of more than one Alzheimer disease
cognitive domain, with memory going first
Dementia with focal neurologic examination Subdural hematoma, tumor
Cognitive impairment limited to memory Mild cognitive impairment
Dementia associated with daytime somnolence, Sleep apnea
loud snoring, apneic episodes

2. Treatment for Alzheimers?

3. 76-year-old man, who is a retired mathematician, comes to office for routine exam: He
asks what mental function he is most likely to lose first as he ages. What will be your
answer?
A. Attention and calculation
B. Orientation
C. Registration
D. Memory

Answer:

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4. 82-year-old man from nursing home is admitted for increasing confusion and the
inability to control his urine. He has slowly progressive memory loss. He is disoriented
to time and place and walks with a wide-based gait. CT shows dilated ventricles with
very little atrophy. Diagnosis?
A. Alzheimer disease
B. Normal pressure hydrocephalus
C. Huntington’s chorea
D. Multi-infarct dementia

Answer:

5. Which has been shown to improve memory in women with late-stage Alzheimer
disease?
A. Estrogen
B. Estrogen/progesterone combination
C. Memantine
D. Vitamin E
E. Aspirin

Answer:

6. 85-year-old woman admitted to nursing home for long-term inability to ambulate after
a hip fracture. She recovers from herpes zoster. Over next several weeks, she is more
confused. She asks about a grandson that died. Her medications are hydrochlorothia-
zide, ibuprofen, lorazepam, and amlodipine. What is next?
A. Transfer to hospital for evaluation
B. Switch ibuprofen to acetaminophen
C. MRI of the brain
D. Consult neurology
E. Stop the lorazepam

Answer:

7. Rapidly progressive dementia with myoclonus.

Diagnosis?
Test?
Treatment?

8. Dementia with VIVID hallucinations. Greatly detailed. Fluctuating. Parkinsons

Diagnosis?
Treatment?

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9. Dementia with choreiform movement with emotional lability.

Diagnostic test?
Treatment?

Summary: Case 16
There are over 100 identifiable causes of dementia in the elderly. Among the many revers-
ible causes of dementia you should consider hypothyroidism, vitamin B12 deficiency, hepatic
or uremic encephalopathy, CNS vasculitis, syphilis, brain abscess, brain tumor (primary
or metastatic), medications (especially anticholinergics), obstructive sleep apnea, central
sleep apnea, trauma, subdural hematoma, normal pressure hydrocephalus, and depres-
sion. Irreversible causes of dementia include progressive multifocal leukoencephalopathy;
Alzheimer disease; dementia with Lewy bodies; frontotemporal degeneration, including Pick
disease; and vascular dementia, including multi-infarct dementia, Binswanger disease, and
Creutzfeldt-Jakob disease (CJD).

The most common cause of dementia is Alzheimer disease. Typically, patients will present
with problems in memory and visuospatial abilities that generally occur early in the course
of the disease. Social graces can be retained despite significant loss of cognitive decline.
Hallucinations and personality changes typically occur late in the course of the disease.

Mild cognitive impairment refers to memory loss without dysfunction of other cognitive
domains. These patients have a higher risk of developing Alzheimer disease later in life but
do not have Alzheimer disease.

Patients with frontotemporal dementias, such as Pick disease will typically present with per-
sonality changes early in the course of their disease with relative sparing of their visuospatial
function.

Dementia with Lewy bodies (DLB) can be confused with delirium and is characterized
by fluctuating cognitive impairment. Dementia secondary to Parkinson disease should be
accompanied with clinical findings consistent with that disease.

Dementia secondary to CJD is characterized by a shorter (weeks to months), more aggressive

course than Alzheimer disease. Patients with CJD will present with dementia and myoclonus.

Vascular dementia is divided into multi-infarct dementia, which typically has a stepwise pro-
gression associated with discrete cerebrovascular events; and Binswanger disease, involving
the subcortical white matter, which presents with a slowly progressive course.

Normal pressure hydrocephalus will present with prominent gait abnormalities early in the
course of the disease that usually precede the onset of cognitive impairment. There will also
be associated incontinence.

All patients with cognitive impairment should be assessed with a Mini-Mental Status
Examination to identify the areas of cognitive impairment.

Initially, the workup should focus on ruling out reversible causes of the dementia. If a revers-
ible cause is identified it should be treated, with the hope that cognitive function can be
recovered. Laboratory studies should include a complete blood count, electrolytes, calcium,
creatinine, liver function studies, glucose, TSH, vitamin B12, RPR, and HIV. Brain imaging
should be reserved for patients who have focal neurologic examination, seizures, gait abnor-
malities, and an acute or subacute onset of their symptoms.

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Treatment of dementia revolves around ensuring that the family and the patient have the
proper medical and emotional support to cope with the disease. Caregivers are at increased
risk for depression and anxiety. Their concerns and frustrations should be addressed at fre-
quent intervals.

Pharmacotherapy with donepezil has been shown to improve cognitive function in mild to
moderate dementia. Other anticholinesterase inhibitors (rivastigmine, galantamine) appear
to be have similar efficacy. If the caregiver reports no improvement within 3 to 6 months,
pharmacotherapy should be discontinued.

Case 17: Headaches

23-year-old woman with bilateral headaches occurring since her menstrual periods
began at age 15. Describes bilateral, throbbing headaches that peak between 6
and 12 hours after they start. Sometimes before headache starts she sees “flashing
lights.” Neuro exam nonfocal.

1. Diagnosis?

2. Treatment?

After 2 weeks of daily headaches, patient returns to clinic. Despite taking the
prescribed medication, her headaches are worsening.

3. When is prophylaxis?

4. What to use?

5. Most common precipitant of migraine?

A. Foods
B. Emotions
C. Sex
D. Exercise

Answer:

6. Which of the following is a contraindication to using triptans?

A. Pregnancy and coronary disease
B. Hyponatremia
C. Seizure disorder
D. Depression and schizophrenia

Answer:

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If a patient presents with headache and one of the

following, seriously consider CT scan or MRI of the brain:

• “Thunderclap” headache
• “This is the worst headache of my life!”
• Progressively worsening headache
• Onset at age >40 years
• Precipitation of headache with cough, sneeze, strain, or bending
• Fever, weight loss, malaise, scalp tenderness, jaw claudication
• Focal neurologic examination

Summary: Case 17 Note

Primary headache syndromes include migraine and cluster and tension-type headaches. Visual disturbance most in
Secondary causes of headache include intracranial hemorrhage, brain tumor, meningitis, migraines.
temporal arteritis, and glaucoma.

A history of recurrent symptoms makes the diagnosis of a primary headache disorder more
likely. A history of a first-time headache, especially when severe and rapidly peaking, speaks
strongly for serious underling pathology. Additionally, any patient who presents with the
following should be considered to have a secondary headache syndrome: complaints of “the
worst headache of my life;” worsening symptoms over days to weeks; an abnormal neurologic
examination; fever; vomiting preceding the headache; headache induced by coughing, bend-
ing, or lifting; or onset after 55 years of age. Headache with fever and nuchal rigidity suggests
meningitis as the underlying cause. Conversely, a headache that is described as “the worst
headache of my life,” is thunderclap in onset, and is accompanied by nuchal rigidity without
fever suggests an intracranial hemorrhage as the underlying cause.

Patients with brain tumors will present complaining of headache that is described as a deep,
dull, aching pain that disturbs sleep. The history of vomiting that precedes the onset of head-
ache by a number of weeks or a history of headache induced by coughing, lifting, or bending
is typical of posterior fossa brain tumors.

Patients with temporal arteritis complain of a unilateral pounding headache associated with
visual changes, described as a dull and boring with superimposed lancinating pain. Patients
will also complain of polymyalgia rheumatica, jaw claudication, fever, weight loss, and scalp
tenderness (difficulty combing hair or lying on a pillow). Temporal arteritis is a disorder of
the elderly generally presenting in patients older than 50 years. Patients with glaucoma will
usually give a history of eye pain preceding the onset of their headache.

1. Man with right-sided sharp pain in head, red eye, runny nose. Had 4 episodes in last
2 days. Gets 8-10 of these every year at this time.
Best abortive therapy?
Prophylactic therapy?
A. Triptans
B. Verapamil
C. SSRI
D. Valproic acid

Answer:
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2. 47-year-old woman gets severe headaches 2–3 times every few months. They are a few
days apart. She is not pregnant and has no heart disease. Best therapy?
A. Propranolol daily
B. Verapamil daily
C. Sumatriptan as needed
D. 100% oxygen
E. Prednisone

Answer:

3. Patient with recurrent migraine headaches almost daily. They respond well to injection
of sumatriptan. Your plan?
A. Oral sumatriptan daily
B. Cyproheptadine daily
C. Head CT scan
D. Propranolol daily

Answer:

4. Who is more likely to have Horner’s syndrome?

A. Cluster headache
B. Tension headache
C. Migraine
D. Pseudotumor cerebri

Answer:

5. Which of the following is most like to have focal neurological defects?

A. Migraine
B. Cluster

Answer:

6. 54-year-old man with moderate severe headaches. Going on for years. Associated with
nausea. BP 150/85. Fundus normal. No focal deficits. No neck stiffness. History coro-
nary disease.
What next?
A. MRI head
B. Lumbar puncture
C. Naprosyn and prochlorperazine
D. Sumatriptan

Answer:

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Migraine headaches are defined as a benign and recurrent syndrome of headache, nausea/
vomiting, and other varying neurologic dysfunction. Patients will describe the headache as
throbbing, unilateral, and aggravated by minor movement. Other associated features include
photophobia, phonophobia, and the time to maximal pain (4 to 72 hours). Migraine is a
likely diagnosis when a typical trigger can be identified. Typical triggers include alcohol;
certain foods, such as chocolate, cheeses, and monosodium glutamate; hunger; or irregular
sleep patterns. Migraine without aura is a migraine without a preceding focal neurologic
deficit. Migraine with aura (classic migraine) is a migraine headache that is accompanied by
a preceding aura, which consists of motor, sensory, and/or visual symptoms. Focal neurologic
symptoms usually occur during the headache rather than as a prodrome. The pathogno-
monic aura for classic migraine is the scintillating scotoma. Migraine equivalent is defined as
focal neurologic symptoms without the classic complaints of headache, nausea, and vomit-
ing. Complicated migraines are migraines with severe neurologic deficits that persist after
the resolution of pain. Basilar migraines are migraine headaches associated with symptoms
consistent with brain-stem involvement (vertigo, diplopia, ataxia, or dysarthria).

Cluster headaches begin without warning and are typically described as excruciating, perior-
bital, and peaking in intensity within 5 minutes of onset. They are rarely described as pulsatile
in nature. The attacks will last from 45–90 minutes and occur 1–3 times a day for a 4- to 8-week
period. Symptoms associated with cluster headache include rhinorrhea, reddening of the eye,
nasal stuffiness, and nausea. Emotion and food rarely will trigger a cluster headache.

Tension-type headaches are described as tight, bandlike headaches that occur bilaterally.
Patients may also describe their headache as “viselike” and perhaps associated with tight-
ness of the posterior neck muscles. Patients will describe their pain as one that builds slowly
and may persist for several days with or without fluctuations. Movement will not generally
exacerbate the headache.

The patient who presents with severe, sudden onset of a first-time headache accompanied by
strong evidence for an underlying cause on history or physical examination should have a CT
scan of the head to rule out any secondary causes.

Effective management of migraine headaches should always begin with an attempt to identify
probable triggers for the individual patient and to modify the person’s lifestyle by avoiding
the things that trigger the symptoms. Most patients will require pharmacotherapy in addition
to any nonpharmacologic intervention that is advised.

The pharmacologic treatment for migraine headaches can be divided into management of an
acute episode and prophylaxis. Initially, in mild migraine that is defined as headache in the
absence of nausea or vomiting, nonsteroidal anti-inflammatory medications may be used.
Acutely, abortive therapy consists of sumatriptan, which acts as a serotonin receptor agonist.
The “-triptans” are contraindicated in patients with known cardiovascular disease. These
medications can be given orally, intranasally, or even subcutaneously, depending on the
severity of the headache. Alternatively, ergotamine can be given for acute abortive therapy.
Dopamine antagonists, such as metoclopramide, can be given acutely as an oral formulation
to aid in the absorption of other abortive medications. When given parenterally, dopamine
antagonists can provide relief acutely for migraine headaches.

Prophylactic treatment for migraine therapy should be started when patients have acute
migraine headaches more than 3 times per month. Propranolol, timolol, valproic acid, and
methysergide are all considered first therapy for migraine prophylaxis. These medications
take 2 to 6 weeks to have an effect and can be discontinued gradually over 6 months once
clinical stabilization has occurred. Methysergide is associated with valvular and retroperito-
neal fibrosis.

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Note Opioid analgesics are not routinely recommended for the treatment of migraine because of
the possibility of developing addiction. Opioids are used only in patients with severe, infre-
Migraine quent migraines that are unresponsive to other therapy.
• Beta blocker Treatment for tension headache consists of relaxation. Patients should be encouraged to find
Cluster activities that are relaxing for them. Initial pharmacotherapy consists of acetaminophen and
NSAIDs.
• Verapamil
Cluster headaches are prophylactically treated with verapamil, prednisone, or lithium.
Acutely, the most effective treatment is triptans or 100% oxygen. Sumatriptan is equal to
100% oxygen. Cluster headaches are best prevented with verapamil.

Case 18
35-year-old woman with headache that started slowly approximately 6 months
ago. Located bilaterally. Worse upon awakening. Associated with blurry vision
and transient visual obscurations. Also complains of “ringing in her ears” that
corresponds to her heartbeat. The patient is obese. Funduscopic exam significant
for papilledema.

1. Diagnosis?

2. Which is most often found?

A. Diplopia
B. Neck stiffness
C. Paralysis

Answer:

3. Next step in management?

4. What test confirms your diagnosis?

5. Initial treatment?

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6. When would you consider surgery?

7. Most common long-term manifestation?

A. Visual field loss
B. Stroke
C. Hydrocephalus

Answer:

Summary: Case 18
In an obese woman of childbearing age who presents with headache and papilledema, the
most likely diagnosis is pseudotumor cerebri. The next step in the management of this patient
is to obtain an MRI of the brain to rule out a space-occupying lesion as a cause of her papill-
edema. Magnetic resonance venography can be useful to rule out cerebral venous thrombosis.
Other causes of pseudotumor cerebri include hypoparathyroidism, Addison disease, hyper-
vitaminosis A, oral contraceptives, tetracycline use, and rapid corticosteroid withdrawal. The
best initial treatment is weight loss and treatment of any identified underlying cause. The next
step in the management of these patients includes therapy with acetazolamide and steroids.
Surgery should be considered for patients with vision loss or refractory headaches.

Case 19: Altered Mental Status

You are called to bedside by the nurse regarding a patient who is agitated. While
giving his medication the elderly man became acutely agitated and combative,
attempting to strike her. As you enter room patient shouts, “Get away from me!”

1. Name of this syndrome? How is it different from dementia?

2. Next step in management?

3. What med to give?

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When this is in the history and physical The most likely diagnosis is . . .
examination . . .
IDU, alcohol abuse, substance abuse Withdrawal or intoxication
Fever, elevated WBCs, nuchal rigidity Acute infection

Consider UTI, pneumonia, meninigitis,

encephalitis, cellulitis
Paradoxical movement of abdomen on Hypoxemia, hypercapnia
inspiration, respiratory distress
History of carcinoma with focal Metastatic carcinoma to the brain
neurologic examination
Jaundice, ascites, and edema Hepatic encephalopathy
Bilateral asterixis Metabolic encephalopathy, i.e., hepatic,
hypercapnia, drug ingestion
Coagulopathy with focal neurologic Intracranial bleed
examination
Myoclonic jerking and tremor in an Uremia or antipsychotic medication
awake patient
Anticholinergic medication Reaction to the drug
Urinary or fecal incontinence, or tongue Seizure
lacerations
Postoperative Hyponatremia

4. Woman comes to clinic without complaint. On exam, when light is shined in left eye
there is no pupillary constriction. When light is shined in right eye, both pupils con-
strict normally. Funduscopic exam is normal. Diagnosis?
A. Retinal detachment
B. Essential hypertension
C. Optic neuritis
D. Glaucoma

Answer:

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5. Man comes to clinic complaining of face stiffness. Face feels “it is being pulled to one
side.” Symptoms began suddenly. Denies pain but “hearing soft noises loudly.” Exam:
right-sided facial palsy with restricted eye closure. What would you recommend?
A. Observation
B. Aspirin
C. Surgical decompression
D. Prednisone and eye lubrication

Answer:

6. Woman with gradual vision loss over last 5 years. Funduscopic exam, you note moder-
ate neovascularization and round, yellowish spots haphazardly distributed at the pos-
terior pole of the retina. Next step in management of this patient?
A. Radiotherapy
B. Laser photocoagulation
C. Observation
D. Pilocarpine eye drops

Answer:

Summary: Case 19
In any patient who presents with acute delirium, the next step is to identify an underlying
cause of the patient’s symptoms. Indications for sedation include engagement in harmful
activity or subjective distress from hallucinations associated with the delirium. Haloperidol
is the agent of choice for the pharmacologic management of the acutely delirious individual.

Case 20: Psychiatric Disorders

45-year-old woman comes complaining of palpitations. She has begun to feel her
heart racing once a day for last 3 weeks. She has visited your clinic in the past for
evaluation of chest pain, headache, back pain, and bloating. All previous workups
have been negative. Exam today is normal.

1. Diagnosis?

2. Best management?

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3. 25-year-old woman comes to emergency department after an episode of severe

headache when she attempted to board an airplane earlier that day. Symptoms were
accompanied by right-sided chest pain that resolved within 5 minutes of onset. She felt
smothered and also had some shortness of breath. This is her first such episode. Exam
normal. EKG shows no abnormalities. Best initial therapy?
A. Education and relaxation techniques
B. Cognitive behavioral therapy
C. Diazepam
D. Fluoxetine

Answer:

4. 18-year-old girl comes to office at behest of her mother because of dramatic weight loss
over the last 3 months since she broke up with her boyfriend. During interview she is
constantly fixing her makeup. When asked about her weight, she immediately appears
horrified and asks, “Do I look fat?” Vehemently denies binge eating. Has not menstru-
ated for last 5 months. She appears emaciated. You note dry, scaly skin and parotid
gland enlargement. Diagnosis?
A. Bulimia nervosa
B. Hyperthyroidism
C. Lymphoma
D. Anorexia nervosa
E. Celiac sprue

Answer:

5. 57-year-old man complaining of feeling worthless since he lost his job 4 weeks ago. He
feels sad and empty and does not find pleasure in his grandchildren. He has also noticed
extreme fatigue and low energy on a daily basis. Wife states that her husband has been
agitated and irritable over the last 2 weeks. Exam normal. EKG: old LBBB (left bundle
branch block). Initial therapy?
A. Desipramine
B. Amitriptyline
C. Citalopram
D. Selegiline

Answer:

6. Woman complaining of recurrent anxiety about cleanliness of her home. States that her
thoughts have begun to interfere with her daily living, because she feels it necessary to
clean every doorknob before she can touch it. Exam normal. Best initial therapy?
A. Behavior modification
B. Cognitive therapy
C. Fluoxetine
D. Clomipramine

Answer:
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7. Known alcoholic is acting very bizarrely because of the inability to find alcohol. Which
medications should you AVOID in this situation?
A. Glucose and thiamine
B. Lorazepam
C. Naloxone
D. Carbamazepine
E. Haloperidol

Answer:

8. Patient on clozapine for psychosis. Blood count reveals leucopenia. What would you do?
A. Discontinue clozapine and use another antipsychotic medication
B. Bone-marrow biopsy
C. HIV testing
D. Repeat the CBC in 10 days
E. Colony-stimulating factor

Answer:

9. 74-year-old man has chronic constipation, prostatic hypertrophy, and glaucoma. He

presents with depression requiring an antidepressant. What is the drug of choice?
A. Desiprimine
B. Nortriptyline
C. Amitriptyline
D. Fluoxetine

Answer:

10. An antidepressant is started. How long should you wait to see the maximum effect?
A. 2 months
B. 9 months
C. 1–2 years
D. Indefinitely

Answer:

11. A patient is depressed following the death of her husband. There is plenty of support
from the family. They bring her to you. Which is most appropriate management?
A. Supportive therapy and return in 2 weeks
B. Paroxetine
C. Diazepam
D. Electroconvulsive therapy

Answer:

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12. Man comes with multiple physical complaints referable to different organs. Patient is
impulsive, dramatic, and demanding. You do not believe symptoms are intentionally
produced or feigned. Diagnosis?
A. Somatoform disorder
B. Conversion disorder
C. Hypochondriasis
D. Factitious illness

Answer:

13. Young female complains to you that your partner is treating her improperly. She says
that he avoids her questions and really does not care, and that she likes your style bet-
ter. She has missed many appointments and has had three different primary doctors.
After the visit, she calls you many times to ask different medical questions. Diagnosis?
A. Paranoid personality disorder
B. Borderline personality disorder
C. Panic attacks
D. Conversion disorder

Answer:

14. Which is associated with highest lifetime risk of suicide?

A. Bipolar disorder
B. Major depression
C. Borderline personality
D. Anxiety disorder

Answer:

Summary: Case 20
In a patient who complains of ≥4 or more unexplained symptoms, the most likely diagnosis
is abridged somatization. Somatization disorder requires a patient to have at least 8 unex-
plained medical symptoms.

Patients with somatization should have 1 primary care provider and should have frequent,
brief, scheduled visits. In the absence of associated depression or panic disorder, medications
are unlikely to be of any benefit.

Haloperidol should be avoided in those with alcohol abuse, because it lowers the threshold to
have a seizure. Clonazepine is associated with neutropenia and if the medication is given and
neutropenia develops, the clonazepine should be stopped immediately. Tricyclic antidepres-
sants should be avoided in elderly patients particularly if there is constipation, urinary reten-
tion, or glaucoma. Antidepressant medications will reach a maximum therapeutic benefit in
about two months. Brief reactive depression, such as from a death in the family, should not
be treated with medication.

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Somatoform disorder is multiple physical complaints referable to different organs. The

patient is often dramatic, impulsive, and demanding. In somatoform disorder, the patient
is not purposely feigning the symptoms. In a conversion disorder, there is a specific motor
or sensory disorder, such as aphonia, paralysis, or pseudo seizures, but the symptoms are
not intentionally produced. Hypochondriasis is when a person believes that he or she has
a serious medical illness, despite reassurance and appropriate medical evaluation. In facti-
tious illness, physical symptoms are produced voluntarily and the sick role is itself a source
of gratification.

Case 21: Peripheral Neuropathy

54-year-old man comes for evaluation of blurry vision. Pupils are not the same
size, with left greater than right. Left eye cannot look up, down, or in; and left
eyelid is drooping.

1. Diagnosis?
A. Horner syndrome
B. Eaton-Lambert syndrome
C. Myasthenia Gravis
D. Third cranial nerve palsy

Answer:

2. 63-year-old woman, who has a history of diabetes, presents with inability to move her
right eye up, down, or in. There is ptosis of the same eye. Pupils react normally. Most
common cause?
A. Intracranial neoplasm
B. Cavernous sinus thrombosis
C. Diabetes mellitus
D. Lung cancer at the apex of the lung

Answer:

3. After an injury to elbow, patient develops hyperextension of the metacarpophalangeal

joints with flexion of the interphalangeal joints. Hand looks like a claw. Most likely
diagnosis?
A. Ulnar nerve palsy
B. Radial nerve palsy
C. Peroneal nerve palsy
D. Tarsal tunnel syndrome
E. Morton’s neuroma
F. Meralgia paresthetica (lateral cutaneous nerve)

Answer:

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4. Man falls asleep drunk in bar with arm over back of chair. When he wakes up, he is not
able to extend his wrist (‘wrist drop’). Diagnosis?
A. Ulnar nerve palsy
B. Radial nerve palsy
C. Peroneal nerve palsy
D. Tarsal tunnel syndrome
E. Morton neuroma
F. Meralgia paresthetica (lateral cutaneous nerve)

Answer:

5. Obese pregnant woman with pain and numbness of the outer aspect of one thigh that
seems to worsen with sitting. Diagnosis?
A. Ulnar nerve palsy
B. Radial nerve palsy
C. Peroneal nerve palsy
D. Tarsal tunnel syndrome
E. Morton neuroma
F. Meralgia paresthetica (lateral cutaneous nerve)

Answer:

6. Pain and numbness in the ankle and sole of the foot with parasthesias that worsens with
walking. Diagnosis?
A. Ulnar nerve palsy
B. Radial nerve palsy
C. Peroneal nerve palsy
D. Tarsal tunnel syndrome
E. Morton neuroma
F. Meralgia paresthetica (lateral cutaneous nerve)

Answer:

7. A woman comes in with weakness of the foot with loss of dorsiflexion and eversion. She
wears high boots and often sits with her legs crossed. Diagnosis?
A. Ulnar nerve palsy
B. Radial nerve palsy
C. Peroneal nerve palsy
D. Tarsal tunnel syndrome
E. Morton neuroma
F. Meralgia paresthetica (lateral cutaneous nerve)

Answer:

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8. 45-year-old woman with sharp intermittent pain radiating into the toes. She feels
better when she takes off her shoes. There is tenderness when pressure is applied
between the heads of her second and third metatarsals. Most likely diagnosis? Note
A. Ulnar nerve palsy
Nocturnal awakening with
B. Radial nerve palsy
hand pain is carpal tunnel.
C. Peroneal nerve palsy
D. Tarsal tunnel syndrome
E. Morton neuroma
F. Meralgia paresthetica (lateral cutaneous nerve)

Answer:

9. Man with a history of healed trauma to the foot comes with severe pain when a sheet
touches his foot. The foot is pale. Diagnosis?
A. Acute gout
B. Tarsal tunnel syndrome
C. Reflex sympathetic dystrophy
D. Fractured ankle

Answer:

10. Avid biker, with wasting of hypothenar eminence and interosseous muscles. No sensory
loss. What is involved?
A. Radial nerve
B. Median nerve
C. Brachial nerve
D. Ulnar nerve

Answer:

11. Sensory loss of lateral aspect of thigh. Which spinal segment is involved?
A. L4
B. L5
C. S1
D. L2

Answer:

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12. Patient with facial droop and blisters on the external auditory canal. Etiology?
A. Herpes simplex
B. Varicella zoster
C. Epstein-Barr virus
D. Cytomegalovirus

Answer:

13. Most common cause of mononeuritis multiplex?

A. Multiple sclerosis
B. Sarcoidosis
C. Diabetes mellitus
D. Malignancy
E. HIV

Answer:

14. 35-year-old athlete has pain in right heel. He has tenderness on palpation of anterior calca-
neus. Pain is extremely severe when arising in morning with first few steps. Improves with
walking and stretching. Diagnosis?
A. Plantar fasciitis
B. Tarsal tunnel syndrome
C. Osteoarthritis
D. Peroneal nerve palsy

Answer:

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Summary: Case 21
Third cranial nerve palsy results in the inability to lift the eyelid, or to look up, down, or in.
When diabetes causes third nerve palsy, the ability to constrict the pupil is retained. Injury
at the elbow resulting in a ‘claw’ hand results from ulnar nerve palsy. ‘Saturday night palsy’ is
damage to the radial nerve that results in wrist drop. This is often transient in nature. Injury
to the lateral cutaneous nerve of the thigh or ‘meralgia paresthetica’ is more common in obese
patients, pregnancy, and with sitting cross-legged. It is an injury of the L2 nerve root. Tarsal
tunnel syndrome is like carpal tunnel syndrome occurring in the foot. Prolonged exercise
makes it worse. Plantar fasciitis gets BETTER with stretching and exertion and is maximally
painful with the first few steps, then improves. Tarsal tunnel causes both pain and numb-
ness of the sole and ankle. Plantar fasciitis is extremely painful and tender at the calcaneus.
There is no numbness with plantar fasciitis. Peroneal nerve palsy occurs more often in those
who wear high boots and is associated with foot drop. Morton’s neuroma causes pain that
is markedly improved when shoes are removed. It is near the 2nd and 3rd metatarsal heads.
Reflex sympathetic dystrophy is clearly associated with healed trauma to the foot and results
in severe pain with relatively minor contact, such as a sheet touching the foot. Injury to the
ulnar nerve can also occur from repetitive minor trauma to the hand while riding a bicycle.
There is wasting of the hypothenar eminence as well. Ramsay-Hunt syndrome is a Varicella-
zoster reactivation with blisters in the external auditory canal as well as facial droop. Diabetes
is the most common cause of mononeuritis multiplex by far.

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 Nephrology
4
ACUTE RENAL FAILURE

Case 1
70-year-old man admitted with diverticulitis confirmed by abdominal CT scan. Was
placed on ampicillin, gentamicin, and metronidazole. History of benign prostatic
hypertrophy and cirrhosis. Over last 2 days, condition has worsened, including
vomiting. BP 90/60 mm Hg, pulse 105. Repeat abdominal CT scan shows a possible
peridiverticular abscess. BUN now 34; and creatinine 2.4.

1. Most likely cause of renal failure?

A. Prerenal azotemia
B. Acute tubular necrosis
C. Allergic interstitial nephritis
D. Postrenal obstruction
E. No clear diagnosis at this time

Answer:

2. What to do next to confirm the diagnosis?

Prerenal Azotemia Acute Tubular Necrosis

U/A
BUN: Creatinine 20:1 BUN: Creatinine 10:1
Urine sodium Urine sodium

FeNa FeNa
Urine osmolality Urine osmolality

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3. How would hepatorenal syndrome fit this diagnostic scheme? Therapy?

4. What test specifically confirms gentamicin as cause of renal failure? What about
Note
contrast?
3 Reasons for Renal Failure
• Pre-renal
• Intrinsic Disease
• Post-renal 5. Treatment proven effective for drug-induced acute tubular necrosis?

Summary: Case 1
Prerenal azotemia can result from any cause of decreased perfusion of the kidney. The pres-
ence of renal insufficiency is not dependent on the total body fluid status. All that matters is
Note how much perfusion the kidney is getting. The kidney experiences renal artery stenosis the
same way that it would life-threatening hypotension. The juxtaglomerular complex does not
Causes of Pre-renal Azotemia know that the systemic blood pressure is elevated. Even though a patient may have severe
Volume Depletion edema from congestive failure or the low oncotic pressure of hypoalbuminemia or cirrhosis,
the kidney experiences this in the same way it would severe dehydration. Hepatorenal
• Vomiting
syndrome gives the same numbers as prerenal azotemia, i.e., an elevated BUN:creatinine
• Diarrhea ratio and a low urine sodium, because there is an intense renal vasoconstriction resulting
• Bleeds in decreased renal perfusion. When the BUN:creatinine ratio cannot help you distinguish
the source of the renal failure, you can also use the urine osmolality. In prerenal azotemia,
• Burns
the urine osmolality is >500. In acute tubular necrosis (ATN), renal concentrating ability is
defective, the kidney can neither concentrate nor dilute the urine, and the urine osmolality is
<350. The urine sodium can easily substitute for the fractional excretion of sodium (FeNa). If
the urine sodium is low, the FeNa will be <1%. Hypomagnesemia is suggestive of gentamicin-
induced renal failure. There is no specific therapy to reverse ATN. Hydration, diuretics, and
low-dose dopamine have never been shown to alter the overall outcome.

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Case 2
Man with tuberculosis was placed on isoniazid, rifampin, ethambutol, and
pyrazinamide. Initially became afebrile. He developed a new fever (38.8°C/102°F)
and rash. Having visual disturbance as well. History significant for gout and seizure
disorder. On phenytoin for many years, taking allopurinol off and on for long time.
BUN and creatinine rise to 28 and 2.4.

1. Type diagnosis of renal insufficiency? Note

ATN: UA
Muddy granular casts, no
2. Cause? RBCs

3. Initial test?

4. Most specific test?

5. Initial therapy?

6. Why does he have visual disturbance?

The renal insufficiency has caused an accumulation of ethambutol that has caused the
optic neuritis resulting in his visual disturbance.

7. At what BUN and creatinine to start dialysis?

8. What in history/physical/labs tell you to use dialysis?

Refractory hyperkalemia, metabolic acidosis, pericarditis, fluid overload, encephalopathy

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Summary: Case 2
Allergic or acute interstitial nephritis (AIN) can be caused by virtually any medication that
Note results in an allergic reaction in the kidney. The drugs are the same drugs that patients are
allergic to in general, such as penicillins, sulfa drugs, phenytoin, allopurinol, and rifampin.
ACE does not cause interstitial Fluoroquinolone antibiotics are also a prominent source of interstitial nephritis. Diuretics
nephritis. such as thiazides and furosemide are sulfa derivatives as well. AIN can also be caused acutely
as a reaction to virtually any bacterial, viral, or Rickettsial infection. The main way to clini-
cally distinguish AIN from other causes of acute renal failure is the presence of fever, rash,
joint pains, and eosinophils in the blood and urine. Urinalysis will show white cells but can-
not specifically identify them as eosinophils. You need to specifically test for them with either
a Wright stain or Hansel stain of the urine. The majority of cases will respond to simply ceas-
ing the offending medication. This is why it is important to determine a specific diagnosis;
otherwise, you cannot be sure which medication to stop. In very severe cases of AIN that seem
to be progressing into end-stage renal disease, despite ceasing the offending medication, the
therapy is steroids. Dialysis use is not based on a specific BUN or creatinine level; it is based
on the development of life-threatening symptoms that cannot be corrected by other means,
such as hyperkalemia, severe metabolic acidosis, pericarditis, fluid overload, or neurologic
abnormalities such as encephalopathy.

Case 3
30-year-old man has just finished 25-mile bicycle ride after a triathlon. He has a
seizure, while on ground gets run over by car. History of hypercholesterolemia.
Takes pravastatin, simvastatin, atorvastatin, lovastatin, and extra helpings of
cerivastatin. He is now in ED. You are first person to see him after he has been
lying in bed in hallway for 4 hours.

1. Most urgent step? (How will he die first?)

2. First test to establish specific diagnosis?

A. EKG
B. Urinalysis
C. CK
D. Urine myoglobin
E. Renal ultrasound

Answer:

3. Initial therapy?

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Summary: Case 3
Rhabdomyolysis can be caused by any form of severe muscular injury, such as that from a
seizure, strenuous exercise, cocaine, or even prolonged pressure. Just lying on a hard floor Note
for several hours from syncope can result in muscular necrosis. Heat stroke, neuroleptic
malignant syndrome, and malignant hyperthermia can all cause severe muscle necrosis. On Rhabdomyolysis lowers
the other hand, hypothermia causes it as well. A low level of potassium or phosphorus and calcium.
the use of statin medications and alcohol can also lead to muscle necrosis. Endocrine myopa-
thies, such as those associated with hyperthyroidism and hypercortisolism do not elevate the
muscle enzyme levels. The urinalysis is the best initial way to determine if a patient has severe
rhabdomyolysis. The urine will be dipstick-positive for blood, but on microscopic examina-
tion, you will not find any red cells. The CPK (creatine phosphokinase) will be markedly
elevated into the thousands, but this takes more time. The most urgent step in life-threaten-
ing rhabdomyolysis, however, is either to check a potassium level or to check the EKG (elec-
trocardiogram). Patients can die very acutely from abnormalities of the cardiac conduction
system. Rhabdomyolysis is treated acutely with hydration and bicarbonate. The bicarbonate
will correct a severe acidosis as well as prevent the precipitation of myoglobin at the kidney
tubules. Hydration will decrease the contact time of the myoglobin with the kidney tubule.

Case 4
Man with history of hypertension admitted for chest pain is treated for unstable
angina. Pain has resolved. Coronary angiography is being planned. Patient has
long-standing mild renal insufficiency with creatinine 1.7–2.0.

1. What to do prior to angiography to decrease risk of worsening renal failure?

After the angiography and angioplasty, he develops worsening renal failure

anyway. There is bluish discoloration of toes and livedo reticularis but no skin
necrosis. Pulses intact. There are white cells in urine found to be eosinophils.
Tests show eosinophilia, leukocytosis, hypocomplementemia, and elevated ESR
(erythrocyte sedimentation rate).

2. Diagnosis?

3. Specific test?

4. Therapy?

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5. Which acute renal failure (kidney injury) is the fastest?

A. Contrast
B. Allergic
C. Gentamicin

Answer:

Summary: Case 4
Note It is very common for patients who need angiographic procedures to have moderate degrees
of renal insufficiency. When radiocontrast causes renal failure, it usually starts to do so within
Fleet’s Phosphosoda Enema 12 hours after the administration of the contrast. It is associated with a low FeNa (<1%) and
causes acute renal failure. resembles prerenal azotemia. The case in question concerns what can be done to prevent
contrast-induced renal insufficiency. Evidence is accumulating that hydration with 1–2 liters
of intravenous normal saline can prevent renal insufficiency. In addition, bicarbonate and
N-acetylcysteine may also help prevent renal insufficiency from nephrotoxic contrast agents.
If cholesterol emboli develop anyway, there is no specific therapy to reverse the renal insuf-
ficiency that develops. Clues to the presence of cholesterol emboli are the development of
eosinophilia, leukocytosis, low complement levels, and an elevation of the sedimentation rate.
The cholesterol emboli also lead to bluish discoloration of the toes. The most specific test,
although rarely performed, is a biopsy of either the skin lesions or the kidney. Steroids and
anticoagulation are the most common wrong answers.

Casts Significance
Hyaline Dehydration; these casts develop as an accumulation of the
normal amount of tubular protein; they do not necessarily
mean disease
Red cell Glomerulonephritis
Broad, waxy Chronic renal failure
Granular Also called “dirty” or “muddy”; they are associated with acute
tubular necrosis and represent accumulated epithelial cells
White cell Pyelonephritis, interstitial nephritis

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Case 5
34-year-old woman with sickle cell disease has increased pain. She is taking a Note
markedly increased number of Percodan® tablets (aspirin/oxycodone). She has
Radionuclide scan can tell if
sudden onset of flank pain, fever, chills, and hematuria. Also passing strange
kidney is viable.
necrotic material in urine. Urinalysis shows red cells and white cells but no casts.
BUN 32, creatinine 3.5.

1. Diagnosis?

2. Most accurate test?

A. Urinalysis
B. CT scan
C. Intravenous pyelogram
D. BUN, creatinine
E. Kidney biopsy

Answer:

3. Therapy?

Summary: Case 5
Analgesics are destructive to the kidney in several ways. They are directly toxic to the kidney
tubule and can result in acute tubular necrosis. In addition, they can give allergic interstitial
nephritis. They can result in papillary necrosis, particularly in those who have sickle-cell
disease or are diabetic. Finally, the inhibition of prostaglandins at the afferent arteriole
results in decreased renal perfusion. Clues to the occurrence of acute papillary necrosis are
the sudden onset of flank pain, fever, chills, and hematuria. The most specific finding is
passing pieces of necrotic material in the urine. The most specific test for this disorder is
CT scan. The intravenous pyelogram (IVP) is no longer done. CT scan shows just as much
information or more than IVP with no contrast exposure. There is no specific therapy to
reverse the necrosis.

Which one does NOT cause papillary necrosis?

A. Analgesics
B. Sickle trait
C. Pyelonephritis
D. Diabetes
E. Aminoglycosides

Answer:

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END STAGE RENAL DISEASE (ESRD)

Note All patients should be on:

Avoid Aluminum and • Erythropoietin

Magnesium Containing • Vitamin D
antacids in ESRD. • Calcium
• Oral phosphate binders
º  Calcium carbonate
º Lanthanum
º Sevelamer

1. The most common cause of death is:

A. Infection
B. Acidosis
C. Coronary disease

Answer:

2. Patient on dialysis has fever. What to do?

A. Nothing. It is expected
B. Draw blood cultures, treat if positive
C. Draw cultures and load with Vancomycin and gentamicin

Answer:

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GLOMERULAR DISEASE

Case 1
50-year-old man comes because of fatigue, shortness of breath, and edema.
Uses no medications and denies hypertension or diabetes. Afebrile bilateral lower
extremity edema. BUN and creatinine mildly elevated at 34 and 2.8. Red blood
cells in urine as well as red blood cell casts.

If THIS is in . . . Then THIS is THIS is the best THIS is the best

the history and the most likely initial test and most initial therapy
physical . . . diagnosis specific test(s)
Pulmonary AND Goodpasture Anti-basement Plasmapheresis
renal symptoms, syndrome membrane with steroids or
dyspnea antibodies cyclophosphamide

Biopsy
Wegener C-ANCA

Biopsy

Polyarteritis
nodosa

Recent viral Berger disease (IgA Normal complement If proteinuria, use

infection (most nephropathy) level ACE inhibitors;
common), fish oil minimally
recurrent episodes Biopsy; skin and effective
of hematuria kidney show IgA

Gastrointestinal
symptoms,
joint pain, renal
problems, skin
lesions
Hypertension,
periorbital edema,
1 week after throat
infection, 2 weeks
after skin infection
Cryoglobulinemia

Anything from SLE (systemic Low complement

normal to lupus erythemato-
nephrotic syn- sus) 24-hour urine
drome
Biopsy
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1. Which disorders in the table cause rapidly progressive glomerulonephritis?

2. Which disorders cause nephrotic syndrome?

3. Woman with edema of legs and face. Takes atenolol, procainamide, and naproxen. BP
146/86 mm Hg, BUN 86 mg/dL, creatinine 6.0, serum albumin 2.5, and ANA is positive at
1:60 with a speckled pattern. Urine shows 4+ protein and oval fat bodies. 24-hour protein
shows 5.6 grams of protein. The complement levels and anti-double stranded DNA are
normal. Diagnosis?
A. Procainamide-induced lupus nephritis
B. Naproxen-induced nephrotic syndrome
C. Amyloidosis
D. Membranous nephropathy

Answer:

4. Patient with recurrent hematuria and:

•  Displaced Lens of Eye
•  Sensorineural hearing loss
•  Family history

Diagnosis?

Summary: Case 1
Glomerulonephritis of all types have several things in common. They all present with
hematuria and red cell casts. They also have mild proteinuria, <2 grams per 24 hours (urine
protein:creatinine ratio <2). Beyond this, the diagnosis is based on what unique features
there are to each disease, such as pulmonary involvement in Goodpasture syndrome or both
upper and lower respiratory involvement in Wegener granulomatosis. Wegener is systemic
vasculitis that affects the joints, eyes, GI tract, skin, and brain just like polyarteritis nodosa
(PAN) does. The main difference is that PAN does not involve the lungs. Henoch-Schönlein
purpura is relatively unique in that it is the only one that gives GI, joint, and renal involve-
ment with purpuric skin lesions, particularly on the buttocks and lower extremities. Berger
disease is diagnosed by excluding the other causes of glomerulonephritis with blood tests and
then performing a renal biopsy. The only frequently found clue to the diagnosis of Berger on
a board test is the history of a recent viral infection followed by hematuria. Although the IgA
level can be elevated in Berger, this is found in only 50% of patients and therefore is relatively
insensitive. The absence of anti–basement membrane antibodies helps exclude Goodpasture.
C-ANCA should be found in Wegener. Post-streptococcal glomerulonephritis should follow
a pharyngeal or skin strep infection by 1–3 weeks. In addition, there are very good blood
tests, such as anti-streptolysin O, low C3 levels, and anti-DNAse, to help diagnose post-
streptococcal disease. Cryoglobulinemia is suggested by the presence of chronic hepatitis B
or C infection and the presence of cryoglobulins on blood testing. The most accurate test for
all forms of glomerulonephritis is a kidney biopsy. The biopsy should be performed in all
forms of this disorder except for post-streptococcal disease and Henoch-Schönlein purpura.
These diseases resolve spontaneously >95% of the time, and a renal biopsy is not necessary.

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Goodpasture is treated with plasmapheresis and steroids. PAN, Wegener, lupus nephritis, and
cryoglobulinemia are all treated with a combination of steroids and cyclophosphamide. One
exception to this is cryoglobulinemia secondary to chronic hepatitis in which the best initial
therapy is to treat the infection with interferon or lamivudine (for hepatitis B) or to combine
the interferon with ribavirin (for chronic hepatitis C). All forms of glomerulonephritis can
lead to nephrotic syndrome if there is sufficient damage to the kidney.

Drug-induced lupus, such as that from procainamide, usually does NOT affect the kidneys
or central nervous system. Drug-induced lupus does not result in a positive double-stranded
DNA antibody or lower the complement levels.

Low Serum Complement Normal Serum Complement

• Post streptococcal • IgA Nephropathy (Berger disease)
• SLE • Goodpasture syndrome
• Cryoglobulinemia • Wegener granulomatosis
• Idiopathic membranoproliferative • Polyarteritis nodosa
• Henoch-Schönlein purpura
• TTP/HUS

1. Nephrotic syndrome, large tongue, dysphagia, and dysarthria. Diagnosis?

A. Myxedema
B. Angioedema
C. Acromegaly
D. Amyloidosis

Answer:

2. Associated with positive rheumatoid factor most?

A. Post-streptococcal
B. Cryoglobulinemia
C. IgA nephropathy
D. SLE
E. Polyarteritis nodosa

Answer:

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Case 2
1. 20-year-old college football player is sent to you because of fever developing in the
second week of his 2-week football training camp in Florida. Normal exam. Urinalysis
shows 1+ proteinuria. Most appropriate management?
A. No further evaluation is needed.
B. Repeat the sample again in 2–3 weeks
C. Urinary protein-to-creatinine ratio on a spot or random urine
D. Split the urine measurement with a 16-hour daytime sample and a nighttime sample
E. 24-hour urine collection
F. Renal biopsy

Answer:

2. 27-year-old man for pre-employment physical exam. Healthy. Uses no medications.

Urinalysis shows 1+ proteinuria. Follow-up measurements 2 and 3 weeks later show no
protein. Most appropriate management?
A. No further evaluation is needed.
B. Repeat the sample again in 2–3 weeks
C. Urinary protein-to-creatinine ratio on a spot or random urine
D. Split the urine measurement with a 16-hour daytime sample and a nighttime sample
E. 24-hour urine collection
F. Renal biopsy

Answer:

3. 27-year-old man has mild proteinuria on several urinalyses over several months. Works
as a waiter. Asymptomatic. No past medical history. Most appropriate management?
A. No further evaluation is needed.
B. Repeat the sample again in 2–3 weeks
C. Urinary protein-to-creatinine ratio on a spot or random urine
D. Split the urine measurement with a 16-hour daytime sample and a nighttime sample
E. 24-hour urine collection
F. Renal biopsy

Answer:

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Summary: Case 2
Transient proteinuria is found in 1–10% of the general population. If it resolves on subse- Note
quent urine samples, then no further evaluation is necessary. A transient functional protein-
uria can also occur with transient stressors, such as vigorous exercise, fever, sleep apnea, and NSAIDs cause proteinuria.
with congestive failure. This proteinuria resolves when the stress resolves. Orthostatic pro-
teinuria is found in generally healthy patients under the age of 30. The diagnosis is confirmed
by finding the proteinuria only during the daytime while a nighttime sample is normal. If the
first morning urine is normal, then it is most likely orthostatic in nature. If the proteinuria
persists, then further evaluation is necessary. A spot urine for the protein:creatinine ratio is
a useful approximation of the amount of protein found in a 24-hour urine collection. If the
ratio is 1, then you find 1 gram over 24 hours, etc. If there truly is an elevated protein level
that persists, then a renal biopsy should be performed.

1. Woman with 2 weeks of cough, hemoptysis, dyspnea, and dark-colored urine. Chest
x-ray shows infiltrates. Urinalysis shows 50–100 red cells and many red cell casts.
Complement and ANA are normal. Renal biopsy will show:
A. Granulomatous necrotizing vasculitis
B. Linear deposits of IgG by immunofluorescence
C. Granular sub-endothelial deposits of IgG
D. IgA deposits in the mesangium

Answer:

2. Muscle cramps and weakness. BP 110/70. BUN and creatinine normal. Potassium
2.6 mEq/L (normal 3.5–5.4); bicarbonate 32 mEq/L; urine sodium 50 mEq/L; urine
potassium 60 meq/L. Renin and aldosterone levels are elevated. Diagnosis?
A. Renin-producing kidney tumor
B. Primary hyperaldosteronism
C. Ectopic ACTH production
D. Bartter syndrome

Answer:

Goodpasture syndrome consists of renal and pulmonary involvement. Biopsy will show lin-
ear deposits of IgG. Bartter syndrome will give an elevated level of both renin and aldosterone
in response to increased urinary loss of sodium and chloride. Bartter syndrome is a form of
secondary hyperaldosteronism, therefore the blood pressure will be normal or slightly low.

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Case 3
Note
63-year-old woman has lower extremity edema. History of lymphoma. Takes no
Urgent dialysis medications. Generalized edema that is much worse in the lower extremities.
• Hyperkalemia Normal BUN and creatinine, albumin 2.6 (normal 3.5–5.5), cholesterol of 285
• Encephalopathy (elevated). Urinalysis shows 4+ proteinuria.
• Fluid Overload
1. Diagnosis?

2. What caused her problem?

3. What test next?

4. Most accurate test?

A. Urinalysis
B. Kidney biopsy
C. 24-hour urine
D. Protein : creatinine ratio
E. Nuclear renal flow scan

Answer:

5. Initial therapy?

Summary: Case 3
Nephrotic syndrome refers to a syndrome of hyperproteinuria leading to hypoproteinemia
leading to edema. Hyperlipidemia is caused by the urinary loss of the lipoprotein signals
on lipids that would normally signal the body to remove them from circulation as well as to
inhibit production. Although not part of the diagnostic criteria, a hypercoagulable state occurs
secondary to the urinary loss of antithrombin III. Any of the causes of glomerulonephritis can
potentially lead to nephrotic syndrome, if the severity is sufficient to lead to massive protein-
uria. In other words, nephrotic syndrome refers to a severity of disease rather than a specific
etiology. In addition to the causes of glomerulonephritis, a number of systemic diseases not
primarily referable to the kidney also result in nephrotic syndrome. These are diabetes, HIV,
hepatitis, heroin use, amyloidosis, and cancer, such as lymphoma. Nephrotic syndrome can
also be caused by a number of drugs, such as ACE inhibitors, gold, and penicillamine use.
Finally, there are several primary kidney diseases, such as minimal change disease in children,
focal-segmental disease, mesangial, and membranous disease, that can occur without a specific
relationship to a specific systemic disease. They are all diagnosed initially with a urinalysis,
24-hour urine, and, most accurately, a kidney biopsy. They are all treated with steroids, some-
times in combination with cyclophosphamide, if the steroids are not sufficient.

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1. BP 150/90. History of injection drug use. Urinalysis has hematuria as well as 4+ pro-
teinuria. 24-hour urine 4 grams protein. Etiology?
A. Focal segmental glomerulosclerosis
B. Minimal change disease
C. Hypertensive nephropathy
D. Membranous glomerulonephropathy

Answer:

2. 24-year-old Asian man evaluated for progressive hematuria over several weeks.
Hematuria began few days after upper respiratory tract infection. BUN and creatinine
normal. Urinalysis shows 2+ proteinuria and some blood. Serum complement is
normal.
A. Post-streptococcal glomerulonephritis
B. Berger disease (IgA nephropathy)
C. Goodpasture syndrome
D. Wegener granulomatosis

Answer:

3. Man undergoes a renal biopsy for glomerulonephritis with linear deposits. Most likely
diagnosis?
A. Wegener granulomatosis
B. Goodpasture syndrome
C. Post-streptococcal glomerulonephritis
D. SLE

Answer:

4. Why does nephrotic syndrome lead to bone lose and iron deficiency anemia?
A. Malabsorption
B. Liver dysfunction
C. Urine loss of carrier protein

Answer:

5. Fish oil may be good for:

A. Focal segmental
B. Minimal change
C. IgA

Answer:

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Case 4
32-year-old man comes for evaluation of generalized edema that is worse in lower
extremities. UA: 4+ protein; urine protein:creatinine ratio is 5 to 1; serum albumin
level decreased. He was diagnosed with HIV several years ago, and he used indinavir
in past. Currently, takes no medications. CD4 count 28.

1. Most effective therapy?

A. Steroids
B. Cyclophosphamide
C. Azathioprine
D. Trimethoprim/sulfamethoxazole
E. Antiretroviral therapy
F. ACE inhibitors

Answer:

Summary: Case 4
Although indinavir can cause kidney stones and renal insufficiency, it is generally a cause of
kidney stones in only 4% of the patients who use it. Indinavir is not generally associated with
glomerulonephritis. HIV itself causes nephrotic syndrome. HIV-induced glomerulonephritis
is treated with antiretrovirals. As the HIV is treated and the CD4 count rises above 100, the
glomerulonephritis will resolve.

1. 72-year-old man evaluated for fatigue, anemia, bone pain, lytic lesions, and frequent
infections. Creatinine 2.4 urinalysis shows 1+ protein. 24-hour urine, however, shows
5 grams of protein. What is the most likely explanation for this finding?
A. False-positive 24-hour urine; do nothing
B. Repeat the 24-hour urine
C. Perform a kidney biopsy
D. Light chains react weakly with the dipstick; do a urine immunoelectrophoresis
E. Post-infectious glomerulonephritis; perform an ASLO titer

Answer:

2. 64-year-old chronically uremic patient with chest pain and fever. Exam reveals pericardial
friction rub and clear lungs. What’s next?
A. Intravenous fluids
B. Intravenous furosemide
C. Pericardiocentesis
D. Immediate hemodialysis

Answer:

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3. Patient on hemodialysis for end-stage renal disease on calcium acetate every morning
to treat high phosphate without improvement. Next step?
A. Calcium acetate with meals
B. Parathyroidectomy
C. Switch to calcium carbonate every morning

Answer:

4. Mechanism of action of ACE inhibitors in slowing rate of progression of diabetic

nephropathy?
A. Decrease in afferent arteriolar pressure
B. Increase in glomerular filtration pressure
C. Decrease in efferent arteriolar pressure

Answer:

5. Patient started on ACE inhibitor. Creatinine rises from 1.2 to 1.5

A. Stop
B. Continue

Answer:

6. 22-year-old man with sudden severe testicular pain. Minimal redness and the affected
testis is elevated and lying in a transverse direction. Diagnosis?
A. Orchitis
B. Epididymitis
C. Hydrocele
D. Testicular torsion

Answer:

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Acute uremic pericarditis is an indication for urgent dialysis. If once daily phosphate bind-
ers are insufficient, they should be used multiple times a day with meals. Calcium carbonate
has no greater efficacy than calcium acetate. ACE inhibitors dilate the efferent arteriole and
decrease intraglomerular hypertension. Although there is a temporary decrease in GFR, the
overall effect is to decrease the rate of progression of diabetic renal disease. Testicular torsion
causes sudden severe pain with elevation of the testis. The normal position of the testis is now
changed to the transverse direction.

Chronic Renal Failure

Dialysis is used if there is a form of life-threatening abnormality that cannot be corrected another way:
• Pericarditis
• Severe acidosis
• Fluid overload
• Encephalopathy
• Hyperkalemia
Bone abnormalities can be treated with calcium and vitamin D replacement. Calcium carbonate
and phosphate binders are given with meals to decrease phosphate absorption. The most common
cause of death in end-stage renal disease is from infection and from cardiovascular disease from
accelerated atherosclerotic disease. Renal transplantation is always better than long-term dialysis
because of an increased survival.

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SODIUM DISORDERS

Case 1
27-year-old man with bipolar disorder for evaluation of polyuria. He is on lithium.
He has been urinating 10–20 times per day. He just bought 4 CD players, 6 toaster
ovens, and 5 TVs. His psychiatrist says he drinks 15 liters per day and he urinates
15 liters per day. Sodium level 140.

1. Diagnosis?

2. Which will have low urine osmolality: psychogenic polydipsia or nephrogenic

diabetes insipidus from the lithium?

3. Which will have low urine sodium: psychogenic polydipsia or nephrogenic diabetes
insipidus from the lithium?

4. What would give the first clue as to diagnosis?

A. Polyuria
B. Polydipsia
C. Low urine sodium
D. Low urine osmolality
E. Nocturia

Answer:

5. Best initial diagnostic test?

6. Most sensitive test to diagnose nephrogenic diabetes insipidus?

7. Treatment for nephrogenic diabetes insipidus?

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Summary: Case 1
Both psychogenic polydipsia and diabetes insipidus can cause polyuria, and by itself a high
volume of urine is not enough to distinguish the two. If the serum sodium were low, then
the diagnosis would be psychogenic polydipsia. If the sodium were high, then it would be
nephrogenic diabetes insipidus (NDI). The point of this case is that when there is an exactly
normal sodium, you need to rely on different diagnostic criteria to establish an etiology. In
diabetes insipidus, the thirst mechanism is intact; and as long as oral intake is adequate, the
sodium level will not rise. Both NDI and polydipsia will result in a low urine osmolality and
low urine sodium. These tests cannot reliably be used to establish the diagnosis. The most
common wrong answer to what is the most reliable diagnostic test is a water deprivation test.
If the patient on history says he only urinates once or twice at night, then the diagnosis is
psychogenic polydipsia. When the patient stops drinking, he will stop urinating. In NDI or
central DI (CDI), the patient will continue to produce a high volume of urine even at night,
because the level of urination is not dependent on water intake, which is exactly the nature
of the problem. Water deprivation is still the best initial test. Looking for a decreased urine
output as a response to the administration of vasopressin (DDAVP) is the way to distinguish
between a central and nephrogenic cause of the DI. The most sensitive test, however, is to
look for an elevated level of vasopressin (ADH) in NDI and a low ADH level in central DI.
The treatment of CDI is to give vasopressin. The management of NDI is to correct the under-
lying cause by stopping lithium or demeclocycline or to correct causes, such as hypokalemia
or hypercalcemia. If these drugs are not the cause and there are no correctable electrolyte
abnormalities, then the treatment is to use a thiazide diuretic or an NSAID, such as indo-
methacin. The inhibition of prostaglandins increases renal concentrating ability. Sickle-cell
disease, amyloidosis, and Sjögren syndrome are examples of diseases in which you cannot
correct the underlying cause.

Note 1. 39-year-old woman is brought to ED for confusion of one day’s duration. She begins
seizing. Sodium 112 (normal 135–145), potassium 4.1, BUN 8 mg/dL, plasma osmo-
Every 100 glucose above lality 230 mOsm/l (normal 280–300), urine sodium10 mEq/L, and urine osmolality
normal is 1.6 drop in Na. 50 mOsm/l. Urine output is 2 liters in 3 hours. Diagnosis?
A. Addison disease
B. SIADH
C. Psychogenic polydipsia
D. Salt-losing nephropathy
E. Diabetes insipidus

Answer:

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Case 2
Woman admitted to ICU because of very severe neurologic abnormalities and
profoundly low sodium at 114. She has not had a seizure yet. History of type 2
diabetes and congestive heart failure. Maintained on ACE inhibitor and diuretic.
Pulse 125/min, BP 98/68 mm Hg, severe confusion, and disorientation. Sodium
114, serum bicarbonate 24, chloride 112, BUN 80, potassium 5.0, glucose 1,750,
calcium 10.2, and magnesium normal. Chest x-ray normal.

1. Diagnosis?
Non-ketotic hyperosmolar syndrome

2. Best initial therapy?

Several liters of normal saline and insulin

3. What is her serum osmolality?

Serum osmolality:
2 × (Na) + BUN/2.8 + glucose/18
228 + 80/2.8 + 1,750/18
228 + 32 + 97 = 357 (normal 280–295)

Summary: Case 2
This patient is hyperosmolar but has a normal serum bicarbonate and therefore is not ketotic
or acidotic. Although the sodium level is profoundly low at 114, it is a pseudohyponatremia
from the extreme hyperglycemia. For every 100 increase in glucose above normal, there is a
1.6-point drop in the sodium level. This person’s glucose is 1,600 above normal; therefore,
the corrected sodium level is actually 140 once it is corrected for the hyperglycemia. She is
hyperosmolar. The equation is:

(2 × sodium) + (BUN/2.8) + (glucose/18) = 357

The management is to correct the massive volume depletion with normal saline and to give
a little insulin.

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Case 3
82-year-old woman with colon cancer with increasingly severe confusion over
2 days. She has metastases to brain and lung. She is profoundly confused and
disoriented. No seizures and is not comatose. On exam, there is no orthostasis.
No edema. Sodium 114, potassium 4.5, glucose 80, uric acid low at 2.2, and a
BUN of 12.

1. Diagnosis?

2. What else in brain can cause this problem?

3. What else in lung can cause this problem?

4. Best initial test?

5. Best initial therapy?

6. Best long-term management?

A. Furosemide
B. Carbamazepine
C. Tolvaptan
D. 3% sodium chloride with furosemide
E. Salty potato chips

Answer:

7. Patient with sodium 112 and confusion is given hypertonic saline, diuretics and
conivaptan. Sodium rises to 128 over 3 hours. Patient develops cranial nerve defects and
quadriparesis. Diagnosis?

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Summary: Case 3
Hyponatremia with a normal volume status is usually from SIADH, if the serum sodium
level is truly low and not a pseudohyponatremia from hyperglycemia or hyperlipidemia.
Other causes of euvolemic hyponatremia are psychogenic polydipsia and hypothyroidism,
which do not seem in any way to be present from the details presented in the case. Any brain
or pulmonary pathology can lead to SIADH. The normal response to hyponatremia would
be to have low urine sodium and low urine osmolality. It is a sign of disease to have a urine
sodium >20 or a urine osmolality >100 in the presence of hyponatremia. Mild cases of
SIADH with very mild symptoms or no symptoms are managed with fluid restriction. More
severe symptoms, such as confusion, disorientation, and seizures, are managed with hyper-
tonic saline and sometimes with additional saline combined with a diuretic. Diuretics lead to
a net-free water loss. In the long-term, when you cannot correct the underlying cause of the
SIADH, the treatment is tolvaptan. Demeclocycline is a tetracycline antibiotic that induces
a nephrogenic diabetes insipidus and therefore increases urinary-free water loss. Tolvaptan
is an ADH, V2-receptor antagonist that has replaced demeclocycline as the best long-term
therapy for SIADH.

Case 4
42-year-old man with sickle-cell disease is admitted to ICU for head trauma. He
continues to be disoriented because of a persistently high sodium of 160. He is
producing 10–15 liters per day of urine. Glucose is normal. He weighs 100 kg.
No response in urine volume or osmolality to intranasal or subcutaneous
administration of vasopressin.

1. Diagnosis?

2. What is the estimated volume deficit?

[(Na/140)−1] × (weight × 0.6) or (Patient Na − 140/140) × (weight × 0.6)
160/140 = 1.14 or (160 − 140/140) × 60 = 8.57 liters
1.14 − 1.00 = 0.14
0.14 × 60 = 8.57

3. What electrolytes would you measure?

4. These electrolytes are normal, and you cannot find a correctable underlying cause.
What is the therapy?

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Summary: Case 4
Nephrogenic diabetes insipidus is caused by sickle-cell disease as well as hypokalemia, hyper-
calcemia, and amyloidosis. The fluid deficit is calculated by:

(patient sodium − 140/140) × (weight in Kg × 0.6)

This formula is useful because the physician will often underestimate the fluid deficit and give
far too little saline replacement. If you cannot correct the underlying cause, then the treat-
ment is with thiazide diuretics or NSAIDs (nonsteroidal anti-inflammatory drugs).

NSAID EFFECTS
1. Which of the following describes the effects of NSAIDs on the kidney?
A. Prostaglandin inhibition dilates the efferent arteriole
B. Increases renin release
C. Increases aldosterone production
D. Constriction of afferent arteriole

Answer:

2. NSAIDs induce all of the following EXCEPT:

A. Increased hypertension
B. Acute tubular necrosis
C. Interstitial nephritis
D. Nephrotic syndrome
E. Hypokalemia

Answer:

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POTASSIUM DISORDERS

Case 1
High-school wrestler brought to ED by family because of muscular pain, weakness
as well as polyuria and polydipsia. He tried a week’s worth of cathartics but didn’t
lose much weight. He induced vomiting over the last several days and used inhaled
beta agonist 14 times a day for exercise-induced asthma. His potassium is now 2.4.

1. What caused hypokalemia?

Diarrhea at first. Then vomiting. The inhaled beta agonists then drove his potassium into
the cells.

2. What will urine potassium be?

Very low: <20 mEq/L

3. Most urgent test and what will it show? (How will he die now?)

4. Maximum rate of oral administration of potassium?

5. Maximum rate of intravenous administration of potassium?

6. What intravenous fluids to avoid in hypokalemia?

A. Normal saline
B. D5W
C. Half normal saline
D. Ringer’s lactate

Answer:

7. How much potassium will he need to raise his level to a normal value of 4.5 from 2.4?

8. After several hours of vigorous replacement, the potassium level rises by only 0.2. What
will you test for?

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Summary: Case 1
Most causes of the loss of gastrointestinal fluids result in hypokalemia. Diarrhea and vomit-
ing both cause hypokalemia with a low urine potassium. The manifestations of hypokalemia
are muscular weakness, cardiac rhythm disturbances, and sometimes a nephrogenic diabetes
insipidus. The earliest electrocardiographic finding is u waves. U waves are an extra positive
deflection after the t wave that indicates Purkinje fiber repolarization. There is generally no
limit to the maximum rate of the oral replacement of potassium because the bowel wall will
regulate potassium absorption so that there will be no rebound hyperkalemia. You should
not use dextrose-containing fluids in patients with hypokalemia, because there may be an
increase in insulin secretion stimulated by the additional sugar. This insulin release can
intracellularly drive potassium and further lower the potassium level. Do not give potassium
faster than 20 mEq per hour intravenously, because it may lead to an arrhythmia. Because the
majority of potassium is intracellular, you must give 4–5 mEq per kilogram of body weight in
order to raise the serum potassium by one point. Most of what you administer will enter the
cells. Hypokalemia that is refractory to potassium replacement is sometimes from hypomag-
nesaemia. There are magnesium-dependent channels in the kidney that will give an increased
urinary wastage of potassium, if the magnesium level is low.

Case 2
Man recently diagnosed with stage 3A Hodgkin disease has just received his first
dose of ABVD (adriamycin, bleomycin, vinblastine, dacarbazine) chemotherapy.
He has developed muscular weakness and pain. Potassium level has risen to 6.7.

1. Diagnosis?

2. Most urgent step?

A. EKG
B. Urinalysis
C. Phosphorous level
D. Calcium level
E. Magnesium
F. Uric acid level

Answer:

3. Best initial step if this is abnormal?

Summary: Case 2
Other causes of hyperkalemia besides cellular destruction, such as rhabdomyolysis, are drugs,
such as ACE inhibitors, beta blockers, heparin, trimethoprim, and NSAIDs. The most com-
mon factitious causes of hyperkalemia are thrombocytosis, the placement of a tourniquet for
a long time, and hemolysis in a blood sample left standing for too long or drawn through too
narrow a needle, resulting in hemolysis. When there are EKG abnormalities from hyperka-
lemia, the best initial step is to administer calcium chloride or gluconate in order to protect
the heart from an arrhythmia.
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PHOSPHATE DISORDERS
1. Which causes LOW phosphate levels?
A. Hypoparathyroidism
B. Vitamin D overdose
C. DKA
D. Starvation
E. Fanconi Syndrome

Answer:

2. Which causes HIGH phosphate levels?

A. Hyperparathyroidism
B. Celiac disease
C. Chronic pancreatitis
D. Alcoholism
E. Renal failure

Answer:

Hypomagnesemia
• Starvation
• Diuretics/Renal Loss
• Amphotericin

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METABOLIC ACIDOSIS

Case 1
Three men are brought to ED completely drunk. They have been at a wild party
in the garage of a photocopy repairman. All three show signs of inebriation. The
chemistries show a serum bicarbonate of 14 (normal 24), sodium of 137, chloride
of 98, and glucose of 240.

1. How will you best determine to test for an ethanol level, a methanol level, or an ethylene
glycol level?

Note
2. Which will have metabolic acidosis?
Compensation Respiratory
Acidosis
↓ pCO2 10 = ↑ HCO3–
3. Which will give an elevated anion gap of 25?
1 Acute
3.5 Chronic
4. Best initial test for ethylene glycol intoxication? Methanol?
Note

Respiratory Alkalosis
5. Best initial therapy for methanol intoxication? Ethylene glycol? Ethanol?
↓ pCO2 10 = ↑ HCO3—

2 Acute
5 Chronic
6. What makes you think of an overdose of isopropyl alcohol?

Summary: Case 1
Metabolic acidosis is divided into those that increase the anion gap and those with a normal
anion gap. The anion gap is calculated by sodium – (bicarbonate + chloride). Normally, the
anion gap should be <12. The two main causes of a metabolic acidosis with a normal anion
gap are renal tubular acidosis and diarrhea. An elevated anion gap with metabolic acidosis
is caused by “LA MUDPIE”. “L” is for lactic acid, which occurs with any type of anaerobic
metabolism, such as hypotension or sepsis. “A” is for aspirin. Look for hyperventilation and
a respiratory alkalosis. “M” is methanol. “U” is for uremia. The creatinine should be signifi-
cantly elevated to a level >3 to result in acidosis. “D” is for diabetic ketoacidosis. No matter
what the glucose is, you should have an elevated acetone level in the blood. Acetone in the
urine is not particularly significant and can occur with even minor degrees of starvation,
such as skipping 1 or 2 meals. “P” is for paraldehyde, which is an old pediatric anti-seizure
medication. “I” is for isoniazid, the antituberculosis medication. “E” is for ethylene glycol,
which is particularly common with intentional overdoses of antifreeze and the development
of hypocalcemia, and renal insufficiency from stone formation and acute tubular necrosis.
A low anion gap is seen in those with myeloma.

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RENAL TUBULAR ACIDOSIS (RTA)

Proximal RTA (II) Distal RTA (I) Type IV RTA

Case 1
Man with history of Fanconi syndrome and amyloidosis from myeloma presents
with osteomalacia. Potassium 3.2, serum bicarbonate 18. Chloride elevated at 110;
sodium normal at 138. Urine pH 5.2.

1. Diagnosis?
A. Proximal RTA (Type II)
B. Distal RTA (Type I)
C. Type IV RTA

Answer:

2. What is the confirmatory test?

3. Best therapy?

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Case 2
Note
A woman is on amphotericin for fungal endocarditis. Her creatinine is 1.8. The
Early renal failure does not Potassium is low at 3.0; chloride is 115; sodium is 136; and serum bicarbonate is
increase anion gap. 12. She has developed some nonobstructive kidney stones. Her urine pH is 6.7.

1. Diagnosis?
A. Proximal RTA (Type II)
B. Distal RTA (Type I)
C. Type IV RTA

Answer:

2. Confirmatory test?

3. Therapy?

Case 3
Diabetic man with well controlled glucose has normal anion gap metabolic
acidosis. Serum bicarbonate 20; potassium is elevated at 5.8.

1. Diagnosis?
A. Proximal RTA (Type II)
B. Distal RTA (Type I)
C. Type IV RTA

Answer:

2. Diagnostic test?

3. Therapy?

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Summary: Cases 1–3

All forms of RTA result in a metabolic acidosis with a normal anion gap. Type IV RTA is
usually associated with diabetes. There is a hyporeninemic hypoaldosteronism. Because there
is a decrease in aldosterone effect, there is an elevation in potassium and the retention of
hydrogen ions. Type IV RTA is the only one that gives hyperkalemia. The treatment is with
aldosterone replacement in the form of fludrocortisone. Type I distal RTA results from the
decreased ability of the kidney to excrete hydrogen ions or acid into the kidney tubule and
hence remove it from the body. This results in a basic urine with a pH above 5.5. In this basic
urine, the patient will form stones and nephrocalcinosis. The test is to administer acid in the
form of ammonium chloride. A normal person will excrete the extra hydrogen ions into the
urine. Patients with type I RTA cannot excrete hydrogen ions into the urine, and therefore
the urine stays basic (pH >5.5) despite a worsening acidosis. Because the proximal tubule
is intact in type I RTA, the treatment is with oral bicarbonate. Bicarbonate is absorbed pri-
marily in the proximal tubule and therefore will be successfully absorbed in a distal tubular
problem. In a type II, or proximal RTA, the kidney cannot absorb bicarbonate in the proximal
tubule. The urine pH is alkalotic at the beginning. When the body has become depleted of
bicarbonate, then the urine pH becomes acidic (pH <5.5). In an acidic urine, only cysteine
stones will form. Most calcium-containing stones will not form, and therefore type II RTA is
not associated with kidney stones. Because the proximal tubule is not working, it is difficult
to treat with bicarbonate. The best therapy is to administer a diuretic. The volume depletion
causes a volume contraction alkalosis.

Compensation for Respiratory Disorders

Respiratory Acidosis: For every 10 increase in pCO2, bicarbonate will rise 1 point acutely,
3.5 points chronically

Respiratory Alkalosis: For every 10 decrease in pCO2, bicarbonate will decrease 2 points
acutely, 5 points chronically

1. Which is found in multiple myeloma?

A. Distal RTA
B. Low anion gap
C. High parathyroid hormone level
D. Low urine calcium

Answer:

2. Patient with Na 132, K 3.2, Cl 110, HCO3– 18

Only one of these is NOT likely to have these numbers:
A. Diarrhea
B. Ureterosigmoidoscopy
C. RTA
D. Early Renal Failure
E. Chronic Renal Failure

Answer:

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METABOLIC ALKALOSIS

Case 1
Depressed obese woman comes because of increasing fatigue and diarrhea. She
has nothing to do all day except work in her candy store and eat licorice. She has
worsening hypertension despite use of thiazide diuretic. BP is 144/92. Potassium
3.0. Bicarbonate 32. Urine chloride of 42 (elevated). Urinary potassium low at 24.

1. Most likely cause of ?

2. How do you know she does not have Conn syndrome?

3. How do you know this is not Bartter syndrome?

4. Single most important lab value to determine etiology of metabolic alkalosis?

Urine Chloride.

5. Most common EKG finding in this case?

6. Treatment?

Summary: Case 1
Metabolic alkalosis is characterized by an elevated serum bicarbonate level. The main key
to the diagnosis is the urinary chloride level. A low urine chloride level (<10) is found with
upper gastrointestinal fluid loss, diuretics, villous adenomas, and posthypercapnia. A high
urine chloride (>20) is found with mineralocorticoid excess, such as in Conn syndrome or
primary hyperaldosteronism, Cushing syndrome, Bartter syndrome, and licorice ingestion.
Hypokalemia also contributes to alkalosis, because hydrogen ions move into the cells in
order to have them release potassium ions. Bartter syndrome is from the inability of the loop
of Henle to reabsorb sodium and chloride. This results in a secondary hyperaldosteronism.
Volume depletion of any kind also makes you relatively alkalotic. The kidney reabsorbs the
same amount of bicarbonate into a smaller bodily volume, raising the bicarbonate concen-
tration. In addition, there is a secondary form of hyperaldosteronism to try to correct the
volume depletion. Aldosterone makes you excrete the cations potassium and hydrogen in
exchange for reabsorbing sodium.
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TUBULAR DEFECTS

Case 2
Match the following:
A. Bartter’s Syndrome

B. Gitelman’s Syndrome

C. Liddle’s Syndrome

D. All of the above

E. None of the above

1. Ascending loop of Henle, decreased transport

2. Distal tubule, decreased transport

3. Distal tubule, ENaC increased transport

4. Hypertension

5. High urine chloride

6. Hypokalemia

Saline-Responsive (most common) Saline-Unresponsive

Normal blood pressure

Low urine chloride (<10 mEq) High urine chloride (>20 mEq)
Etiology: Etiology:

• Diuretics • Increased mineralocorticoids

• Vomiting • Conn syndrome

• Diarrhea • Bartter syndrome

• Nasogastric suction • Licorice

• Exogenous alkali • Liddle’s syndrome

• Posthypercapnia • Gitelman’s syndrome

• Volume contraction
Infuse sodium chloride, KCl Block the aldosterone; spironolactone

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ACID-BASE COMPLEX PROBLEMS

Respiratory acidosis (increased pCO2)

For every 10 increase in pCO2
º  Acutely: HCO3– rises 1 point
º  Chronicly: HCO3– rises 3.5 points
Respiratory alkalosis (decreased pCO2)
For every 10 decrease in pCO2
º  Acutely: HCO3– drops 2 points
º  Chronicly: HCO3– drops 5 points

1. Na 142, K3.3, Cl 95, HCO3– 38

BP 150/100
Renin level: low
Urine Chloride: >20
Patient most likely has:
A. Diuretic induced metabolic Alkalosis
B. Licorice ingestion
C. Bartter syndrome
D. Gitelman’s syndrome

Answer:

2. Exacerbation COPD and CHF

pCO2 70, pH 7.34, Intubated and ventilated PCO2 now 50
Furosemide, steroids, albuterol, tiotropium
Extubated and discharged on same
One week later
pH 7.46, pCO2 60, PO2 55, Bicarbonate 40
A. COPD
B. Diuretics

Answer:

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3. Patient ingests unknown substance

pH 7.18 pCO2 23, pO2 98, HCO3– 8
Serum
Na 136 Cl 100 HCO3– 10
This patient has:

A. Gapped Metabolic Acidosis

B. Non-Gapped metabolic acidosis
C. Gapped and non-gapped acidosis

Answer:

4. Na 135, CL 80, HCO3– 24, BUN/Creat 110/11

pH 7.4, pCO2 37, HCO3– 22
This patient most likely has;
A. Normal Acid Base
B. Metabolic acidosis with Metabolic alkalosis
C. Metabolic alkalosis with Respiratory alkalosis

Answer:

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NEPHROLITHIASIS

Case 1
47-year-old man comes to ED within an hour of the sudden onset of severe,
overwhelming flank pain radiating to groin and scrotal area. He has hematuria
with reddish tinge to urine but denies dysuria. He is HIV-positive maintained on
lamivudine, zidovudine, indinavir, and trimethoprim/sulfa. Temperature normal.
Tenderness. The urine pH is 6.5.

1. Best initial step?

A. Urinalysis
B. Analgesics
C. Straining the urine
D. X-ray
E. Ultrasound
F. CT scan

Answer:

2. Most accurate diagnostic test?

The following day all of his symptoms have resolved. This is his first episode of
pain. A followup ultrasound shows a 1.5-cm stone in the renal pelvis.

3. Cause of his kidney stone?

4. Most appropriate management at this time?

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Case 2
47-year-old man comes for recurrent kidney stones. No past medical history.

1. Most likely cause?

2. Parathyroid hormone level, calcium level, uric acid, creatinine, urine volume, and urine
culture are all normal. Best therapy?

3. What would be different if an abdominal x-ray failed to show the stones and the urine
pH were low?
A. Uric acid
B. Struvite
C. Calcium oxalate

Answer:

Summary: Cases 1–2

Nephrolithiasis is far more likely in men. All stones are radio-opaque except for uric acid
and cystine stones. The most common cause of calcium oxalate stones is idiopathic hyper-
calciuria. Although they form in an alkaline environment, an extremely high pH >7.5 should
raise the possibility of a struvite stone. Struvite crystals are rectangular prisms resembling
a coffin lid. This is an important distinction, since it is essential to eradicate infection with
struvite stones; and the chronic use of methenamine mandelate may be necessary for this
purpose. Hyperuricosuric patients with uric acid stones must have their urine alkalinized
with potassium citrate and be treated with allopurinol. Cystine stones should be suspected
in those presenting at a young age, such as those in their 20s. Cystine stone formers are
treated with alkalinizing the urine with potassium citrate and sometimes acetazolamide.
Type I renal tubular acidosis also presents with stone formation because of the high urine
pH. Inflammatory bowel disease and others with a malabsorption of fat present with oxalate
stones. The malabsorbed fat chelates with calcium in the gut, leading to increased oxalate
absorption and increased stone formation.

Patients with Crohn disease have fat malabsorption particularly after resection of large
amounts of the small bowel. Fat malabsorption leads to calcium malabsorption as well. The
leaves oxalate free to be absorbed in increased amounts from the gastrointestinal tract, which
is associated with the development of kidney stones.

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1. Woman with history of Crohn disease and surgical resection of terminal ileum presents
with severe left flank pain radiating to groin for 4 hours. Urinalysis shows 2+ blood; Flat
plate shows a small ureteric stone. Most likely cause of renal stone?
A. Increased excretion of calcium in urine
B. Increased oxalate absorption from gastrointestinal tract
C. Increased calcium absorption form gastrointestinal tract
D. Medication adverse effect
E. Dehydration

Answer:

2. Patient has stone 6 mm in renal pelvis. No passage after several weeks. What should be
done next?
A. Lithotripsy
B. Percutaneous removal
C. Tamsulosin and Nifedipine

Answer:

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POLYCYSTIC KIDNEY DISEASE

Case 1
32-year-old woman comes for evaluation of flank pain, hematuria, and recurrent
urinary tract infections and kidney stones. No other past medical history. Ultrasound
of kidney reveals polycystic kidneys.

1. Most common associated anatomic problem?

2. What other anatomic problems are likely to be found?

Mitral valve prolapse, diverticular disease, and circle of Willis aneurysms

3. Most common cause of death in patient like this?

4. Will you offer her a cerebral angiogram?

Summary: Case 1
Polycystic kidneys most often present with chronic hematuria and recurrent stone forma-
tion. The most common cause of serious injury and death is from end-stage renal disease.
Recurrent stone formation and infection results in chronic scarring of the kidneys, which
leads to renal failure. Polycystic kidney disease is also associated with cysts of multiple organs
of the body, with the most common site being the liver. In addition, these patients have mitral
valve prolapse, colonic diverticula, and aneurysms of the circle of Willis; however, subar-
rachnoid hemorrhage is not the most common cause of death. You do not have to perform
routine angiography in these patients, unless there is a history of subarrachnoid hemorrhage
or there is a prominent family history of hemorrhage.

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URINARY INCONTINENCE

Case 1
Note 70-year-old woman comes because of intense urinary urgency. Over last several
Sono relatives BEFORE HLA months, she has developed urinary frequency, and sudden overwhelming urge
type to donate to polycystic to urinate that she is unable to control. Unless she is near a bathroom, she has
family urinary leakage that is extremely embarrassing. Renal and pelvic ultrasound and
urinalysis are normal.

1. Diagnosis?

2. Most accurate diagnostic test?

Urodynamic studies to measure bladder pressures. Fill the bladder up with water and mea-
sure the pressures.

3. Over next few weeks, you try behavioral therapy and instruct patient to void every
1–2 hours while awake and then increase intervals between urinations by 30 minutes.
This fails as does biofeedback. What would you try next? Most common adverse effect?

4. 82-year-old, nursing-home patient with indwelling urinary catheter has cloudy urine.
There are no white cells, but culture grows >10,000 colony forming units of pseudomo-
nas. There is no fever and no recent change in the condition of the patient. Next step?
A. Repeat urinary culture
B. Observation
C. Trimethoprim/sulfamethoxozole for 3 days
D. Trimethoprim/sulfamethoxozole for 7 days

Answer:

5. Bethanechol is used for:

A. Neurogenic bladder
B. Stress incontinence
C. Urge incontinence

Answer:

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Action Diagnosis
Do residual volume, if >100 mls Bladder outlet obstruction
Do residual volume, if <100 mls, instill 250 mls If urge to void, it is urge incontinence
If there is no urge to urinate with instilling If there is no urge to urinate with 600
250 mls, then instill a total of 600 mls mls instilled, then there is impaired
bladder contraction

Case 2
Elderly woman comes because of urinary leakage. Every time she laughs or
coughs, she wets herself. Ultrasound of urinary tract is normal. You instruct her
to stand up and cough. As you bend down to look, you find you have a wet face.

1. Best initial therapy?

2. What to do therapeutically if this is not effective?

Summary: Cases 1–2

Urinary incontinence is divided into two forms. In one form, there is urge incontinence from
an overactive detrusor muscle. These patients have urinary frequency with extreme urgency.
The patients develop an overwhelming urge to urinate. This also can be associated with pain
and discomfort. The test is to measure the pressure in the bladder by filling up the bladder
with water, inserting a manometer into the bladder with a catheter, and measuring the
pressure. The treatment is initially behavior modification: asking the patient to void every
1–2 hours while awake, followed by increasing the time between urinations by 30 minutes.
If this does not work, anticholinergic medications, such as oxybutynin, imipramine, or tolt-
erodine, are used. The most common side effect of these medications is a dry mouth from
the anticholinergic activity of these medications. The other type of incontinence is stress
incontinence in which there is weakness of the muscle of the pelvic floor. When the patients
cough or laugh, intra-abdominal pressure increases. This leads to leakage of urine out of the
bladder. The best initial therapy for this form of incontinence is with pelvic floor muscular
exercises, such as Kegel exercises or estrogen cream. If this is not effective, then surgery to
tighten the urethra is indicated.

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HYPERTENSION

Case 1
47-year-old man comes for advice about smoking cessation. No current complaints
and no past medical history. He is not black, white, Asian, or Hispanic. Blood
pressure 155/92. The following week his blood pressure is 152/94. It is the same
the following week.

1. Next step in management?

Lifestyle modifications, such as exercise, sodium restriction, smoking cessation, dietary
alteration, relaxation methods, and weight loss

2. Which of these will be most effective?

3. Which has weakest data to suggest efficacy?

4. After 4 months of attempts, he comes back and says he would rather just be fat and lazy,
keep smoking, and take some pills. Best initial medical therapy?
Diuretics such as thiazide

5. What will you do in the 30% of patients who are not adequately controlled with this
regimen?
Add a beta blocker, calcium blocker, ACE inhibitor, or ARB (angiotensin receptor blocker)

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Special Circumstances
When you see THIS …Use THIS as the best
in the history… initial therapy
Diabetes ACE
Myocardial infarction Beta blocker, ACE

CHF ACE, beta blocker

Isolated systolic Thiazides
hypertension

Migraine headaches

BPH Alpha blockers

Asthma Avoid beta blockers
Depression Avoid beta blockers
Peripheral arterial disease

Osteoporosis

Pregnancy

6. In primary management of hypertension, when you should answer each of the

following.
Hydralazine:
Clonidine:
Minoxidil:
Alpha-methyldopa:

7. What antihypertensive medication is associated with the worst depression?

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Summary: Case 1
In the initial management of hypertension, the first step is to confirm that the patient is truly
hypertensive by repeating the measurement several times over a few weeks. Once the hyper-
tension is confirmed, then you should try to modify the patient’s lifestyle, if the hypertension
is mild. The most effective lifestyle modification is to lose weight. There is a 1-to-1 linear
Note reproducible relationship between weight and blood pressure. For every kilogram lost, the
blood pressure drops by 1 mm of mercury. The best initial medical therapy for hypertension
Ephedra (Ma Huang) is an is with diuretics in patients without compelling indications. Diuretics are still the best initial
herb causing high BP. therapy for isolated systolic hypertension as well. This is true only in patients who do not
have any other significant past medical history.

If there is a history of diabetes, systolic congestive heart failure, or microalbuminuria, then

the best therapy is with an ACE inhibitor. Microalbuminuria is any protein level >30 mg per
day. Microalbuminuria is detected by the radioimmunoassay or ELISA.

Those with a history of a myocardial infarction or migraine headaches should be started first
on a beta blocker and not a diuretic, because there is a greater mortality benefit. Beta blockers
should be avoided in asthma, depression, and peripheral arterial disease. Thiazide diuretics
can increase calcium reabsorption, which is useful in patients with osteoporosis. Hydralazine
and alpha-methyldopa can be safely used in pregnancy. However, any therapy except ACE
inhibitors, ARB, and diuretics can be used safely in pregnancy. Clonidine, hydralazine, and
alpha-methyldopa should not be used routinely as either first- or second-line therapy in
the routine management of hypertension. They do not have greater efficacy than the other
medications and have more adverse effects. The goal with diabetes or chronic kidney disease
is <130/80 mm Hg.

Case 2
1. Man has hypertension, history of myocardial infarction, and hyperlipidemia. First
drug to control his BP?

Case 3
1. Woman has hypertension, history of myocardial infarction, and diabetes. First drug to
control BP?

2. What is the BP goal of therapy?

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Case 4
1. Man has history of mild myocardial infarction, hypertension, and asthma.

2. First drug to control his BP?

Summary: Cases 1–4

You should expect to find questions on the Boards that require you to analyze multiple vari-
ables in the management of hypertension. Many cases will have both an indication for and
against specific medications. You should answer based on what factor indicates the greater
mortality benefit. Although hyperlipidemia and diabetes are relative contraindications to the
use of beta blockers, it is more important in patients with a history of a myocardial infarction
to use medications, such as beta blockers, that lower mortality. When the patient is diabetic,
increase therapy until the blood pressure is <130/80.

Case 5
1. 54-year-old man comes to emergency department with several hours of headache,
blurry vision, mild confusion, and dyspnea. BP is 210/130.

2. Best therapy?
Labetalol, nitroprusside, nicardipine

Case 6
1. 24-year-old woman comes because of headaches. You find blood pressure 160/105.

2. Who will you evaluate for secondary (potentially correctable) causes of hypertension?
The young (<30), the old (>60). Those with very hard to control hypertension. Those
who have something specific in the history and physical suggestive of a cause of secondary
hypertension.

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Special Circumstances—Secondary Hypertension

When you see THIS in the Then THIS is the most Treatment
history or physical… likely diagnosis
Abdominal bruits Renal artery stenosis
Muscular weakness,
hypokalemia
Striae, buffalo hump, truncal
obesity, easy bruising
Lower extremity wasting and
upper > lower BP
Episodic, flushing, wheezing,
sweating
Hirsute female

Summary: Case 6
Less than 5% of patients with hypertension will have secondary causes of hypertension. You
do not have to evaluate all the patients for secondary hypertension. If in a question you need
to know that the patient has one of these diseases, there will be something in the history
or physical. The first clue indicating that the patient has secondary hypertension is if they
present a patient who is especially young (<30) or old (>60). Statistically, the most common
cause of secondary hypertension is renal artery stenosis. If you see hypertension combined
with muscular weakness and hypokalemia, then you should suspect primary hyperaldoste-
ronism or Conn syndrome. If the patient has higher blood pressure in the upper extremi-
ties than in the lower extremities, then you should investigate for coarctation of the aorta.
Pheochromocytoma is suggested by the presence of episodic hypertension associated with
wheezing, flushing, and diarrhea.

1. 44-year-old man with headaches, BP 188/100 mm Hg, potassium 2.8, elevated bicar-
bonate level. The renin level is low; and the aldosterone level is elevated after salt
loading. Diagnosis is:
A. Renovascular hypertension
B. Primary hyperaldosteronism
C. Bartter syndrome
D. Low renin essential hypertension

Answer:

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Case 7
24-year-old woman comes with headaches. BP is 160/105. You find a bruit on one
side. Renal ultrasound shows both kidneys are small and one kidney is slightly
smaller than the other.

1. Next diagnostic step?

2. Most accurate diagnostic test?

3. Best initial therapy?

Summary: Case 7
The most common cause of secondary hypertension is renal artery stenosis. A clue to the
diagnosis is the presence of hypertension occurring outside the normal age range. Bruits are
found in <30% of patients, but they have a fairly high degree of specificity. An ultrasound
can show small kidneys, but this is relatively nonspecific. The most accurate diagnostic test
overall is the renal angiogram. Before you do the angiogram, however, there are several dif-
ferent forms of noninvasive testing that can be performed. The duplex ultrasound is the most
technically variable and may have up to 90% sensitivity, if the test is accurately performed.
A captopril nuclear renogram is less accurate, if there is a significant amount of renal insuf-
ficiency. The magnetic resonance angiogram (MRA) is extremely sensitive and specific. You
should generally not be asked to choose between them in the same question, because all three
have about the same accuracy. If the question occurs, however, you should choose the MRA.
The initial therapy for renal artery stenosis is with balloon angioplasty and the placement of
a stent. ACE inhibitors should only be used if angioplasty is unsuccessful and the patient is
not a surgical candidate. ACE inhibitors will control the blood pressure but will not prevent
the progression to renal failure.

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Case 8
Note A man is about to undergo surgery for removal of a pheochromocytoma.

Small cuffs overestimate BP.

1. What medication do you give him prior to surgery?

2. What class does it belong to?

Note

Large cuffs underestimate BP.

Summary: Case 8
The standard of care for controlling blood pressure prior to the removal of a pheochromocy-
toma is phenoxybenzamine. This is an alpha adrenergic receptor blocker.

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 Endocrinology
5
Hypothalamus Pituitary Axis

HYPOTHALAMUS CRH TRH GnRH GHRH, Somatostatin Dopamine

PITUITARY ACTH TSH LH,FSH GH Prolactin TRH

TARGET ORGAN Adrenal Thyroid Testes, Ovary Liver Liver

HORMONE Cortisol T3,T4 Estrogen IGF-1 IGF-1

Questions
1. With target organ failure, what will happen to the levels of the corresponding pituitary
hormone? Why?

2. If pituitary hormone levels are normal in the presence of target organ failure, what is
the diagnosis?

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Case 1
32-year-old complaining of weakness and fatigue. Decreased tolerance to low
temperature and losing her pubic and underarm hair. No period for at least
5 months. + Latent untreated TB. Examination for dry, pale, finely textured skin.
Chemistries hyponatremia with normal potassium.

1. Next step in management?

A. TSH
B. β-HCG
C. Stat MRI of the brain and pituitary
D. Cosyntropin stimulation test
E. Neurosurgery consult for pituitary biopsy

Answer:

2. Diagnosis?
A. Hypothyroidism
B. Adrenal insufficiency
C. Growth hormone deficiency
D. Cushing syndrome
E. Pan-hypopituitarism

Answer:

3. Pituitary MRI is performed, showing a 2.2 cm mass in the anterior pituitary. TSH 0.33,
free T4 0.56, A.M. cortisol 2.1, ACTH 3, IGF-1.54 (<10th percentile for age), prolactin 53.
Most likely etiology?
A. Hashimoto’s thyroiditis
B. Acromegaly
C. Non-functioning pituitary adenoma
D. Cushing disease
E. Craniopharyngioma

Answer:

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4. 26-year old is 3 months post-partum, giving birth to a healthy boy without complica-
tion via C-section. Her period has not returned as of yet. Unable to breast feed the baby
due to lack of milk. Diagnosis?
A. Lymphocytic hypophysitis
B. Sheehan syndrome
C. Non-functioning pituitary adenoma
D. Prolactinoma
E. Sarcoidosis
Answer:

5. 44-year-old African American man presents with headache, worsening. Shortness of

breath on exertion. He has positive orthostatics, positive bibasilar crackles, and bitempo-
ral visual field deficits. Chest x-ray shows significant mediastinal lymphadenopathy, and
head CT shows enlargement of the sellar area. An HIV test is negative. Diagnosis?
A. Toxoplasmosis
B. Metastatic lung cancer
C. Glioblastoma multiforme
D. Sacroidosis
E. Histoplasmosis
Answer:

6a. What is pituitary apoplexy?

6b. How would you treat pituitary apoplexy?

7. 24-year-old man complaining of significant polyuria and polydipsia. Drinks ice water,
and is constantly urinating. Sodium of 151, and a 24-hour urine collection has a volume
of 3.2 L. Pituitary MRI reveals “thickening of the pituitary stalk.” Negative chest x-ray,
remaining serum chemistries are normal. Diagnosis?
A. Histiocytosis X
B. Sarcoidosis
C. Diabetes
D. Pituitary adenoma
E. Lymphocytic hypophysitis
Answer:

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Summary: Case 1
Panhypopituitarism is the most likely diagnosis because the patient exhibits deficiency of all
glandular hormones controlled by the trophic hormones of the anterior pituitary. Thyroid
hormone deficiency is manifested as cold intolerance, weight gain, or fatigue. The loss of
pubic hair and the absence of menstruation are manifestations of gonadotropin deficiency.
The presence of hyponatremia with a normal potassium strongly indicates cortisol deficiency
with intact aldosterone secretion. Patients with growth hormone deficiency often complain
of feelings of social isolation. Any time a female patient of reproductive age presents with
complaints of menstrual irregularities, always send a pregnancy test!

Sheehan syndrome is postpartum pituitary necrosis secondary to blood loss experienced dur-
ing delivery. It most commonly presents in women who deliver via C-section. The pituitary
naturally hypertrophies during pregnancy to sustain the hormonal demands of the mother
and fetus. The acute blood loss that occurs during labor—or even more so in C-section (the
second bloodiest surgery in medicine)—coupled with the increased demand, leads to pitu-
itary necrosis. Patients typically complain of cold intolerance, weakness, lack of menstrua-
tion, and difficulty lactating after giving birth.

The laboratory investigations you would order to evaluate pituitary functioning include TSH,
FT4, A.M. cortisol, ACTH, cosyntropin stimulation test, IGF-1 levels, and prolactin.

Because the patient is hemodynamically stable, MRI (magnetic resonance imaging) would be
the next step in the management of this patient. MRI of the brain with gadolinium is used
to determine the etiology of this patient’s panhypopituitarism. In our case, the etiology is a
non-functioning pituitary macro-adenoma, which is the most common cause.

Panhypopituitarism can be caused by Sheehan syndrome, TB, ischemia, sickle-cell disease,

aneurysms, autoimmune disease, radiation, trauma, encephalitis, Langerhans cell histiocyto-
sis, sarcoidosis, hemochromatosis, and pituitary adenomas.

Patients with pituitary apoplexy will complain of headache, nuchal rigidity, and photophobia
with symptoms of hypoadrenalism. When the CSF (cerebrospinal fluid) is analyzed, there
will be little evidence of bacterial meningitis. Pituitary apoplexy occurs when there is bleed-
ing or infarction of a preexisting pituitary macroadenoma. Treatment involves emergent
surgical decompression and replacement of deficient hormones (especially steroids).

Treatment for panhypopituitarism involves replacement of all the hormones that are
deficient. These include thyroid hormone, steroids, sex hormones, and growth hormone.
Generally, you will replace steroids before initiating therapy with thyroid hormone, as thy-
roid hormone may increase the metabolism of endogenous steroids, causing acute adrenal
insufficiency.

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Case 2
44-year man with headaches, sweating, and generalized weakness. Symptoms have
been gradually worsening over last 3 months, glove and ring sizes have increased
over the last several years. Desire to have sex with his wife has decreased over the
last year. History is for hypertension diagnosed 2 months ago, for which he takes a
low-dose diuretic. Physical examination is noteworthy for a soft, doughy, and sweaty
handshake. You also note coarse facial features and protrusion of his lower jaw.

1. Diagnosis?

2. Initial test?
A. Growth hormone
B. IGF-1
C. TSH
D. Prolactin
E. IGF binding protein
Answer:

3. Test would confirm?

4. Laboratory testing?

5. MRI or CT (computed tomography) scan of the head be indicated?

6. Major complications of disease?

7. Best initial treatment?

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8. Two weeks later the patient presents with acute cholecystitis. What medication what
most likely added?
A. Pegvisomant
B. Lanreotide
C. Bromocriptine
D. Cabergoline
E. Methimazole
Answer:

9. When is cabergoline or bromocriptine indicated?

10. When is radiation therapy indicated?

Summary: Case 2
Patients who present with enlarging ring or hat sizes, protrusion of the jaw, coarsening of the
facial features, and deepening of the voice are most likely to have acromegaly. Amenorrhea,
headaches, and visual field deficits (bitemporal hemianopsia) are also consistent with the
diagnosis.

The best initial test for the diagnosis of acromegaly is to measure levels of IGF-1 (somato-
medin C). The most accurate test to diagnose acromegaly is the oral glucose suppression test.
In normal individuals, a glucose load should suppress growth hormone levels to <1 µg/L
within 2 hours after a 75-gm load of glucose. In acromegaly, growth hormone levels are not
suppressed.

You would order laboratory investigations to evaluate the functioning of the thyroid and
adrenal glands and ovaries/testes. The panel would include TSH, FT4, cosyntropin stimula-
tion test, IGF-1 levels, and prolactin.

Once you have established the presence of elevated growth hormone, you will pursue the
presence of a pituitary macroadenoma (>1 cm) by obtaining a CT scan/MRI of the pituitary
gland. In over 60% of cases, the tumor is a macroadenoma with significant cavernous sinus
invasion at the time of diagnosis.

Major complications of acromegaly are diabetes, hypertension, with LVH and CHF, colonic
polyps, sleep apnea, carpal tunnel, osteoarthritis, and pseudogout.

The best initial therapy for acromegaly is surgical removal of the macroadenoma.

Surgical intervention is the current treatment of choice for acromegaly. The somatostatin
analogues octreotide and lanreotide have multiple indications based on the updated acro-
megaly guidelines. These include adjunctive therapy post-operatively to further decrease
residual tumor, and initial therapy to shrink the tumor allowing it to be more amenable to
pituitary resection.

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10–20% of acromegalics co-secrete prolactin along with growth hormone. This is the popu-
lation group that has a chance of benefit with the addition of dopamine agonists. They are
most effective when used together with somatostatin analogues.

After surgical and medical interventions fail, stereotactic radiosurgery is indicated.

Case 3
27-year-old man complains of infertility and inability to smell his dinner. He is
also color-blind and deaf. LH/FSH and testosterone are reduced.

1. Diagnosis?

2. Treatment?

Summary: Case 3
Kallmann syndrome is an X-linked dominant disorder characterized by deficiency of GnRH.
LSH, FSH, and testosterone are also reduced. Hypothalamic hypogonadism in women is
manifested by amenorrhea or oligomenorrhea and is caused by weight loss, emotional or
physical stress, or athletic training. Anorexia nervosa and obesity can also cause hypotha-
lamic hypogonadism. These patients will have normal prolactin levels and reduced LH and
FSH with their amenorrhea.

The most common mutation involved in Kallmann syndrome in men is a mutation of the
KAL-1 gene, which is responsible for allowing the proper migration of embryonic gonado-
trope cells to their place in the sella turcica. This same migration defect is responsible for
the sensory deficits associated with Kallmann. The treatment for Kallmann depends on the
goals of the patient; if the goal is just virilization and return of androgen function, then just
testosterone is indicated. If the goal includes fertility, then gonadotropes (LH/FSH) need to
be replaced.

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Case 4
32-year-old woman complaining of fluid discharge from her breast. That her
symptoms started 3 months ago. Denies headache but attests to a 6-month
history of infrequent and irregular menses. unable to become pregnant despite
attempts over the last year. Physical examination NL.

1. Diagnosis?

2. Best test?

3. Next step?
A. Start bromocriptine
B. Refer to neurosurgery
C. Refer for radiation therapy
D. Order MRI
E. Observe

Answer:

4. Major causes of secondary hyperprolactinemia?

Causes of Hyperprolactinemia

Physiologic Causes Pharmacologic Causes Pathologic Causes

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5. When would you initiate therapy?

6. Treatment of choice for a prolactinoma?

A. Surgery
B. Cabergoline
C. Radiation therapy
D. Observation

Answer:

7. When is surgery indicated?

8. 27-year-old woman recently diagnosed hyperprolactinema. After a negative pregnancy

test is sent for MRI a 2.7 cm pituitary mass. Prolactin level is 67. No visual field deficits.
Next step?
A. Repeat prolactin with hemodilution
B. Repeat MRI
C. TSH
D. Cabergoline
E. Immediate radiation therapy

Answer:

Summary: Case 4
Amenorrhea and galactorrhea in a young woman who is unable to conceive strongly suggests
the diagnosis of a prolactin-secreting adenoma. Hyperprolactinemia in men presents with
decreased libido, erectile dysfunction, and infertility.

The best initial test to determine the presence of hyperprolactinemia is to measure the pro-
lactin levels in the blood. When prolactin levels exceed 200 ng/mL, the likelihood of having
a prolactin-secreting pituitary adenoma is high. When prolactin levels are under 100 ng/mL,
other causes for the hyperprolactinemia should be sought by history, physical examination,
and laboratory evaluation.

After establishing the presence of hyperprolactinemia, the next diagnostic step is to get a CT
scan/MRI of the pituitary.

Therapy is initiated when a macroprolactinoma (>1 cm) is present in the visual field defi-
cits, hypogonadism, infertility, significant galactorrhea, headaches, acne, or hirsutism and
significant loss of bone density. Bromocriptine can be used as initial therapy for hyperpro-
lactinemia, but because patients may have less tolerance of the adverse effects associated with
this medication (GI upset, hypotension), it should be chosen only when cabergoline is not

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given as an answer choice. Cabergoline is the dopamine agonist of choice for the initial treat-
ment of a prolactinoma. Approximately 90% of treated patients will respond to this therapy.

Surgery is the initial therapy of choice with pituitary apoplexy, or the patient cannot toler-
ate or fails medical therapy with a dopamine agonist. Even in patients with optic chiasm
compression, dopamine agonists are the initial treatment of choice, as the majority will have
resolution of visual field deficits within 48 hours of initial therapy.

In patients with microprolactinomas, estrogen replacement therapy and oral contraceptives

may be taken without danger of enlarging the pituitary.

Patients with galactorrhea and normal prolactin levels should be evaluated for hypothyroid-
ism with TSH and free T4 levels. Low thyroid hormones result in increase in TRH which
increases prolactin secretion. Symptoms abate with thyroid replacement therapy.

Case 5
56-year-old man admitted for cardiac bypass surgery. You note an elevated
alkaline phosphatase. The other liver enzymes are normal; and direct and
indirect bilirubin are normal. Serum calcium and phosphate are normal. Physical
examination is normal.

1. Next step in the management of this patient?

2. Diagnosis?

3. Best initial test?

4. Most accurate test?

A. Bone scan
B. Skeletal survey
C. CT scan
D. MRI
E. PET scan

Answer:

5. What is the treatment?

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6. When is calcitonin the treatment?

7. 62-year-old man. 12-year history of Paget disease presents with a “deep and achy” pain
in his right hip over the past 6 months. X-ray of right hip and pelvis shows new sclerotic
changes in proximal femur. The patient has not required bisphosphonates and doing
well with acetaminophen. Next best course of action?
A. Start alendronate
B. Start zoledronic acid
C. Perform a bone biopsy of the femur
D. Add ibuprofen to the patient’s pain regimen
E. Start calcitonin

Answer:

Summary: Case 5
The next step in the evaluation of this patient’s isolated rise in alkaline phosphatase is to
determine the source of the enzyme in the serum. To determine whether the alkaline phos-
phatase is coming from the bone or the liver, you would check a GGT or a bone-specific
alkaline phosphatase. When the GGT is within normal limits, the most likely cause of an
isolated elevation of alkaline phosphatase with normal serum calcium and phosphorus is
Paget disease of the bone.

The best initial test for the diagnosis of Paget disease is a radiologic bone survey. The most
accurate test for the diagnosis of Paget disease is a nuclear bone scan. The treatment of choice
for Paget disease is always a bisphosphonate. Asymptomatic Paget disease of the bone is usu-
ally left untreated. Therapy is initiated when patients complain of bone pain or if there is
significant cranial involvement leading to deafness. In the past, calcitonin was the therapy of
choice for the treatment of Paget disease. The advent of bisphosphonates has made calcitonin
obsolete in the treatment of this disease. The primary role of calcitonin today is for the acute
management of bone pain.

In addition, patients with Paget disease have a 100-fold increased risk for osteosarcoma
compared to age-matched patients without Paget; thus, if a patient with long-standing Paget
presents with new types of pain or radiological findings, a bone biopsy is mandatory to rule
out osteosarcoma of the involved bone.

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Case 6

65-year-old woman with back pain. Pain started suddenly 2 days prior been
progressively worsening. No fever or cancer. PE: for point tenderness over
the lower spine. Lumbar spinal x-ray is positive for a compression fracture of
L5. Marked osteopenia of all the vertebra. Calcium, phosphorus, and alkaline
phosphatase all falling within normal.

1. Diagnosis?

2. Most accurate test for osteoporosis?

3. What is the next best step in the management of this patient?

A. MRI of spine
B. Estrogen levels
C. Vitamin D and TSH
D. Prolactin

Answer:

4. Secondary causes of osteoporosis?

5. Best initial therapy for osteoporosis?

6. When is hormone replacement therapy (HRT) used?

7. Treatment of choice for osteoporosis?

8. When is raloxifene used?

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9. 78-year-old woman complaining of severe back pain. Lumbar spinal x-ray reveals
severe osteopenia of L4 and L5. Best therapy?
A. Calcitonin
B. Raloxifene
C. Hormone replacement therapy
D. Risedronate

Answer:

10. 62-year-old woman comes for results of a recent bone densitometry scan, which
showed a T score of 1 SD below normal. Denies history of bone fractures. Physical
examination 139 lb and is otherwise normal. Next step?
A. Begin alendronate
B. Begin hormone replacement therapy
C. Begin raloxifene
D. Begin calcium and vitamin D

Answer:

11. 47-year-old woman complains of hot flashes and requesting hormone replacement
therapy. Advise past medical history of uterine fibroids.?
A. No evidence to suggest that HRT will affect her fibroids
B. HRT will decrease fibroid size.
C. HRT will increase fibroid size.

Answer:

12. 55-year-old man is found to have vertebral compression fracture. Normal calcium
phosphate levels. Testosterone levels decreased. Most appropriate management?
A. Alendronate
B. Calcitonin
C. Testosterone
D. Vitamin D and calcium supplementation

Answer:

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13. Postmenopausal woman is found to have T score of -1.5. She exercises regularly, has a
BMI 21, and is currently a smoker. History of recurrent prednisone use due to COPD.
Most appropriate next step in management?
A. Alendronate
B. Increase intensity of exercise
C. Raloxifene
D. Parathyroid hormone
E. Vitamin D and calcium supplementation

Answer:

14. Postmenopausal woman with osteoporosis and history of DVT on alendronate therapy
for 18 months. She presents with a hip fracture. Next step in management of this patient?
A. Continue alendronate and start estrogen plus progesterone
B. Continue alendronate and start calcitonin
C. Continue alendronate and add raloxifene
D. Discontinue alendronate and start raloxifene
E. Discontinue alendronate and start parathyroid hormone

Answer:

15. Premenopausal woman with breast cancer develops chemotherapy-induced ameonor-

rhea. Which agent is appropriate to prevent bone loss?
A. Alendronate
B. Calcitonin
C. Parathyroid hormone
D. Raloxifene
E. Tamoxifen

Answer:

What amount of vitamin D has been shown to aid in decreasing fractures?

800 IU/day

Most common cause of secondary hyperparathyroidism is Vitamin D. What is the

number 1 cause?
Bariatric surgery

60-year-old male with bone pains and osteopenia. X-ray shows Looser’s Zone.
Diagnosis?
Osteomalacia due to Vit D deficiency

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What is the biggest risk factor for osteoporosis?

Sedentary lifestyle

Elderly female with hip fracture and Dexa of -1.5. Which factor is most important in
whether to start a bisphosphonate or not? FRAX score is discussed in the book; I put it in?
A. T score
B. Hip fracture
Answer is hip fracture

What can artificially increase the T-score?

OA, trauma, metastasis

Summary: Case 6
In a 65-year-old woman, who presents with osteopenia, the most likely diagnosis is osteopo-
rosis. It is important to distinguish between osteoporosis and osteomalacia both by history
and by laboratory evaluation. Osteoporosis is characterized by decreased bone matrix and
decreased bone mineralization. Osteomalacia is characterized by intact matrix but decreased
mineralization. The most common form of metabolic bone disease is osteoporosis. The
laboratory findings in osteomalacia are characterized by decreased calcium, phosphorus,
and vitamin D. The alkaline phosphatase is typically elevated. In osteoporosis, the alkaline
phosphatase, calcium, and phosphorus are usually normal.

Once osteoporosis is established as the most likely diagnosis of this patient’s metabolic bone
disease, the next step is to evaluate the patient for an underlying cause of her osteoporosis.
When a secondary cause of the osteoporosis is identified, you treat the underlying disease
to treat the osteoporosis. The most accurate test for the diagnosis of osteoporosis is bone
densitometry. The test is interpreted in the following manner: The T score is the score used
to determine your management of the case. If a patient has a T score of >2.5 standard devia-
tions below the mean, that patient has osteoporosis. If the T score lies between 1–2.5 standard
deviations below the mean, then the patient has osteopenia. Any patient with osteoporosis
should receive aggressive therapy for the disease. If the T score lies between 0–2 standard
deviations below the mean, then the patient has osteopenia. Osteopenia in the presence of 2
risk factors or a history of pathologic fracture should be managed like full-fledged osteopo-
rosis. Risk factors for osteoporosis include a history of smoking, low body weight, Caucasian
race, and a family history of the disease. The best initial therapy for osteoporosis is always
calcium and vitamin D.

Patients with risk factors for the development of osteoporosis should be started at an early
age with supplemental calcium and vitamin D. Patients should receive 1,500 mg daily if they
are not receiving hormone replacement therapy (HRT). If they are receiving HRT and are
younger than 50 years of age, then 1,000 mg of calcium per day is sufficient.

HRT should be used for the treatment of osteoporosis when the patient has a history of
hot flashes. HRT should never be used if a patient is pregnant or has unexplained vaginal
bleeding, a history of breast or endometrial cancer, a history of liver disease, or recent vas-
cular thrombosis. For men with osteoporosis and hypogonadism, the treatment of choice is
testosterone.

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The treatment of choice for osteoporosis is bisphosphonates. These medications reduce bone
resorption by inhibiting osteoclastic activity. The bisphosphonates effectively increase bone
density and decrease the risk of pathologic fracture. The major side effect of these medica-
tions is esophagitis.

Raloxifene is a selective estrogen receptor modulator. Like HRT, raloxifene increases bone
density and reduces LDL. Unlike HRT, raloxifene does not confer an increased risk of endo-
metrial cancer, it reduces the risk of breast cancer, and it has no effect on reducing levels of
triglycerides. Like estrogens, raloxifene is contraindicated in pregnancy and should not be
used in patients with a history of deep vein thrombosis. Patients with hot flashes will experi-
ence a worsening of symptoms when taking raloxifene.

Case 7
37-year-old woman complains of tingling of the lips progressively worsening. Also
complaining of tingling of her hands, muscle cramping, and vague abdominal
pain. Physical examination is significant for uncontrollable grimacing when her
cheek is lightly tapped. Serum chemistries calcium of 6.2 mg/dL.

1. Next best step in management?

A. Send PTH
B. Send 25-vitamin D
C. Parathyroid scan
D. Order calcium carbonate
E. Order calcium gluconate

Answer:

2. Next step in the diagnostic workup of this patient’s hypocalcemia? Why?

3. What would you expect to find on EKG?

4. What is the treatment of hypoparathyroidism?

5. What diuretic should be avoided in patients with hypocalcemia?

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Hypoparathyroidism
Diagnosis Calcium Phosphate PTH Alkaline Phenotype
Phosphatase
Primary
hypoparathyroidism
Pseudo- Mental retardation, short
hypoparathyroidism stature, round face, ectopic
bone formation, short fourth
metacarpals, obesity
Pseudo- Mental retardation, short
pseudohypoparathyroidism stature, round face, ectopic
bone formation, short fourth
metacarpals, obesity

6. 19-year-old woman presents for recurrent numbness and tingling in her fingers, legs,
and face. She has a cleft palate, mild cognitive deficiency, and a short fourth metatarsal
of her hands. Calcium 7.1, PTH 128, and phosphate 2.1. Next test?
A. TSH
B. Magnesium
C. 1,25 vitamin D
D. Prolactin

Answer:

Case 8
34-year-old woman comes for a regular checkup. Denies any complaints. Ca2+
elevated at 11.7 mg/dL. Physical examination is normal.

1. Most common causes of hypercalcemia?

2. Symptoms of hypercalcemia?

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Hypercalcemia
Serum Serum PTH
Diagnosis Serum Calcium Phosphate (IRMA Assay)
Primary hyperparathyroidism
Malignancy (multiple
myeloma, breast, lung, and
prostate carcinoma)
Granulomatous disease
(sarcoid, TB)
Familial hypocalciuric
hypercalcemia

3. Best initial treatment?

A. Alendronate
B. Normal saline
C. Furosemide
D. Pamidronate
E. Sensipar

Answer:

4. Pamidronate or zoledronic acid used?

5. Ketoconazole or steroids used?

6. When is surgery the answer? What is the most common complication of surgery?

NOTE 7. 72-year-old man is confused and unresponsive. PMH is significant for metastatic lung can-
cer. Serum calcium is measured at 16.2 mg/dL. Which will lower serum calcium the fastest?
Age < 50
A. Plicamycin
Renal Stones B. Pamidronate
Serum Ca+2 > 1mg above C. Calcitonin
normal D. Prednisone
Symptomatic (T score > 2.5,
Answer:
hip fracture, stones etc)

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8. 28-year-old woman has incidentally discovered hypercalcemia. Her calcium was 11.3
and PTH 67. She denies any history of fracture. A 24-hour urine calcium is 23. Next
appropriate step?
A. Immediate parathyroid surgery
B. Calcitriol
C. Parathyroid scan and neck CT
D. Check her sister’s calcium
E. Renal sonogram

Answer:

Patient with thyroid mass, elevated Ca, elevated calcitonin and calcifications noted in
the mass. Diagnosed with medullary carcinoma. How to screen family members?

26-year-old female with 9 kidney stones in the last 17 months. Ca is 12.1 mg/dl, PO4
2.1, and PTH is 335 pg/ml (10–65). Most appropriate management is:

Summary: Case 8
The most common causes of hypercalcemia are primary hyperparathyroidism and hyper-
calcemia of malignancy. Any granulomatous disease (TB, sarcoid) can induce hypercalcemia
by increased production of vitamin D by the macrophages in the granuloma. Additionally,
thyrotoxicosis and adrenal insufficiency can also induce hypercalcemia. Frequently, patients
with hypercalcemia are asymptomatic. Symptomatic hypercalcemia is manifested by renal
stones, hypertension, polyuria, fatigue, mental status changes, bone pain, osteoporosis, and
pathologic fractures. The initial treatment of any patient with symptomatic hypercalcemia
is to aggressively hydrate the patient with large amounts of normal saline. In patients with
renal insufficiency where the physician’s ability to hydrate may be limited, additional therapy
with calcitonin may be required to acutely lower the serum calcium. Treatment with furo-
semide to induce renal secretion of calcium is effective only when the patient has adequate
hydration and sodium onboard, i.e., normal saline. Pamidronate and zoledronic acid are
the anti-resorptive therapies of choice in patients with hypercalcemia due to malignancy.
The problem with both drugs is that they take 1 to 2 days to have an effect. The fastest way
to decrease calcium levels is with the use of calcitonin. The problem with calcitonin is that
it has a short half-life requiring multiple daily doses; in addition, many patients develop
resistance to calcitonin via immunologic mechanisms, which decreases its efficacy. Patients
who have hypercalcemia secondary to granulomatous disease should be treated with steroids
or ketoconazole. In primary hyperparathyroidism, surgery is indicated if any of the following
are present:
• Symptomatic hypercalcemia
• Calcium >11.5
• Renal insufficiency
• Age <50 years

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• Nephrolithiasis
• Osteoporosis
• Malignant hypercalcemia

It is important to note that preoperatively, in the absence of a history of previous neck sur-
gery, there is no need to image the parathyroid glands to localize the responsible adenoma.

Hypocalciuric hypercalcemia is an autosomal dominant disorder characterized by decreased

calcium secretion by the kidney (<200 mg/day). These patients have asymptomatic and mild
hypercalcemia. The diagnosis is made when the calcium clearance/creatinine clearance is less
than 0.1.

Hyperparathyroidism can be a manifestation of the multiple endocrine neoplasia (MEN)

syndromes:
• MEN I consists of hyperparathyroidism (hyperplasia), pituitary tumors, and pan-
creatic tumors.
• MEN II consists of hyperparathyroidism (hyperplasia), pheochromocytomas, and
medullary carcinoma of the thyroid.
• MEN IIb consists of pheochromocytomas, medullary carcinoma of the thyroid, and
mucous neuromas.

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Case 9
25-year-old man for evaluation of new-onset hypertension. Exam significant for
central obesity, muscle wasting, and multiple bruises. Bluish markings on the
patient’s abdomen. Labs:

Na+: 137 WBC: 17.5

K+: 2.9 Differential: Low lymphocytes

HCO –3: 23 Glucose: 276

1. Most likely diagnosis?

2. Best initial test?

3. Next test in the evaluation of this disease? Why?

4. 46-year-old man is treated with long-term inhaled corticosteroids for severe asthma. He
develops hypertension, moon facies, and abdominal striae. Which laboratory test will
distinguish exogenous corticosteroids from Cushing syndrome?
A. ACTH cosyntropin stimulation test
B. High-dose dexamethasone suppression test
C. Low-dose dexamethasone suppression test
D. Serum aldosterone
E. Serum cortisol and urinary cortisol

Answer:

5. When is surgery the answer?

6. When is ketoconazole or metyrapone the answer?

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Summary: Case 9
The history of new-onset hypertension associated with central obesity, muscle wasting, and
easy bruising strongly suggests Cushing as the most likely diagnosis in this case. The presence
of hyperglycemia and hypokalemia on laboratory investigation also suggests the diagnosis.

The low-dose dexamethasone or the 24-hour urine-free cortisol tests are the initial tests for
the diagnosis of Cushing syndrome. While they are extremely sensitive tests, they are not very
specific. This means that a negative test rules out the diagnosis with a 98% certainty, whereas
a positive test may be falsely positive (due to obesity, stress, alcoholism, estrogen, pregnancy).
The other test approved for screening is the midnight salivary cortisol. The premise behind
this test is that cortisol naturally nadirs around midnight in humans. If the midnight salivary
cortisol is elevated, it is suspicious of Cushing syndrome.

Once hypercortisolism is established, the next diagnostic question is whether the hypercor-
tisolism is ACTH-dependent or -independent. This is determined by measuring the ACTH
level. If the ACTH is low (<5 pg/mL), then the hypercortisolism is ACTH-independent, i.e.,
from the adrenal gland. The main differential for adrenal causes of Cushing is adrenal ade-
noma versus carcinoma. Adrenal carcinoma can be differentiated by high levels of DHEA as
the tumor usually arises in androgen-producing cells. If the ACTH is elevated (>10 pg/mL),
then the hypercortisolism is ACTH-dependent, i.e., ectopic versus pituitary in origin. Since
elevated ACTH stimulates all 3 levels of the adrenals, patients will also complain of hirsutism.

In ACTH-independent disease, the next step will be to order a CT scan of the adrenal glands
to localize an adrenal mass or tumor. In ACTH-dependent Cushing, the next step is to get an
MRI of the brain to localize an adrenal adenoma as the source of the ACTH. If a pituitary
abnormality is identified, surgical excision of the mass is indicated. If no pituitary abnormal-
ity is identified, then inferior petrosal sinus sampling can be used to differentiate between
ectopic ACTH production and a pituitary source of the ACTH. All glucocorticoids, includ-
ing potent inhaled and topical glucocorticoids, can result in the inhibition of ACTH secre-
tion. Thus, in order to differentiate iatrogenic Cushing from Cushing syndrome, the plasma
ACTH, serum cortisol concentrations, and cortisol excretion will all be low.

Overall, pituitary Cushing disease accounts for approximately 70-80% of cases, while adrenal
Cushing accounts for 10-15%.

In Cushing disease with a pituitary source of the ACTH, transsphenoidal resection of any
adenoma is the treatment of choice. In ACTH-independent adrenal disease, laparoscopic
excision of the adrenal mass is the treatment of choice. The larger the adrenal mass (>6 cm),
the higher the likelihood of malignant disease. Ketoconazole and metyrapone can be used to
treat Cushing syndrome in cases where surgical resection is impossible (metastatic adrenal
carcinoma).

The management of the incidental adrenal mass depends on the size of the mass on presenta-
tion. Masses that measure less than 4 cm in size can be followed with a repeat CT scan in 6 to
12 months. In addition, any adrenal incidentaloma greater than .8 cm requires a full hormone
evaluation with measurement of ACTH, low-dose dexamethasone test/24-hour urine free
cortisol, and aldosterone/plasma : renin ratio. All masses greater than 4 cm in size should be
surgically resected regardless of endocrine abnormalities. With masses that are smaller than
4 cm in size and accompanied by hypertension, surgery is indicated only when the endocrine
evaluation is abnormal.

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Case 10
25-year-old man presents for evaluation of new-onset hypertension. Complains
of muscular weakness and frequent urination. No significant PMH. PE normal.
Labs:

Na+: 134 WBC: 7.3

K+: 2.7 Differential: Normal

HCO–3: 32

1. Diagnosis?
A. Coarctation of the aorta
B. Hyperaldosteronism
C. Hypercortisolism
D. Pheochromocytoma

Answer:

2. Best initial test?

A. Adrenal vein aldosterone
B. CT scan of abdomen
C. Aldosterone suppression test
D. Plasma renin activity and aldosterone concentration
E. 24-hour urine potassium

Answer:

3. Most appropriate confirmatory test?

A. Adrenal vein aldosterone
B. CT scan of abdomen
C. Aldosterone suppression test
D. Plasma renin activity and aldosterone concentration
E. 24-hour urine potassium

Answer:

4. Next step in evaluation of primary aldosteronism?

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5. What laboratory abnormality suggests secondary hyperaldosteronism (e.g., reno-

vascular disease)?
A. Increased plasma renin activity; increased plasma aldosterone concentration
B. Decreased plasma renin activity; increased plasma aldosterone concentration
C. Decreased plasma renin activity; decreased plasma aldosterone concentration
D. PAC/PRA ratio is >20.

Answer:

6. What is the gold standard in differentiating adrenal hyperplasia versus adrenal adenoma?

7. When is surgery the most appropriate step in management?

8. Drug of choice in management of hyperaldosteronism?

A. Amiloride
B. Epleronone
C. Spironolactone
D. Triamterene
E. Furosemide

Answer:

9. Patient with adrenal incidentaloma on CT for pneumonia. Next steps to test if

functioning?
Cushings ➔
Pheochromocytoma ➔
Conn’s syndrome ➔
Adrenal Carcinoma ➔

Summary: Case 10
New-onset hypertension accompanied by hypokalemia and metabolic alkalosis strongly sug-
gests the diagnosis of primary hyperaldosteronism, or Conn syndrome. An elevated plasma
aldosterone concentration (PAC) accompanied by a low plasma renin activity (PRA) and a
PAC:PRA ratio >20:1 suggests the diagnosis of Conn syndrome. Secondary hyperaldoste-
ronism (e.g., renovascular disease) should be considered when both the PRA and PAC are
increased and the PAC/PRA ratio is <10. Once the diagnosis of primary hyperaldosteron-
ism has been made, a unilateral aldosterone-producing adenoma or carcinoma must be
distinguished from bilateral hyperplasia. Thus the next step is to obtain a CT scan of the
adrenal glands to localize adenomas that may be present. Measuring aldosterone in samples
of adrenal venous blood is the “gold standard” test to distinguish between an adenoma

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and hyperplasia. In hyperplasia, the aldosterone will be elevated in both venous samples.
In adenomatous disease, aldosterone will be elevated in the venous sample of the affected
gland. For unilateral adrenal adenomas causing primary aldosteronism, the most effective
surgical excision. For patients with bilateral adrenal hyperplasia, surgery will correct the
hypokalemia but will fail to correct the hypertension. Spirinolactone is the most appropriate
therapy for adrenal hyperplasia and also is the most appropriate initial therapy preoperatively
for adrenal adenomas. Epleronone is a highly selective mineralocorticoid receptor antagonist
which may be used if side effects of spirinolactone prevent its use.

Case 11
25-year-old man with new-onset hypertension. He complains of headache
“attacks” associated with profuse sweating and palpitations. Also complains of
paroxysmal attacks of abdominal pain and chest pain. 3-month history of weight
loss. Physical examination normal. Labs:

Na+: 135 WBC: Normal

K+: 3.7 Differential: Normal

HCO–3: 24

1. Diagnosis?

2. Best initial test?

A. Serum epinephrine
B. Serum aldosterone
C. Urine vanillylmandelic acid
D. Urine metanephrines

Answer:

3. Next step in diagnosis?

4. When is surgery the answer?

5. When is phenoxybenzamine the answer?

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6. When is beta blocker used?

A. Prior to use of phenoxybenzamine
B. After use of phenoxybenzamine
C. After use of calcium channel blockers
D. Postoperative management of hypertension

Answer:

7. 38-year-old Asian woman with 4 cm incidental right adrenal nodule found on an

abdominal CT scan. 24-hour urinary free catecholamines shows metanephrines of
5 times normal, and normetanephrines of 4 times normal. Next test?
A. Thyroid sonogram
B. Prolactin
C. TSH
D. Gastrin
E. Pituitary MRI

Answer:

Summary: Case 11
The history of episodic headache, chest pain, and abdominal pain is highly suggestive of
a pheochromocytoma. The best initial test for the diagnosis of a pheochromocytoma is
24-hour urine for vanillylmandelic acid (VMA), metanephrines, or catecholamines. The
most accurate test for the diagnosis of pheochromocytoma is to measure, via direct assay,
urine and blood levels of epinephrine and norepinephrine during or shortly after an attack.
The next step in diagnosis is to obtain a CT scan of the adrenal glands. Approximately 90%
of all pheochromocytomas occur in the adrenal gland.

The treatment of choice for all pheochromocytomas is surgery. Preoperatively, phenoxy-

benzamine is the therapy of choice. The introduction of phenoxybenzamine has drastically
reduced the perioperative mortality associated with surgery.

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Case 12
52-year-old man presents with appendicitis and is found to have a 2-cm adrenal
mass preoperatively. Referred to his primary care doctor for followup of his
adrenal mass. He has otherwise been in his usual state of health. Labs:

Na+: 139

K+: 4.2

HCO–3: 22

Glucose: 92 mg/dL

1. What is the most appropriate next step in management?

A. 24-hour urinary cortisol
B. FNA of adrenal mass
C. Repeat CT scan in 3 months
D. Repeat CT scan in 6 months
E. Serum prolactin

Answer:

2. Repeat CT scan reveals mass is now 4 cm in diameter. Next step in management?

A. 24-hour urinary catecholamine
B. MRI of adrenals
C. Serum cortisol
D. Urinary 17-keto steroid

Answer:

3. 29-year-old presents with chronic abdominal pain, recent weight gain, and easy bruising.
BP of 152/89 mm Hg. Coagulation profile is normal. Basic chemistry profile normal.
CT scan of her abdomen reveals 1-cm mass in the right adrenal gland. The most
appropriate initial test?
A. ACTH level
B. FNA biopsy of mass
C. High-dose dexamethasone suppression test
D. Serum aldosterone
E. TSH

Answer:

Patient with 3.4 cm mass on adrenal found incidentally on CT. All hormone levels are
within normal limits. Next step?
Repeat CT scan in 6 mos
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Summary: Case 12
Incidental adrenal tumors are seen in approximately 1.5% of CT scans performed. The 2
most important factors in the diagnostic workup of an incidental adrenal mass are its size
and “functioning”. Since most adrenal tumors are benign lesions less than 3 cm in diameter
without any accompanying symptoms may be followed with a repeat CT scan in 3 months and
then every 6 months for 2 years, if found to be stable. Lesions >3 cm in diameter or increase
in size of lesion from prior study requires MRI imaging and endocrine evaluation based on
symptoms. Lesions larger than 4-cm diameter are more likely to be malignant and require
surgical removal. Functioning lesions are lesions that result in one of 3 conditions:

1) Cushing syndrome
2) Hyperaldosteronism
3) Pheochromocytoma

Initial step in endocrine assessment is to evaluate for functioning tumors based on symp-
tomatology. Cushing syndrome is suggested by hypertension, central obesity, proximal
muscle weakness, easy bruisability. The appropriate screening test in workup of adrenal
mass with these presenting symptoms is a 24-hour urinary free cortisol or low-dose dexa-
methasone suppression test. Hyperaldosteronism presents with hypertension, hypokalemia,
and hypernatremia. Workup should include plasma renin activity and plasma aldosterone.
Pheochromocytoma is suggested with hypertension, episodic headaches, palpitations, and
diaphoresis. Initial workup for pheochromocytoma is urinary catecholamines. Patients with
abnormal screening laboratory results should be referred for surgical excision, regardless of
the size of the mass.

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Case 13
43-year-old woman complaining of weakness and generalized fatigue, nausea,
vomiting, low-grade fever, and some joint pain. BP of 94/56 mm Hg. Sparse
axillary hair and diffuse hyperpigmentation of the skin. Labs:

Na+: 127 WBC: 2.2

K+: 5.7 Differential: Eosinophils 343

HCO–3: 18

1. Diagnosis? Why?

2. Best initial test?

A. 24- hour urine free cortisol
B. A.M. cortisol
C. Random cortisol
D. Serum aldosterone
E. Serum DHEA-s

Answer:

3. When would you measure ACTH?

4. When is abdominal CT used?

5. When is MRI or CT of the head used?

6. Treatment?

7. For the patient above, the ACTH returns are 57, and A.M. cortisol returns as .8. Which
of the following tests would help confirm the etiology of the patient’s condition?
A. Anti-TPO antibodies
B. Antigliadin antibodies
C. Transglutaminase antibodies
D. Renal and adrenal sonogram
E. Anti-CYP21 antibodies

Answer:

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Summary: Case 13
Hypotension accompanied by hyperkalemia, a non-anion gap metabolic acidosis, and
increased skin pigmentation make the diagnosis of adrenal insufficiency quite likely. It is
important to note that the presence of both skin pigmentation and hyperkalemia implies
that the level of failure is at the adrenal gland itself rather than at the level of the pituitary.
As the adrenal gland fails, the pituitary responds by increasing its secretion of ACTH. ACTH
stimulates the melanocytes in the skin, resulting in hyperpigmentation. The same process in
the adrenal gland that compromises its ability to produce steroid also causes a reduction in
the adrenal gland’s ability to produce aldosterone, resulting in hyperkalemia.

An A.M. cortisol is the best initial screening test for adrenal insufficiency. The ACTH (cosyn-
tropin) stimulation test is the test to confirm the presence of adrenal insufficiency. The 8 a.m.
cortisol is useful for diagnosis only if it is extremely high (>18 µg/dL), when it can rule out
adrenal insufficiency; or if it is extremely low (<3 µg/dL), when it confirms the presence of
adrenal insufficiency. The random serum cortisol is no longer considered useful unless the
patient is under physiologic stress and the cortisol should be >18 µg/dL. If the random serum
cortisol does not rise in response to physiologic stress, then adrenal insufficiency should be
suspected.

After establishing the diagnosis of adrenal insufficiency, the ACTH should be measured to
determine whether the failure is primary (ACTH increased) or secondary (ACTH normal
or decreased). The abdominal CT scan is the next step when primary adrenal insufficiency
is diagnosed. Small adrenals imply an adrenalitis as the etiology of gland failure, whereas
enlarged glands imply tumor or granulomatous disease (TB, sarcoid) as the etiology of the
gland failure. When there is evidence of secondary failure of the adrenal gland, the pituitary
should be imaged to determine the etiology.

Treatment for acute adrenal insufficiency is IV fluids and hydrocortisone. Patients who are
taking steroids for the treatment of chronic disease may acquire the inability of their adrenal
to respond adequately to stress. Generally, a dose of at least 15 mg/day for 4 weeks is needed
to produce any element of adrenal suppression requiring stress doses of steroid.

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Case 14
25-year-old woman comes to clinic complaining of nervousness and restlessness.
States she constantly feels hot and continually reports an irregular menstrual
period. PE: enlarged, nontender thyroid gland, and a thyroid bruit is present. Fine
tremor of both hands bilaterally and the patient’s hair is very fine. Labs:

TSH < 0.1 mU/L Free T4: 2.9 mcg/dL (normal 0.8–1.7)

1. Most likely diagnosis?

A. Toxic multinodular goiter
B. Hashimoto’s thyroiditis
C. Autonomous thyroid nodule
D. Grave’s disease

Answer:

2. When is the diagnosis subacute thyroiditis?

3. Best initial treatment?

4. When is iodine the best treatment?

5. When is radioactive iodine the best initial treatment?

6. 26-year-old woman comes to your office complaining of diarrhea, palpitations, and

heat intolerance after giving birth to a baby girl 4 months ago. Physical examination
is normal except for a tender and swollen thyroid gland. Serum TSH is <0.1 mU/L (nor-
mal 0.5–4); and free T4 is 53 pmol/L (normal 3.5–8). What is the best initial therapy?
A. Prednisone
B. Radioactive iodine
C. Propylthiouracil
D. Propranolol

Answer:

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7. 42-year-old woman was started on PTU and propranolol 3 weeks ago for symptomatic
hyperthyroidism. Which test to follow progress?
A. TSH
B. Free T4
C. Free T3 and TSH
D. T4

Answer:

8. Young woman complains of diarrhea and irritability. Lost 15 lbs over the last 3 weeks.
Thyroid gland is painless. TSH is decreased; free T4 is elevated. Radioactive uptake is
low; and ESR is <25 mm. Diagnosis?
A. Factitious hyperthyroidism
B. Subacute thyroiditis
C. Toxic multinodular goiter

Answer:

9. 65-year-old woman with acute shortness of breath and tachycardia. Currently taking
amiodarone to control atrial fibrillation. ECG sinus tachycardia at 133 bpm, and tro-
ponins negative 3 times over the course of 24 hours. TSH is <.01 (normal 0.5-4). What
would you most commonly expect to see on a thyroid uptake and scan?
A. Increased uptake
B. Decreased uptake
C. Normal uptake
D. Multiple “hot” nodules

Answer:

Elderly patient presents with A fib and low TSH. Patient also admits to heat intolerance
Diagnosis?
Hyperthyroidism

Elderly patient brought with apathy, weight loss, arrhythmias, CHD, diarrhea, and
muscle weakness. TSH decreased. T4 low normal. Diagnosis?
Apathetic thyrotoxicosis

Can you cardiovert afib during thyroid storm?

No

Must wait for 3 months until euthyroid? Why?

Spontaneous conversion is normal

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10. Patient with Graves’s disease had radioiodine ablation and then 10 weeks later has
20 lbs. of weight gain.
A. Wait one month
B. Wait 3 months
C. Start T4

Answer:

Patient with Graves presents with watery eyes and periorbital edema. Exam reveals an
afferent pupillary defect. Pain on eye exams. Diagnosis?
Thyroid ophthalmopathy with optic nerve impingement

How to manage thyrotoxicosis and optic nerve impingement

Surgery

Patient s/p uncomplicated delivery and breastfeeding 14-week-old child. Has enlarged
thyroid and non-tender. Diagnosed with Chronic lymphocytic thyroiditis. Best treatment?
Beta blocker
Why?
Usually transient

Summary: Case 14
Patients who present with tremor, heat intolerance, weight loss, anxiety, and menstrual irreg-
ularity should be suspected of having hyperthyroidism. The elevated FT4 with suppressed
pituitary TSH establishes the hyperthyroidism as primary, with the problem at the level of
the thyroid gland. Patients who present with primary hyperthyroidism and exophthalmos are
likely to have Graves disease. Toxic nodular goiter is diagnosed in patients with a hot nodule
on radioactive iodine scanning. Subacute thyroiditis should be diagnosed when you see a low
uptake scan with primary thyrotoxicosis and a tender thyroid gland on physical examination.

Propranolol is the best initial therapy for any patient with thyrotoxicosis. Propranolol inhibits
peripheral conversion of T4 to T3 and improves the patient’s symptoms. Methimazole and
propylthiouracil are effective only for treatment of high-uptake thyrotoxicosis. In a pregnant
woman, propylthiouracil will always be the treatment of choice. Iodinated contrast agents are
effective in the treatment of both high-uptake and low-uptake thyrotoxicosis. Treatment of
high-uptake thyrotoxicosis with ablative radioactive iodine or surgery should be undertaken
only if the patient has already received medical therapy. Proceeding directly to ablative therapy
can induce thyroid storm. For all patients with high-uptake primary hyperthyroidism, the
ablative therapy of choice is radioactive iodine. Surgery is the ablative therapy of choice in
patients with airway obstruction secondary to goiter or pregnancy and when there is a high
likelihood of thyroid cancer. The effectiveness of therapy is followed with the FT4.

Patients with subclinical hyperthyroidism should not be treated initially, and their thyroid
function studies should be repeated in 6 to 12 months.

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Thyroid Function Tests

Free T4 Serum TSH

Primary hypothyroidism Low High

Secondary hypothyroidism Low Normal or low
Primary hyperthyroidism High Low
Secondary hyperthyroidism High Normal or high

Thyrotoxicosis

Suspect Hyperthyroidism

The next diagnostic test is...

Low FT4 Low TSH Serum TSH, FT4 High FT4 nl/high TSH

High FT4 Low TSH

The next diagnostic test is...

Radioactive Iodine Uptake

High Uptake Low Uptake

Graves disease Subacute thyroiditis
Toxic multinodular goiter Acute Hashimoto
Levothyroxine Iodine loading

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Case 15
56-year-old with 1.5-cm nodule in the right lobe of the thyroid gland. The
patient denies any history of fatigue, cold intolerance, or weight gain. History of
anxiety, palpitations, or diarrhea. There is no history of neck irradiation. Physical
examination is otherwise normal. You note no lymphadenopathy in the head or
neck. TFTs are normal.

1. Why are TFTs essential in any patient with a thyroid nodule?

2. Next step in the management of this patient?

A. Sonogram-guided FNA
B. Surgical biopsy
C. Levothyroxine
D. Repeat sonogram in 6 months

Answer:

Summary: Case 15
Thyroid function studies are essential to the management of a thyroid nodule, because if the
TSH is found to be suppressed, then the workup and management of thyrotoxicosis applies.
Only when the TSH is normal does the workup focus on determining the histology of the
thyroid nodule. Once the TSH test returns normal, the next step in the evaluation of a thyroid
nodule is to perform an FNA. Thyroid ultrasound is the imaging modality of choice that may
precede the FNA to assess for the likelihood of malignancy. Radioactive thyroid scanning is
no longer indicated in the workup of a thyroid nodule.

The decision to send a nodule for surgical excision should be based on the results of the FNA.
When the FNA shows malignancy, the patient is diagnosed with and should be treated for
thyroid cancer. When the FNA shows “follicular neoplasm,” then an excisional biopsy should
be performed.

Thyroid cancer comes in 4 histological types: papillary, follicular, medullary, and anaplastic.
The most common type is papillary carcinoma. Both papillary and follicular carcinoma of
the thyroid are considered differentiated and closely resemble normal thyroid tissue. It is not
surprising that these differentiated cancers of the thyroid have a much better prognosis than
their undifferentiated counterparts. Treatment with surgery followed by follow-up scanning,
ablative therapy with radioactive iodine, and suppression with levothyroxine are effective
only in patients with differentiated cancer of the thyroid.

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Evaluation of Thyroid Nodule

Solitary thyroid nodule

TSH test

Euthyroid Thyrotoxic

Scan

FNA Cold Nodule Hot Nodule

Radioactive
Insufficient Benign Suspicious Malignant iodine or
surgery
Repeat
in 6
Cystic Solid months Frozen section

Regress Recurrence Repeat FNA Benign or Malignant

intermediate

Clinical suspicion Total or partial thyroidectomy

Observe Low High Thyroid lobectomy

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Case 16
34-year-old woman complaining of fatigue, cold intolerance, and constipation.
Complains of feeling “low“ over the last several weeks. Diffusely decreased tendon
reflexes.

1. Diagnosis?

2. Next step in the management of this patient?

3. Treatment for this disease?

4. 72-year-old woman presents with depressed sensorium. Hypothermic and to have a HR

of 38 and RR of 10. Na 122 mEq/dL and glucose 52 mg/dL. Next step?
A. D5W ampoule
B. Hypertonic saline infusion
C. Intravenous glucocorticoids
D. Intravenous T3
E. Empiric antibiotics

Answer:

5. Homeless man brought in on stupurose, hypothermic and bradycardia. + edema

around eyes, and no axillary hair. Diagnosis?
Myxedematous coma
Treatment?
Steroids plus IV T4/T3
Most common cause
Sepsis

6. Rapidly growing neck mass in pt with Hashimoto’s for > 20 years. Most likely diagnosis?
Thyroid Lymphoma

7. If a nuclear meltdown were to occur what would you give to people in the surrounding
area?
Potassium iodide

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Summary: Case 16
34-year-old woman, who presents with fatigue, cold intolerance, and constipation, should
raise the possibility of hypothyroidism. Feelings of depression and decreased tendon reflexes
further support the diagnosis. Patients with hypothyroidism can present with rare manifes-
tations of the disease, including pericardial effusions, sinus bradycardia, hypoventilation,
CHF, and sleep apnea. The best initial test for the evaluation of hypothyroidism is a serum
TSH. Treatment for hypothyroidism consists of levothyroxine to normalize the TSH. Unlike
hyperthyroidism, where the therapy is followed with the FT4, in hypothyroidism you should
follow your therapy with the serum TSH.

In primary hypothyroidism you expect the FT4 to be low with an appropriate increase in
the TSH, which should be elevated. If the FT4 is low and the TSH is normal or low, the next
step is to order an MRI of the brain to evaluate for any pituitary lesion causing a secondary
hypothyroidism. Myxedema coma is a rare complication of hypothyroidism with patients
presenting with coma, bradycardia, heart failure, respiratory failure, and hypotension. The
treatment is to replace the thyroid hormone with IV levothyroxine. Before giving thyroid
hormone, it is imperative to give steroids first.

Euthyroid Sick Syndrome Case 17

• Free T4 is normal or low. 57-year-old man is admitted to the MICU with hypotension and gram-negative
• TSH is normal or low. sepsis. Laboratory results are:
• T3 is low. TSH: 0.2 mU/mL FT4: 7 pmol/L Total T4: 8 µg/dL
• rT3 is high.
1. Diagnosis?

Case 18
46-year-old man states that his father and mother both diabetes mellitus. He
denies any history of polyuria or polydipsia over the last several months, but
attests to generalized fatigue. Physical examination is normal.

1. How is diabetes mellitus (DM) diagnosed?

2. Best initial test for diagnosis of diabetes mellitus?

3. When is oral glucose tolerance testing indicated?

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A. Routine diabetic screening

B. 14 weeks gestation
C. 26 weeks gestation
D. 30 weeks gestation

Answer:

4. When is metformin indicated?

5. When is a sulfonylurea indicated?

6. When are thiazolidinediones indicated?

7. When is insulin indicated, even initially?

8. 65-year-old woman with fasting glucose elevated at >126 mg/dL for the second time.
Her HgA1c was measured at 10%. PE is normal; BMI of 32. Best initial therapy?
A. Glyburide
B. Metformin
C. Pioglitazone
D. Insulin

Answer:

9. 56-year-old man is admitted after breaking his hip. Has history. His treatment regimen
consists of regular and glargine insulin. What would you recommend before surgery?
A. NPO from 9 P.M., stop regular insulin the morning of procedure and replace with
half normal dose of glargine
B. NPO from 9 P.M., IV fluids with 5% dextrose and continue regular insulin and
glargine dose the morning of procedure
C. NPO from 9 P.M., IV fluids with 5% dextrose and regular insulin via IV infusion
D. NPO from 9 P.M., IV fluids with 5% dextrose, and give half dose of regular insulin
while holding glargine

Answer:

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10. 47-year-old man with left ventricular hypertrophy and an EF 37% following a recent
heart attack is diagnosed with diabetes. Most appropriate initial therapy?
A. Metformin
B. Sulfonylurea
C. Thiaglitazone
D. Basal insulin

Answer:

11. Patient with BIM of 32 and FBS of 115. Best way to prevent onset of DM?
A. Metformin decreased progression by 30-40%
B. Diet and exercise decreases progression by 70%

Answer:

12. 38-year-old woman is newly diagnosed with diabetes that has been uncontrolled
despite intensive diet and exercise. Routine workup shows creatinine 2.1, BMI 31, and
HgbA1c 8.4. Most appropriate therapy?
A. Glipizide
B. Glyburide
C. Metformin
D. Thiaglitazone
E. Long-acting insulin

Answer:

13. 24-year-old patient with fasting sugar of 140 mg/dl. Relatives have type 2. What is the
best test to tell if DM is type 1 or type 2?
Anti Glutamic acid dehydrogenase antibodies

Summary: Case 18
There are several ways to diagnose diabetes mellitus. Patients, who are symptomatic (poly-
uria, polydipsia) and have a random serum glucose >200 mg/dL, have a 2-hour postglucose
load of >200 mg/dL, or have a fasting glucose of >126 g/dL, are considered to have diabetes
mellitus. The preferred diagnostic test for diabetes mellitus is the fasting plasma glucose
(FPG). For gestational diabetes or the diabetes associated with polycystic ovarian syndrome,
the oral glucose tolerance test is the preferred test.

The best initial therapy in the treatment of diabetes mellitus is diet and exercise. The HgA1c
should fall between 0.5–1.0% on an appropriate diet and exercise regimen. Patients with
type 1 DM should be treated with insulin from the onset of their disease, because they have
an absolute insulin deficiency. When patients with type 2 DM have persistently elevated glu-
cose levels despite diet and exercise, oral hypoglycemic therapy should be started. In type 2
DM, metformin is the initial oral hypoglycemic of choice. In patients who are not obese, a
sulfonylurea may be considered, but with updated guidelines; sulfonylureas have fallen out

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of favor. In patients already on oral hypoglycemic therapy who need additional medication,
the second medication should be metformin if the patient was initiated on a sulfonylurea.
Thiazolidinediones are reserved for those patients on both metformin and a sulfonylurea
who need further control. Another option is to add bedtime glargine or NPH insulin in
these patients. Diabetics who present with symptomatic hyperglycemia that is >280 mg/dL
may be started on insulin until better glycemic control is achieved. At that point, they may be
switched to a regimen of oral hypoglycemic medications.

Goals for glycemic control include an HgA1c <7%, fasting plasma glucose between 90–130
mg/dL, and a peak postprandial glucose of <180 mg/dL.

Interventions in the Treatment of Type 2 Diabetes Mellitus Note

Resulting What percent does each of
Primary Mechanism of Common Side Decrease in the following reduce A1c by if
Intervention Action Effects HgA1c (%) taken properly?
Diet and exercise Trauma 0.5–2.0 Sulfonylureas 1%

Metformin GI upset, lactic 1.0–2.0 Metformin 1%

acidosis Exenatide 1%
Sulfonylureas Hypoglycemia, 1.0–2.0
weight gain
Note
Repaglinide Hypoglycemia, 1.0–2.0
weight gain In 5% of people what can
metformin case?
Acarbose Flatulence, GI 0.5–1.0
upset, weight gain Lactic acidosis
When is metformin
Thiazolidinediones Edema, weight 0.5–1.0
contraindicated?
gain
Serum Creatinine 1.4 in
Exenatide Nausea, vomiting 0.5–1.0 women, 1.5 in men; CHF no
Liraglutide Abdominal longer a contra-indication
discomfort, weight
loss, pancreatitis
Note
Sitagliptin Abdominal 0.5–1.0
discomfort, Patient is going for CT angio
pancreatitis with contrast. What drug do
you stop?
Metformin
When do you restart
48 hours after the CATH

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Case 19
56-year-old woman with a history of type 2 diabetes mellitus. She is concerned
about complications that may arise from her diabetes. She has no complaints.
Physical examination: elevated blood pressure.

1. Best initial therapy?

A. Hydrochlorothiazide
B. Lisinopril
C. Amlodipine
D. Metoprolol

Answer:

2. Major side effects?

3. How would you screen for diabetic nephropathy?

4. What are other causes of microalbuminuria?

5. What else is important in retarding the progression to renal failure?

The patient heard on the news that her diabetes can affect her eyes and cause
blindness.

6. What would you recommend? Are there other conditions that cause blindness in diabetics?

The patient’s friend told her that she is at higher risk for amputation.

7. What would you recommend to prevent that eventuality?

Several months later, this patient comes to the clinic complaining of a “burning”
sensation in her feet bilaterally. On neurologic examination, you note decreased
position and vibration sense and hyporeflexia of both patellar reflexes.

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8. What is the most appropriate therapy at this time?

A. Morphine
B. Ibuprofen
C. Acetaminophen
D. Duloxetine

Answer:

9. Patient with DM for 11 years on basal bolus regimen presents with hypoglycemic Note
attacks after eating and vomiting. BS is widely varied. Diagnosis?
Most common wrong answer
Gastroparesis
is high fiber in gastroparesis.
Best test
Nuclear gastric emptying study They need low fiber as fiber
slows digesting.
Treatment?
Small frequent meals with low fat and low in fiber

10. When is sildenafil indicated?

Your patient comes to the clinic complaining of fatigue and generalized weakness.

11. What would you do next? Why?

12. Patient with DM2 and elevated BUN/Cr of 40/3.7. What is the best medication
Repaglinide

13. Italian male with diabetes and G6PD presents with HBA1C of 6.5 but with fasting sug-
ars of 200. Why the difference?
2/2 to hemoglobinopathy

14. AA male with MCV of 114 and HBA1C of 11 but fasting blood sugar of 118. Why the
difference
2/2 to decreased RBC turnover in Macrocytosis (low retic)

15. Pt with DM2 and TG of 1500 admitted with pancreatitis. What is the fastest way to
control the triglycerides?
Insulin

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Summary: Case 19
In any diabetic patient with hypertension, the first-line therapy is an ACE inhibitor. The
major side effects of ACE inhibitors are hyperkalemia, renal failure (especially in the context
of renal artery stenosis), hypotension, and angioedema. Diabetic patients who cannot toler-
ate ACE-inhibitor therapy should be started on an ARB.

A spot urine for albumin and creatinine is the most effective way to screen for diabetic
nephropathy. An albumin-to-creatinine ratio that exceeds 30 mg/dL is indicative of abnormal
albuminuria. Hematuria, exercise, sustained upright posture, and GU tract infection are all
causes of proteinuria. It is for this reason that diabetic proteinuria must be confirmed over
a several-month period with repeat urinalysis. Tight glycemic control has been shown to
reduce the incidence of the microvascular complications of diabetes. Retarding the progres-
sion to overt nephropathy requires tight blood pressure control to <120/80 mm Hg, reduced
dietary protein intake, and tight glycemic control.

Diabetic patients have a higher rate of retinopathy than nondiabetics. Annual ophthalmolog-
ic evaluation is essential in preventing the progression of the retinopathy to blindness. Tight
glycemic control is also important. It should be noted that diabetics also have a higher rate
of cataracts and open-angle glaucoma, which are additional factors that predispose diabetic
patients to loss of eyesight.

Inspection of the diabetic patient’s feet at every visit, with education regarding proper foot
hygiene, is important in preventing chronic ulceration that can lead to amputation. Regular
podiatry evaluation is also recommended for the care of diabetic patient’s feet.

Diabetic neuropathy is peripheral neuropathy characterized by a glove-and-stocking distri-

bution. Autonomic neuropathy can manifest as gastroparesis, urinary incontinence, impo-
tence, and orthostatic hypotension. Treatment of the peripheral neuropathy associated with
diabetes consists of gabapentin, pregabalin, and the most recently approved anti-depressant
duloxetine. Metoclopramide is given to treat nausea and vomiting associated with autonomic
neuropathy of diabetes.

Diabetic patients with erectile dysfunction should be treated with sildenafil. The major con-
traindication to the use of sildenafil is concurrent use of nitrates.

Diabetics are at increased risk for the development of coronary artery disease. Additionally,
they do not present with typical symptoms of CAD. Thus, any typical symptoms, such as
fatigue, exertional shortness of breath, or abdominal pain, are indications for cardiac stress
testing in any patient with diabetes. The presence of 2 risk factors for CAD in addition to
diabetes is also an indication for stress testing, even in the absence of symptoms. Preventing
the macrovascular complications of diabetes involves aggressive control of smoking, high
cholesterol, and high blood pressure. Goals for lipid control include LDL <100 mg/dL, tri-
glycerides <200 mg/dL, and HDL >45 mg/dL.

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Case 20
54-year-old diabetic man complains of nausea, vomiting, and abdominal pain.
He has not been taking his medications over the last several days. EKG was
negative. Chemistries:

Na: 132 Cl: 97 Glucose: 763

HCO3: 16 K: 5.4 ABG: 7.14/32/90

1. Diagnosis:

2. Next step?

3. When would you start a glucose infusion in this patient?

A. Glucose <400
B. Glucose <300
C. Glucose <200
D. Glucose <150

Answer:

4. When would you initiate potassium infusion? Phosphate infusion?

5. How would you convert the IV insulin to subcutaneous insulin without causing hyper-
glycemia?

6. When is the answer hyperosmolar nonketotic coma?

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7. Which of the following is an indication to delay initiation of insulin infusion?

A. Anion gap <12
B. Moderate urinary ketones
C. Potassium 3.0
D. Serum bicarbonate >18
E. Venous pH >7.30

Answer:

8. 48-year-old man comes for follow-up of his diabetes. He currently takes 24 units of
glargine at night and 8 units of asparte insulin before meals. His finger stick log book
is as follows:
• AM 170-210
• Pre-lunch 120-150
• Pre-dinner 90-130
• Pre-bed 65-80

Next appropriate step in the management of this patient’s insulin?

A. Nothing, the patient is at goal most of the day
B. Decrease glargine insulin
C. Decrease pre-dinner asparte
D. Increase pre-breakfast asparte

Answer:

9. Patient returns for follow-up 3 months later. His finger stick log book is as follows:
• AM 150-190
• Pre-lunch 100-139
• Pre-dinner 90-130
• Pre-bed 85-120

10. Next appropriate step in the management of this patient’s insulin?

A. Nothing, the patient is at goal most of the day
B. Decrease glargine insulin
C. Decrease pre-dinner asparte
D. Increase pre-breakfast asparte

Answer:

11. Patient does well on glargine insulin and metformin. Repeat A1c shows increase. What’s
the next add on?
Lispro insulin

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Summary: Case 20

In the diabetic, who presents with nausea, vomiting, abdominal pain, and an anion gap
metabolic acidosis on regular chemistries, diabetic ketoacidosis (DKA) is the most likely
explanation. DKA occurs when there is a complete absence of endogenous insulin with unop-
posed action of the cortisol, glucagons, and catecholamines that induce the liver to produce
ketoacids. This counterregulatory surge is usually induced by some physiologic stress, such
as a myocardial infarction, stroke, or occult infection. Patients with DKA are severely dehy-
drated, are usually hypotensive, and require rapid and enormous amounts of normal saline.
The reason for their dehydration is the osmotic diuresis induced by high levels of glucose
present in their serum.

The next step in the management of any patient with DKA is to initiate IV-fluid therapy with
normal saline or Ringer lactate. After starting IV fluids, the next step is to start an insulin
drip. The reason for giving the insulin is to reduce and control the hyperglycemia, but more
importantly to oppose the counterregulatory hormones and reduce the severity of the keto-
acidosis. It is for this reason that you continue therapy with IV insulin despite normalization
of the blood glucose until the anion gap/ketoacidosis resolves. Dextrose is added to the IV
infusion when the glucose approaches 250 mg/dL. IV insulin should be continued until the
anion gap resolves.

DKA can induce false elevations of potassium when in fact the patient’s total body potassium
is reduced. In patients with adequate urine output, normalizing renal function, and a potas-
sium<5 mEq/L, potassium should be added to their IV infusion. Phosphorus is generally not
given unless the patient has severe hypophosphatemia with signs of rhabdomyolysis, heart
failure, or hemolysis.

Conversion to subcutaneous insulin should occur when the patient is beginning to eat. The
first dose of subcutaneous insulin should be given 30–60 minutes before stopping the insulin
infusion. In Type 2 DM, where insulin is present endogenously, it is very rare to develop DKA.
Hyperosmolar nonketotic coma is a metabolic state characterized by hyperglycemia, hyper-
osmolarity, and an absence of metabolic acidosis. These patients tend to be more dehydrated,
requiring a larger amount of fluid, and do not require IV insulin therapy.

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Case 21
45-year-old woman is brought to the emergency department with complaints of
confusion and anxiety. Her blood glucose is measured at 34 mg/dL.

1. Major causes of hypoglycemia?

Summary: Case 21
Patients who present with hypoglycemia can be divided into 3 groups.

1. Diabetics being treated with insulin or an insulin secretagogue. They should have their
treatment regimen adjusted and their blood glucose monitored.

2. The second group consists of patients with an obvious systemic disease that predisposes
them to low levels of blood glucose. End-stage liver disease, renal disease, sepsis, alcohol
abuse, Addison disease, use of pentamidine, or a history of previous gastric surgery are
all systemic disorders that can cause hypoglycemia. The management involves treating the
underlying disease and providing adequate glucose.

3. In the apparently healthy adult, distinguishing between endogenous and exogenous insulin-
caused hypoglycemia involves measurement of the insulin and C peptide in the plasma
during a hypoglycemic episode. Elevated C peptide occurs in patients with insulinomas
and patients who are taking sulfonylureas. Elevated insulin accompanied by normal level
of C peptide implies that the patient is self-administering exogenous insulin.

Hypoglycemia

The next diagnostic test is...

C peptide and plasma insulin

Normal C peptide Elevated C peptide

Elevated plasma insulin Elevated plasma insulin
Use of exogenous insulin Check urine for sulfonylurea
Consider insulinoma

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Case 22
24-year-old woman complaining of a “hairy face.” 1 year ago her voice began
to deepen and her menstrual periods have become irregular. Significant for hair
present on the upper lip and chin. You note severe acne and frontal balding.
Patient is also obese.

1. When is laboratory workup indicated?

2. 38-year-old woman presents complaining of excessive course facial hair over the past
3 months. Serum testosterone level is elevated. Serum cortisol and ACTH are normal.
Next step?
A. ACTH stimulation test
B. CT scan of the adrenal gland
C. DHEAs
D. Mechanical hair removal
E. Spirinolactone

Answer:

4. 27-year-old woman presents to the gynecologist complaining of irregular menstrua-

tions since puberty. Difficult to control hair on her face and abdomen. Blood tests
return with a testosterone of 88. She has been married for 2 years. She has been trying
to become pregnant without success. How to improve her fertility rate?
A. Finasteride
B. Spironolactone
C. Oral contraceptives
D. Metformin
E. Nothing, just time

Answer:

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Summary: Case 22
Hirsutism is caused by increased concentration of the active metabolite of testosterone, dihy-
drotestosterone, in the skin. Testosterone is converted to dihydrotestosterone by the enzyme 5
α-reductase. Hirsute women have higher levels of 5 α-reductase than do nonhirsute women.
The treatment of mild hirsutism usually consists of mechanical hair removal. Tumors of the
ovary, Cushing syndrome, and adrenal tumors can cause hirsutism. Polycystic ovary syn-
drome is also a common cause of hirsutism. Hirsutism can be caused by various medications,
including cyclosporine, steroids, danazol, diazoxide, or phenytoin. The decision to perform a
hormonal evaluation is based on the likelihood of finding a serious underlying cause for the
patient’s hirsutism. The presence of virilization, irregular menses, manifestations of Cushing
syndrome, or rapid progression of the hirsutism should prompt further investigation.

For mild hirsutism, treatment consists of mechanical hair removal. Antiandrogen therapy
in the form of spironolactone is the medical treatment choice for hirsutism. Finasteride is
a 5 α-reductase inhibitor that can be used for the treatment of hirsutism. For patients with
polycystic ovary syndrome (PCOS), metformin is sometimes effective in restoring normal
menses. If patients with PCOS want to become pregnant, clomiphene can be used to induce
ovulation. Most recently, in addition to clomiphene, metformin has been shown to be effec-
tive at improving ovulation by 33%; it also improves the insulin resistance associated with
PCOS. Likely, the insulin resistance is part of the underlying pathology for the anovulation
in PCOS. Patients with congenital adrenal hyperplasia should be treated with antiandrogen
therapy, such as spironolactone, cyproterone, or flutamide. In the pregnant female, all anti-
androgen medication is contraindicated.

Hirsutism

Does the patient have menstrual irregularity?

Virilization? Cushingoid appearance?

YES NO

Hormonal Proceed to
evaluation treatment

Elevated testosterone (>170 ng/dL) = Ovarian or adrenal tumor

Elevated DHEAS = Adrenal source of androgen

Elevated 17-hydroxyprogesterone = Congenital adrenal hyperplasia

(21-hydroxylase deficiency)

Ratio of LH to FSH >2.0 = Polycystic ovarian syndrome

Elevated prolactin = Prolactinoma (causing amenorrhea, not hirsutism)

Elevated LH and FSH = Ovarian failure (causing amenorrhea, not hirsutism)

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Case 23
38-year-old woman recently “lost” her regular menstruation. Menarche was at
the age of 15; and she has had regular menstrual cycles until 1 year ago, when
she noted that her cycle had become irregular. She has not had her period for
4 months. Physical examination normal.

1. First step in the workup of secondary amenorrhea?

2. Next step?

3. 32-year-old with amenorrhea for 5 months. She follows a strict diet, and runs 6 days a
week in preparation for a marathon. Prolactin level is normal. LH and TSH levels are
low. Next step?
A. Advise cutting down on exercise
B. Calcium and vitamin D supplementation
C. MRI of the brain
D. Progesterone challenge test

Answer:

4. 24-year-old woman has not had a menstrual cycle for 7 months. Her menses stopped
shortly after she began intense training for her college lacrosse team. Serum levels of
FSH, LH, prolactin, and beta-hCG are WNL. Next step?

A. MRI of the brain

B. Perform a progesterone withdrawal test
C. Measure TSH
D. Observation

Answer:

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5. 34-year-old woman complains of severe aching pelvic and rectal pain that is constant
and seems to begin 4–5 days before menses. Menstrual periods are also “extremely
heavy.” Pelvic examination reveals tender, indurated nodules in the cul-de-sac. Next
step in the management?
A. Exploratory laparotomy
B. Pelvic ultrasound
C. MRI of the abdomen
D. Prescribe low-dose oral contraceptives

Answer:

6. 23-year-old woman comes to your office complaining of irritability, bloating, ankle

swelling, and depression that starts 10 days before her monthly menses. Physical exami-
nation is within normal limits. Treatment of her mood changes?
A. Luteal phase danazol
B. Fluoxetine
C. A diet that incorporates complex carbohydrates
D. Diazepam

Answer:

Summary: Case 23
In evaluating a woman who complains of secondary amenorrhea, the first step is to rule out
pregnancy with a beta-hCG. If the patient is not pregnant, a serum prolactin and TSH level
should be checked to rule out hyperprolactinemia as the cause of the amenorrhea.

If the prolactin levels are elevated and the thyroid function studies are normal, the next step
is to evaluate the pituitary for the presence of a prolactin-secreting adenoma. If the prolactin
is within normal limits, the next step is to perform a progesterone withdrawal test.

If withdrawal bleeding is present, then the patient’s amenorrhea is secondary to anovulation,

most commonly associated with polycystic ovarian syndrome (PCOS).

If progesterone is given and there is no withdrawal bleeding, the next step is to check the LH
and FSH levels. If LH and FSH are elevated, the amenorrhea is secondary to ovarian failure.
If LH and FSH are found to be low or normal, the next step is to obtain an MRI of the brain
to evaluate for CNS/pituitary dysfunction as the cause of the patient’s amenorrhea.

Testing for the presence of an outflow obstruction as a cause of amenorrhea is indicated only
when there is clinical suspicion of an anatomical problem (e.g., history of uterine surgery). In
outflow tract defects, the prolactin will be normal and there will be no withdrawal bleeding
even when estrogen and progesterone are given together.

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Case 24
47-year-old man complaining of decreased desire to have sex with his wife.
Diminished erections over the last several months. Physical examination
decreased body hair and small testicles.

1. Best initial test?

A. LH and FSH
B. Prolactin
C. Serum free testosterone
D. Serum total testosterone
E. Testicular ultrasound

Answer:

2. Next most appropriate test in the workup?

3. 42-year-old man presents with decreased libido and gynecomastia. Examination

reveals small testes. Serum testosterone is normal; however, LH and FSH are elevated.
Is testosterone therapy indicated in this patient?

4. Most common sexual dysfunction is premature ejaculation.

5. Which medication can cause retrograde ejaculation SSRI?

6. 35 male has decreased libido and low testosterone levels. What’s the next best test?
FSH

7. pt with impotence and normal testosterone? Next step?

Stamp test

8. Best initial test after couple comes to you with difficulty conceiving?
Sperm analysis
How many days must they abstain
3 days prior to testing
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9. Best way to manage ED with patient on nitrates?

Penile tumescent devices

10. How soon after using sildenafil or tadalafil can nitrates be used
24 hours after sildenafil
48 hours after tadalafil

11. Amenorrhea: Primary- woman never had menarche, secondary- woman had menarche
and then menstruation stopped

12. Hirsutism, acute vs. chronic 28/F with significant facial hair and abdominal hair above
her belly button over the past 2 months; DHEA-s elevated, testosterone elevated; wtd
next?
Adrenal CT- r/o adrenal ca; 50% adrenal ca productive with DHEA-s, testo, and most
commonly cortisol
Just acute testosterone rise, scan ovaries

Summary: Case 24
Hypogonadism in males can be secondary to testicular failure (hypergonadotropic hypo-
gonadism) or to an inability of the pituitary to secrete adequate amounts of LH and FSH
(hypogonadotropic hypogonadism). The best initial test for the evaluation of hypogonadism
is to measure the serum testosterone. If the testosterone returns abnormally low, the LH and
FSH should be measured. Causes of testicular failure include Klinefelter syndrome, mumps
infection, radiation therapy, and testicular trauma. Causes of pituitary failure of LH or FSH
secretion in the male include alcohol abuse, leuprolide, Kallmann syndrome, hypothyroid-
ism, hyperprolactinemia, and marijuana use.

Diagnosis Testosterone LH FSH

Hypogonadotropic Low Normal or low Normal or low
hypogonadism
Hypergonadotropic Low High High
hypogonadism

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In testicular failure with hypogonadism, the treatment is to replace testosterone. For hypo-
gonadotropic hypogonadism, management involves evaluating for deficiency or excess of
other pituitary hormones. The treatment will be determined by the specific etiology of the
pituitary dysfunction.

Testosterone should be administered only to a man who has signs and symptoms of androgen
deficiency and a distinctly subnormal serum testosterone concentration. Giving exogenous
testosterone in a man with symptoms suggestive of hypogonadism, but whose testosterone
concentration is normal, will not relieve symptoms and is not currently indicated, even if LH
and FSH levels are above normal.

Primary
Pituitary Disease Craniopharyngioma, Adenoma; low FSH/
LH; + HA, visual disturbances
Mullerian Agenesis Proximal 1/3 of vagina present; distal 2/3
vagina, uterus missing; ovaries are func-
tional and estrogen normal➔ breast devel-
opment
Testicular Feminization/Androgen Atrophic/thin labia; small breast develop-
Insensitivity ment; high testosterone; testes on CT scan
visible
Pituitary Disease HA, visual field disturbances
Imperforate Hymen Painful, bulging vaginal mass monthly;
lower abd pain monthly

Secondary
Pregnancy Most common cause by far
Pituitary Adenomas in adults primarily; s/p radia-
tion Tx/surgery; PRLoma
PCOS Most common pathologic cause in the US;
hirsutism, elevated testo, DHEA-s, irregular
menstruation since menarche; sonogram
NOT NEEDED TO DX!
Thyroid Dysfunction Hyper or hypothyroidism can cause men-
strual abnormalities
Excessive exercise/low body fat Collegiate or professional athletes if body
fat < 4% body thinks woman is starving,
and hypothalamic releases change
Psycho-social stressors Psycho-social history should clarify

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 Hematology/Oncology
6
MICROCYTIC ANEMIA

Case 1
34-year-old woman comes to clinic for insurance forms. She is occasionally fatigued
but otherwise healthy. Uses no medications. Examination normal. CBC (complete
blood count): hemoglobin of 10 g/dL and hematocrit of 32%. MCV is 72 fl (low).

If THIS is in the …THIS is the most …And THIS is the best initial …And THIS is the best
history… likely diagnosis… and most specific tests… initial therapy
Blood loss, Iron deficiency Iron low, ferritin low, TIBC high Iron replacement
menstruation

Chronic disease Iron low, ferritin high, TIBC low Correct underlying cause;
erythropoietin for
end-stage renal disease

Sideroblastic anemia

Thalassemia Normal iron studies,

Electrophoresis

1. Which of the following change first with iron replacement?

A. Hematocrit
B. RDW
C. Reticulocytes
D. Hemoglobin level

Answer:

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2. A person with iron deficiency does not improve with oral ferrous sulfate. What would
you do first?
A. Blood transfusion
B. Vitamin C
C. Folate
D. Intravenous iron

Answer:

3. Which will decrease oral iron absorption?

A. Orange juice
B. Grapefruit juice
C. PPI
D. Pregnancy
E. Blood loss

Answer:

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1. Which is the anemia of chronic disease? NOTE

Iron deficiency
Iron Level TIBC Ferritin • Pica
(Normal 45–160) (Normal 220–420) (Normal 20–320) • Pagophagia (eating ice)
A. 34 450 12
B. 230 275 180
C. 32 440 140
D. 40 210 300

Answer:

2. 63-year-old woman is being evaluated for anemia. She drinks “socially” 6 nights a week.
No symptoms. MCV 70. White cell count normal. Platelet count 636,000. Most likely
cause of thrombocytosis?
A. Essential thrombocytosis
B. Alcoholism
C. Iron-deficiency anemia
D. Chronic myelogenous leukemia
E. Sideroblastic anemia
F. Anemia of chronic disease

Answer:

3. Which of the following is most likely to be Macrocytic?

A. Anemia of chronic disease
B. Renal failure
C. Hypothyroidism
D. One gene deleted alpha thalassemia

Answer:

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Anemias

Microcytic Microcytic Normocytic

MCV <83 MCV <100 MCV 83–100
• Iron deficiency anemia • Vit B12 deficiency • Renal failure
• Anemia of Chronic Dz • Folate deficiency • Hypothyroidism
• Thalassemia • Myelodysplasia • Chronic disease
• Sideroblastic anemia • Alcoholic/ Liver Dz • Aplastic anemia
• Blood loss
• Hypothyroidism
↑ Retic:
• Hemolysis
• Blood loss

4. 63 male with Hg 9 (normal 14-17 g/dl), MCV 72. Ferritin low. Stool negative for blood
three times. What to do?
A. No further evaluation needed
B. Upper endoscopy
C. Repeat stool test in one year
D. Colonoscopy

Answer:

5. Basophilic stippling is most likely to be found in:

A. Sideroblastic anemia
B. Folate deficiency
C. Hemolysis
D. Splenectomy

Answer:

NOTE Summary: Case 1

It is very difficult to answer the question, “Which of the following is the most likely diagno-
Iron saturation is: Iron/TIBC
sis?” of a microcytic anemia by history and physical examination alone. All forms of anemia
25%-45% normal give the same symptoms if they have an equal severity. Iron deficiency with a hematocrit of 22
gives the same symptoms as the anemia of chronic disease with a hematocrit of 22. A history
of blood loss, heme-positive stools, and menorrhagia suggests iron deficiency. Thalassemia
is suggested when the case shows a profoundly low MCV with relatively few symptoms.
Alcohol overuse in the history suggests sideroblastic anemia, as does lead or isoniazid use.
The smear is of very limited utility in the diagnosis of a specific cause of microcytic anemia.
All of the causes are hypochromic.

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Target cells are most likely associated with thalassemia and can be the answer to the question
“What is the best initial test?” The problem with this answer is that alcoholism itself and liver
disease can produce target cells as well. In general, microcytic anemia is a laboratory diag-
nosis. Do not memorize anything in physical examination to help you answer the diagnosis
question. When preparing for your exam, focus on the laboratory section. Iron studies, in
general, are the best way to distinguish between the different types of microcytic anemia.

The most specific iron study for iron deficiency is a low ferritin, although it lacks sensitivity.
The most specific iron study for sideroblastic anemia is a high iron, although it lacks sensitiv-
ity. Although it is rarely done, a bone marrow biopsy is the most accurate test for iron defi-
ciency anemia. Ringed sideroblasts are the most specific finding for sideroblastic anemia, but
they are not visible on routine blood smear. That is why the Prussian blue stain is the most NOTE
specific test for sideroblastic anemia. The iron studies will be normal in thalassemia, and the
most accurate test is the hemoglobin electrophoresis. Hepcidin is a gut peptide
hormone that regulates iron
What is the name of this cell? absorption.

What causes it?

Case 2
78-year-old alcoholic man comes to hospital from nursing home for confusion,
weakness, fatigue, and a pins-and-needles sensation in his hands and feet. He has
continued to drink while in the nursing home. Hematocrit 30% and MCV is 122.

1. Diagnosis?
A. Alcoholism
B. B12 deficiency
C. Folate deficiency
Answer:

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2. What test next?

A. B12 level
B. Folate level
C. Peripheral smear
D. Methylmalonic acid level
E. Homocysteine level
F. Bone marrow biopsy
G. Schilling test
H. Anti-intrinsic factor and anti-parietal cell antibodies
Answer:

NOTE 3. Most accurate diagnostic test?

Phenytoin is associated with A. B12 level

folic acid deficiency. B. Folate level
C. Peripheral smear
D. Methylmalonic acid level
E. Homocysteine level
F. Bone marrow biopsy
G. Schilling test
H. Anti-intrinsic factor and anti-parietal cell antibodies
Answer:

4. Best initial therapy?

B12 or folate replacement

5. Most common initial complication of therapy?

A. Metabolic acidosis
B. Alkalosis
C. Hypokalemia
D. Hyperkalemia
Answer:

6. Man comes for evaluation of fatigue. On exam, he is found to have decreased sensation
in lower extremities. Hematocrit is 32, MCV 112. Smear shows hypersegmented neu-
trophils. B12 and folate levels are normal. Which is the most specific diagnostic test?
A. Schilling test
B. LDH (lactate dehydrogenase)
C. Bone marrow
D. Reticulocyte count
E. Red-cell folate level
F. Methylmalonic acid level

Answer:

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7. Man has developed anemia with MCV 115. Smear shows hypersegmented neutrophils.
No neurologic symptoms. Which medication should be avoided?
A. Alpha-methyldopa
B. Vitamin B12
C. Antibiotics
D. Quinidine
E. Trimethoprim

Answer:

8. Deficiency of which of these increases blood level of homocysteine?

A. Folic acid
B. Vitamin B12
C. Both

Answer:

9. What medication decreases B12 absorption the most?

Summary: Case 2 NOTE

Macrocytic anemia specifically means an elevated MCV. Megaloblastic anemia means that
the MCV is elevated and the neutrophils are hypersegmented. Normally, the average number Iron: increased reticulocytes
of lobes in each white cell is 3.5. If the average is >4 or if there is a single 6-lobed neutrophil,
the diagnosis of megaloblastic is confirmed. Deficiency of vitamin B12 and folic acid cause
almost all types of megaloblastic anemia. Myelodysplasia and certain medications, such as
methotrexate, azathioprine, 6MP, 5FU, and folate antagonists such as trimethoprim, can also
cause megaloblastic anemia. Simple macrocytosis or large cells can be seen with liver disease,
alcoholism, hypothyroidism, and anything that leads to an increased reticulocytosis, such as
blood loss and hemolysis. Reticulocytes are slightly larger than normal red cells.

After a peripheral smear confirms the presence of a megaloblastic anemia, levels of B12 and
folate will help confirm a specific diagnosis. Methylmalonic acid levels are elevated in B12
deficiency but not in folate deficiency. Methylmalonic acid levels are most useful when you
suspect B12 deficiency but the level is normal or equivocal. Schilling test is rarely necessary.
Antibodies against intrinsic factor and parietal cells have sufficient accuracy in the diagnosis
of pernicious anemia that if they are positive, the Schilling test is not necessary. Since B12
and folate deficiency are hematologically identical, treatment with folate can correct the
hematologic abnormalities of B12 deficiency but not the neurologic abnormalities. Beware
of the rapid development of hypokalemia when treating a severe megaloblastic anemia.
Extremely rapid production of cells leads to the intracellular incorporation of potassium
and hypokalemia.

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 IM Board Review Course Book

HEMOLYSIS
If the case history says that the patient’s anemia, or symptoms of anemia, have developed
acutely (in hours to days), then it can only be these 2 types of problems: hemolysis and blood
loss.

All forms of hemolysis have the following lab tests in common:

• Increased reticulocyte count
• Increased LDH
• Increased indirect bilirubin
• Decreased haptoglobin
• Normocytic (sometimes slightly macrocytic)

1. If hemolysis is intravascular, there will also be these other laboratory abnormalities:

2. Which hemolysis will show bilirubin in the urine?

Case 1
Man comes to ED because of several hours of severe chest, back, and thigh
pain. He has a history of sickle-cell disease. He denies a history of cholelithiasis,
osteomyelitis, or hip problems (aseptic necrosis). Temperature 36.6°C (98°F)
orally. No ulcers on legs. Oximeter saturation of 100%.

1. Best initial test to confirm disease?

A. Hemoglobin electrophoresis
B. Bone marrow biopsy
C. Reticulocyte count
D. Peripheral smear
E. LDH and bilirubin level

Answer:

2. Most accurate test?

A. Hemoglobin electrophoresis
B. Bone marrow biopsy
C. Reticulocyte count
D. Peripheral smear
E. LDH and bilirubin level

Answer:
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 Hematology/Oncology

3. Best initial step?

A. Oxygen, fluids, and analgesics
B. Ceftriaxone
C. Hydroxyurea
D. Folate

Answer:

4. When will antibiotics be the best initial step in management, besides when there is an
obvious complaint or site of infection?

5. What antibiotic?

6. Several hours after starting therapy, patient develops dyspnea, worse chest pain, hypoxia, NOTE
and a new pulmonary infiltrate. Troponin levels are normal. Howell-Jolly bodies occur
What will you do now? when spleen is missing.

A. Heparin
B. Antibiotics
C. Exchange transfusion
D. Hydroxyurea

Answer:

7. In the following day or two, the man’s hematocrit drops from 32 to 21 (excluding those
associated with transfusion above).

What is the most likely diagnosis?

8. Best initial test?

A. IgM level
B. Reticulocyte count
C. IgG level
D. Bone marrow biopsy
E. PCR for DNA

Answer:

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9. Most accurate test?

A. IgM level
B. Reticulocyte count
C. IgG level
D. Bone marrow biopsy
E. PCR for DNA

Answer:

10. Best initial therapy?

11. The following day, he is all better. What will you give him on discharge?

12. Sickle cell patient develops sudden HIP PAIN, what is the cause? What test?
A. Osteomyelitis
B. Bone infarct
C. Bursitis
D. Aseptic necrosis

Answer:

Summary: Case 1
Sickle-cell disease gives painful crises of unclear etiology. Although hypoxia, acidosis, and
infection lead to crises, you can still have a crisis in their absence.

The peripheral smear is the initial clue to the diagnosis because the homozygous disease will
have sickled cells and AS disease or trait will not.

You do not have to routinely give antibiotics to all patients. Besides being given to patients
with obvious infections, antibiotics are indicated just for a fever or an elevated white cell
count because adult patients who have sickle-cell disease have no functioning spleen.
Ceftriaxone or the fluoroquinolones, such as levofloxacin, moxifloxacin, or gemifloxacin, are
good choices. Patients with life-threatening crises, such as a stroke, priapism, or lung involve-
ment leading to pulmonary infarction and hypoxia, should undergo exchange transfusion.

Parvovirus B19 infection in the presence of a hemoglobinopathy is recognized when there

is a sudden drop in the hematocrit that cannot be explained otherwise. Although the IgM
antibody to parvovirus is fairly specific, the most specific diagnostic test is the PCR for par-
vovirus B19 DNA. Severe anemia secondary to parvovirus B19 is treated with intravenous
immunoglobulins. For long-term management, all patients with sickle-cell disease should
receive pneumococcal vaccination and folic acid supplementation. Those with frequent crises
should also receive hydroxyurea to decrease the frequency of crises.

Although bone marrow transplantation can be curative for sickle-cell disease, it is rarely—if
ever—performed in an adult.

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Case 2
20-year-old man on hemoglobin electrophoresis, is found to have sickle-cell trait or
heterozygous disease.

1. What would you find on CBC and smear?

2. Man wants to know what he can expect to happen to him. What do you tell him?
A. Visual disturbance
B. Splenic infarction
C. Renal involvement
D. Osteomyelitis
E. Gallstones

Answer:

3. Could this be SC (sickle-hemoglobin C) disease, and how will you distinguish it from
SS (sickle-cell anemia) and AS disease?

4. What presentation is most unique for SC disease?

A. Visual disturbance
B. Splenic infarction
C. Renal involvement
D. Osteomyelitis
E. Gallstones

Answer:

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5. Recently married couple each is HETEROzygous (AS) for sickle cell. What is the chance
of homozygous (SS) child?
A. 25%
B. 50%
C. 75%
D. 100%

A S
A
S

Answer:

Summary: Case 2
Sickle-cell trait is most often a clinically silent disorder. The most common manifestation
is painless hematuria and a renal tubular concentrating defect. Sickle-cell trait rarely gives a
painful crisis, and this usually occurs only under conditions of extreme hypoxia, dehydration,
and acidosis. SC disease is seen in a patient who is heterozygous for both sickle-cell disease
and hemoglobin C. SC disease acts as if it were halfway between SS and AS diseases. There
is anemia and reticulocytosis, but they are milder than with SS disease. There are painful
crises with SC disease, but they occur less frequently and with fewer infections than with SS
disease. The most characteristic feature of SC disease is the retinopathy. Life expectancy with
SC disease is 20 years longer than with SS disease, and there is much greater preservation of
spleen function.

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Case 3
If THIS is in the …Then THIS is the …And THIS is the best initial …And THIS is the best
history… most likely diagnosis… and most specific tests… initial therapy
Collagen-vasc diseases, Autoimmune hemolysis, Coombs test Steroids; splenectomy
SLE, RA, CLL lymphoma, drug-induced if recurrent; IV
IBD, viral infections; immunoglobulins if
drugs: penicillin, severe
quinidine, methyldopa;
50% idiopathic
Mycoplasma, EBV, Cold agglutinin disease Coombs positive for complement
lymphoma, CMV Agglutination in the cold
IgM antibody titer

Hereditary spherocytosis Splenectomy; folic acid

supplementation
Hemolytic uremic
syndrome (HUS)
Thrombotic
thrombocytopenic
purpura (TTP)
Glucose-6-phosphate
dehydrogenase
deficiency
Paroxysmal nocturnal
hemoglobinuria
Definition of abbreviations: CLL, chronic lymphocytic leukemia; CMV, cytomegalovirus; EBV, Epstein-Barr virus; IBD,
inflammatory bowel disease; RA, rheumatoid arthritis; SLE, systemic lupus erythematosus.

1. Woman at 37 weeks of pregnancy with right upper quadrant abdominal pain and vomit-
ing. BP 168/108 mm Hg, normal temperature, and modest peripheral edema. Her hema-
tocrit is 25, with 16% reticulocytes, platelets of 24,000, schistocytes and fragmented cells on
peripheral smear, and a urinalysis with marked proteinuria. AST and ALT elevated at 370
and 390. Prothrombin time and aPTT (activated partial thromboplastin time) are normal.
What is the diagnosis, and what should you do about it?
A. TTP; do plasmapheresis
B. DIC; give platelets
C. HUS; do plasmapheresis
D. Pre-eclampsia; give hydralazine or labetalol
E. HELLP syndrome; perform immediate delivery

Answer:

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Summary: Case 3
Given that all forms of hemolysis have an elevated reticulocyte count, LDH, and indirect
bilirubin level, the emphasis in studying the other forms for hemolytic anemia is to assess
what one or two things are unique about each one to allow you to decide on the best initial
and the most accurate diagnostic tests.

Autoimmune hemolysis is idiopathic in up to half of cases, but it is difficult to ask you a

Board question about the idiopathic type unless there is a positive Coombs test or negative
tests for all the other diseases. Autoimmune hemolysis is suggested by the presence of other
autoimmune diseases, such as SLE, rheumatoid arthritis, viral infections, and lymphocytic
malignancies such as CLL or lymphoma. Autoimmune hemolysis is also suggested by the
presence of allergen-stimulating medications in the history, such as penicillin, quinidine,
alpha-methyldopa, or sulfa drugs. The best initial therapy for autoimmune hemolysis is the
same as for the autoimmune disease, namely, steroids. If the hemolysis continues to recur,
the best next step in management is splenectomy. Cold agglutinin disease is treated with
rituximab or alkylating agents to shut off antibody production.

The most important thing to know about cold agglutinin disease is that it is not treated with
steroids or splenectomy.

Hereditary spherocytosis is the cause of hemolysis most commonly associated with spleno-
megaly. This is because it is the type of hemolysis that is most often a chronic, long-term
disorder. Splenomegaly needs time to develop. Although autoimmune hemolysis can be
associated with the presence of spherocytes, hereditary spherocytosis has the greatest degree
of increase in osmotic fragility.

Although hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura

(TTP) are really different spectra of the same disease, HUS is suggested by the presence of
E. coli 0157:H7 on history. TTP is suggested by the additional presence of fever and neuro-
logic abnormalities in an adult. Ticlopidine in the history suggests TTP. Both HUS and TTP
are diagnosed by the clinical/laboratory triad (HUS) or pentad (TTP), and neither really has
a unique specific test. Both are treated with plasmapheresis in adults.

TTP should not be treated with platelet transfusions.

Although sulfa drugs lead to hemolysis in glucose-6-phosphate dehydrogenase (G6PD) defi-

ciency, the most common cause of hemolysis is infection. Remember that immediately after
the hemolysis the G6PD level will be normal. Use the peripheral smear and the presence of
Heinz bodies and bite cells to establish a diagnosis. Avoiding oxidant stress in this disorder is
the only long-term management.

The most common cause of death in paroxysmal nocturnal hemoglobinuria (PNH) is

thrombosis.

Do not choose Ham test or the sugar water test as the diagnostic test. Use the CD55-59 assay,
which is the decay accelerating factor. Severe PNH is treated with eculizumab.

1. Which form of hemolysis is most likely to occur in men?

2. Which form of hemolysis is most likely to occur in men only? Check spelling?
Check with author whether this is what he wanted?

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Hemolysis Review Questions

1. A patient is post upper endoscopy and suddenly turns cyanotic and short of breath.
EKG normal.
ABG: pO2 96, Pulse Oximeter: 85% saturation
What is the diagnosis?

What is the treatment?

2. Heinz bodies are found in:

A. Sickle cell
B. Alpha thalassemia
C. Leukemia
D. Cold agglutinin disease

Answer:

3. 42 patient comes with dry cough bilateral interstitial infiltrates x-ray. After start of
azithromycin, there is a drop in the hematocrit and the smear shows clumping of red
cells. What is the most likely diagnosis?
A. Autoimmune, warm IgG antibodies
B. Autoimmune, cold IgM antibodies
C. Glucose 6 phosphate dehydrogenase deficiency
D. Hereditary spherocytosis
E. Hemolytic uremic syndrome (HUS)

Answer:

4. 23 African-American with sickle-cell disease comes with rhinorrhea with clear dis-
charge. Hematocrit drops from 36% to 28%; and platelets drop to 75,000. No rash, but
there are severe joint pains. What is the most likely diagnosis?
A. Acute rheumatoid arthritis
B. Drug reaction
C. Parvovirus infection
D. Allergic reaction
E. Splenic sequestration

Answer:

Summary
The presence of Heinz bodies on smear after the use of a sulfa medication indicates glucose
6 phosphate dehydrogenase deficiency. Splenomegaly with bilirubin gallstones and increased
osmotic fragility indicate the presence of hereditary spherocytosis. Mycoplasma pneumonia
is associated with IgM cold antibodies. Sickle cell disease and other forms of hemoglobin-
opathy put a patient at risk of parvovirus B19 infection. Parvovirus also causes arthralgia.

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Methemoglobinemia is an idiosyncratic reaction to anesthetics such as the benzocaine for upper

endoscopy or bronchoscopy. The patient becomes cyanotic with dissociation between pO2 and
oxygen saturation. The saturation is even lower than would be expected from the pO2.

Acanthocytes = SPUR cell = Liver disease

Echinocytes = BURR cells = Uremia/Low Erythropoietin

BLISTER cells = Bite cells

Which of these has schistocytes?

A. ITP
B. DIC
C. Autoimmune
D. Thalassemia
E. Spherocytosis

Answer:

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LEUKEMIAS

Case 1
70-year-old man with fatigue over last several months. Spleen is normal. CBC
reveals a pancytopenia that is mild. MCV is mildly elevated and reticulocyte count
low. Smear shows oval cells. No hypersegmentation to neutrophils, although some
are bilobed. Very small number of blasts are seen (<1%). B12 and folate levels are
normal. Bone marrow shows a hypercellular marrow with ringed sideroblasts on
Prussian blue stain and a small number of blasts (<5%).

1. Diagnosis?

2. What will you see on smear?

3. Most important measure of severity or prognosis described?

A. Platelet count
B. Hematocrit
C. Presence of hypersegmentation
D. Percentage of blasts

Answer:

4. Most common cause of death?

A. Bleeding and infection
B. Acute myelogenous leukemia
C. Acute lymphocytic leukemia
D. Leukostasis reaction
E. Lymphoma

Answer:

5. Best initial and most effective therapies?

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Summary: Case 1
Myelodysplasia is a clonal stem-cell disorder resulting in a hypercellular marrow with pancy-
topenia. It can resemble B12 and folate deficiency and gives an elevated MCV as well as possi-
ble megaloblastic changes. The peripheral smear is characterized by macro-ovalocytes as well
as the bilobed Pelger-Huet abnormality. The main prognostic factor is the number of blasts.
As the number of blasts increases, the disease acts more like acute myelogenous leukemia.
The most common cause of death, however, is not AML but rather infection and bleeding
from bone marrow failure. Although only 20% of patients respond, it is important to give a
trial of erythropoietin. In patients under age 60 with severe disease, allogeneic bone-marrow
transplantation may be curative. It is associated with deletions of chromosome 5q and 7.

Case 2
56-year-old woman comes because of fever of 38°C (100.5°F), progressive fatigue,
and easy bruising. She was born in Nagasaki in 1945, went to the University of
Chernobyl, and worked briefly in Bhopal, India. She is currently a benzene taste-
tester in a radon detection company. WBC count 3,000, hematocrit 23, platelet
count 34,000.

1. Diagnosis?

2. What test would you do first to confirm her diagnosis?

Diagnosis Acute Myelogenous Leukemia Acute Lymphocytic Leukemia

(AML) (ALL)
Specific diagnostic
tests
Treatment

3. Most important indicator of prognosis in this patient? And what is the impact of that
on management?

4. When is ATRA (all-trans retinoic acid) indicated?

5. What therapy in a patient who has relapsed after first remission?

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Summary: Case 2
Acute leukemia presents with the signs of pancytopenia, such as fever, infections, fatigue from
the anemia, and increased bleeding from the low platelet count. Although more than half of
patients with acute leukemia may have a normal or elevated white cell count, the cells are not
functioning normally and the patients still have infections. The presence of >20% blasts helps
distinguish acute leukemia from other causes of pancytopenia. Stains such as Sudan black,
myeloperoxidase, and Auer rods can point to a diagnosis of acute myelogenous leukemia,
but using monoclonal antibodies to look for specific antigens is more specific. Daunorubicin
(or idarubicin) combined with high-dose cytosine arabinoside (ARA-C) is the most com-
mon regimen used in this disorder. Bone marrow transplantation is used in any patient who
relapses after initial induction chemotherapy. The hardest question is whether or not to do
a transplantation after the initial induction therapy. Primary transplantation is performed
initially after chemotherapy when there is the presence of poor cytogenetic markers, such as
9:22 translocation in ALL as well as monosomy 5 and 7 in AML.

43-year-old woman with fever, leukocytosis, and erythematous plaques and nodules on face/
extremities. Lesions are tender.

Biopsy shows dense neutrophilic infiltrates. Blood smear shows blasts.

What is the diagnosis?

Case 3
Man comes because of left upper quadrant pain, early satiety, and fatigue. He has
left upper quadrant tenderness and enlarged spleen. CBC reveals a white blood
cell (WBC) count of 85,000.

1. Best initial test?

A differential of the white cell count, which in this case reveals 91% neutrophils

2. Most likely diagnosis?

3. What else on the CBC is characteristic of this disorder?

Increased basophils and occasionally an elevated platelet count

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4. Most accurate diagnostic test?

A. Bone marrow biopsy
B. Philadelphia chromosome (BCR/ABL) by PCR
C. Leukocyte alkaline phosphatase (LAP) score
D. Beta 2 microglobulin level
E. Abdominal CT scan

Answer:

5. Best initial therapy?

A. Bone marrow transplantation
B. Interferon
C. Imitanib (Gleevec®)
D. Hydroxyurea
E. Busulfan

Answer:

6. The following day, before you are able to give any therapy, the patient begins to complain
of headache, dyspnea, confusion, and priapism. His white cell count has risen to 218,000.

What is the best immediate therapy for this man?

7. What other therapy will lower the WBC count?

8. When is busulfan indicated?

Summary: Case 3
Chronic myelogenous leukemia is diagnosed when you find an elevated white-cell count that
is mostly neutrophils and a reactive leukemoid reaction has been excluded. The leukocyte
alkaline phosphatase will be elevated in reactive leukocytosis but will be decreased with a leu-
kemia. The Philadelphia chromosome will be present in chronic myelocytic leukemia (CML).
Initial therapy of CML is with the tyrosine kinase inhibitor imatinib or Gleevec. There is an
excellent hematologic response in as many as 90% of cases, and the Philadelphia chromo-
some becomes negative in as many as 60%. If this response is not sustained, then all patients
under the age of 50 with a matched donor should undergo transplantation before the disease
converts into acute leukemia.

Interferon is no longer used. It has far less efficacy and far more adverse effects.

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Case 4
74-year-old man for routine visit. No specific complaints, beyond a little fatigue.
Normal physical exam with no organomegaly. 45,000 white cells that are 98%
lymphocytes. Platelet count and hemoglobin are normal. CD5 and CD23 are
positive.

1. Most likely diagnosis?

2. Best therapy?
A. No treatment necessary
B. Fludarabine
C. Melphalan
D. Chlorambucil
E. Bone marrow transplantation

Answer:

Case 5
60-year-old man with fatigue and anemia. Coombs test is negative, but you
are concerned about the palpable lymph nodes and the white count of 80,000
lymphocytes.

1. Best therapy?
A. No treatment necessary
B. Fludarabine
C. Melphalan
D. Chlorambucil
E. Bone marrow transplantation

Answer:

Summary: Cases 4–5

The therapy for CLL depends upon the stage of the disease. In older patients with early stage-
zero or stage-one disease, there is no evidence that treatment alters survival. Older patients
with severe disease can be treated with chlorambucil and prednisone as palliation to reduce
the cell count. Advanced-stage disease in relatively younger patients (<70) is treated with
fludarabine. There is virtually no use of bone marrow transplantation in CLL because the
patients are too old to survive an allogeneic transplantation.

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Case 6
55-year-old man with gradual onset of fatigue, early satiety, and abdominal pain.
Exam massive splenomegaly. CBC shows a pancytopenia, and a bone marrow
biopsy shows an inaspirable dry tap.

1. Diagnosis?

2. Most specific diagnostic test?

3. Therapy?

Summary: Case 6
Pancytopenia has a large differential, including any metastatic cancer to the marrow; infec-
tions, such as tuberculosis; fungi invading the marrow SLE; HIV; paroxysmal nocturnal
hemoglobinuria; and viruses, such as EBV, CMV, and hepatitis B and C. Most of these will
be diagnosed by the bone marrow biopsy. When there is a dry, inaspirable marrow combined
with splenomegaly and the absence of blasts in a middle-aged male patient, your answer as
the most likely diagnosis should be hairy cell leukemia. Aplastic anemia should not have
splenomegaly. Splenic sequestration would be associated with a fully productive marrow.
Hairy cell is confirmed by finding the cytoplasmic projections on the lymphocytes as well
as immunotyping of the cells. The treatment of hairy cell leukemia is 2-CDA or cladribine.

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CLL Review Questions

1. 71-year-old man admitted for hernia repair. White cell count is 30,500 with 79% lym-
phocytes. There are smudge cells visible on smear. Platelet count is 146,000, hematocrit
37%. Bone marrow biopsy shows that 70% of marrow is replaced with lymphocytes.
Best management?
A. Observation
B. Chlorambucil
C. Fludarabine
D. Prednisone
E. Bone marrow transplantation

Answer:

2. 73-year-old man with CLL has recurrent episodes of streptococcal pneumonia. White
cell count is 30,000. What is the etiology?
A. Effects of chemotherapy
B. Bone marrow infiltration
C. Hypogammaglobulinemia
D. Neutrophil dysfunction

Answer:

3. 78-year-old man with CLL has palpable spleen and anemia with a reticulocyte count of
8%. What is the treatment of his anemia?
A. Splenectomy
B. Erythropoietin
C. Interferon alpha
D. Prednisone

Answer:

Summary
Smudge cells are a sign of CLL. The nucleus of the cells is fragile; and when the cover slip is
placed, it ruptures the cells so they appear ‘smudged’. Stage 0 CLL is the presence of an elevat-
ed white cell count only. This does not require specific therapy for the CLL. The main reason
people with CLL have recurrent bacterial infections is that the lymphocytes do not produce
antibodies. These patients are often treated with monthly infusions of intravenous immu-
noglobulins. CLL is also associated with Coomb positive hemolytic anemia. This is treated
with prednisone. The key to knowing it is autoimmune hemolysis is the elevated reticulocyte
count. If anemia is from progression of the CLL, the reticulocyte count will be low.

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MYELOPROLIFERATIVE DISORDERS

Case 1
60-year-old man comes because of headache, dizziness, blurred vision, and
fatigue. Generalized pruritus, which is especially severe after he comes out of
the shower. Does not smoke. Exam: splenomegaly and some dried blood in the
nostrils. Hematocrit 62, MCV is 75, white cell count is 19,000, and platelet count
is 700,000.

1. Diagnosis?

2. What information in history and physical suggests primary polycythemia?

A. Pallor
B. Erythema
C. Splenomegaly
D. Lymphadenopathy

Answer:

3. What tests will you perform first?

• Arterial blood gas on room air to exclude hypoxia as a cause of the increased hematocrit
• Renal ultrasound to exclude an erythropoietin-secreting cyst or tumor
• The erythropoietin level should be decreased and the B12 level

4. Most accurate diagnostic test?

A. BCR-ABL
B. JAK-2
C. Myeloperoxidase
D. Smudge cells
E. CBC

Answer:

5. What medication can cause a hematologic picture like this one?

A. Testosterone
B. Growth hormone
C. Erythropoietin
D. None

Answer:

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6. Best initial therapy?

7. Best medical therapy if patient does not respond?

8. Most common cause of death?

Summary: Case 1
Polycythemia can occur as a reaction to hypoxia, so the first step in diagnosing polycythemia
vera is to find an oxygen saturation >92% on room air. Reactive polycythemia is not associ-
ated with splenomegaly or an elevation of the white cell or platelet count. In addition, there
are only 3 causes of a microcytic erythrocytosis: polycythemia vera, thalassemia, and hypoxia.
After excluding hypoxia, an elevated red cell mass confirms the diagnosis.

The most common cause of death in polycythemia vera cases is thrombosis. The mainstay of
therapy is phlebotomy. If phlebotomy does not control the red cell count, or if the white cell
and platelet counts are very high, then hydroxyurea will help lower the cell counts. Although
testosterone can cause an elevated red cell count, it should not elevate either the white cell
or platelet count. In addition, reactive erythrocytosis and testosterone effect on the marrow
will not enlarge the spleen.

Case 2
73 with polycythemia vera well maintained with phlebotomy for several years.
Hematocrit lower over last several months. Now very tired. Spleen big. Hematocrit
28% Blood smear: teardrops shaped cells. Nucleated red cells.

1. Most likely etiology?

A. Parvovirus B19
B. Myelofibrosis
C. Hemolysis
D. Splenic sequestration

Answer:

2. What is the therapy?

A. IVIG
B. Steroids
C. Lenalidomide
D. Hydroxyurea

Answer:
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Case 3
55-year-old woman comes because of painful burning of her hands. Hands are
red and there is mild splenomegaly. CBC reveals platelet count of 1,400,000, white
blood cell count of 14,000. Hematocrit is normal.

1. Diagnosis?

2. Most common complication of this disorder?

A. Bleeding
B. Thrombosis
C. Both bleeding and thrombosis
D. Transformation to acute myelogenous leukemia

Answer:

3. Therapy?

4. Which of the following is most likely to progress to acute leukemia within 5 years?
A. Polycythemia vera
B. Essential thrombocythemia
C. Chronic myelogenous leukemia
D. Chronic lymphocytic leukemia

Answer:

5. 34 man with 750,000 platelets on CBC done for other reasons. Feels well. Takes no
meds. What to do?
A. Anagrelide
B. Hydroxyurea
C. Observation
D. Steroids
E. Thrombopheresis

Answer:

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Summary: Case 3
Essential thrombocytosis is an uncommon disorder in patients over age 50. It presents with
either bleeding or thrombosis. If the patient is asymptomatic or has only the painful, red
hands known as erythromelalgia, then anagrelide is the best therapy to lower the platelet
count with aspirin to control the erythromelalgia. If your question shows a patient who is
over the age of 65 with thrombosis, then your answer is hydroxyurea and aspirin. It is bet-
ter to avoid hydroxyurea in people under age 65 because of the risk of possible secondary
malignancy. If your patient presents with bleeding and an extremely high platelet count, then
thrombopheresis is the answer.

Which of the following causes of leukocytosis will have a normal vitamin B12 level?
A. CML
B. Leukemoid reaction
C. Polycythemia vera

Answer:

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PLASMA CELL DISORDERS

Case 1
62-year-old man with sharp pain in his chest when he coughs. X-rays of his ribs
reveal several fractures as well as several lytic lesions. Hemoglobin 9, mildly
elevated calcium level of 11.7, creatinine 1.8, total protein elevated.

1. Diagnosis?

2. Most specific diagnostic test?

A. Serum protein electrophoresis
B. Urine electrophoresis for Bence-Jones protein
C. Bone marrow biopsy
D. Skeletal survey
E. Peripheral smear

Answer:

3. What other laboratory abnormalities are associated with this disorder?

Bence-Jones protein in the urine, an elevated beta-2 microglobulin, hyperuricemia, elevat-
ed ESR (erythrocyte sedimentation rate), low anion gap, rouleau formation of red cells on
peripheral blood smear, and suppression of the other immunoglobulin lines (such as IgM
or IgA) on the SPEP

4. Most effective therapy?

A. No treatment
B. Melphalan, prednisone, lenalidomide
C. Thalidomide
D. Autologous stem cell transplant
E. Vincristine, adriamycin, dexamethasone

Answer:

Summary: Case 1
Autologous stem cell transplantation is attempted in patients with myeloma who are under
the age of 70. The degree of immunosuppression and the duration of absolute neutrope-
nia are far less with autologous transplantations than with an allogeneic transplantation.
Mortality in a bone marrow transplantation is directly related to the duration of the neutro-
penia. Stem cells engraft much sooner and result in a viable bone marrow and resolution of
cytopenias in a few days. In addition, there is no graft-versus-host disease with autologous
transplantation. This is why transplantation can be performed even in older patients.

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Case 2
You are evaluating a 72-year-old man because of an elevated total protein found
on a routine blood test. He has a monoclonal spike of 3.2 g/dL. Hemoglobin,
calcium level, and urine for Bence-Jones protein are normal. Skeletal survey
normal, and bone marrow biopsy reveals 4% plasma cells.

1. Diagnosis?

2. Management?
A. No treatment
B. Melphalan and prednisone
C. Thalidomide
D. Autologous stem cell transplant
E. Vincristine, adriamycin, dexamethasone

Answer:

Summary: Case 2
As the population ages, monoclonal gammopathy of uncertain significance (MGUS) is only
going to become more common because it is a disease seen almost exclusively in those above
the age of 70. You recognize the case because of an increased total protein that, on electropho-
resis, is found to have an elevated monoclonal spike. The size of the spike is lower than that
of myeloma. MGUS does not have any of the usual manifestations of myeloma, such as bone
lesions, hypercalcemia, Bence-Jones protein in the urine, or renal failure. In addition, there
will be <10% plasma cells in the marrow. Although 1% of MGUS cases per year progress to
myeloma, you cannot predict which cases will do so. There is no effective therapy.

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Case 3
67-year-old man with fatigue, confusion, vertigo, and blurry vision. Retinal veins
are engorged. Hematocrit 32 and total protein elevated. Head CT scan and calcium
levels are normal. Protein electrophoresis shows IgM monoclonal spike. Skeletal
survey is normal. No Bence-Jones protein in urine.

1. Diagnosis?

2. Therapy?
A. Steroids
B. Plasmapheresis
C. Fludarabine
D. Melphalan

Answer:

Summary: Case 3
Waldenström macroglobulinemia has many similarities to myeloma in that it presents with
an elevated total protein with a monoclonal IgM spike. One of the main differences is that the
spike is IgM, not IgG. Unlike myeloma, there are no bone lesions, hypercalcemia, Bence-Jones
proteins, or renal failure. The only clear therapy is plasmapheresis for the hyperviscosity
syndrome. This patient has confusion and blurry vision, which suggests the need to rapidly
reduce the serum viscosity with plasmapheresis. Long-term therapy is less clear. You can treat
with chlorambucil and prednisone or fludarabine, as with CLL, or with cladribine.

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APLASTIC ANEMIA

Case 1
48-year-old woman admitted to hospital for fatigue, easy bruising, and multiple
recent infections. No past medical history. Uses no medications. Spleen is normal
in size. Pancytopenia on CBC.

1. What tests will you perform?

HIV, EBV, CMV, hepatitis B and C serology, ANA, and B12 and folate levels

2. Most sensitive test?

3. Best therapy?
A. Autologous bone marrow transplant
B. Allogeneic bone marrow transplant
C. Cyclosporine and antithymocyte globulin
D. Adriamycin and methotrexate

Answer:

4. Best therapy if she were older (>50)?

A. Autologous bone marrow transplant
B. Allogeneic bone marrow transplant
C. Cyclosporine and antithymocyte globulin
D. Adriamycin and methotrexate

Answer:

5. 48-year-old woman lives with her father and a 12-year-old boy who are healthy. The
woman has recently undergone an allogeneic bone marrow transplantation for leuke-
mia. She is maintained on prednisone, sirolimus, and azathioprine. Which of the fol-
lowing should NOT be used in this family?
A. Tetanus vaccine
B. Pneumococcal vaccine
C. Influenza vaccine
D. Oral polio vaccine
E. MMR

Answer:

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6. Deficiency of which of the following vitamins causes pancytopenia?

A. B12
B. B6
C. C
D. B1

Answer:

7. Woman comes for evaluation of fatigue. CBC reveals anemia, low platelet count, and
leukopenia. Which is the most likely etiology?
A. Cobalamin deficiency
B. Disseminated intravascular coagulation
C. Autoimmune thrombocytopenic purpura
D. Cirrhosis

Answer:

Summary: Case 1
Aplastic anemia can be caused by a number of toxins, such as benzene, chemotherapeutic
agents, and radiation. Several viral infections can also lead to aplastic anemia, such as CMV,
EBV, HIV, and hepatitis B or C. If a patient does not have any of these, the disorder is from
abnormal function of the T-killer cell. The best treatment in patients who are under age 50
and have a matched donor is allogeneic bone marrow transplantation. If transplantation is
not possible, then T-cell suppressive therapy with cyclosporine and antithymocyte globulin
is the treatment.

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LYMPHOMA

Case 1
22-year-old man with several weeks of increasing neck pain and swelling. No past
medical history. Uses no medications. No other complaints besides the isolated
neck mass. The mass is in the cervical area and is nontender and rubbery. The
lesion persists despite the use of antibiotics over the next 10 days.

1. Next step in management of this patient?

2. What other diagnostic testing is appropriate for this patient?

Chest x-ray, chest/abdomen/pelvic CT scans, bone marrow biopsy and LDH level.
Lymphangiography and laparotomy are no longer used.

3. Which type of Hodgkin has best prognosis?

A. Lymphocyte predominant
B. Lymphocyte depleted
C. Mixed cellularity
D. Nodular sclerosing

Answer:

4. Which type has worst prognosis?

A. Lymphocyte predominant
B. Lymphocyte depleted
C. Mixed cellularity
D. Nodular sclerosing

Answer:

5. If all the other tests are normal, what is the best initial therapy for this patient?
A. Radiation therapy
B. ABVD chemotherapy
C. CHOP chemotherapy
D. Bone marrow transplantation

Answer:

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6. What is the best therapy if the diagnostic tests do find other disease?
A. Radiation therapy
B. ABVD chemotherapy
C. CHOP chemotherapy
D. Bone marrow transplantation

Answer:

7. What is the therapy if the patient relapses after radiotherapy?

A. Radiation therapy
B. ABVD chemotherapy
C. CHOP chemotherapy
D. Bone marrow transplantation

Answer:

8. What is the therapy if he relapses after chemotherapy?

A. Radiation therapy
B. ABVD chemotherapy
C. CHOP chemotherapy
D. Bone marrow transplantation

Answer:

9. If the patient has received chemotherapy and is pancytopenic and in need of a blood
transfusion, what type of transfusion should he receive?

Summary: Case 1
The diagnosis of lymphoma is the presence of adenopathy that is not warm or tender. An exci-
sional biopsy is necessary because a needle biopsy will reveal only normal-looking lympho-
cytes. After you find the Reed-Sternberg cells characteristic of Hodgkin disease, then the key is
to make sure that the disease is localized to just one or two lymph node groups (stage I or II).
Radiation is used to treat localized stage I and II disease. The other stages or the presence of
“B” symptoms, such as fever, weight loss, or night sweats, indicate the need for chemotherapy
with ABVD (Adriamycin [doxorubicin], bleomycin, vinblastine, dacarbazine). MOPP (mech-
lorethamine, vincristine, procarbazine, prednisone) was used in the past but is associated
with an unacceptably high rate of developing aplastic anemia and leukemia. Laparotomy and
lymphangiography are no longer used as a routine part of staging evaluation.

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1. Patient underwent radiation for Hodgkin disease 10 years ago. Which is the patient at
greatest risk for?
A. Acute leukemia
B. Lung cancer
C. Brain cancer
D. Ovarian cancer

Answer:

2. Patient underwent chemotherapy for Hodgkin disease 10 years ago. Which is the most
common treatment-related complication?
A. Breast cancer
B. Lung cancer
C. Acute leukemia
D. Hypothyroidism

Answer:

Case 2
47-year-old woman comes with fever and weight loss. She gets up at night to
change her sheets because they get soaked through with sweat. Lymph nodes
palpable in cervical and axillary region. Abdominal and pelvic CT scans are normal.
Excisional lymph node biopsy reveals diffuse, large B-cell non-Hodgkin lymphoma.
LDH level is elevated. Bone marrow biopsy reveals the same lymphoma.

1. Best next step in the management of this patient?

Start treatment; more tests won’t change therapy

2. Best initial therapy?

3. What further tests alter her management?

4. What to do differently if these further tests were positive?

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5. What would you do for her if she relapses after the initial therapy?
A. Re-treat with CHOP
B. Switch to ABVD
C. Radiation therapy
D. Bone marrow transplantation

Answer:

Summary: Case 2
Non-Hodgkin lymphoma is almost always treated with chemotherapy with CHOP because
80 to 90% of patients present with stage III or IV disease. Rituximab is an anti-CD20 anti-
body, and it is used with any form of non-Hodgkin lymphoma that expresses this antigen.

Sterility is extremely common in both men and women after chemotherapy. Myelodysplasia
and acute leukemia can occur even years after the end of chemotherapy. Radiation treatment
can lead to hypothyroidism, accelerated coronary disease, and pericarditis. Lymphoma is
also associated with Coombs positive hemolytic anemia. The case will describe an elevated
reticulocyte count and spherocytes on smear. Remember that autoimmune hemolysis is
also associated with spherocytes as the antibodies remove pieces of the red cell membrane.
Interestingly, although non-Hodgkin lymphoma presents in stage III or IV MUCH more
often than Hodgkin disease, systemic symptoms are more common with Hodgkin disease.

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TRANSFUSION REACTIONS

Case 1
Man with non-Hodgkin lymphoma has become severely pancytopenic after
chemotherapy in preparation for autologous bone marrow transplantation. During
transfusion he experiences chills, fever, dyspnea, back pain, and dark urine.

1. What is the reaction?

Major blood group ABO incompatibility

2. Next step in management of this case?

Stop the transfusion. Recheck the blood type. Hydrate the patient and consider forced
diuresis with mannitol. Possible use of bicarbonate.

3. What type of blood transfusion should you use in this patient in the future?
A. Whole blood
B. Packed red blood cells
C. Filtered
D. Filtered and irradiated
E. IgA deficient donor blood

Answer:

4. How will you recognize a minor blood group (Kell, Duffy, Lewis, Kidd, Rh)–incompatible
donation?

5. After massive (6–8 units) of transfusion, which is most likely to occur?

A. Hypokalemia
B. Hypernatremia
C. Hypocalcemia
D. Hyperuricemia

Answer:

6. Which of the following happens the FASTEST?

A. IgA deficiency reaction
B. ABO incompatibility
C. RH incompatibility

Answer:

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Case 2
Patient develops a mild febrile reaction with transfusion. With second unit of
transfusion he develops severe shortness of breath and pulmonary infiltrates.
Although the dyspnea is severe, it resolves within 24 hours. Hematocrit raises the
expected amount with transfusion. Bilirubin and LDH are normal. Smear is normal.

What is the reaction?

A. Febrile nonhemolytic reaction; use white-cell filter
B. Anaphylaxis, IgA deficiency; use washed red cells
C. Leukoagglutination reaction; no treatment necessary
D. Urticarial reaction; diphenhydramine, use washed red cells

Answer:

Case 3
Patient develops mild urticarial reaction after a blood transfusion. No evidence of
hemolysis. Bilirubin and LDH are normal. It resolves over the next day.

What is the reaction and how will you treat it?

A. Febrile nonhemolytic reaction; use white-cell filter
B. Anaphylaxis, IgA deficiency; use washed red cells
C. Leukoagglutination reaction; no treatment necessary
D. Urticarial reaction; diphenhydramine, use washed red cells

Answer:

Case 4
Patient develops an anaphylactic type of reaction after a blood transfusion.

What is the diagnosis and how will you treat it?

A. Febrile nonhemolytic reaction; use white-cell filter
B. Anaphylaxis, IgA deficiency; use washed red cells
C. Leukoagglutination reaction; no treatment necessary
D. Urticarial reaction; diphenhydramine, use washed red cells

Answer:

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Case 5
Person receiving a blood transfusion develops a 1°C elevation in temperature but
is otherwise asymptomatic. No back pain, no dyspnea, and no urticaria, and the
blood pressure and pulse are normal.

1. What is the reaction and how will you treat it?

A. Febrile nonhemolytic reaction; use white-cell filter
B. Anaphylaxis, IgA deficiency; use washed red cells
C. Leukoagglutination reaction; no treatment necessary
D. Urticarial reaction; diphenhydramine, use washed red cells

Answer:

2. Leukocyte reduction filters are meant to reduce the incidence of which of the following
reactions?
A. Acute hemolytic reactions
B. Urticarial reactions
C. IgA reactions
D. Leukoagglutination reactions
E. Febrile nonhemolytic reactions

Answer:

Summary: Cases 1–5

Leukoagglutination reactions (Case 2) occur when the recipient’s white cells are bound by
antibodies from the plasma of the donor. The recipient’s white cells transiently agglutinate
in the lung, causing shortness of breath. The disorder resolves spontaneously and there is no
specific therapy. This is also known as transfusion-related acute lung injury. Many patients
can develop a mild allergic reaction to plasma proteins in the transfused blood. This results
in urticaria (Case 3). It is treated with an antihistamine such as diphenhydramine. The man-
agement in the future will be to use red cell transfusions that have been washed of all the
plasma proteins. Patients deficient in IgA may produce antibodies to IgA (Case 4). If blood
that has not been washed is transfused into a patient who is IgA-deficient, an acute anaphy-
lactic reaction may occur. The solution is to use washed red cells or blood from a donor that
is IgA-deficient as well. Donor blood contains small amounts of white blood cells. Sometimes
a patient will develop a mild reaction to these antigens, resulting in a mild elevation in tem-
perature (Case 5). Because the antibodies are directed against white blood cell antigens and
not to red cells, there is no hemolysis. Because there is fever but no hemolysis, it is called a
febrile nonhemolytic reaction. The management is to use a filter with future transfusions.
The filter removes white cells and the antigens that they carry.

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COAGULATION DISORDERS

Case 1
37-year-old woman has come to see you because of epistaxis. The episodes have
been recurrent and hard to control. Uses no medications. She has petechiae.
Spleen is not palpable. Laboratory tests show:

Platelets: 227,000 PT: 12 sec PTT: 52 sec (prolonged)

1. Diagnosis?

2. What type of bleeding is this?

3. What is the Most accurate diagnostic test?

A. Bleeding time
B. Von Willebrand’s factor level and ristocetin cofactor assay
C. Bone marrow biopsy
D. Anti-platelet antibodies
E. Mixing study

Answer:

4. Initial therapy?
A. Desmopressin (DDAVP)
B. Factor VIII replacement
C. Steroids
D. Plasmapheresis
E. Cryoprecipitate
F. Fresh frozen plasma
G. Vitamin K
H. Epsilon aminocaproic acid

Answer:

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5. What if this therapy does not work?

A. Desmopressin (DDAVP)
B. Factor VIII replacement
C. Steroids
D. Plasmapheresis
E. Cryoprecipitate
F. Fresh frozen plasma
G. Vitamin K
H. Epsilon aminocaproic acid

Answer:

6. Epistaxis and petechiae with a normal platelet count. aPTT normal Platelets don’t stick
to vessel wall (adherence) Giant platelets, defect of glycoprotein Ib/IX receptor
A. Von Willebrand
B. Bernard-Soulier
C. Glanzmann’s thrombasthenia
D. Heparin overdose

Answer:

7. Epistaxis and petechiae with a normal platelet count. aPTT normal. Platelets don’t stick
to each other (abnormal aggregation). Defect of glycoprotein IIb/IIIa receptor
A. Von Willebrand
B. Bernard-Soulier
C. Glanzmann’s thrombasthenia
D. Heparin overdose

Endothelial Cells

Collagen
Adhesion
F
VW Ib
GP

GPIIb/IIIa
Aggregation

Fibrinogen
Activated platelet

Factor Interaction
on Platelet surface
Release
ADP

Answer:
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 IM Board Review Course Book

8. A patient has a motor vehicle accident and gets 12 units of transfusion. They start ooz-
ing at venipuncture sites and have petechiae. Platelets at 24,000 Why?

9. Woman with several children had anemia from heavy periods. A week after blood trans-
fusion she develops purpura and petechiae. Platelets drop.
Diagnosis?

Treatment?

Factor Platelet

Deep Superficial
Muscles, hematoma Gingiva, gums, mucosa
Joint, hemarthrosis Epistaxis, skin, purpura, petechiae

Summary: Case 1
Von Willebrand disease (VWD) presents with platelet type of bleeding with a normal platelet
count. Because factor VIII and Von Willebrand factor (VWF) travel bound to each other,
50% of patients with VWD will have a prolonged aPTT. Deficiency is confirmed by find-
ing an abnormal Ristocetin cofactor assay, and the diagnosis is confirmed by a low level of
VWF. Although it is usually preferable to use DDAVP in the treatment of VWD, you must
exclude type IIb VWD first because DDAVP can worsen this subtype. Factor VIII replacement
includes VWF. Cryoprecipitate will also treat VWD, but because it is a pooled blood product
it carries a higher risk of transmitting disease. Bernard-Soulier has defective adherence to
vessel wall. Glanzmann’s is defective aggregation to other platelets. Post-transfusion purpura
happens in women sensitized by pregnancy and transfusion to human platelet antigen-1.
Treat with IVIG.

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Case 2
37-year-old woman has come to see you because of epistaxis. Episodes have been
recurrent and hard to control. Uses no medications. She has petechiae. Spleen not
palpable. Laboratory tests show:

Platelets: 27,000 PT: 12 sec PTT: 32 sec (normal)

1. Diagnosis?
ITP (idiopathic thrombocytopenic purpura)

2. Best initial test?

3. Best initial therapy?

Steroids

4. What will you do if the thrombocytopenia continues to recur? Bernaud-Soulier

A. Splenectomy • Giant platelets
B. Romiplostim
• Ib/IX missing
C. Eltrombopag
D. Vincristine • N
 o adherence to
E. Platelet transfusion endothelium

Answer:

Summary: Case 2 Glanzmann’s

ITP is largely a diagnosis of exclusion. There is no truly specific test to diagnose the disorder.
Antiplatelet antibodies are sensitive but extremely nonspecific because many normal persons • IIb/IIIa defect
can harbor the antibodies. A bone marrow biopsy is useful primarily to exclude other causes • N
 o aggregation to other
of thrombocytopenia. The initial therapy is with steroids. If the patient’s thrombocytopenia platelets
is serious and continues to recur after stopping the steroids, then the best therapy is with
a splenectomy. More than 70% of patients will experience a sustained improvement in the
platelet count after the splenectomy.

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Post-transfusion purpura Case 3

• W
 omen sensitized by 37-year-old woman with epistaxis. Episodes have been recurrent and hard
pregnancy to control. In past 24 hours, she has had a subdural hematoma, an epidural
• Week after transfusion hematoma, melena, a subarachnoid hemorrhage, hemoptysis, hematemesis, and
• Give IVIG
heavy periods. Uses no medications. She has petechiae. Spleen is not palpable.
Laboratory tests show:

Platelets: 7,000 PT: 12 sec PTT: 32 sec (normal)

Antiplatelet antibodies: Positive Abdominal ultrasound: Normal

Bone marrow biopsy: Increased numbers of megakaryocytes

1. Best initial therapy?

2. 17-year-old boy admitted with bloody diarrhea. Recently ate a chicken hamburger.
Has purpuric spots on his legs. Hematocrit is 27. White cell count 7,200. Platelet count
of 23,000 elevated LDH. Smear shows fragmented red cells. BUN and creatinine are
elevated normal PT (prothrombin time) and aPTT. Most likely cause?
A. Campylobacter
B. Yersinia
C. Salmonella
D. E. coli 0157:H7

Answer:

Case 4
22-year-old woman with epistaxis and menorrhagia. She has lifelong history of
poor wound-healing and has had several spontaneous abortions. She was told she
had increased bleeding at birth, and she has had a lifelong history of easy bruising.
The PT, PTT, and platelet counts are normal. The bleeding time is normal.

1. Diagnosis?

2. Diagnostic test?

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3. What is the therapy if serious bleeding develops?

A. Desmopressin (DDAVP)
B. Factor VIII replacement
C Plasmapheresis
D. Cryoprecipitate
E. Fresh frozen plasma
F. Vitamin K

Answer:

Summary: Case 4
Factor XIII is also known as “clot-stabilizing factor.” After the initial fibrin clot forms, it is
subject to lysis by the body’s own natural fibrinolytic system, based on plasmin. Factor XIII
normally functions to stabilize the clot and stop the lysis by plasmin. There is increased
degradation of the clot in 5-molar urea. Treatment is initially with FFP, although factor XIII–
specific replacement does exist. You recognize the case based on increased mucosal bleeding
combined with normal PT, PTT, platelet count, and bleeding time. Factor XII deficiency
gives, in a way, the opposite presentation: there is an elevation of the PTT but no history of
bleeding.

Case 5
32-year-old woman comes to hospital with an episode of increased bleeding after
a surgical procedure. She has had two episodes of increased bleeding with trauma
in the past several years. Her prothrombin time is normal at 12 sec and the aPTT
is prolonged at 52 sec. Her platelet count and bleeding time are normal.

1. Diagnosis?

2. What test will you order first?

A. Bleeding time
B. Von Willebrand’s factor level and ristocetin cofactor assay
C. Bone marrow biopsy
D. Anti-platelet antibodies
E. Mixing study
F. Specific factor level

Answer:

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3. Most accurate diagnostic test?

A. Bleeding time
B. Von Willebrand’s factor level and ristocetin cofactor assay
C. Bone marrow biopsy
D. Anti-platelet antibodies
E. Mixing study
F. Specific factor level

Answer:

4. Initial therapy?

A. Desmopressin
B. Fresh frozen plasma
C. Cryoprecipitate
D. Vitamin K
E. Steroids

Answer:

Summary: Case 5
When the aPTT is prolonged and the PT is normal, the possibilities are hemophilia A or B,
factor XI and XII deficiency, and Von Willebrand disease and factor inhibitors. The fact that
this patient is female virtually excludes hemophilia A or B because these are both X-linked
recessive disorders; women are almost exclusively carriers, with men expressing the disease.
A mixing study will help greatly in distinguishing a factor deficiency from an acquired factor
inhibitor. The mix corrects the aPTT to normal in a deficiency but stays elevated in the pres-
ence of a factor-inhibiting antibody. Factor-inhibiting antibodies are treated with plasma-
pheresis in severe disease and sometimes with steroids and gammaglobulin. Massive infusion
of porcine factor VIII should be able to overcome an inhibitor because it does not cross-react
with the inhibitor. With Von Willebrand disease there should be mucosal bleeding, and the
bleeding time should be abnormally prolonged. Factor XII deficiency gives a prolonged
aPTT but never results in bleeding. The lupus anticoagulant should result in thrombosis, not
bleeding. Factor XI deficiency gives increased bleeding after trauma and surgery. It is treated
acutely with FFP (fresh frozen plasma).

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Coagulation Review Questions

1. 41-year-old patient bleeds excessively after dental extraction. aPTT is prolonged at
65 seconds and prothrombin time is normal. After 50:50 mixing study with normal
plasma, aPTT is 62 seconds and the bleeding time is prolonged. Most likely diagnosis?
A. Acquired Von Willebrand’s factor antibody
B. Dysfibrinogenemia
C. Factor VIII deficiency
D. Factor XI deficiency

Answer:

2. Which is consistent with disseminated intravascular coagulation?

Fibrin split D-dimers Fibrinogen Platelets PT aPTT

products
A. High High Low Low High High
B. Low Low Low Low High High
C. High High High Low High High

Answer:

3. Which causes schistocytes?

A. Autoimmune hemolytic anemia
B. Disseminated intravascular coagulation
C. Uremia
D. Folic acid deficiency

Answer:

4. Man has been on Coumadin for atrial fibrillation. Comes with multiple episodes of
hematemesis. INR is 8.0. Besides holding the Coumadin for 2 doses, what else should
be done?
A. Vitamin K orally
B. Vitamin K subcutaneously
C. Fresh frozen plasma
D. Protamine sulfate

Answer:

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5. A patient comes in with massive bleeding requiring multiple transfusions. What would
you expect to see on blood testing?
A. Hyponatremia
B. Hypocalcemia
C. Hyperphosphatemia
D. Hypokalemia

Answer:

6. Patient for pre-operative evaluation. Prolonged aPTT 90 seconds. PT normal. aPTT

corrects with mixing study. What is the most likely deficiency?
A. VIII, IX, XI, XII
B. V or VII
C. II
D. XIII

Answer:

7. A patient with mild hemophilia A with 10% factor VIII levels is going for dental extrac-
tion, what to do?
A. Nothing
B. DDAVP
C. Transfuse factor VIII
D. Cancel procedure

Answer:

8. Patient with A fib on warfarin and INR 5.0 No bleeding.

A. Lower dose
B. Hold for three days and restart lower
C. Vitamin K

Answer:

9. Patient needs surgery. Has Afib. Warfarin stopped for 3 days. INR 1.4 on morning of
surgery.
A. Cancel procedure
B. Vitamin K
C. Proceed with surgery

Answer:

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10. Alcoholic with portal hypertension and ascites from cirrhosis. PT markedly elevated.
Platelet 90,000. Hb 9. Needs portocaval shunt. What to do about bleeding?
A. Nothing, proceed
B. Cancel procedure
C. FFP
D. FFP and platelets

Answer:

11. Patient with atrial fibrillation for a day. EKG with rate 125/minute. Diltiazem started.
What else to do?
A. Nothing until echo done
B. Admit for IV heparin
C. Low molecular weight heparin and warfarin
D. Warfarin or Dabigatran

Answer:

12. Patient admitted for syncope. Platelets 200,000 on day one. Second day platelets, 5,000.
Normal PT/aPTT/INR. No bleeding. No clotting. What to do?
A. Steroids
B. Anti-platelet antibodies
C. Smear to look for clumping

Answer:

Summary
Excessive bleeding after dental extraction is a sign of platelet dysfunction. An elevated aPTT
and prolonged bleeding time indicate Von Willebrand’s disease. If a 50:50 mixing study does
NOT correct the aPTT to normal, this is a sign of an antibody inhibitor being present. DIC is
associated with elevated PT/aPTT, D-dimers and fibrin split products. Schistocytes are pres-
ent in DIC, HUS, and TTP. Uremia and autoimmune hemolysis do not give fragmented red
cells on smear. If a patient has acute bleeding, such as hematemesis, from Coumadin over-
dose, fresh frozen plasma is the fastest way to correct the coagulopathy. Multiple transfusions
are associated with hypocalcemia from chelation with the citrate buffer in the transfused
blood.

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Case 6
69-year-old woman was admitted for pulmonary embolus 3 days ago. She was
started on intravenous unfractionated heparin and warfarin. On fourth hospital
day, her laboratory tests reveal a PTT of 70 sec and platelet count of 115,000. On
the following day, her labs show an INR of 1.4 and platelet count of 95,000.

1. Diagnosis?
Heparin-induced thrombocytopenia

2. Most common clinical manifestation?

A. Stroke
B. Myocardial infarction
C. Venous thrombosis
D. Arterial thrombosis

Answer:

3. What would you do next in the management of this patient?

Stop the heparin

4. What will you use as an anticoagulant for the pulmonary embolus or if the patient
develops an arterial clot of her radial artery?

5. Best diagnostic test?

6. 27-year-old woman comes in for evaluation of a coagulopathy. Recently become preg-

nant. History of a deep venous thrombosis. She is G2, P0020 with two spontaneous
abortions. What will lab testing most likely reveal?
A. Increased aPTT
B. Protein C deficiency
C. Increased INR
D. Thrombocytosis
E. Thrombocytopenia

Answer:

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Summary: Case 6
Heparin induces thrombocytopenia by two mechanisms. There is a mild thrombocytopenia
induced by the direct effect of heparin on the platelets, and a more severe form caused by the
induction of antiplatelet antibodies that are heparin-dependent. This can occur with even the
smallest amount of heparin, such as heparin flushes of an intravenous catheter or a heparin-
coated catheter. Venous emboli occur in 75% of patients developing clots. Only 25% of heparin-
induced clots are arterial “white” thrombi. Low molecular-weight heparin has a smaller risk of
developing thrombocytopenia, but once the antiplatelet antibodies develop, all forms of heparin
must be stopped. On the exam, the most common error is switching to low molecular-weight
heparin after the patient develops thrombocytopenia with unfractionated heparin. The test is to
try to detect antibodies against platelets that are heparin-dependent. Specifically, you look for
antiheparin/platelet factor 4 antibodies, or antibodies that agglutinate platelets only after the
addition of heparin. The treatment for clots in those who cannot use heparin is with derivatives
of the direct thrombin inhibitor hirudin, such as lepirudin. Another therapy is argatroban.

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Case 7
36-year-old woman admitted for severe headache and left-sided weakness. She
has livedo reticularis. She denies an increased incidence of bleeding. Her only
medication is oral contraceptive pills.

PT: 11 sec; aPTT: 54 sec (elevated) Platelets: 185,000

VDRL: Positive FTA: Negative

1. What is the cause of the hemiplegia?

A. Oral contraceptives
B. Complex migraine
C. Factor V Leiden
D. Antiphospholipid syndrome

Answer:

2. Best initial test?

3. Most accurate diagnostic test?

4. What will you prescribe for next pregnancy?

A. Steroids
B. Aspirin
C. Heparin
D. Heparin and steroids
E. Aspirin and heparin

Answer:

Summary: Case 7
A lupus anticoagulant is an IgG or IgM antiphospholipid antibody that causes thrombosis
through an unknown mechanism. Although it exclusively causes thrombosis clinically, it
results in an elevated aPTT in vitro. On mixing study, there is a failure of the aPTT to cor-
rect to normal with a 50% 1:1 mix with normal plasma. All deficiencies will correct with the
addition of normal plasma. The lupus anticoagulant often results in a falsely positive VDRL.
Russell viper venom assay is the most specific test for the lupus anticoagulant. Treatment for
thrombosis is with heparin. When a patient has frequent spontaneous abortions from the
lupus anticoagulant, the treatment is to use aspirin and heparin throughout the pregnancy.
Steroids have no proven efficacy in decreasing the frequency of spontaneous abortion, and
may be harmful. If the lupus anticoagulant or antiphospholipid syndrome has not resulted
in a clinical manifestation, then no therapy is necessary.
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Case 8
27-year-old woman comes to ED with pulmonary embolus. She has DVT
(deep vein thrombosis) on the lower-extremity Doppler. These have each been
treated with 6 months of warfarin. No history of cancer or recent surgery.

1. What is the most common cause of this patient’s condition?

A. Antithrombin deficiency
B. Protein C deficiency
C. Protein S deficiency
D. Lupus anticoagulant
E. Factor V leiden mutation
F. Homocysteine elevation

Answer:

2. Therapy?

3. How will you recognize protein C deficiency?

4. How will you recognize antithrombin deficiency?

Thrombophilia Questions
1. Asymptomatic patient comes in for evaluation. His mother has factor V mutation and
has had her third episode of clotting. He is homozygous for the mutation. What do you
recommend?
A. Warfarin lifelong
B. IV Heparin followed by warfarin
C. No treatment necessary
D. Low molecular weight heparin subcutaneously lifelong

Answer:

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 IM Board Review Course Book

2. 30-year-old woman, who has been taking oral contraceptives for 10 years, is admitted
with an acute DVT. Her brother has a DVT at age 35 as well. She is started on heparin
and warfarin; and the oral contraceptives are stopped. The warfarin is stopped at 6
months. Two weeks later testing shows factor V mutation. What should you do?
A. Restart warfarin indefinitely
B. Restart warfarin, and add aspirin
C. Advise her not to use oral contraceptive without additional therapy
D. Restart warfarin indefinitely with an INR of 3–4

Answer:

3. Which of the following is most likely to have a recurrent clot?

Which of the following is most likely to need lifelong warfarin?
A. Factor V mutation
B. Factor II 20210 defect
C. Antiphospholipid mutation
D. Protein C, S
E. Anti-thrombin III deficiency

Answer:

Summary: Case 8
Factor V Leiden mutation is the most common cause of thrombophilia. The only cause of
thrombophilia that is associated with baseline abnormalities in the coagulation times is one
of the phospholipids syndromes, such as a lupus anticoagulant or anticardiolipin antibod-
ies. Protein C deficiency is the answer as the most likely diagnosis when there is evidence
of thrombosis and skin necrosis after the start of warfarin. Your answer is antithrombin
deficiency when the case describes an unexplained clot and there is no prolongation of the
aPTT with the start of heparin. Heparin works through the potentiation of antithrombin. If
there is a deficiency of antithrombin, the heparin will not be able to work and the aPTT will
remain low.

Asymptomatic factor V Leiden mutations do not need therapy. A single clot only needs
6 months of therapy of the usual intensity of an INR of 2–3. You do NOT give a higher INR
or longer duration based on a single clot. Lifelong warfarin would only be considered if there
were recurrent clots.

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Case 9
Patient maintained on dialysis comes to emergency department with melena.
Also has some epistaxis. Endoscopy reveals no specific lesion and he continues to
bleed despite the transfusion of blood. PT, aPTT, and platelet count are normal.
Hematocrit is 25.

1. What is the cause of bleeding?

2. How will you treat?

Summary: Case 9
Uremia is a cause of bleeding because of platelet dysfunction. There is an acquired storage
pool disorder. The platelet count is normal, but uremia inhibits the degranulation of the
platelet so the patients have the same manifestations as they would if they were thrombocy-
topenic or had Von Willebrand disease. The release of subendothelial stores of factor VIII and
Von Willebrand factor causes a brief increase in platelet function in uremia.

1. 62-year-old female patient consults you because of recurrent ecchymosis and a large
hematoma in gluteal area. Aspiration of knee reveals gross blood. Bleeding time is nor-
mal, aPTT is prolonged at 80 seconds, and the PT is normal. When the patient’s plasma
is mixed 1:1 with normal plasma, the aPTT fails to correct. Most likely diagnosis?
A. Disseminated intravascular coagulation
B. Anti-phospholipid syndrome
C. Acquired factor VIII inhibitor
D. Acquired factor V inhibitor

Answer:

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Case 10
Young man with:

• Recurrent sinopulmonary infections and immunosuppression

• Eczematous skin lesions
• Mild thrombocytopenia with small platelets
• Petechiae and bruising

What is the diagnosis?

A. Bernard-Soulier
B. Ouchy-Itchy-Bleedy syndrome
C. Wiskott-Aldrich syndrome
D. Itchiness-Crapsky syndrome

Answer:

Summary: Case 10
Recurrent sinopulmonary infections in young people suggest primary immune defects.
With eczema and small platelets in low number we have the Wiskott-Aldrich syndrome.
Dysfunctional platelets are removed by the spleen leading to their low number. Severe bleed-
ing needs platelet transfusion or splenectomy. Immune defects need IVIG. Bone marrow
transplant is curative.

No XII
bleed
Rare XI
bleed
Mod IX
bleed
Severe VIII
PTT bleed

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PROSTATE CANCER

Case 1
65-year-old man comes to office wanting to know what he should do about being
screened for prostate cancer. He is not anxious to find anything abnormal, but he
wants to be responsible if there is a test that can help him.

1. What do you tell him?

A. Perform PSA
B. Refuse to do the PSA because it is not proven to lower mortality
C. Perform transrectal ultrasound

Answer:

Man’s prostate-specific antigen is mildly elevated at 8. There are no masses found

on rectal examination. He wants to “go all the way,” since he has already taken the
test and he has now started feeling anxious.

2. What do you do next in his evaluation?

A. Prostatectomy
B. Transrectal ultrasound with biopsy
C. Repeat the PSA in 6 months
D. Flutamide

Answer:

Case 2
74-year-old man comes in for evaluation of an elevated PSA at 9 and a small mass
found in one lobe of his prostate. Transrectal ultrasound reveals a small, isolated
lesion limited to one lobe of his prostate. A biopsy finds adenocarcinoma.

1. Most important prognostic finding for this lesion?

2. What would you recommend as therapy for this man’s localized disease?
A. Radiation or surgery
B. No treatment
C. Leuprolide
D. Flutamide
E. Orchiectomy

Answer:
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Case 3
78-year-old man comes to see you because he has developed locally metastatic
prostate cancer after his initial therapy several years ago.

1. Best therapy?

Summary: Cases 1–3

The prostate-specific antigen (PSA) test is not generally recommended as a screening test for
prostate cancer. Although it is elevated in prostate cancer, there is still no evidence that screening
with this test reduces mortality. The same is true for the digital rectal examination. If the PSA is
elevated, the best step is to palpate for a nodule and biopsy that nodule. If no nodule is palpated,
then the best next step in management to exclude disease is to perform a transrectal ultrasound.
If a lesion is identified, then you should biopsy the lesion found. If the PSA is elevated and no
mass can be found by either examination or ultrasound, then the only way to exclude cancer
is with multiple blind biopsies. The most important prognostic factor besides the size of the
lesion is the Gleason stage. There is still no clear survival advantage confirmed to allow you to
choose between a radical prostatectomy, external beam radiation, or implanted radioactive seeds
(brachytherapy). Metastatic disease is managed with antiandrogens. The gonadotropin-releasing
hormone agonists leuprolide or goserelin can cause a flare initially with the disease, and they
should be combined with antiandrogens such as flutamide, ketoconazole, or orchiectomy.

There is no benefit at all to PSA above age 75 and if the question describes someone >75 wanting
PSA the answer is: refuse.

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TESTICULAR CANCER
1. 22-year-old homosexual man with a painless testicular mass, which is non-illuminat-
ing. Ultrasound confirms it to be solid mass. Serum alpha fetoprotein and beta-hCG are
elevated. CT scans of the abdomen and pelvis are negative. Which is the best choice?
A. Scrotal orchiectomy
B. Inguinal orchiectomy
C. Observe
D. Fine needle biopsy via the scrotum
E. Excisional biopsy via the scrotum

Answer:

2. 21-year-old man has a growing painless left testicular mass that is solid. All the
serum markers are normal (hCG, LDH, alpha-fetoprotein). Left orchiectomy shows
a non-seminomatous germ cell cancer. Patient is asymptomatic. CT scans as well as
retroperitoneal lymph node dissection are normal. Which of the following is the most
appropriate after surgery?
A. Regular follow up; no chemotherapy unless it recurs
B. Empiric adjuvant chemotherapy
C. Chemotherapy and radiation
D. Bone marrow transplantation

Answer:

Case Summary
Testicular cancer presents as a painless mass in the scrotum. The best method of diagnosis is
with removal of the gonad through a high inguinal approach. Incisions or needle biopsy of the
scrotum should not be done. Seminomatous cancer does NOT raise the level of alpha fetoprotein
(AFP). An elevated AFP always means a non-seminomatous cancer. Beta-hCG can be elevated
with either of them. LDH can also be used to follow prognosis. Non-seminomatous cancer does
not need adjuvant chemotherapy or radiation, if the disease was localized to the scrotum and
the lymph nodes are free of disease. Non-seminomatous cancer is cured by orchiectomy alone,
if it is limited to the scrotum.

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BREAST CANCER

Case 1
A woman comes to see you on her 50th birthday. She has not seen a doctor in 20
years—since her last child was born—and now she is here to have “the works.” She
still has periods. Her physical examination, including breast exam and stool for occult
blood, is negative.

1. What screening tests should you perform?

Mammogram shows several small calcifications. Core needle biopsy shows

infiltrating ductal carcinoma. At surgery, she is found to have a 3-cm cancer that is
estrogen receptor–positive.

2. What is “sentinel node” biopsy, and how does it change your management?

All of the woman’s lymph nodes are freely mobile and not fixed to each other.
Three of 14 nodes are found to be positive for cancer.

3. What therapy?

4. What adjuvant therapy?

5. What is trastuzumab?
A. All women
B. HER-2/Neu positive
C. Estrogen-receptor positive
D. Progesterone-receptor positive

Answer:

6. When are aromatase inhibitors (anastrazole, letrozole, exemestane) indicated?

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7. 52-year-old woman has a mother and aunt with breast cancer. Her last mammogram
was normal 6 months ago. How would you best follow this patient?
A. Mammography now
B. Mammography yearly
C. Carcinoembryonic antigen level
D. CA-125 level

Answer:

Summary: Case 1
Aromatase inhibitors stop the conversion of androgens to estrogen. Examples of aromatase
inhibitors are anastrazole, letrozole, and exemestane. The best evidence for the use of these
drugs is in estrogen receptor–positive patients who have received 2 to 5 years of tamoxifen.
These aromatase inhibitors are used after tamoxifen to decrease the risk of recurrent disease.
The use of aromatase inhibitors is generally limited to postmenopausal women because
these drugs have fewer efficacies in overcoming the higher estrogen level produced by the
premenopausal patient.

In the general population, screening for breast cancer with mammography should begin at
age 50 and be performed every 1 to 2 years. If a breast mass is palpated on examination, an
ultrasound is used to exclude a cystic lesion. Solid lesions found on either mammography or
ultrasound should be biopsied. At resection, a dye is injected into the operative field. The first
node that the dye goes to is the “sentinel node.” If this node contains cancer, an axillary lymph
node dissection is the best next step. If this sentinel node is free of disease, then the axillary
dissection is unnecessary. This sentinel node is an acceptable marker for the occurrence of
disease in the other nodes.

Breast Cancer Questions

1. Which most accurately determines prognosis in patient with breast cancer?
A. Lymph node involvement
B. Estrogen receptors
C. Progesterone receptors
D. Tumor size
E. Age of the patient

Answer:

2. 38-year-old woman is found to have a 3-cm breast cancer without lymph-node involve-
ment. Best management?
A. Mastectomy without chemotherapy
B. Lumpectomy without chemotherapy
C. Chemotherapy and radiation
D. Lumpectomy with adjuvant chemotherapy
E. Lumpectomy with adjuvant chemotherapy and local radiation

Answer:

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3. 60-year-old woman, who is post menopausal, has lumpectomy and axillary dissection
for breast cancer. The estrogen receptors are positive, and 3 of 17 nodes are positive for
cancer. What do you recommend?
A. Radiation followed by tamoxifen and chemotherapy
B. Tamoxifen alone
C. Radiation followed by chemotherapy
D. Chemotherapy alone

Answer:

Lumpectomy with breast radiation is equal in efficacy to modified radical mastectomy.

Tamoxifen should be given to all patients whose estrogen receptors are positive on the
cancer. Adjuvant chemotherapy with cyclophosphamide, methotrexate, 5-fluorouracil, and
sometimes doxorubicin is given if cancer is found in the lymph nodes in any premenopausal
patient. Taxanes, such as paclitaxel or docetaxel, can be used in conjunction with cyclophos-
phamide and doxorubicin. Adjuvant chemotherapy is also given to any patient with a primary
cancer larger than 1 cm. Trastuzumab is a monoclonal antibody against an antigen (known as
Her-2/neu) that is often expressed in increased amounts in patients with breast cancer.

Case 2
Healthy 45-year-old woman with breast cancer. Mother was diagnosed at 55 and
was estrogen and progesterone receptor–positive. She had one sister with breast
cancer.

1. What do you suggest to reduce this woman’s risk of cancer?

A. Soy diet
B. Low-fat diet
C. Hormone receptor therapy
D. Tamoxifen

Answer:

2. Most common adverse effect of tamoxifen?

3. Which statements concerning BRCA is true?

A. All patients should receive tamoxifen.

B. BRCA is associated with ovarian cancer.
C. BRCA is associated with colon cancer.
D. Patients who are BRCA positive should undergo mastectomy.
E. Mammography screening should be done more frequently.
F. BRCA should be routinely performed.

Answer:
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4. Which is NOT a side effect of tamoxifen?

A. Osteoporosis
B. Endometrial hyperplasia
C. Deep venous thrombosis

Answer:

Summary: Case 2
There is mounting evidence that for those patients at extremely high risk of breast cancer,
tamoxifen and possibly raloxifene as primary prophylaxis will reduce the risk of developing
breast cancer. There is no clear evidence that dietary manipulation will reduce the risk of
cancer before it has occurred. The most common long-term adverse effects of tamoxifen
therapy are DVT and endometrial cancer.

BRCA is associated with ovarian cancer. There is NO clear indication of how to use the test
clinically. It is NOT a routine test. Tamoxifen use in BRCA carriers did NOT reduce the risk
of developing cancer; and there is no indication for mammography more often than the usual
recommendation of yearly. Other risks for breast cancer are nulliparity, early menarche, late
menopause, and first pregnancy after age 30. The only risk in which there is a proven change
in management is with family history. Patients with multiple first-degree relatives should be
offered tamoxifen.

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GYNECOLOGIC MALIGNANCIES

Cervical Cancer
1. Which is the appropriate screening for cervical cancer?
A. Every year, starting at age 18
B. Every year, starting at age 21 and stopping at age 65
C. Yearly for 3 years starting at age 21, then every 3 years
D. Yearly for 3 years starting at age 21, then every 3 years, then stopping at age 65
E. Every 3 year from 21–29, then HPV and Pap every 5 years to age 65

Answer:

2. On Pap smear, a 50-year-old woman is found to have high-grade dysplasia. What is the
most appropriate?
A. Repeat Pap smear
B. Repeat Pap smear after a course of antibiotics
C. Hysterectomy
D. Colposcopy for biopsies
E. Human papillomavirus typing

Answer:

3. Which screening test has the greatest reduction in mortality?

A. Pap smear
B. Colonoscopy
C. Prostate-specific antigen
D. Mammography after age 40
E. Mammography after age 50

Answer:

Endometrial Cancer
1. Which is the most common risk factor for endometrial cancer?
A. Obesity
B. Oral contraceptives
C. Multiple sexual partners
D. Ovarian carcinoma

Answer:

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2. 42-year-old woman comes to see you because of vaginal spotting in between periods for
the last several months. Thyroid function tests are normal. She has had yearly Pap smears
that were normal for the last 2 years. Most appropriate management of this patient?
A. Conization biopsy
B. Colposcopy
C. Endometrial biopsy
D. Dilatation and curettage
E. Observation

Answer:

Ovarian Cancer
1. Woman with increasing abdominal girth and ascites. There is an ovarian mass; and the
ascitic fluid is positive for malignant cells. What is best therapy?
A. Combination chemotherapy
B. Chemotherapy and radiation
C. Bilateral salpingo-oophorectomy, hysterectomy, omentectomy, and maximum tumor
removal followed by chemotherapy
D. Intraperitoneal chemotherapy

Answer:

Summary: Gynecologic Malignancies

The standard of care for screening for cervical cancer changes somewhat depending on the
recommending organization. Screening should, at the least, occur every 3 years between the
ages of 21 and 65. It is acceptable to perform screening more frequently for the first 2 to 3
years. Patients with atypical squamous cells of unknown significance (ASCUS) should be
treated for infection if it is present and be re-screened in 4 to 6 months. Human papillo-
mavirus (HPV) testing combined with Pap smear extends screening length to 5 years. HPV
testing in a person with ASCUS can be used to differentiate between who should go on to
colposcopy versus who can just have their tests repeated. If colposcopy is to be performed,
there is no point in testing for the specific type of HPV. The boards have avoided controver-
sial areas such as this.

Dysfunctional bleeding should be evaluated by endometrial biopsy. Although Pap smear,

colonoscopy, and mammography all lower mortality, mammography above the age of 50
lowers it the most.

Ovarian cancer most often presents at advanced stage. CA-125 is NOT specific for ovarian
cancer. There is no routine screening test for ovarian cancer that should be offered. Ovarian
cancer should undergo maximum cytoreduction at the time of surgery. This is unusual for
cancer. Although metastatic, survival and prognosis is based on how much of the tumor can
be removed. Chemotherapy will further cytoreduce the malignancy.

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 Pulmonology/Critical Care
7
Case 1
55-year-old woman with shortness of breath. Progressive 40-year history of
smoking. Acutely short of breath and is using her accessory muscles to breathe.
Diffuse wheezing noted bilaterally. Arterial blood gas: 7.20 / 80 / 42 / 32.

1. How do you calculate the A–a gradient?

2. What information does it provide regarding this patient’s respiratory status?

3. When do you have hypoxia with a normal A–a gradient?

A. Pulmonary embolism
B. Guillian-Barre syndrome
C. Acute Respiratory Distress Syndrome
D. Pneumonia
E. Emphysema
Answer:

4. What is this patient’s current acid–base status?

Summary: Case 1
In evaluating any patient with hypoxia, the first question to ask yourself is whether the
hypoxia is associated with a rise in the partial pressure of carbon dioxide. If the low oxygen
is associated with a rise in the PCO2, the next question you have to ask is whether the A–a
gradient is abnormal. The formula to calculate the A–a gradient on room air is the following:

150 – PCO2 – ¼(PCO2) – PaO2.

An abnormal A–a gradient establishes the hypoxia as being due to some degree of V/Q
mismatch.

When you observe the combination of hypoxia associated with a normal A–a gradient in the
presence of elevations in the PCO2, the hypoxia is due solely to hypoventilation. A normal
A–a gradient essentially rules out V/Q mismatching as the mechanism for low oxygenation

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in the blood. Hypoxia with a normal A–a gradient establishes the etiology of the hypoxia as
being secondary to lower partial pressure of oxygen in the alveolus rather than an intrinsic
pulmonary problem. Most commonly, the gas displacing oxygen is carbon dioxide that
accumulates when a patient hypoventilates. Common causes of hypoventilation without
pulmonary involvement include opioid overdose and neuromuscular disease. High altitude
is another explanation for persistent hypoxia with a normal A–a gradient. In summary,

Increased A-a gradient with normal pCO2 Normal A-a gradient with increased
pCO2
Ventilation/perfusion mismatches (V/Q) Central nervous system trauma/insult
Pulmonary embolism Neuromuscular disorders
ARDS Scoliosis
Pneumonia Demyelinating disorders (i.e., Guillain-
Barre, multiple sclerosis)

When you identify acidosis with elevations in the carbon dioxide, the acid–base disorder is
most likely to be a respiratory acidosis. To determine the acuity of the respiratory acid–base
disorder, it is helpful to calculate the change in pH and change in the HCO3 concentration
relative to the rise or fall in PCO2.

Change in pH Change in HCO3

per 10 mm Hg per 10 mm Hg change
change in PCO2 in PCO2
Acute respiratory pH decreases 0.08 per 10 HCO3 increases by 1.0–2.0 per
acidosis mm Hg increase in PCO2 10 mm Hg increase in PCO2
Chronic respiratory pH decreases 0.03 per 10 HCO3 increases by 3.0–4.0 per
acidosis mm Hg increase in PCO2 10 mm Hg increase in PCO2
Acute respiratory pH increases 0.08 per 10 HCO3 decreases by 2.5 per
alkalosis mm Hg decrease in PCO2 10 mm Hg decrease in PCO2
Chronic respiratory pH increases 0.03 per 10 HCO3 decreases by 5.0 per
alkalosis mm Hg decrease in PCO2 10 mm Hg decrease in PCO2

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Case 2
45-year-old man with abdominal pain and vomiting. Type 1 diabetes mellitus.
Smoking two packs of cigarettes. Vital signs are as follows: temperature 38.3ºC
(101ºF), BP 112/54 mm Hg, HR 116, RR 23. + abdominal pain. Stat arterial blood
gas analysis with standard chemistries shows the following:

7.05 / 90 mm Hg 132 102 63

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5.8 8 2.3

1. What is the primary acid–base disorder?

2. Is there an additional respiratory acid–base disorder?

3. Is there an additional metabolic acid–base disorder?

4. What are the major causes of anion gap metabolic acidosis (AGMA)? Non-anion gap
metabolic acidosis (NAGMA)?

Summary: Case 2
The presence of acidemia associated with a low bicarbonate makes the diagnosis of a meta-
bolic acidosis. Once you identify a metabolic acidosis, an easy way to classify the disorder is
to calculate the anion gap. The anion gap is calculated by subtracting the serum bicarbonate
and chloride from the serum sodium. A normal anion gap is around 12 ± 4. Anything above
16 should be considered to indicate the presence of an anion gap metabolic acidosis (AGMA).
Causes of AGMA include: diabetic ketoacidosis (DKA), alcohol, renal failure, lactic acidosis,
iron poisoning, starvation, seizures, sepsis, ASA poisoning, rhabdomyolysis, ethylene gly-
col, isoniazid, methanol, and paraldehyde. Non-anion gap metabolic acidosis (NAGMA)
is caused by uretosigmoidostomy, saline, early renal failure, diarrhea, carbonic anhydrase
inhibitors, adrenal insufficiency, renal tubular acidosis (RTA), hyperparathyroidism, triam-
terene, and spironolactone. In this case we have an AGMA. We use Winter’s formula and the
delta anion gap to determine if there are any concomitant respiratory or metabolic acid–base
disorders in addition to the AGMA that we have already identified.

Winter’s formula (expected PCO2 = 1.5 × bicarbonate + 8) is used to calculate an expected

PCO2 that would result if the only acid–base disorder would be our AGMA. If the actual
PCO2 (as measured on the ABG) is higher then our expected value, then there was a

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pre-existing respiratory acidosis. If the actual PCO2 (as measured on the ABG) is lower than
the expected value, then there was a pre-existing respiratory alkalosis. The delta anion gap is
used to determine the existence of an additional metabolic acid–base problem. The calculated
anion gap minus the normal anion gap gives the delta anion gap. The delta anion gap plus the
measured bicarbonate should equal 25 (normal serum bicarbonate). If the delta anion gap
plus the measured serum bicarbonate <25, it means there was pre-existing NAGMA. If the
delta anion gap plus the measured serum bicarbonate >25, it means there was a pre-existing
metabolic alkalosis.

1. A 27-year-old witnessed seizure: sodium 135, potassium 4.2, chloride 95, bicarbonate 18,
with a pH of 7.22 and a lactic acid of 12.7 on ABG. Next step?
A. Infuse bicarbonate intravenously
B. Begin NS at 200 cc/hour
C. Observation with repeat chemistries in 2 hours
D. Dialysis

Answer:

2. A 23-year-old man found with several bottles of rubbing alcohol opened near him.
Lab results are: sodium 141, potassium 4.2, chloride 106, bicarbonate 23, glucose 127. Next
diagnostic test?
A. Urine sodium
B. Measure serum osmolarity
C. Urine toxicology
D. Urinalysis for crystals
E. Gastric lavage

Answer:

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Case 3
A 63-year-old woman with severe shortness of breath. History of congestive heart
failure. Chest x-ray on admission shows cardiomegaly and frank pulmonary edema.
Aggressive diuretic therapy initiated. On day 3 ABG: 7.50 / 45 / 85 / 36.

1. What is the primary acid–base disorder?

2. Is there an additional respiratory disorder?

3. What test would you order to confirm your diagnosis?

4. A 19-year-old woman with near syncope. Always “light headed,” but worsening.
Pregnancy test is negative. Blood pressure 98/58, heart rate 88, respiratory rate 12,
afebrile. Labs:

Na+: 138 Cr 1.1

K+: 3.2 Ca 8.9
Cl: 103 Mg 0.6 (low)
HCO3 32 Urine potassium elevated
BUN 31

What is the most likely diagnosis?

A. Conn syndrome
B. Gitelman syndrome
C. Glycyrrhetinic acid excess
D. Cushing syndrome

Answer:

Summary: Case 3
Alkalemia on the ABG in the presence of elevated serum bicarbonate makes the diagnosis of a
metabolic alkalosis. To determine whether there is an additional respiratory acid–base disor-
der, the expected PCO2 should be calculated using the following formula:

expected PCO2 = 0.7 × bicarbonate + 20.

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If the actual PCO2 (as measured on the ABG) is higher than the expected value, it means
there is an additional respiratory acidosis. If the actual PCO2 (as measured on the ABG) is
lower than the expected value, it means that in addition to the metabolic alkalosis there is a
respiratory alkalosis.

To determine the etiology of a metabolic alkalosis, the next step is to measure a urine chlo-
ride. A urinary chloride of <10 mEq/L suggests the presence of a saline-responsive meta-
bolic alkalosis. The metabolic alkalosis would respond to fluid replacement. Most commonly,
saline-responsive metabolic alkalosis results from overzealous diuresis. Other causes include
vomiting or nasogastric suction. A urinary chloride >10 mEq/L indicates saline-unresponsive
metabolic alkalosis. Conn syndrome, Liddle syndrome, and Bartter syndrome are some
etiologies of a saline-unresponsive metabolic alkalosis.

Case 4
A 55-year-old homeless man with changed mental status. Unconscious. He is
disheveled and obtunded; pupils are midpoint and reactive. CT scan of the head,
CBC, and LFTs are normal. ABG:

pH 7.25 Potassium 5.4

PCO2 30 Bicarbonate 15
PO2 90 Serum osmolarity (measured) 411 mmol/kg
Serum bicarbonate 15 Creatinine 1.8
Na 135 BUN 43
K 5.4 Glucose 134
Sodium 135

1. What is the acid–base disorder?

2. What is the differential diagnosis?

3. How do you calculate serum osmolarity?

4. When would you measure serum osmolarity?

Summary: Case 4
In the presence of an unexplained anion gap metabolic acidosis, or when a suspicion of
poisoning exists, it is extremely important to measure the osmolar gap. The osmolar gap
is defined as the difference between the calculated serum osmolarity and the measured

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serum osmolarity. An osmolar gap >10 suggests an exogenous source of an osmotically active
substance (methanol or ethylene glycol). Ethylene glycol commonly produces oxalate crystal-
luria, and methanol will produce visual changes or blindness.

Calculated serum osmolarity = 2[Na] + Glucose/18 + BUN/2.8

Other causes of an increased osmolar gap include poisoning with paraldehyde, ethanol, or
isopropyl alcohol (elevated osmolar gap without a metabolic acidosis); mannitol ingestion;
hyperlipidemia; and hyperproteinemia. To calculate the serum concentration of the unmea-
sured osmoles the following formula is used:

Serum concentration = change in osmoles × (molecular weight/10).

Molecular weights are: ethanol 46, methanol 32, ethylene glycol 60, and isopropanol 60.

Case 5
45-year-old with community-acquired pneumonia. On day 3 vital signs are 38.8ºC
(102ºF), BP 87/42, HR 124, RR 27.

1. Next step in management?

A. Norepinephrine
B. Dopamine
C. Normal saline
D. Observation

Answer:

2. Most likely etiology of hypotension?

3. If fluid resuscitation is unsuccessful, what vasopressor will you begin?

4. What other causes of shock should you consider?

Summary: Case 5, Questions 1–4

The next step in the management of this patient is to initiate aggressive fluid resuscitation
with normal saline or Ringer lactate. In this case, the most likely etiology of this patient’s
hypotension is septic shock, simply because you are given a history of fever associated with a
pulmonary infection. If the blood pressure does not respond after aggressive fluid resuscita-
tion, the next step would be to initiate therapy with Norepinephrine. Norepinephrine is the

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vasopressor of choice in septic shock. It has recent data supporting improved outcome in
septic shock patients, primarily from the surviving sepsis campaign. The second vasopressor
of choice is dopamine. Both norepinephrine and dopamine have alpha and beta activity, thus
improving cardiac contractility and increasing cardiac output.

In evaluating any patient with hypotension, it is important to categorize the various mecha-
nisms by which shock can occur. Cardiogenic shock is hypotension secondary to arrhyth-
mias, pump failure, acute valvular disease, or a dramatic event such as a ventricular wall
rupture. Obstructive shock causes hypotension by reducing venous return to the heart.
Common causes of obstructive shock include tension pneumothorax, cardiac tamponade,
and pulmonary embolus. Hypovolemic shock causes hypotension by decreased intravascu-
lar volume. Common causes include bleeding, burns, and dehydration. Distributive shock
is hypotension due to pathologic vasodilation of the peripheral vasculature. It is the only
type of shock associated with a decreased systemic vascular resistance (SVR) on Swan-Ganz
catheterization. Common causes include septic shock, anaphylactic shock, and acute adrenal
insufficiency.

When you have shock with a …Then the most And the treatment is …
history of … likely diagnosis is …
Urticaria; insect sting
Hyperkalemia with hyponatremia
and a non-anion gap metabolic
acidosis; hyperpigmentation
Malignancy; DVT immobilization;
hip surgery; widely split S2 with
loud P2
Myocardial infarction or a new
pansystolic murmur loudest at
axilla or a pansystolic murmur
maximal at the left sternal border
Pneumonia; UTI; cellulitis
Bleeding anywhere; recent
procedure (biopsy, CVP
placement, etc.); dehydration; or
vomiting
Distended neck veins; pulsus
paradoxus; cardiomegaly with
clear lungs on chest x-ray

A stat EKG shows diffuse ST-segment elevations, and the patient’s cardiac enzymes
are positive.

5. What is the next step in the management of this patient?

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Shock State Cardiac Output PCWP SVR

Hypovolemic
Cardiogenic
Cardiac
tamponade*
Pulmonary
embolus**
Distributive
*Tamponade is characterized by equalization of right atrial and right ventricular diastolic pressure, pulmonary
artery diastolic pressure, and PCWP
**Massive pulmonary embolism is characterized by sudden increase in the pulmonary artery pressure with a normal
or decreased PCWP

Summary: Case 5, Question 5

When the cardiac enzymes return positive, the etiology of the patient’s hypotension becomes
unclear. The two possible causes of shock include sepsis and cardiogenic shock secondary
to an acute myocardial infarction. It is at this point in time that right heart catheterization
would be important in distinguishing between these two possible etiologies of the patient’s
hypotension.

Case 6
A 23-year-old woman with community-acquired pneumonia. + respiratory distress.
You elect to intubate immediately. Chest x-ray shows diffuse bilateral pulmonary
infiltrates. Arterial blood gas on 100%:

7.42 / 32 / 150 / 100%.

1. Diagnosis?

2. Next step in the evaluation of this patient?

A. Right heart catheterization
B. Left heart catheterization
C. CT angiogram
D. 2-dimensional echocardiogram
E. Start enoxaparin

Answer:

3. When is increasing PEEP the answer?

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4. What are plateau and peak pressures? Why are they important?

5. When should you suspect intrinsic PEEP?

A. Chronic obstructive pulmonary disorder
B. Acute respiratory distress syndrome
C. Acute lung injury
D. Pulmonary embolism

Answer:

6. What can happen to the blood pressure when PEEP is increased?

7. 42-year-old man is intubated for severe exacerbation of COPD. He is found to have

infiltrate on chest x-ray. He is found to be hypotensive. The following graph is obtained
from the ventilator:

i
n
s
p
i
F r
e
L
O
e
W x
p
TIME i
r
e

What is the most appropriate next step in management?

A. Apply external PEEP

B. Administer dopamine
C. Give bronchodilators
D. Increase tidal volume
E. Increase respiratory rate

Answer:

8. What is permissive hypercapnia? Why is it important?

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9. An ICU patient is currently being mechanically ventilated. You are required to increase
PEEP from 5 to 10 cm H2O in order to maintain oxygenation status. Which of the fol-
lowing would suggest that increasing PEEP may have a detrimental effect to the patient?
A. Decrease in (A–a) gradient
B. Decrease in blood pressure
C. Increased urinary output
D. Increase in lung compliance

Answer:

10. In which of the following conditions will you only see an increase in the peak pressure?
A. Air leak
B. Cuff rupture
C. CHF
D. Mucus plug
E. Pneumonia

Answer:

Summary: Case 6
The acute onset of respiratory failure 12 to 24 hours after an initial lung injury is consistent
with the diagnosis of acute respiratory distress syndrome (ARDS). ARDS is characterized
by increased oxygen requirements reflected by a PaO2/FiO2 ratio of <200. The next step in
the evaluation of this patient is to exclude cardiogenic pulmonary edema by performing an
echocardiogram or by inserting a Swan-Ganz catheter to document normal left-sided pres-
sures and/or a PCWP <18 mm Hg. Positive-end expiratory pressure (PEEP) is pressure pro-
vided by the ventilator at the end of expiration. PEEP improves oxygenation in patients with
ARDS by optimally distending collapsed alveoli to optimize gas exchange. In patients who
are persistently hypoxemic despite increasing levels of FiO2 (>50%), PEEP should be added
to reduce oxygen requirements.

The management of ARDS consists of treating the underlying cause of the lung inflamma-
tion. Supportive therapy consists of respiratory support to keep the PaO2 at 60 mm Hg or
the SaO2 at 90%. To maintain these levels of oxygenation it is often necessary to increase the
PEEP, which, in turn, may cause hypotension by decreasing venous return to the right side
of the heart. This may also require the addition of vasopressor therapy to maintain blood
pressure at acceptable levels. The concept of permissive hypercapnia consists of reducing
tidal volume and thus allowing the levels of carbon dioxide to rise. By reducing tidal volume,
the amount of barotrauma is reduced. The mortality of patients with ARDS is thus reduced
by up to 10%.

As stated above, PEEP applied through a mechanical ventilator is referred to as applied or

extrinsic PEEP to improve oxygenation. PEEP may also be referred to as auto or intrinsic

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PEEP if it is secondary to incomplete exhalation, usually due to obstructive airways disease.

There are three common situations in which auto PEEP is found:

1. high minute volume ventilation: large TV (too much air to expire) and/or a high RR
(not enough time to exhale)

2. expiratory flow limitation: airway collapse, bronchospasm, inflammation (common

in COPD)

3. expiratory resistance: narrow diameter or kinked endotracheal tube, inspissated

secretions

The concept of auto PEEP or intrinsic PEEP can be explained as follows: If after a tidal vol-
ume is delivered, the amount of time in expiration is insufficient for the exhalation of the
inspired tidal volume, auto PEEP may develop.

New equilibrium
↑ Elastic recoil phase (inspired and
Hyperinflation
of lungs exhaled volumes
of alveoli
become equal)

Inadequate time for

Volume of air (mL)

exhalation of inspired VT
Dynamic
hyperinflation

Expiratory flow
ON

EX

continues until next

I
AT

HA

inspiratory breath
IR

LA
SP

is initiated
TIO

Development
IN

of auto PEEP
FRC
Time (sec)

At the end of inspiration, after the entire tidal volume has been delivered, when the expira-
tory port is occluded and there is no air movement, the plateau pressure can be measured.
In absence of flow, airway resistance becomes almost nonexistent. Therefore, the plateau
pressure reflects the compliance of the lung parenchyma and chest wall. Higher plateau
pressures reflect decreased compliance of the lung parenchyma and chest wall. When auto
PEEP occurs, the treatment depends on the underlying cause. Prolonging the expiratory
phase is the best ventilatory intervention. This is achieved by decreasing the tidal volume or
decreasing the RR to allow the lungs time to exhale. Bronchodilators, steroids, and antibiot-
ics are helpful when the underlying cause is obstructive airways disease. In patients with an
expiratory flow limitation, small amounts of applied PEEP (approximately 50% of auto PEEP
levels) can counter the effects of auto PEEP. But only give applied PEEP in the setting of
expiratory flow limitation otherwise it will worsen respiratory dynamics and predispose the
patient to barotraumas and hypotension.

The peak inspiratory pressure (PIP) is measured at the end of inspiration while the tidal
volume is being delivered. The way to determine whether increased PIP reflects an airway

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(bronchospasm) or a parenchymal problem is to measure the plateau pressure. If a parenchy-

mal problem is causing an increased stiffness of the lung, both the plateau and peak pressures
will be elevated. In an airway problem, you would expect the PIP to be increased with normal
plateau pressures.

Mechanical ventilation can be delivered in various modes. Controlled mechanical ventilation

(CMV) and assist/control (A/C) are modes that guarantee delivery of a minimum number
of breaths per minute. If the patient triggers the ventilator by attempting to breathe, the
machine will deliver the prescribed tidal volume. Synchronized intermittent mandatory ven-
tilation (SIMV) is identical to CMV except that when the patient attempts to breathe on his/
her own, the machine will not deliver the prescribed tidal volume. When the ventilator only
provides pressure support for the patient’s spontaneous breathing, the mode of ventilatory
support is called continuous positive pressure ventilation.

Control SIMV

Ventilator
Insp Constant flow

Spontaneous
. Constant
Flows (V)
(L/min)

flow Insp
Start of
exhalation
0
Rapid Exhal
rise of
flows

Ventilator
Exhal
Airway pressure (Paw)

Spontaneous
(+)
(cm H2O)

Exhal
Insp

Insp
(–) Synchronized with
spontaneous breath

End of VT delivery Ventilator

Spontaneous
Volume (VT)
(mL)

Insp Exhal Exhal

Insp
0

1. 65-year-old man intubated for COPD exacerbation. Ventilator alarms off. Peak inspira-
tory pressure to be 40 cm H2O and the plateau pressure to be 36 cm H2O. Diagnosis:
A. Mucus plug
B. Worsening obstruction
C. Increased secretions
D. Lobar atelectasis

Answer:

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2. 65-year-old man intubated for COPD exacerbation. Ventilator alarms went off. Peak
inspiratory pressure to be 40 cm H2O and the plateau pressure to be 10 cm H2O.
Diagnosis?
A. Pneumothorax
B. Cardiogenic pulmonary edema
C. Occluded ET tube
D. Pneumonia

Answer:

3. 78-year-old woman is intubated. ABG showed PaO2 of 44 mm Hg. Post-intubation,

with an FiO2 of 0.50, the patient’s PaO2 improved to 83 mm Hg. Repeat ABG shows a
PaO2 of 34 mm Hg. PIP and plateau pressures are noted to be 23 cm H2O and 25 cm
H2O, respectively. Physical exam shows no interim change. Diagnosis:
A. Pulmonary edema
B. ARDS
C. Bronchospasm
D. Pulmonary embolism

Answer:

NOTE 4. 23-year-old woman with asthma is intubated. What is the best intervention to reduce
her risk of ventilator-associated pneumonia?
Chlorhexidine VAP as well
A. Continuous suctioning of respiratory secretions
B. Antibiotic prophylaxis
C. Frequent respiratory tubing changes
D. Semi-recumbent position

Answer:

The following table summarizes the common problems in ventilator management.

Problem What does it mean? Examples

Elevation of peak Indicates problem or obstruction in the
pressure only bronchial tree
Elevation of both Indicates a problem in the chest cavity of
peak and plateau parenchyma
pressures
Decrease in both Indicates a problem in the air flow
peak and plateau through the tube
pressures

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Pulmonary Function Testing (PFT)

1. What pattern on PFT is consistent with obstructive lung disease?

2. What pattern on PFT is consistent with restrictive lung disease?

Explanations: Pulmonary Function Testing

Pulmonary function testing (PFT) consists of two parts: the measurement of lung volumes,
and spirometry. Lung volumes are used as a measure of static compliance and are used pri-
marily to diagnose restrictive lung disease. Spirometry is a measure of dynamic compliance
and is used primarily to diagnose obstructive lung disease. It is important to realize that both
of these classifications of lung disease have many etiologies. Restrictive lung disease can be
caused by fibrosis, sarcoidosis, obesity, or chest wall deformity. Obstructive lung disease can
be due to asthma, COPD, or bronchiectasis. Thus, the history and physical exam become
essential in arriving at a correct diagnosis.

Spirometry is considered abnormal when the FEV1 falls below 80% of its predicted value.
Lung volumes are considered to indicate restrictive disease when they are less than 80%
of predicted.

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Flow Volume Loops

Flow Flow Flow
8 8 8

6 6 6

4 4 4

2 2 2

0 0 0

–2 –2 –2

–4 –4 –4

–6 –6 –6
–1 0 1 2 3 4 –1 0 1 2 3 4 –1 0 1 2 3 4

Volume Volume Volume

(1) (2) (3)

Flow Flow Flow

8 8 8

6 6 6

4 4 4

2 2 2

0 0 0

–2 –2 –2

–4 –4 –4

–6 –6 –6
–1 0 1 2 3 4 –1 0 1 2 3 4 –1 0 1 2 3 4

Volume Volume Volume

(4) (5) (6)

1. What is the diagnosis?

Match the appropriate flow-volume loops with the following patients:

A. A 17-year-old girl with moderate asthma and nocturnal symptoms undergoes spirom-
etry with methacholine.

B. A 23-year-old immigrant from South America presents with a large goiter. She com-
plains of dyspnea at rest and exercise.

C. A 65-year-old with a 35-pack/year history of smoking undergoes spirometry to assess

worsening of symptoms. He has had 4 hospitalizations in the past 12 months for dys-
pnea and increased sputum production. Baseline oxygen saturation is 86% on room air.

D. A 28-year-old college basketball player has a history of asthma which occurs usually 1–2
times a month and is worse in winter months. There are no nocturnal symptoms.

E. A 68-year-old man suffers a stroke. After the stroke he is noted to have hoarseness in his
voice and difficulty swallowing foods and liquids, with frequent episodes of coughing
during meals.

F. A 55-year-old man presents with dyspnea at rest which is progressively worsening over
the past 2 years. He was previously employed as a subway construction worker for many
years. Fine crepitations are heard diffusely on lung examination. Diffusing capacity for
carbon monoxide is decreased.
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2. 38-year-old man with history of lung cancer and radiation therapy to chest is being eval-
uated for progressive dyspnea. Restrictive pattern is found on spirometry. Measurement
of which of the following can differentiate interstitial fibrosis from pleural fibrosis?
A. DLCO
B. FEV1
C. FEV1/FVC
D. RV
E. TLC

Answer:

3. In which of the following patients will you find reduced lung volume and normal
DLCO?
A. Asthma
B. Congestive heart failure
C. Interstitial lung disease
D. Phrenic nerve paralysis
E. Pulmonary embolism

Answer:

4. In which of the following patients will you find reduced lung volume and decreased
DLCO?
A. Asthma
B. Emphysema
C. Neuromuscular disorders
D. Pulmonary edema
E. Pulmonary embolism

Answer:

Summary: Flow Volume Loops

The diffusion capacity of carbon monoxide (DLCO) is used as a measure of the lung’s ability
to perform gas exchange at the level of the alveolus and capillaries. The amount of CO that
is able to diffuse across the alveolus into the capillary depends on the amount of hemoglobin
in the blood and the integrity of the alveolar structure. Thus, any process that increases the
amount of blood in the pulmonary capillaries will increase the DLCO. Such processes include
asthma and heart failure. Diseases that reduce the DLCO include any interstitial process such
as fibrosis or inflammation. When reduced lung volumes are measured and the DLCO is also
reduced, the most likely diagnosis is some sort of intrinsic lung disease such as parenchymal
fibrotic process or pulmonary edema. The presence of a restrictive pattern with reduced lung
volumes and a normal DLCO speaks for an extrapulmonary cause of the restrictive disease,
i.e., obesity or chest wall deformity, or neuromuscular disease. Decreased DLCO in the setting
of obstructive airway disease suggests emphysema.

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Case 7
A 57-year-old woman with shortness of breath progressively worsening. Baseline,
distance of 3 city blocks. At this time she can walk only one block. Chronic cough
that is productive of whitish sputum that has become greenish and more copious
over the last several days. 20-year history of smoking. Bilateral wheezing.

1. Most likely diagnosis?

A. Asthma
B. Chronic bronchitis
C. Emphysema
D. Pulmonary edema
E. Pulmonary fibrosis

Answer:

2. Best initial therapy?

A. Albuterol
B. Corticosteroids
C. Oxygen
D. Mucolytics
E. Tiotropium

Answer:

3. Are antibiotics indicated? If so, what antibiotics would you give?

4. Which of the following blood gases is most likely found in this patient?
pH PO2 (mm Hg) PCO2 (mm Hg) HCO3 (mEq/L)
A. 7.15 98 33 11
B. 7.15 98 24 8
C. 7.30 56 80 38
D. 7.40 100 40 25
E. 7.50 100 33 25

Answer:

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5. All of the following are indications for home oxygen therapy, except
A. pO2 <55
B. Left ventricular failure
C. Right ventricular failure
D. Hematocrit >56%

Answer:

6. Upon review of her EKG, she is found to have increased amplitude of P waves in NOTE
lead II. Which of the following changes in management is indicated?
Major risk factor for COPD
A. Diuretic therapy
Smoking
B. Home oxygen
C. Oral prednosine
D. Prophylactic antibiotics
E. Theophylline

Answer:

7. Which of the following is indicated for reduced mortality and progression of COPD? NOTE
A. Long-acting anticholinergics Supplemental O2 reduces
B. Lung volume reduction surgery mortality as well as lung
C. Influenza vaccine volume reduction surgery
D. Inhaled corticosteroids
E. Smoking cessation

Answer:

8. 69-year-old man is discharged COPD exacerbation. He is discharged on home O2, NOTE

inhaled fluticasone, and albuterol. On follow-up arterial blood gas is: pH 7.44, pO2 57,
pCO2 51, and FEV1 40% of expected. Next step: Young patient started on
salmeterol. Best counseling.
A. Phlebotomy to goal of hematocrit <56%
Oral hydration 2/2 to dry
B. Change inhaled fluticasone to oral prednisone
mouth
C. Add ipratropium
D. Add azithromycin
E. Recommend possible lung resection surgery

Answer:

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Summary: Case 7
In any patient who presents with progressively worsening shortness of breath associated
with wheezing and has a significant history of smoking, COPD should be considered in the
differential diagnosis. The history of chronic cough and sputum production also speaks for
the diagnosis of COPD. Patients with emphysema complain mostly of dyspnea and rarely
complain of cough. Patients tend to be thin and appear short of breath, using accessory
muscles of breathing. Peripheral edema is rare. Chest x-ray shows hyperinflation, and the
PaCO2 tends to be normal or reduced. Hemoglobin is normal. Spirometry shows obstruc-
tion, and the residual volume may be markedly increased. DLCO is usually reduced. In
patients with chronic bronchitis, the major complaint is productive cough lasting months at a
time. Dyspnea is usually mild, and patients often appear overweight and cyanotic. Peripheral
edema is common. PaCO2 can be markedly elevated, and polycythemia is more common
than in emphysema. Spirometry reveals obstruction. The DLCO is usually normal.

The best initial therapy for patients with COPD is oxygen via nasal cannula, and ipratropium
is the bronchodilator of choice. Antibiotic therapy is indicated for the treatment of acute
COPD exacerbations when there has been a documented increase in the amount of sputum
or a change in the quality of the sputum. Commonly used antibiotics include doxycycline,
fluoroquinolones, and macrolide antibiotics.

The role of steroids in the management of COPD is only during an acute exacerbation. They
are generally not useful in the management of chronic disease, with benefit in less than 10%
of patients with chronic COPD. The presence of hypercapnia on the ABG is an indication
that the FEV1 has fallen below 50% of predicted. If a room-air ABG shows the PaO2 to be
below 55 mm Hg, oxygen therapy may be initiated. Oxygen therapy may also be started if
the PaO2 lies between 56 and 59 mm Hg and the patient has a hematocrit >56% or has a
P pulmonale on EKG or has clinical evidence of right-sided heart failure on physical exam.
Oxygen therapy is the only intervention besides smoking cessation that has been shown to
reduce mortality secondary to COPD. Smoking cessation is always a goal that is desirable in
the treatment of COPD. It is the only nonpharmacologic intervention shown to reduce mor-
tality and progression of COPD. The main indication for lung resection surgery in patients
with chronic COPD is an FEV1 less than 40% of expected, with primarily upper lobe disease.
The procedure does not improve mortality, but it improves frequency of exacerbations. It
also allows the lower lobes of the lungs with less disease to expand better, thus improving
ventilation.

Case 8
A 23-year-old woman with dry cough. She denies any shortness of breath, acid
reflux, or heartburn. + “spring allergies.” Positive nasal polyps bilaterally.

1. Most common causes of cough?

2. How would you treat this patient’s nasal polyps?

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The patient is sent for pulmonary function tests (PFTs), which return as normal
with no evidence of airways obstruction.

3. What test is indicated at this time?

A. Methacholine challenge
B. Ipratropium challenge
C. Chest CT
D. Repeat PFTs in 3 months
E. Repeat PFTs in 6 months

Answer:

4. Are the normal PFTs inconsistent with the diagnosis of asthma? Why or why not?

Patient with perennial allergies, rhinitis and nasal polyps. He has an allergy to NSAIDs
as well. Diagnosis?
ASA sensitivity

Summary: Case 8, Questions 1–4

The most common causes of cough include GERD, postnasal drip, and cough variant asthma.
The treatment of nasal polyps includes nasal steroids. Generally, if on spirometry the FEV1
improves by more than 12% after administration of a bronchodilator, the diagnosis of
asthma is made. Because asthma is defined as reversible airways obstruction, normal spirometry
does not rule out the diagnosis. Thus, if after spirometry the diagnosis is suspected, then the
next step is to perform a methacholine challenge test. This test is very sensitive for the diagnosis,
so a negative result excludes asthma with a 95% certainty.

A week later symptoms worsening. She regularly wakes up at night with a dry,
hacking cough that is relieved by her inhaler.

5. What medication would you start at this time?

A. Salmeterol
B. Fluticasone
C. Theophylline
D. Zafirlukast
E. Omalizumab

Answer:

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NOTE Treatment of Asthma

Most accurate way to treat Classification Daytime Nighttime Therapy

asthma is to remove the Symptoms Symptoms
causative agent Mild intermittent
Mild persistent
Moderate persistent
NOTE
Severe persistent
Empiric PPI have not shown
to make a change in the
symptoms of asthma You start the patient on a steroid inhaler in addition to her inhaled bronchodilator.
Two weeks later she came in complaining of shortness of breath with wheezing.

NOTE 6. What is the best initial test at this time?

Mainstay of therapy for A. Chest x-ray

asthma is inhaled steroid B. Peak flow
C. Skin allergy testing
D. Chest CT
E. Methacholine challenge

Answer:

7. What is the best initial therapy?

8. When will you give ipratropium bromide?

9. What other medications are indicated at this time?

The resident does an ABG and reads you the following report:

7.35/ 38 / 90 / 25.

NOTE 10. What is the next step in the management of this patient?

Post Nasal Drip is now A. Discharge patient home with oral prednisone for 7 days
called Upper Airway Cough B. Discharge patient home with higher dose inhaled fluticasone
Syndrome. C. Admit to 23-hour observation unit
D. Stat MICU consult and probable intubation

Answer:

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11. Young college graduate with history of asthma and eczema moves to NYC and has
worsening asthma. Remove rug, carpeting, and use allergy proof warps for mattress,
pillows, and box spring.

12. Patient with a history of asthma maxed on medical symptoms, and changed lifestyle to
remove allergens. She has a dog but doesn’t want to get rid of it. Serum IgE level is high.
Next step?
Add omalizumab

Summary: Case 8, Questions 5–10

The best initial diagnostic test in the management of an acute asthma exacerbation is to
measure the peak expiratory flow. This test is quick and provides a tremendous amount of
information regarding the severity of the exacerbation. Additionally, it provides a baseline
against which to measure the effectiveness of therapy. The best initial therapy for an acute
asthma exacerbation is a short-acting beta agonist. Ipratropium is the bronchodilator of
choice in asthma exacerbating secondary to beta-blocker ingestion. Steroids should be used
for the treatment of an acute asthma exacerbation. The findings on ABG in a patient with
asthma should reflect the patient’s hyperventilation. Thus, when the pCO2 is normal and not
low, as in the present case, it may be a sign of impending respiratory failure. Thus, the next
step in management of this patient would be to intubate and provide mechanical support of
her respiratory function.

The difference between the acute management of COPD and asthma is important. In COPD,
the bronchodilator of choice is always ipratropium, whereas in asthma the bronchodilator
of choice is a short-acting beta agonist. The chronic management also differs between the
two diseases. In asthma, the role of inhaled steroids is clearly defined and has proven benefit;
whereas in COPD, the only interventions that have been shown to improve mortality are
smoking cessation and chronic oxygen therapy.

1. A 32-year-old with medical history of asthma. Severe shortness of breath that began
2 days ago. – fever, + substernal chest pain that worsens with deep inspiration. Diffuse
wheezing. What is the best initial test?
A. Arterial blood gas
B. Chest x-ray
C. Pulse oximetry
D. Pulmonary function testing
E. Peak flow

Answer:

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2. A 23-year-old man with asthma + nighttime symptoms twice a week and has to use his
albuterol inhaler three times per week. Only medication is albuterol inhaler. Next step?
A. Add an inhaled steroid and theophylline
B. Add zafirlukast and an inhaled steroid
C. Add prednisone and salmeterol
D. Add an inhaled steroid and salmeterol
E. Add salmeterol

Answer:

3. A 37-year-old man with asthma that has worsened over the past 8 months. Daily, asso-
ciated with brown-tinged productive sputum. Symptoms are no longer well controlled
with long-acting beta-agonist and inhaled corticosteroids. Elevated eosinophil count
and elevated IgE levels. Next step?
A. Blood cultures
B. High resolution CT scan of the chest
C. Serum precipitins
D. Skin test to aspergillus antigens
E. Sputum culture

Answer:

NOTE 4. The patient is found to have centrally located bronchiectasis on CT scan of the chest.
Which of the following is the most appropriate management?
If you see a fungus ball on
A. Omalizumab
CT but pt asymptomatic =
monitor B. Antibacterial agents
C. Corticosteroids
Fungus ball on CT with
D. Iptratropium
hemoptysis = surgery
E. Itraconazole

Answer:

NOTE Patient with steroid dependent asthma presents with cough wheezing and brownish
mucus. IgE is extremely high
Psittacosis Complement
Diagnosis?
fixation and serology = most
ABPA
accurate diagnostic test
Treatment? Increased Steroids
Young patient who works with birds presents with cough and ground glass appearing
CXR. WBC (–) for eosinophils and PFTs are restrictive. Diagnosis?
Bird fancier lung
38-year-old woman with night sweats, and low fever. + cough (–) sputum. CXR –
peripheral infiltrates. High eosinophils. ESR 90
Diagnosis?
Chronic eosinophillic PNA
Treatment? Steroids

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5. 50-year-old woman with severe asthma requiring long-term corticosteroids presents

with rhinitis. + palpable purpuric lesions of extensor surfaces. Chest x-ray reveals
patchy opacities in bilateral lung fields. CBC reveals eosinophilia. Next step?
A. ANA
B. c-ANCA
C. p-ANCA
D. Lung biopsy
E. High resolution CT scan

Answer:

6. 45-year-old man with history of diabetes, asthma, allergic rhinitis, and angina presents
with worsening of his asthma symptoms. Taking albuterol daily aspirin. Next step?
A. Add anticholinergic
B. Add inhaled corticosteroid
C. Add theophylline
D. Stop aspirin
E. Switch to long-acting beta agonist

Answer:

7. 21-year-old woman complains of dyspnea and coughing during exercise – symptoms at

night or during the day. Next step?
A. Anticholinergic inhalers
B. Avoidance of strenuous activity
C. Cromolyn sodium
D. Long-acting B agonist
E. Short-acting B agonist

Answer:

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Case 9
43-year-old man with chronic cough. Cough started after pneumonia 6 months
ago. Production of copious amounts of sputum, foul-smelling, streaked with blood.
Crackles bilaterally at the lung bases.

1. Diagnosis?
A. Allergic bronchopulmonary aspergillosis
B. Bronchiectasis
C. Goodpasture syndrome
D. Systemic lupus erythematosus
E. Wegener granulomatosis

Answer:

2. What is the best initial diagnostic test?

3. What is the diagnostic test of choice?

4. What would you expect to find on PFTs?

5. 22-year-old man with complaints of increased shortening of breath. Mild bilateral

basilar rales. Prescribed levofloxacin and an albuterol inhaler. 2 weeks later no improve-
ment, more short of breath. A chest CT reveals multiple bullae in the lower lobes of the
lungs bilaterally. A CBC returns:

WBC 6.1 HCT 37

Hgb 11.1 Plt 254

The serum chemistries return:

Na 140 Ca 9.8
K 4.7 AST 145 (elevated)
Cl 110 ALT 233 (elevated)
HCO3 28 Alk Phos 340 (elevated)
BUN/Cr 30/1.2 Total Bili 0.9

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What is the next best test in the management of this patient?

A. Sweat chloride test
B. Bronchoscopic lung biopsy
C. Sperm motility test
D. Alpha-1 antitrypsin
E. Anti-neutrophilic antibody

Answer:

Summary: Case 9
In a patient who presents with a history of recurrent pneumonia and chronic productive
cough, the most likely diagnosis is bronchiectasis. The best initial test is the chest x-ray.
Radiographic findings include “train tracks” and “ring shadows.” The diagnostic test of
choice is a CT scan of the chest, which shows dilated airways. The findings on spirometry are
consistent with an obstructive pattern, with reduced FEV1. The most common underlying
disease predisposing to bronchiectasis is cystic fibrosis. Other immunodeficiencies, such as
common variable immunodeficiency, can also predispose to bronchiectasis. Granulomatous
infection (TB, histoplasmosis), rheumatoid arthritis, inflammatory bowel disease, SLE, and
alpha-1-antitrypsin disease are uncommon causes of bronchiectasis. Treatment for bronchi-
ectasis includes chest physical therapy to mobilize secretions, bronchodilators, and antibiot-
ics based upon sputum cultures.

Case 10
32-year-old African-American complaining of chest pain. Pain began suddenly
yesterday. EKG, cardiac enzymes, and all subsequent lab work are negative. Slightly
elevated calcium level. Chest x-ray reveals bilateral hilar lymphadenopathy. PE: NL.

1. Diagnosis?

2. Test?
A. Lymph node biopsy
B. ACE levels
C. Serum calcium
D. Chest x-ray
E. Chest CT

Answer:

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3. When are steroids the answer?

4. What do you expect to find on PFTs?

Summary: Case 10
In a young African-American patient who is found incidentally to have hilar lymphadenopa-
thy on CXR, the diagnosis of sarcoidosis is likely. The diagnostic test of choice is a biopsy of
mediastinal or hilar lymph nodes to identify noncaseating granulomas. Measuring ACE levels
is not sensitive or specific enough to be relied upon for the diagnosis. ACE levels are only used
after treatment to determine efficacy of therapy. Indications for therapy for patients with
sarcoidosis include ocular, cardiac, CNS involvement, or symptomatic Stage II pulmonary
disease. Also, patients with malignant hypercalcemia and constitutional symptoms should
be treated with systemic steroid therapy. The appropriate management of the asymptomatic
patient with Stage I sarcoid is to withhold therapy; 75–80% of these patients will go into
spontaneous remission on their own. Most patients with pulmonary involvement demon-
strate a restrictive pattern when lung volumes are measured.

Sarcoidosis Staging

Stage Findings
0 No radiologic findings

1 Hilar lymphadenopathy and no pulmonary infiltrates

2 Hilar lymphadenopathy and pulmonary infiltrates

3 No lymphadenopathy and significant pulmonary infiltrates

4 Diffuse pulmonary fibrosis

Case 11
35-year-old man with sudden onset of chills, fever, cough, and shortness of breath.
Nausea that began yesterday evening. Works in a cork factory. Symptoms began
5 hours after first day. Tachycardic and tachypneic, and lung auscultation reveals
that he has bibasilar crackles. Chest x-ray is significant for small nodular densities
sparing the bases and apices of the lungs.

1. When is the most likely diagnosis hypersensitivity pneumonitis?

2. When is the most likely diagnosis bacterial pneumonia?

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3. What laboratory findings will distinguish hypersensitivity pneumonitis from bacterial

pneumonia?

4. What is the best initial test for diagnosis of hypersensitivity pneumonitis?

A. Blood cultures
B. Pleural biopsy
C. Skin antigen testing
D. Anti-neutrophilic antibody

Answer:

5. What is the treatment for hypersensitivity pneumonia?

Summary: Case 11
Hypersensitivity pneumonitis is characterized by sudden onset of chills, malaise, cough, and
shortness of breath 4 to 8 hours after exposure to the occupational hazard. The white blood
cell count is usually elevated with a left shift. Notably, there is mild eosinophilia associated
with hypersensitivity pneumonitis. Diagnosis is made by the presence of precipitating anti-
body in the serum to the offending agent. Treatment is with oral steroids.

Case 12
57-year-old man with difficulty breathing over the last 9 months. + exertional
symptoms that have limited his ability to walk. + cough, dry. No history of heart
disease or smoking. Worked as an accountant for the last 35 years. No medication.
Clubbing of his fingers and bilateral “dry” crackles over both lung bases.

1. What is the best initial test?

2. What is the most sensitive non-invasive test?

3. What is the best test for the diagnosis of this disease?

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4. What would you expect to find on PFTs?

5. 49-year-old man with idiopathic pulmonary fibrosis. Patient is on oral prednisone and
inhaled albuterol daily with little improvement in FEV1 and SO2. Next best treatment
option?
A. Start methotrexate
B. Start infliximab
C. Start rituximab
D. Refer for a heart-lung transplant

Answer:

Summary: Case 12
In a 57-year-old man who presents with insidious onset of shortness of breath associated
with clubbing and “Velcro” rales on lung auscultation, the most likely diagnosis is idiopathic
pulmonary fibrosis. The best initial test for the diagnosis is a chest x-ray that may reveal
increased linear or reticular opacities. Areas of normal lung may be present next to areas of sig-
nificant fibrosis. Pathologically, the pattern is referred to as usual interstitial pneumonia (UIP)
characterized by patchy, non-uniform distribution of fibrosis. CT scanning of the chest can,
in certain situations, obviate the need for a biopsy to establish the diagnosis. In patients who
are over 65 years of age and have progressive fibrosis and honeycombing on chest CT scan and
a restrictive pattern on pulmonary function testing, a biopsy to diagnose pulmonary fibrosis
may be unnecessary. The most accurate test for the diagnosis is a lung biopsy. Treatment for
pulmonary fibrosis consists of steroid therapy. The response to steroids depends on the
histological type. Patients with UIP respond to steroid therapy 15% of the time. Their prognosis
is poor, and median survival is approximately 3 years from the time of diagnosis.

Additional cases
45-year-old man comes to your office complaining of SOB and a dry cough that
began 3 months ago after an URTI. Complains of fatigue and weight loss over
the same period of time. PE is significant for respiratory crackles noted bilaterally.
Chest x-ray shows diffuse opacities with normal lung volume.

The most likely diagnosis in this case is bronchiolitis obliterans organizing pneumonia (BOOP).
The relatively acute onset of symptoms following a flu-like illness strongly suggests the diagno-
sis. The best initial test is a chest x-ray, and the most accurate test is lung biopsy showing buds
of loose connective tissue (Masson bodies) and inflammatory cells filling the alveoli and distal
bronchioles. Treatment consists of steroids.

42-year-old man comes to your office complaining of progressively worsening

SOB, fatigue, and weight loss. He also complains of a nonproductive cough.
Physical exam is significant for crackles auscultated bilaterally in both lung
fields. Chest x-ray is significant for bilateral symmetrical lower-lobe opacities in a
“bat-wing” distribution.
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The most likely diagnosis is pulmonary alveolar proteinosis. This condition is characterized by
accumulation of phospholipids within the alveolar spaces. The “bat-wing” appearance on chest
x-ray is typical. The most accurate test is a bronchoalveolar lavage that demonstrates PAS-
positive material. Treatment is whole-lung lavage through a double lumen endotracheal tube.

32-year-old woman comes to the office complaining of progressively worsening

shortness of breath. PMH is significant for several spontaneous pneumothoraces,
and chest CT scan reveals thin-walled cysts surrounded by normal lung.

In a young woman with recurrent pneumothoraces and thin-walled cysts on CT scan of the
chest, the most likely diagnosis is pulmonary lymphangioleiomyomatosis. The disease is char-
acterized by atypical proliferation of smooth-muscle cells and subsequent cyst formation. Other
complications include early-onset emphysema, chylothorax, chylous pleural effusions, and chy-
loperitoneum. Disease typically accelerates during pregnancy and is retarded by oophorectomy.
Treatment consists of oophorectomy, luteinizing hormone, tamoxifen, or progesterone. The most
effective therapy is lung transplantation.

Occupational Lung Disease

When there is interstitial lung . . . Then the most Characteristics unique to

disease and the history is . . . likely diagnosis is . . . the disease are . . .
Rock mining, quarrying, Eggshell calcifications on
stonecutting, sandblasting chest x-ray; higher risk of
pulmonary TB
Mining, insulation, Pleural plaques and
construction, shipbuilding calcifications on chest x-ray;
barbell-shaped fibers on lung
biopsy
Coal mining Caplan syndrome: necrotic
rheumatoid nodules in the
periphery of the lung in a
coal worker with RA
Machining and handling Can mimic sarcoidosis
beryllium alloys. Beryllium and show noncaseating
miners do not get berylliosis. granulomas on lung biopsy.
Lymphocyte proliferation test
diagnostic. Responds to early
treatment with prednisone.

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Case 13
27-year-old man with daytime sleepiness. Sudden inability to stay awake that
occurs sometimes during important meetings. Describes falling down while
laughing at a friend’s joke. Describes being unable to move, despite being fully
alert, while waking in the morning. Begun to hear voices before falling asleep in
the evening. Physical exam is normal.

1. Diagnosis?

2. Diagnostic test of choice? What do you expect to find?

3. Treat this disorder?

Patient with daytime sleepiness and moderate muscular weakness and hallucinations at
sleep onset.
Diagnosis?
Narcolepsy
Treatment?
Modafinil

Summary: Case 13
Narcolepsy is characterized by the following tetrad of symptoms:
• Sleep attacks
• Cataplexy—sudden loss of muscle tone with emotion
• Sleep paralysis—temporary paralysis upon waking
• Hypnagogic hallucinations
The diagnostic test of choice is sleep studies. The finding indicative of narcolepsy is the onset
of REM sleep within minutes of falling asleep. Treatment consists of methylphenidate for
sleep attacks and SSRIs for cataplexy, sleep paralysis, and hallucinations.

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Case 14
47-year-old brought by wife who says her husband’s snoring keeps her up at night.
He sometimes stops breathing at night and “chokes” until he clears his throat and
begins to breathe again. Patient complains of excessive sleepiness during the day,
morning headaches, and some confusion. Past medical history for high blood
pressure and obesity. Physical exam elevated blood pressure.

1. Diagnosis?

2. What is the best initial test?

3. What is the most sensitive test?

4. What is the best initial treatment?

A. Oxygen
B. Tracheostomy
C. Albuterol
D. Continuous positive airway pressure
E. Uvulopalatopharyngoplasty

Answer:

5 A daytime arterial blood gas shows the following: 7.35 / 50 / 78 / 28. Do these results
change your diagnosis?

6. What medications can you give at this time?

Patient with daytime sleepiness and irritability with leg edema. Wife says he snores and
stops breathing at night intermittently. Diagnosis?
OSA

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Summary: Case 14
In a 47-year-old obese man who complains of daytime somnolence and has apneic episodes
witnessed by others, the most likely diagnosis is obstructive sleep apnea. The best initial test
is overnight pulse oximetry. In the absence of any overnight desaturations, the likelihood of
having obstructive sleep apnea is very low. The most accurate test is sleep studies document-
ing at least 30 overnight apneic episodes lasting 10 seconds each. The best initial therapy is
CPAP. Uvulopalatopharyngoplasty is the next step in management when CPAP therapy fails.
Tracheostomy is the most effective therapy and is generally reserved for those patients who
are refractory to medical treatment or whose disease is associated with malignant cardiac
arrhythmias.

The presence of carbon dioxide retention on an ABG is diagnostic of obesity hypoventilation,

or Pickwickian syndrome. In patients with obesity hypoventilation, the treatment consists of
acetazolamide or progesterone to stimulate the central respiratory centers.

Case 15
46-year-old man with cough. Began 2 months ago progressively worsening over
the last 2 weeks. Nonproductive. Denies cigarette smoking or heart disease.
Decreased breath sounds in the right lower lobe with dullness to percussion and
decreased fremitus. Chest x-ray shows right-sided pleural effusion.

1. Diagnosis? Why?

2. Next step in the management?

3. When is thoracentesis the answer? What tests would you order on the pleural fluid? Why?

A thoracentesis is performed and 200 mL of serosanguineous fluid is drained. The

following results are obtained:

Serum LDH: 120 Pleural fluid LDH: 80

Serum protein: 6.2 g/dL Pleural fluid protein: 4.5 g/dL

Cytology: Negative for malignant cells

Stain for AFB: Negative

Pleural fluid amylase and glucose: All normal

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4. Transudate or an exudate? Why?

5. Which of the following is a cause of transudative effusions?

A. Pneumonia
B. Pancreatitis
C. Ovarian cancer
D. Rheumatoid arthritis
E. Cirrhosis
Answer:

6. What are the most common causes of exudative effusions?

7. 22-year-old woman with large, bilateral pleural effusions right greater than left. Saturating
92–94% on 2L nasal cannula. A pleurocentesis is performed, with the following results:

Pleural LDH 800 Lipase negative

Pleural protein 6.1 Fluid stain and culture negative
Pleural WBC 0-2 Pleural biopsy negative for culture
Pleural glucose 23 Serum LDH 930
Pleural amylase Serum protein 7.5

What is the most likely underlying diagnosis causing this patient’s pleural effusions?
A. Post-obstructive pneumonia
B. Rheumatoid arthritis
C. Pancreatitis
D. Esophageal rupture
E. Cholangiocarcinoma
Answer:

A V/Q scan is done and shows a low probability for pulmonary embolus.

8. What would you do next?

9. When is the answer pleurodesis?

10. What is a complicated parapneumonic effusion? How will it change your management
of this case?

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Summary: Case 15
In any unexplained pleural effusion, the next step is to obtain a decubitus film. The purpose
of this study is to ascertain the presence of free-flowing fluid in the thorax. If the fluid layers
and is >10 mm thick, then a blind thoracentesis may be performed to obtain pleural fluid
for analysis. If on the decubitus film the fluid is not free-flowing, then the thoracentesis
should be performed under ultrasound guidance. When an underlying systemic disease is
identified that can cause a pleural effusion, the underlying disease is usually treated before a
thoracentesis is performed. Only if the effusion is asymmetric, is persistent after treatment of
the underlying disease, or is associated with a fever is thoracentesis done immediately before
initiating therapy for the underlying disease.

The distinction between a transudative effusion and an exudative effusion is made by

measuring the LDH protein in the pleural fluid. Transudative effusions have a pleural fluid
LDH-to-serum LDH ratio of less than 0.6, a pleural fluid protein-to-serum protein ratio of
less than 0.5, and an absolute LDH level that is below 200. To classify an effusion as exuda-
tive, only one of the above criteria must be consistent with an exudative effusion. The most
common causes of a transudative effusion are heart failure, cirrhosis, nephrotic syndrome,
hypothyroidism, and pulmonary embolus. Exudative effusions are caused by pneumonia,
malignancy, tuberculosis, sarcoidosis, and pulmonary embolism.

Typically, a patient with a pleural effusion secondary to rheumatoid arthritis will have pleural
fluid glucose <40 g/dL. When the amylase is elevated in the pleural fluid, you should suspect
pancreatitis or esophageal rupture as the cause of the pleural effusion. In complicated para-
pneumonic effusions (frank pus, glucose <60 g/dL, pleural fluid pH <7.2, pleural fluid pH
7.2–7.3, and LDH >1,000), the next step is to place a chest tube for drainage. If the fluid does
not drain, then lavage with streptokinase may be attempted to break down the loculations. If
this is unsuccessful, then surgical debridement and drainage are indicated.

In any unexplained pleural effusion the next step is to rule out a pulmonary embolus, which
can cause both a transudative or exudative effusion. If the workup for a pulmonary embolism
is negative, the next step is to pursue the diagnosis with a video-assisted thoracic surgical
(VATS) biopsy or open pleural biopsy. The most accurate test for the diagnosis of the etiology
of a pleural effusion is the open pleural biopsy.

1. 33-year-old female immigrant complaining of gradual onset of shortness of breath

began 4 months ago. + low-grade fever and night sweats. Chest x-ray reveals a right-
sided pleural effusion. Thoracentesis is performed, which shows an exudative effusion.
Cultures for viral, acid-fast bacillus (AFB), and bacteria are all negative. Next step?
A. Open pleural biopsy
B. Repeat thoracentesis
C. Begin empiric treatment for pulmonary TB
D. Ventilation perfusion scan
E. Begin IV heparin

Answer:

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2. 47-year-old man with HIV and tuberculosis develops recurrent pleural effusions.
2 therapeutic pleural taps in the past 3 weeks. Next step?
A. Chest tube drainage
B. Pleurectomy
C. Pleurodesis
D. Pleuroperitoneal shunting
E. Thoracic duct ligation

Answer:

3. 32-year-old woman presents with fever, cough, and dyspnea. Chest x-ray shows small
right-sided pleural effusion that layers on right lateral decubitus film. Pleural fluid analysis
shows pH 7.28. Gram stain and culture results and sensitivities are obtained. Next step?
A. Antibiotic therapy
B. Chest tube drainage
C. Decortication
D. Pleurodesis
E. Thoracoscopy

Answer:

4. 53-year-old man presents with fever, cough, and dyspnea. Chest x-ray shows moderate
right-sided pleural effusion with irregular contour. The effusion does not layer on lateral
decubitus film. Pleural fluid analysis shows pH 7.19. Gram stain and culture results and
sensitivities are obtained. Next step?
A. Antibiotic therapy
B. Chest tube drainage
C. Decortication
D. Pleurodesis
E. Thoracoscopy

Answer:

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Case 16
56-year-old man comes to your office for evaluation of a 2-cm “coin lesion”
found on chest x-ray. The surrounding lung appears normal and without infiltrate,
atelectasis, or adenopathy. The patient states that he has not had any chest x-rays
performed previous to the present one.

1. What is the central question in the management of the solitary pulmonary nodule?

2. What will you do next?

3. When is infection the answer?

A. Doubling time <30 days
B. Doubling time <90 days
C. Doubling time <180 days
D. Doubling time <360 days

Answer:

4. When is hamartoma the answer?

5. When is a granuloma the answer?

6. The patient subsequently has a high definition chest CT done, showing a 2.4 cm
spiculated mass with irregular borders ~5 mm from the right hilum. What is the next
appropriate step in the management of this patient?
A. Bronchoscopy
B. Trans-thoracic needle biopsy
C. Mediastinoscopy
D. CT/PET scan

Answer:

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7. 57-year-old man newly discovered pulmonary nodule during a pre-operative evalua-

tion for a voluntary cholecystectomy. + smoker. CT of the chest shows a 2.8 cm mass in
the lateral aspect of the right middle lobe. Next step?
A. Bronchoscopy
B. PET scan
C. Trans-thoracic needle biopsy
D. Wedge resection/partial lobectomy

Answer:

Summary: Case 16
The evaluation of a solitary pulmonary nodule centers on determining the likelihood of
malignancy. The answer to this question is determined by the history and radiographic
appearance of the pulmonary nodule. Patients who smoke, are older than 30 years of age, and
have history of previous malignancy are at increased risk for having a malignant pulmonary
nodule. Radiographically, the first step in the evaluation of a solitary pulmonary nodule is to
compare the current x-ray with previous studies. If the nodule demonstrates stability and no
growth for 2 years or more on chest radiography, the nodule is not malignant. Comparison
with old radiographs also helps in determining the doubling time. If the doubling time is <35
days, infection becomes the likely cause of the pulmonary nodule. If the doubling time is >465
days, then the nodule is likely to be benign. A doubling time between 35 and 465 days sug-
gests a malignancy as the underlying diagnosis. After reviewing previous chest x-rays, the next
step is to obtain a CT scan of the chest. The CT scan provides essential information regarding
the radiological appearance of the nodule, which determines the likelihood of malignancy.
Margins that are smooth-appearing suggest a benign lesion, whereas unclear or spiculated
margins suggest malignancy. A peripheral halo or a lobulated appearance also suggests an
underlying malignancy. Laminated or dense central calcifications suggest a benign diagnosis;
eccentric or stippled calcification suggests malignancy. Popcorn calcifications are charac-
teristic of hamartomas. An “onion-skin” or “bull’s-eye” calcification suggests an underlying
granuloma.

Patients who have pulmonary nodules that have a high likelihood of malignancy should have
excisional biopsy performed. This procedure is both diagnostic and therapeutic. Patients with
pulmonary nodules that have a low probability of malignancy should be followed with serial
chest radiography and CT scans. Intermediate risk nodules traditionally have been managed
either with transthoracic needle biopsy (if located in the periphery) or bronchoscopy (if
located centrally). PET scanning is an emerging modality for evaluation of the solitary pul-
monary nodule and is used for intermediate risk nodules that are greater than 1 cm in size.

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Case 17
65-year-old woman complaining of cough productive of blood-streaked sputum.
20-lb weight loss that has occurred over the last 3 months. Denies shortness of
breath. + 40-pack-year history of cigarette smoking. Physical exam is significant for
bronchial breath sounds in the left lower lobe. + palpable supraclavicular lymph
nodes on the left side.

1. What is the next step in the management of this patient?

A chest x-ray reveals a left lower lobe mass. CT scan of the chest reveals hilar
lymphadenopathy with several nodes 2–2.5 cm in size.

2. What would you do next?

A. PET scan
B. MRI of chest
C. Mediastinoscopy
D. Send for surgical resection

Answer:

A diagnosis of adenocarcinoma of the lung is made after biopsy.

3. What is the next step in the management of this patient?

4. Would your management change if the diagnosis were small cell carcinoma? How?

5. When is surgery the answer?

6. Before resection, what must you check?

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Summary: Case 17
The next step in the evaluation of a patient who presents with hemoptysis is to obtain a chest
x-ray. Chest CT scan and bronchoscopy are indicated only after the chest x-ray does not
reveal an underlying cause of the hemoptysis. The amount of hemoptysis does not reflect the
seriousness of the underlying disease. Thus, even a small amount of hemoptysis should be
evaluated aggressively. Once a pulmonary mass is identified, the next step is to pursue a tissue
diagnosis. The preferred site of biopsy is the site that is associated with the least morbidity.

In the management of lung cancer, the most important distinction to make is between small
cell carcinoma (which is sensitive to chemotherapy and radiation but tends to metastasize
early in the disease course) and non–small cell carcinoma (which is resistant to chemother-
apy and radiation and whose definitive treatment consists of surgical excision). Thus, if the
biopsy returns as a non–small cell carcinoma of the lung, the next step is to determine the
stage of the lung cancer. This is important because surgery is indicated only in Stage I, II, and
IIIa disease. Staging of non–small cell cancer of the lung consists of a CT scan of the chest
and abdomen up to the adrenal glands. If mediastinal lymphadenopathy greater than 1 cm
in size is identified, then mediastinoscopy with lymph node biopsy is indicated. The therapy
for lung cancer is summarized in the table below.

Treatment of Lung Cancer

Stage Feature Non–Small Cell Stage Small Cell

Carcinoma Carcinoma
I <3 cm Surgery Limited Etoposide, cisplatin,
and radiotherapy of
hemithorax
II 3-5 cm (A) Surgery Extensive Etoposide, cisplatin,
and palliative
5-7 cm (B) radiotherapy
IIIa Ipsillateral and Surgery (if non-
carinal lymph bulky disease)
nodes plus carboplatin,
etoposide
IIIb Contrallateal Radiation plus
lymph nodes carboplatin,
etoposide, no
surgery
IV Distant mets Carboplatin,
etoposide with good
performance status,
otherwise palliative
care

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Case 18
A 23-year-old woman complaining of shortness of breath started 7 months ago.
She also complains of fatigue, and recently had a fainting spell while walking
briskly down the street. – heart disease and – family history of heart disease.
Physical exam is significant for splitting of the second heart sound with a loud
pulmonary component and a parasternal heave. EKG:

I V1 V4
aVR

II aVL V2 V5

III aVR V3 V6

VI

Chest x-ray shows enlarged central pulmonary arteries.

1. What is the most likely diagnosis? Why?

2. What is the next step in the management of this patient? What is the logic?
A. Two-dimensional echocardiogram
B. Right heart catheterization
C. Chest CT
D. Aspirin
E. Enoxaparin

Answer:

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3. After establishing the diagnosis, what would you do next? How would the results affect
your management of the case?

4. When is warfarin the answer?

5. When is prostacyclin the answer?

6. When are calcium channel blockers the answer?

7. When is heart–lung transplantation the answer?

Summary: Case 18
In a young woman who presents with exertional shortness of breath, syncope, and fatigue
associated with a prominent P2 and evidence of elevated right heart pressures on EKG, the
most likely diagnosis is pulmonary hypertension. At this point the etiology of the pulmonary
hypertension is unknown. The next step in the management of this patient is to account
for the pulmonary hypertension that is being observed clinically. Initially, a 2-dimensional
echocardiogram is necessary to assure proper left ventricular function. Lung disease, acidosis,
chronic hypoxia, chronic recurrent pulmonary emboli, decreased LV systolic function, dia-
stolic dysfunction, and increased blood viscosity are all causes of pulmonary hypertension
that should be considered and excluded in this patient. Only after all causes of pulmonary
hypertension have been excluded can the diagnosis of primary pulmonary hyperten-
sion be considered. The management of primary pulmonary hypertension is determined
by the response of the vasculature to vasodilatation during right heart catheterization. If
when the patient is given adenosine, prostacyclin, or nitrous oxide there is a marked reduction
in the pulmonary vascular resistance, the next step is to challenge with calcium channel blockers.
If the vasculature responds, then the treatment of choice is calcium channel blockers. If there
is no response to IV vasodilators, then the management depends on the functional status of
the patient. In patients with New York Heart Association (NYHA) Class I or II symptoms,
the treatment is sildenafil. In patients with NYHA Class III symptoms, the treatment is
with bosentan. For Class IV symptoms, treatment is prostacyclins or prostacyclin analogue.
Transplantation should be considered only for those patients who progress to right-sided
heart failure while being treated with prostacyclin, or those patients who cannot tolerate
medical therapy.

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Categories of Pulmonary Hypertension

Idiopathic (treat with warfarin)
• Primary pulmonary hypertension
• HIV
Pulmonary-Venous Disease (treat left heart disease)
• Left heart disease
• Compression (tumors)
Hypoxic (treat with oxygen)
• COPD
• Interstitial lung disease
• Obstructive sleep apnea
• High altitude
Embolic Disease (treat with warfarin)
• Chronic pulmonary embolism
Pulmonary Vascular Disease (treat underlying process)
• Inflammation
• Sarcoidosis
• Schistosomiasis

Case 19
56-year-old man is admitted after falling and breaking his hip. Has an uncomplicated
postoperative course. Patient states that he is experiencing SOB, associated with
chest pain that worsens with deep breathing. Five minutes prior no distress.
Physical exam tachycardia, lungs are clear to auscultation bilaterally. ABG:

7.49 / 28 / 90 / 24.

Chest x-ray shows no significant cardiopulmonary disease.

1. What is the most likely diagnosis? Why?

2. When is thrombolytic therapy the answer?

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3. Which of the following will confirm the diagnosis?

A. Cardiac stress test
B. Echocardiography
C. Pulmonary function tests
D. Right heart catheterization
E. V/Q scan
Answer:

4. If the patient is a pregnant female, what will you use for long-term treatment?
A. Warfarin
B. Aspirin
C. IVC filter
D. Dalteparin
E. Lepirudin
Answer:

5. How long would you treat?

6. What are the major risk factors for this disease?

7. A 19-year-old woman presents to the emergency department with acute shortness of

breath. She has just returned on an 8-hour-flight from a business trip to Greece. In the
emergency department she has an oxygen saturation of 89–91% on room air. What is
the next best step to diagnose this patient?
A. 2-dimensional echocardiogram
B. Spiral CT
C. D-dimer
D. V/Q scan
E. Lower extremity Doppler
Answer:

Summary: Case 19
In any patient who presents with unexplained shortness of breath, hypoxia, or tachycardia,
the diagnosis of pulmonary embolism should be suspected. Thrombolytic therapy in the
treatment of PE is indicated when there is hemodynamically unstable PE in the absence of
any contraindications to thrombolytic therapy. For the pregnant female who requires chronic
anticoagulation after a thromboembolic event, low molecular-weight heparin for 6 months
is the treatment of choice. Major risk factors for the development of a pulmonary embolism
include immobility, malignancy, nephrotic syndrome, oral contraceptives, pregnancy, obesity,
hormone replacement therapy. and heart failure. The imaging modality of choice is a high

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resolution spiral CT, which provides the best sensitivity and specificity for clinically signifi-
cant pulmonary emboli. The “gold standard” is still a pulmonary angiogram, but this is a
very high risk procedure, and rarely done. The diagnostic workup of pulmonary embolism
is summarized below.

Clinical suspicion of PE
Chest radiograph and ABG

PE The next diagnostic test is… Treat as

excluded Normal High prob.
V/Q scan or spiral CT scan PE

Low or intermediate
probability with
clinical suspicion for a
pulmonary embolus

PE Negative
excluded The next diagnostic test is…
Lower extremity Duplex with D-dimer

The next diagnostic test is…

Pulmonary angiogram

• The best initial test for PE is V/Q scan or spiral CT scan

• The most sensitive test for PE is pulmonary angiogram

1. 63-year-old woman complaining of right lower extremity swelling. History is sig-

nificant for recent GI bleed that required multiple blood transfusions. Lower-extremity
duplex shows right superficial femoral vein thrombosis. Next step in the management?
A. Subcutaneous heparin
B. Warfarin and IVC filter placement
C. Low-dose warfarin
D. IVC filter placement
E. Observation

Answer:

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2. 46-year-old woman evaluation of difficulty breathing that began suddenly after flying.
Upon arrival at the emergency department, IV heparin is started and she is sent for a
V/Q scan, which shows an intermediate probability for a pulmonary embolism. Next
step in the management?
A. Chest CT scan
B. Pulmonary artery magnetic resonance angiography
C. Lower-extremity ultrasound
D. Begin warfarin to INR 2.0–3.0

Answer:

3. 52-year-old with breast cancer presents with acute dyspnea and tachypnea. BP is
80/55 mm Hg and is unresponsive to intravenous fluids and vasopressors. CT angio-
gram confirms pulmonary embolism. EKG shows high amplitude P wave in lead II and
right axis deviation. Most appropriate next step in management?
A. Low-molecular-weight heparin alone
B. Unfractionated heparin
C. Thrombectomy
D. Thrombolytic
E. Warfarin

Answer:

4. 49-year-old man with history of renal cell carcinoma with brain lesions presents with
cough, hemoptysis, and dyspnea. Exam reveals S3, prominent JVD. Hypotension is not
responsive to fluid resuscitation. CT angiogram confirms large pulmonary embolus.
Most appropriate next step in management?
A. Thrombolytic
B. Thrombectomy
C. Low-molecular-weight heparin alone
D. Low-molecular-weight heparin with thrombolytic
E. IVC filter placement

Answer:

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Case 20
A 42-year-old man presents with cough, fever, and dyspnea of 4 days duration.
Chest x-ray shows diffuse alveolar opacities. Laboratory profile shows increased
WBC and creatinine, and decreased hematocrit. DLCO is increased. Urinalysis
reveals 2+ proteinuria.

1. Which of the following is the most likely diagnosis?

A. Alveolar hemorrhage
B. Interstitial fibrosis
C. Septic emboli
D. Pleural effusion
E. Pulmonary edema

Answer:

2. Which of the following is the most appropriate initial diagnostic test?

A. Blood cultures
B. Bronchoalveolar lavage
C. CT scan of chest
D. Pulmonary function tests
E. Renal ultrasound

Answer:

3. Renal biopsy is performed and shows IgG along the glomerular capillaries. Which of the
following is most likely to have positive serological tests?
A. Anti-GBM antibodies
B. Antiphospholipid antibodies
C. Antistreptococcal antibodies
D. Antinuclear antibody
E. Rheumatoid factor

Answer:

4. Which of the following illicit drugs should be suspected in a patient with fever, dyspnea,
cough, and patchy infiltrates on chest x-ray?
A. Crack cocaine
B. Heroin
C. LSD
D. Marijuana
E. Opium

Answer:

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Summary: Case 20
Diffuse alveolar hemorrhage (DAH) presents with cough, fever, dyspnea, and unexplained
diffuse alveolar infiltrates present less than a week. Vasculitis should be considered in such
patients particularly with new-onset renal insufficiency or known connective tissue disor-
der. Up to one-third of patients with diffuse alveolar hemorrhage do not have hemoptysis.
Diagnosis is made with bronchoalveolar lavage, with samples showing a persistently bloody
fluid. Chest x-ray shows new patchy or diffuse alveolar opacities. A sensitive marker for DAH
is an increase in diffusing capacity for carbon monoxide (DLCO). An elevated white blood
cell count and falling hematocrit are also common. Based on the history, you should consider
the following underlying conditions and appropriate workup:

• Wegener granulmomatosis in patients who present with upper and lower respiratory
tract lesions and glomerulonephritis (proteinuria, hematuria, oliguria, and elevated
creatinine). Antineutrophil cytoplasmic antibodies, (c-ANCA) favors the diagnosis.
The p-ANCA suggests microscopic polyarteritis or Churg-Strauss syndrome.
• Goodpasture syndrome in patients with glomerulonephritis. Anti-glomerular base-
ment membrane antibodies (anti-GBM) is diagnostic.
• SLE in patients that present with multiple organ vasculitis, including purpuric skin
lesions, joint disorders, kidney, neurological involvement. Antinuclear antibodies
and anti-DNA antibodies are diagnostic.
• Antiphospholipid syndrome when the patient has a history of venous or arterial
thromboses, recurrent fetal loss, or thrombocytopenia. Presence of anticardiolipin
antibodies, anti-β2-glycoprotein I, and/or a lupus anticoagulant diagnostic.
• Poststreptococcal glomerulonephritis in a patient with fever, malaise, and history
of throat infection 1–2 weeks ago (suggesting group A beta hemolytic streptococ-
cus). Antistreptococcal antibodies aid in the diagnosis.
• Cocaine-induced pulmonary hemorrhage when drug abuse is suspected.

Corticosteroids are the management of choice. Plasmapheresis is generally used in the treat-
ment of DAH associated with Goodpasture syndrome. Recurrent episodes of DAH may lead
to pulmonary fibrosis and interstitial opacities.

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Pt develops SOB and pulmonary edema while skiing at 7800 ft. Next step?
O2
Next step?
Bring him to lower altitude
Best way to prevent future occurrence
Acetazolamide

Case 21
A 26-year-old mountaineer presents prior to leaving for Nepal. He is planning
a hiking trip at the base of the Himalayas. He reports that on his last trip he
developed nausea, headaches, and difficulty sleeping.

1. Which of the following is an appropriate therapy for the prophylaxis of his symptoms?
A. Acetazolamide
B. Albuterol inhaler
C. Furosemide
D. Metaclopramide
E. Aspirin

Answer: The correct answer is A. Acetazolamide

2. On ascent, he complains of dyspnea, headache, and nausea. Which of the following is

the most appropriate next step in management?
A. Bed rest
B. Descent to lower altitudes
C. Furosemide
D. Oxygen therapy
E. Mannitol

Answer:

The most common form of high altitude illness is acute mountain sickness (AMS) and
is usually mild. Acetazolamide should be considered the first-line medication for AMS.
Dexamethasone should be used in individuals who cannot tolerate sulfa. However, the most
important prevention is slow ascent, hydration, and rest.

More severe forms of altitude disease include high altitude pulmonary edema and high
altitude cerebral edema (HACE). They require immediate descent as soon as possible.
Medications (nifedipine, inhaled nitric oxide, and dexamethasone), oxygen, and use of the
portable hyperbaric chamber may be very helpful, but they should never be used to delay
transporting a victim to lower altitude.

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8
Case 1
40-year-old woman brought to ED by husband after she ingested bottle of pills
in attempt to commit suicide. She came 30 minutes after the ingestion. Exam
normal.

1. What to do first?
Ipecac is always wrong. Perform a gastric lavage if she has an altered mental status after
prophylactic intubation to protect her airway. About 25 to 50% of the ingested pills can
be removed within the first hour of ingestion. There is no efficacy of gastric emptying
after 2 hours.

2. In whom is this intervention contraindicated?

3. When is charcoal dangerous?

4. When will your answer be hydration and simple forced diuresis?

Summary: Case 1
Gastric emptying is rarely used any more in the management of overdoses. With gastric emp-
tying, only 50% of pills at 1 hour after ingestion can be removed and only 15% at 2 hours, with
nothing significant removed after that. Ipecac is never an emergency department therapy; it
is mostly used for people at home, too far from the hospital, or in children immediately after
the ingestion. Charcoal is the mainstay of toxicology management. It has no adverse effects
and can remove toxins from the bloodstream even after they have been absorbed.

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Case 2
40-year-old woman brought to ER after she ingested a bottle of pills. She is
severely confused and lethargic. Comes within 30 minutes after the ingestion. On
exam, she is sleepy, dopey, stuporous, disoriented, and obtunded.

1. What to do first?

2. What will you do if your answer to question 1 has already been done?

3. When will your answer be flumazenil?

4. Most common complication of flumazenil use?

Case 3
72-year-old man comes to emergency department (ED) because of altered
mental status, dyspnea, tinnitus, and hyperventilation developing over the last
several hours. History of severe osteoarthritis. 38ºC (100.3ºF) orally. Chest x-ray
noncardiogenic pulmonary edema. Mildly elevated BUN and creatinine, low
serum bicarbonate of 14, mildly elevated prothrombin time, and a blood gas with
pH 7.49 and a pCO2 of 23.

1. Likely diagnosis?

2. Best initial therapy?

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Summary: Case 3
The key to the diagnosis is hyperventilation combined with metabolic acidosis and tinnitus.
Salicylates are a complex metabolic poison. They directly stimulate the brain stem, causing
hyperventilation, and are toxic to the lungs as well, causing adult respiratory distress syn-
drome (ARDS) with a chest x-ray that looks like pulmonary edema. Analgesics in general are
toxic to the kidney tubule, resulting in acute tubular necrosis (ATN). Aspirin also interferes
with the vitamin K–dependent production of clotting factors, resulting in prolongation of
the prothrombin time. The outcome is a lactic acidosis, resulting in a metabolic acidosis with
an increased anion gap.

1. Elderly woman with bilateral hearing loss and tinnitus. She has to turn
the volume higher on the TV. Takes aspirin for rheumatoid arthritis. What is the
diagnosis?
A. Aspirin toxicity
B. Presbycusis
C. Cerebellopontine angle tumor
D. Cerumen impaction

Answer:

Case 4
Woman with long history of depression comes after attempting to commit
suicide by ingesting bottle of amitriptyline.

1. Most important step now?

A. Blood level
B. Urine level
C. Bicarbonate
D. EKG
E. Charcoal

Answer:

2. Best initial therapy if at toxic level?

Summary: Case 4
Tricyclic antidepressants are directly toxic to many parts of the body because of their anti-
cholinergic effects. Although you get blurred vision, dry mouth, urinary retention, and
constipation, you don’t die from them. When questions ask you for “the most URGENT” or
“the most IMPORTANT” step for an overdose patient, they are asking if you know how the
patient will die first. Tricyclics kill via seizures and arrhythmias. The first clue to an imminent
cardiac problem is a wide QRS.

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Case 5
Escaping from very tall burning building, a 64-year-old man walks down more
than 50 flights of stairs. Many floors are on fire. No direct burns. History of aortic
stenosis that has been moderate in severity. Carboxyhemoglobin level is 32%.

1. What to do first?

2. Most important diagnostic test at this time? (or, how is he going to die first?)

3. What will a pulse oximeter show?

Alternate for Case 5

Elderly man and family live in a house that uses a wood stove for heat. They
have not been able to get out of the house because of heavy snow. They now
have headaches, dizziness, nausea, and shortness of breath. The father feels
better when shoveling snow.

1. What to do?
A. Switch to oil heat
B. Open the window
C. Call an ambulance
D. Give supplemental home oxygen
E. Check RBCs

Answer:

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Case 6
A 31-year-old Marine captain has been brought because of heavy terrorist activity
at Madison Square Garden, where chemical agents may have been released. He
has shortness of breath, excessive salivation, diarrhea, weak muscles, polyuria,
and abdominal cramping. Exam: constricted pupils and wheezing.

1. Most likely diagnosis?

2. What should you do first?

A. Remove clothes
B. Wash the patient
C. Atropine
D. Pralidoxime
E. Red cell cholinesterase level
F. Intubation

Answer:

3. Most specific treatment?

Case 7
78-year-old woman with congestive heart failure is brought to the emergency
department with several days of confusion, nausea, abdominal pain, vomiting,
palpitations, and altered color perception, such as yellow halos around objects.
She is maintained on an ACE inhibitor, digoxin, furosemide, and metoprolol.
Digoxin level is elevated.

1. Most common manifestation of digoxin toxicity?

Gastrointestinal disturbance

2. What cardiac rhythm disturbances are possible?

A. Atrial
B. Ventricular
C. Ectopy
D. Anything

Answer:

3. Most common cardiac dysrhythmia with digoxin toxicity?

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4. Most urgent step in this patient?

EKG

5. What will patient’s potassium level be?

6. Strongest indication for digoxin immune Fab (Digibind®)?

Case 8
47-year-old man lives in rural West Virginia being one of the 2% of Americans left
without indoor plumbing. As he is sitting in his outhouse, he experiences sudden
pain in perineum. Bitten by local insects. He develops waves of muscular pain
that extend to his abdomen. Abdomen extremely painful and rigid.

1. Diagnosis?

2. Most common electrolyte abnormality?

A. Low magnesium
B. High sodium
C. Low calcium
D. High potassium
E. Low potassium

Answer:

3. What are the initial and most effective therapies?

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Case 9
It is February in Chicago and one of the local homeless alcoholics has been
brought in because of lethargy worse than his usual episodes of drunkenness.
His body temperature is found to be 31.1ºC (88ºF).

1. What is the most urgent test?

A. Urinalysis
B. EKG
C. Arterial blood gas
D. Calcium
E. Urine toxicology

Answer:

2. What would be the most specific finding?

Case 10
82-year-old homebound woman brought to ED because of bleeding gums,
ecchymoses on her legs, and several nonhealing ulcers. She is malnourished
and gives a history of living on a “tea and toast” diet. PT, PTT, and platelet count
are normal.

1. Most likely diagnosis?

Case 11
Generally healthy athlete comes to ED because of headache, bone pain, nausea,
vomiting, and vertigo. Found to have papilledema. His only medications are
numerous vitamin tablets. Head CT scan is normal. There is some ataxia,
alopecia, and laboratory evidence of hepatic toxicity. He also has dry skin, dry
eyes, and mild confusion.

1. What is the most likely diagnosis?

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Case 12
34-year-old man on skiing trip in Swiss Alps at altitude 14,000 feet. He is a healthy
nonsmoker but develops severe shortness of breath. Rales heard on lung exam.
EKG unremarkable. Chest x-ray consistent with vascular congestion.

1. Best management?
A. Rapid descent to a lower altitude
B. Oral acetazolamide
C. Intravenous diuretics
D. Corticosteroids
E. ACE inhibitors
F. Diphenhydramine

Answer:

Case 13
57-year-old vegetarian is visiting her family from the ashram where she has
become a devout Hindu. She has memory loss, diarrhea, malnutrition, and thick
skin that is hyperpigmented.

1. Which is the Most likely etiology of her problem?

A. Zinc deficiency
B. Atopic dermatitis
C. Riboflavin deficiency
D. Nicotinic acid deficiency

Answer:

Case 14
Patient on long-term total parenteral nutrition (TPN) develops new-onset
hyperglycemia.

1. Deficiency of which of these caused it?

A. Selenium
B. Chromium
C. Copper
D. Zinc

Answer:

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Case 15
An Elderly hypertensive man maintained on chronic hyperalimentation and
captopril. He develops dermatitis of extremities and altered taste (dysgeusia).

1. What is the cause?

A. Selenium
B. Chromium
C. Copper
D. Zinc

Answer:

Case 16
Following ingestion of home canned food, a young woman has sudden onset of
blurred vision, diplopia, dysarthria, dysphagia, and constipation. She has muscle
weakness in a descending fashion. Tongue is dry and pupils are fixed and dilated.

1. Diagnosis?
A. Guillain Barré syndrome
B. Myasthenia gravis
C. Botulism
D. Salmonella
E. Ciguatera food poisoning

Answer:

Case 17
1. Which is consistent with ethylene glycol toxicity?
A. Increased osmolar gap
B. Increased anion gap
C. Calcium oxalate crystals in urine
D. Low serum calcium
E. All of the above

Answer:

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ACETAMINOPHEN OVERDOSE

Case 1
35-year-old man comes to hospital 4 DAYS after he ingested bottle (60 tablets)
of acetaminophen. He came today because of developing nausea, vomiting,
and abdominal pain. Mildly elevated bilirubin, AST, and prothrombin time, with
mildly decreased albumin level.

1. Next step in management?

Case 2
35-year-old man comes to hospital 4 HOURS after he ingested bottle (60 tablets)
of acetaminophen. He came because of developing nausea, vomiting, and
abdominal pain.

1. Next step in management?

Case 3
35-year-old man comes to hospital 30 MINUTES after he ingested a large
bottle (60 tablets) of acetaminophen. He came because of developing nausea,
vomiting, and abdominal pain.

1. Next step in management?

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ADVERSE EFFECTS AND DRUG TOXICITY

Case 1
1. Which juice increases blood levels of cyclosporine?
A. Orange
B. Tomato
C. Grapefruit
D. Apple
E. Pineapple

Answer:

Case 2
A 36-year-old woman with long-term inflammatory bowel disease is about to
start using infliximab to close up a fistula she has developed.

1. What to do prior to starting the infliximab?

A. Start prednisone
B. PPD skin testing
C. Increase the mesalamine
D. Start azathioprine

Answer:

Case 3
Man with a history of bipolar syndrome comes because of unsteady gait, muscle
twitching, tremor, dysarthria, and fasciculations.

1. Which caused this presentation?

A. Fluoxetine
B. Amitriptyline
C. Lithium
D. Prochlorperazine

Answer:

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Alternate for Case 3

Patient who is disoriented and confused is brought to the emergency department
unresponsive with muscle twitching. He has a history of bipolar disorder. He is
dehydrated and mildly hypotensive. There is a mild leukocytosis and the serum
sodium is 170 with a potassium of 4.0.

1. Diagnosis?
A. SIADH
B. Nephrogenic diabetes insipidus
C. Dehydration
D. Salicylism
E. Central diabetes insipidus

Answer:

Case 4
20-year-old man is brought to ED in comatose state. He was visibly intoxicated
at a party with his friends earlier in the evening. Serum bicarbonate is low at 22,
but anion gap is normal. Ethanol level is zero, and there is an elevated osmolar
gap. Urinalysis is normal.

1. Diagnosis?
A. Isopropyl alcohol
B. Methanol
C. Ethylene glycol
D. Aspirin

Answer:

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Case 5
32-year-old man with sexual dysfunction. He is able to achieve erection but not
orgasm. History of depression and recently started on new antidepressant.

1. What led to this problem?

A. Bupropion
B. SSRIs
C. Mirtazapine
D. Risperdal
E. Tricyclic antidepressants

Answer:

Case 6
50-year-old man with schizophrenia is brought to emergency department
because of confusion and a temperature of 40.4ºC (104.8ºF). Recently started on
chlorpromazine. He has some muscular rigidity, and CPK is elevated. Urinalysis
is normal.

1. What is most appropriate management?

A. Head CT scan
B. Lumbar puncture
C. Brain biopsy
D. Stop chlorpromazine and use cooling blanket
E. Stop chlorpromazine and use bromocriptine and dantrolene

Answer:

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Case 7
46-year-old woman is brought to ED for nausea, diarrhea, agitation, disorientation,
tremor, and restlessness. She is usually on nefazodone for her depression.
Recently started on new medication for her depression, but she does not recall
the name.

1. Which medication was most likely started?

A. Bupropion
B. Methylphenidate
C. SSRIs
D. Tricyclic antidepressants
E. Valproic acid

Answer:

Case 8
37-year-old man with generalized discomfort. Has pupillary dilation, lacrimation,
rhinorrhea, piloerection, and yawning. In addition, he has diarrhea and needle-
sticks on his arms. He admits to abusing pills, but he refuses to tell you the
specific names.

1. Most appropriate therapy?

A. Benzodiazepines
B. Naloxone
C. Methadone
D. Flumazenil

Answer:

Case 9
1. Most common adverse effect of bupropion?
A. Rhythm disorder
B. Headache
C. Seizures
D. Xerostomia

Answer:

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Case 10
Patient comes to ED because of nausea from gastroenteritis. Patient is treated
with metoclopramide and develops distortion of the neck and eyes.

1. How to treat?
A. Benzodiazepines
B. Diphenhydramine
C. Prochlorperazine
D. Chlorpromazine

Answer:

2. 82-year-old patient has recently started to take aspirin, vitamin E, glucosamine, ginkgo
biloba, and calcium carbonate. He comes to you with the recent onset of constipation.
Which caused the constipation?
A. Aspirin
B. Calcium carbonate
C. Ginkgo biloba
D. Glucosamine
E. Vitamin E

Answer:

3. Which is associated with pulmonary fibrosis?

A. Quinolone
B. Quinidine
C. Warfarin
D. Amiodarone
E. Cholestyramine

Answer:

4. Why should metformin be stopped in diabetic patients with renal failure?

A. Hyperkalemia
B. Lactic acidosis
C. Hypoglycemia
D. Ketoacidosis
E. Renal toxic

Answer:

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5. 55-year-old woman with severe shortness of breath and low oxygen saturation.
Temperature is normal. Earlier she had an outpatient, upper endoscopy, which included
xylocaine spray. What is the cause?
A. Aspiration pneumonia
B. Pulmonary embolism
C Diaphragmatic paralysis
D. Methemoglobinemia

Answer:

6. Which of the following is most likely to have an increased end-tidal CO2?

A. Asthma
B. Malignant hyperthermia
C Anemia

Answer:

7. A patient with hypertension and coronary disease takes an overdose of 40 metoprolol

pills. BP 76/40, pulse 36/minutes with AV block. After IV fluids, the BP is still low. What
would you add to therapy?
A. Norepinephrine
B. Epinephrine
C Glucagon

Answer:

8. Overdose of diltiazem. BP 70/40, pulse 44/minute. In addition to IV fluids, what should

you do?

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EPIDEMIOLOGY

1. What is the fastest growing segment of the US population?

A. 15–25
B. 35–45
C 65–75
D. > 85

Answer:

2. What is the most common reason to be admitted to the hospital in the United States?
A. MI
B. CHF
C Falls
D Pneumonia

Answer:

3. Most common hospital acquired infection?

A. Urine
B. Lung
C Skin

Answer:

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IMMUNOLOGY

Case 1
43-year-old farmer from rural area is being discharged after an immediate
hypersensitivity (anaphylactic) reaction secondary to a bee sting. He was treated
with epinephrine, fluids, and steroids.

1. What is the best long-term management?

A. Reassurance that all will be well
B. Chronic oral steroids
C. Recommend that the patient change professions
D. Desensitization (immunotherapy)
E. Chronic antihistamine use (diphenhydramine, ranitidine)

Answer:

Summary: Case 1
Venom-specific immunotherapy offers 98% protection from immediate hypersensitivity
reactions. This is done after specific allergic testing to confirm the precise etiology of the
immediate hypersensitivity reaction. This patient has no guarantee that he will not be stung
again by a bee even if he does change his profession. Patients with anaphylaxis should not be
maintained on beta blockers. These drugs block the effect of the epinephrine that is used in
the management of acute anaphylactic reactions.

1. Patient with a history of allergic rhinitis is coming to the office for immunotherapy to
desensitize him to an environmental allergen. He has a history of hypertension main-
tained on propranolol and hydrochlorothiazide. What is most important prior to the
initiation of immunotherapy?
A. Stop hydrochlorothiazide
B. Stop propranolol
C. Start oral prednisone
D. Admit to intensive care unit

Answer:

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Case 2
Patient recently been started on multiple medications after admission to nursing
home. The patient has developed swollen lips consistent with angioedema.

1. Which of the following is most likely responsible?

A. Vitamin E
B. Aspirin
C. Beta blockers
D. Nifedipine
E. Enalapril

Answer:

Case 3
Anaphylactic shock occurs is a 53-year-old man, following a bee sting. His is
on labetolol for hypertension. Epinephrine and diphenhydramine are given in
the emergency department, but the BP is only 85/60 and the pulse is 56. What
should you do next?

A. More epinephrine
B. Ephedrine
C. Glucagon
D. Ranitidine

Answer:

Case 4
Patient with asthma and other allergies wants to avoid exposure to mites. Which
is most useful?

A. Vacuum carpets frequently

B. Wash clothes once a week
C. Use insecticide
D. Keep humidity in the room at 75–80%
E. Encase mattress, pillows, and box spring in zippered covers

Answer:

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Summary: Case 2–4

Most chronic urticaria is caused by pressure, cold, vibration, and exercise. The medication
most likely to be associated with angioneurotic edema is ACE inhibitors. This is usually due
to the patient having a hereditary deficiency of C1 esterase. Patients with hereditary deficien-
cies of C1 esterase inhibitor are treated with anabolic steroids or with infusion of the deficient
inhibitor.

Beta blockers can be reversed with the use of glucagon. Glucagon can also be effective in
beta–blocker overdose.

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COMPLEMENT DEFICIENCY

Case 1
31-year-old patient with SLE comes with pleuritic chest pain and yearly sinusitis.
CH50 is undetectable.

1. Which deficiency is most likely?

A. C2 deficiency
B. C3 deficiency
C. C5-C8 deficiency
D. C9 deficiency

Answer:

Case 2
14-year-old female student develops non-pruritic and non-pitting facial edema
after a pillow fight with roommate. The patient’s sister has had similar symptoms.

1. Diagnosis?
A. Insect sting
B. C1 inhibitor deficiency
C. Contact dermatitis
D. Factor XIII deficiency

Answer:

Case 3
Child presents with angioedema.

1. What test would you order first?

A. C2 and C4 levels
B. IgE levels
C. Patch testing
D. C1 esterase inhibitor level

Answer:

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Case 4
1. Most effective treatment of hereditary angioedema?
A. Doxepin
B. Danazol
C. Diuretics
D. Prednisone chronically

Answer:

Case 5
14-year-old boy with swollen hand and forearm that developed after shop class
on wood chopping. He has had 5 trips to the emergency department with a
negative evaluation. Father died at age 30 of unknown cause.

1. Which are you most likely to find?

A. Increased IgE levels
B. White count of 7,800 with 10% basophils
C. Decreased C2 and C4 levels
D. Abnormal allergic skin tests for dust

Answer:

Case 6
31-year-old patient has an itchy and runny nose when flowers are blooming in
spring.

1. What is your advice?

A. Nasal steroids
B. Nasal saline
C. Monteleukast (singulair)
D. Nasal oxymetazoline

Answer:

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Case 7
73-year-old man with fever and hypotension. Two years ago he was involved in
a car accident following which he had abdominal surgery for internal bleeding.

1. Why did he get fever and hypotension?

A. Late complement deficiency
B. Early complement deficiency
C. Pneumococcal sepsis
D. IgA deficiency

Answer:

Summary:

Complement Deficiency
C2 complement deficiency is the most common in North-American Caucasians. C2 comple-
ment deficiency is also associated with SLE and other autoimmune diseases. It is also associ-
ated with recurrent sinopulmonary diseases. Hereditary angioedema can be brought on by
both physical and emotional trauma. A woman being hit in the face with a pillow can develop
an attack of this severe, non-pitting, and non-pruritic edema. It is caused by C1 esterase
inhibitor deficiency. ACE inhibitors can also provoke attacks. When initially diagnosing C1
esterase inhibitor deficiency, C2 and C4 levels should generally be done first. If BOTH choices
are in the answer, then choose C2 and C4 as the best INITIAL diagnostic test. C2 and C4
levels are always low due to chronic over-activation. They go even lower during acute attacks.
C1 levels can sometimes be artificially normal; and the level of activity can be harder to
perform as a diagnostic test. Long-term treatment of hereditary angioedema is best achieved
with danazol or stanazol, which are synthetic androgens that have few adverse effects. Allergic
rhinitis is routinely treated with nasal steroids. A motor vehicle accident can result in a sple-
nectomy, which markedly increases the risk for pneumococcal sepsis. Complement deficiency
cannot result from trauma or surgery and does not begin at an older age, such as 73. A car
accident cannot cause or unmask complement deficiency.

Common Variable Immunodeficiency

Common variable immunodeficiency (CVID) is a disease of the of the second to fourth
decade of life; as opposed to X-linked agammaglobulinemia, which starts to show clinical
manifestations as early as the first year of life. The clinical manifestations of both diseases are
not clinically distinguishable, and they consist of an increased frequency of sinopulmonary
infections. CVID is characterized by a normal number of B cells with a low function. They
do not develop into antibody-producing plasma cells. In addition, there is an increased inci-
dence of autoantibody diseases such as hemolytic anemia, atrophic gastritis, bronchiectasis,
pernicious anemia, giardiasis, and gastric carcinoma. CVID is treated with immunoglobulin
replacement.

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X-Linked Agammaglobinemia
X-linked agammaglobulinemia is a disease of the first year of life. There are no B cells, and
there is hypoplasia of the tonsils and lymph nodes. Germinal centers are not present. As with
CVID, there is an increased incidence of sinopulmonary infections. X-linked agammaglobu-
linemia occurs only in males. Treatment is with immunoglobulin replacement.

Selective IgA Deficiency

Selective IgA deficiency is the most common immunodeficiency syndrome, with a 1-in-500
incidence. It is characterized by recurrent respiratory infections and a high frequency of
atopic diseases. There is often a sprue-like diarrheal disease that sometimes responds to a
gluten-free diet. There is increased frequency of giardiasis. There is no specific treatment;
infections are treated as they occur, and the patient must receive IgA-deficient blood or
washed red cells.

1. 30-year-old man presents with recurrent sinusitis and pneumonia. He has normal
white cell count with normal number of B and T cells. IgG, IgM, and IgA levels are low.
Diagnosis?
A. Malabsorption
B. Multiple myeloma
C. Bruton’s X-linked agammaglobulinemia
D. Common variable immunodeficiency

Answer:

Hyper IgE Syndrome

Hyper IgE syndrome is a disorder characterized by recurrent abscesses involving the skin and
lungs. Increased staphylococcal infection is common to all patients. Prophylaxis with anti-
staphylococcal penicillins or cephalosporins is the primary management.

1. Which of the following should not be given to someone with IgA deficiency?
A. Pneumococcal vaccine
B. Influenza vaccine
C. Intravenous immunoglobulins
D. Prednisone

Answer:

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9
PSORIASIS
This disorder affects 2% of people throughout the world. One-third of patients have a joint
disorder associated with it. Physical examination reveals silvery scale on the extensor surfaces.
Psoriasis can also be widely disseminated throughout the entire body. The Koebner phenom-
enon is a worsening of the disease, with trauma to the skin.

Initial treatment consists of topical steroids to localized disease. In order to have the steroid
penetrate the skin, keratolytic agents such as salicylic acid derivatives remove the superficial
scales.

Long-term therapy involves the use of vitamin D ointments such as calcipotriene, and vita-
min A derivatives such as tazarotene gel. All patients will find emollients to soften the skin
useful, such as Aveeno® oatmeal baths, Lubriderm®, and Eucerin®. Coal tars and anthralin
can be used for disease not responsive to these measures.

Widespread disease involving >30% of the body surface area is hard to treat with topical
therapy alone. These patients can use ultraviolet light and methotrexate. Patients with joint
problems should also be treated with methotrexate.

Antihistamines and topical antibiotics are of no use in treating psoriasis.

Lichen Planus
Lichen planus is an inflammatory process usually consisting of violaceous papules that have
a polygonal outline and flat top, and it tends to favor flexural surfaces of skin and the buccal
mucosa of the oral cavity. It sometimes affects follicles and nail units as well. Just as is the case
for all inflammatory diseases of the skin, lesions of lichen planus begin as flat, pink or darker
red macules and very quickly become violaceous papules. The papules sometimes become
confluent to form plaques. In general, lesions of lichen planus tend to persist for months and
usually begin to wane within a year. If lesions are rubbed persistently, as is the situation for
what is called hypertrophic lichen planus, the condition may last for years. Vesicles of lichen
planus come into being rapidly and also disappear rather quickly.

Topical corticosteroids, intralesional injections of corticosteroids, and, for acute, widespread

lesions, even a short course of oral corticosteroids may be appropriate. Other modes of treat-
ment for widespread lesions are phototherapy (psoralen ultraviolet A [PUVA], ultraviolet B
[UVB]) and retinoids. Painful oral lesions may be assuaged by topical corticosteroids in a
coated base, by a mouthwash that contains cyclosporine, or by topical application of tacroli-
mus (FK506).

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IMMUNE AND HYPERSENSITIVITY REACTIONS

Urticaria
The most common forms of acute urticaria are associated with medications such as penicil-
lin, with infections, and with foods. Chronic urticaria is associated with pressure (dermatog-
raphism), cold, and vibration. The therapy is to treat the underlying cause. Acute medical
management is with antihistamines such as hydroxyzine and diphenhydramine (Benadryl®).
Extremely severe cases are treated like anaphylactic shock, with epinephrine and steroids.
Chronic urticaria is treated with nonsedating antihistamines such as loratadine, fexofenadine,
and cetirizine. Doxepin is a tricyclic antidepressant with strong antihistaminergic effects.

Drug Eruptions
Drug eruptions can range from a very mild macular rash from an allergic reaction (morbil-
liform rash) all the way up to life-threatening toxic epidermal necrolysis. When the reaction
is more severe, it is called erythema multiforme (EM). EM can occur in association with the
drug phenytoin as well as with infections such as herpes simplex. When the disease is even
worse and involves the eye, it is known as Stevens-Johnson (SJ). EM and SJ both involve
mucous membranes, but SJ is worse and can result in sloughing of respiratory mucosa that
might lead to the necessity of mechanical ventilation. Toxic epidermal necrolysis (TEN) is
the most severe form of drug-related eruption, although the etiology of all of these disor-
ders includes medications such as phenytoin. There is no specific therapy for any of these
disorders except to stop taking the offending drug. Steroids have never proved to benefit
Stevens-Johnson.

Morbilliform < EM < SJ < TEN

Specific treatment should be given for the condition that induced the disorder, if known (e.g.,
antiviral for herpes simplex). Local applications of soothing lotions and general supportive
measures are indicated. If systemic corticosteroids are to be used in a patient with severe,
widespread, life-threatening disease, they must be given in very large doses. Such a patient
is best managed in a burn unit, or in the equivalent of one. Parenthetically, intravenous
administration of immunoglobulins is reputed to be beneficial in a circumstance of EM that
potentially is fatal.

Pemphigus Vulgaris and Bullous Pemphigoid

Pemphigus vulgaris and bullous pemphigoid are both autoimmune phenomena resulting in
antibodies against the epidermis, causing the loss of skin. Pemphigus vulgaris often involves
the mouth and is associated with intraepidermal antibodies that split off the skin and result
in flaccid bullae that rupture easily. Bullous pemphigoid is associated with antibodies in
between the epidermis and dermis; therefore, the bullous lesions are most likely to stay intact
because they are thicker.

Depending on their severity, both of these phenomena may need to be treated with steroids.
Mortality is higher in pemphigus vulgaris, presumably from a greater loss of the protective
skin surface; the condition acts more like a burn.

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Erythema Nodosum
Erythema nodosum (EN) is a self-limited disorder most often found in association with
sarcoid, pregnancy, and coccidioidomycosis. It is most frequently seen in patients who are
taking sulfonamides, penicillin, salicylates, and/or oral contraceptives. Infections such as
syphilis and streptococcal disease are also associated with this skin disorder. NSAIDs provide
some symptomatic relief.

Pityriasis Rosea
Pityriasis rosea is a generalized macular, red rash that is hard to distinguish from secondary
syphilis. The VDRL of RPR will be negative. The clue to the diagnosis is that the rash starts
out as a single lesion (or “herald” patch) before it disseminates. Generally, it lasts for 8 weeks
and heals without scarring and without therapy. Severe cases with a lot of pruritus can be
treated with topical or systemic steroids. Soothing lotions, topical hydrocortisone cream, and
ultraviolet light may each ameliorate the signs, but they do not hasten the disappearance of
the lesions, which takes about 6 weeks regardless of treatment.

Porphyria Cutanea Tarda

Porphyria cutanea tarda can be caused by a congenital inborn error interrupting the normal
production of the hemoglobin molecule. Acquired disease can be associated with hepatitis
C, alcohol, estrogen exposure, or iron overload. Light reacts with abnormal precursors of
porphyrin, resulting in blistered skin on sun-exposed areas. Treatment consists of stopping
the offending drug. Patients who have porphyria cutanea tarda along with hepatitis C are
treated with interferon and ribavirin. Those with a metabolic disorder could undergo phle-
botomy as a treatment. This will prevent the accumulation of the abnormal free erythrocyte
precursors.

ECZEMATOUS DISEASE

Atopic Dermatitis
Atopic dermatitis is an idiopathic disorder that has a prominent hereditary component.
Individual patients often will give a history of asthma and hay fever. The disease is more fre-
quent on flexor surfaces. Because of dryness and scratching, the patient will “impetiginize”
staphylococcus into the skin. It is like doing a PPD. Patients scratch the staph antigens into
the skin, which causes more itching. This is also why topical and occasionally systemic anti-
staphylococcal medications can be effective.

Lifestyle alterations in the treatment include avoiding wool-containing fabrics and switching
to cotton, which is less irritating. Patients should also avoid excessive soap and hot water use
when bathing because this further dries the skin and worsens the itching.

Emollients and lotions to soothe the skin and to make it less dry and itchy are essential to
case management. This would basically amount to any form of non-irritating topical, such
as Aveeno® oatmeal baths and soaps, Eucerin®, Lubriderm®, Aquaphor®, Dermasil®, Nivea®,
or even Vaseline®.

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Specific therapy includes:

First-line (“best initial therapy”): Topical steroids, antihistamines (such as loratadine),

cetirizine, fexofenadine, and systemic antihistamines (such as doxepin or Chlor-Trimeton®).
Emollients.

Further therapy includes tacrolimus, sirolimus, and pimecrolimus.

If the question is “What would you do next if the symptoms persist?”: Your answer is topi-
cal or oral antibiotics. Tacrolimus and picrolimus are T cell–inhibiting agents that help keep
patients off chronic steroids. Coal-tar preparations are also good.

Contact Dermatitis
Contact dermatitis can potentially occur from contact with almost anything, in the sense
that a patient could be allergic to any medication. Common etiologies of contact dermatitis
include soaps, poison ivy, nickel, and cosmetics. Latex allergy is particularly problematic
because it can result in anaphylaxis. Look for a history of a health care worker with unex-
plained syncope; it results from putting on latex gloves.

Treatment is by avoiding the allergen and by applying topical steroids.

Asteatotic Eczema
Asteatotic eczema, an idiopathic loss of sebaceous glands, results in profoundly dry skin
that constantly flakes off. The treatment is topical emollients, as described above for atopic
dermatitis.

Seborrheic Dermatitis
Seborrheic dermatitis is what the rest of the world calls dandruff, with yellow, flaky, oily skin.
It is treated with topical steroids. There is an unexplained association with the dermatophyte
Pityrosporon ovale, which is why selenium sulfide and ketoconazole shampoos are effective
as well.

Stasis Dermatitis
There’s not much you can do for the patient with stasis dermatitis. Try to fix the underlying
disease and elevate the patient’s legs. Inflammation and secondary infection occur, and then
you treat with antibiotics and topical steroids.

Acne
Types of Lesions
Non-inflammatory lesions or primary lesions: microcomedones, closed comedones, open
comedones.

Treatment of comedonal acne is with topical tretinoin (Retin-A®) and benzoyl peroxide. For
papular or pustular acne, add topical and systemic antibiotics. Cystic acne is treated with
systemic antibiotics and isotretinoin (Accutane®).

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Side Effects of Isotretinoin (13-cis-retinoic Acid)

• Xerosis, dermatitis, cheilitis, sticky skin, peeling skin
• Epistaxis
• Conjunctivitis
• Hair loss
• Arthralgia/myalgia
• Hyperlipidemia (hypertriglyceridemia and hypercholesterolemia)
• Increased LFTs, leukopenia

Greatest risk: Teratogenicity (patient needs reliable contraception during therapy and for at
least 1 month after use of the drug is discontinued)

BENIGN, MALIGNANT, AND PRE-MALIGNANT LESIONS

Seborrheic Keratosis
Seborrheic keratosis is a common hyperpigmented lesion in older patients. These keratoses
vary from light to dark brown and have a “stuck on” appearance. They are like a lipoma in
that they have no malignant potential and are removed only if they get in the way or are
cosmetically displeasing.

Nevi
Benign moles are small (<6 mm) and well circumscribed, with well-defined borders and
a single shade of pigment without change for years. They have regular edges and uniform
color; they don’t change much in size over time; and they are smooth, even, and symmetrical
in shape.

When do you consider a mole suspicious? Remember ABCD: Look for asymmetry, border
irregularity, color variegation, and diameter >6 mm; or the history of a changing mole.
Ulceration and bleeding are ominous signs.

Melanoma
When it is the opposite of what is described above for a suspicious mole, it is time for a
biopsy. Do not perform a shave biopsy. The prognosis is entirely dependent on the lesion’s
thickness, which cannot be determined if you shave. Remove the lesion rather than shaving
it. Also, remember that large margins (>5 cm) are no longer recommended. Lesions <1 mm in
thickness need only a 1-cm margin. Metastatic disease is treated with interferon. Local recur-
rences are treated with radiation.

Incidence: 13 cases/100,000 persons

What are the risk factors for developing melanoma?

• Fair complexion
• Exposure to sunlight for short, intense periods of time
• Numerous atypical-appearing melanocytic nevi
• Large congenital melanocytic nevi
• Personal or family history of melanoma

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What determines the prognosis of melanoma? Clark and/or Breslow levels: Assess the
depth of the lesion, measuring directly with a micrometer.

How do you treat melanoma? Treatment of melanoma primarily consists of excision.

After histologic diagnosis, the area is usually re-excised, with margins dictated by the thick-
ness of the tumor, as follows:
• In situ: 0.5-cm border of normal skin
• Breslow’s depth of <1.0 mm: 1-cm margin
• Breslow’s depth of 1–2 mm: 1–2-cm margin
• Breslow’s depth of 2–4 mm: 2-cm margin
• Breslow’s depth >4 mm: 3-cm margin

Actinic Keratosis
Actinic keratosis is like cervical dysplasia of the face. These keratoses are premalignant
squamous cell cancer lesions of the skin. You will find them in the Scotch/Irish/Australian
person who works outside a lot. They are treated with cryotherapy or topical 5-FU for
removal. This is similar to what you would do for cervical dysplasia.

Squamous and Basal Cell Carcinomas

Squamous and basal cell carcinomas occur most frequently on sun-exposed areas. The man-
agement is primarily to biopsy and then remove them. Both have <1% mortality. Neither has
good chemotherapy for the rare person with metastatic disease. These carcinomas are the
most common cutaneous malignancies. Basal cell occurs with a 4:1 frequency, as compared
with squamous cell carcinoma. Both kinds are of low-grade malignancy. Even when aggres-
sive, they are only locally invasive. They are primarily related to sun exposure.

Clues for recognition: Look for a papule or nodule that may or may not be ulcerated or pig-
mented. Basal cell gives a waxy, “pearly,” or transparent appearance, and telangiectasia.

What is the most effective treatment for basal cell carcinoma? Mohs surgery has the highest
cure rates, at 98%. This surgery involves removal of the tumor, followed by immediate frozen
section and histopathologic examination of the margins, with subsequent re-examination of
tumor-positive areas.

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Sunscreen and Protection from Skin Cancer

Besides its acute effect (sunburn), sun exposure has a number of chronic effects:
• Skin wrinkles and abnormal pigmentation
• Precancers (actinic keratoses)
• Basal and squamous cell carcinoma (more related to lifetime cumulative sun exposure)
• Malignant melanoma (more related to history of sunburns)
• Cataracts
Different spectra of UV radiation have different effects on human skin:
• U
 VB (wavelength: 290–320 nm): causes DNA formation of thymine dimers, leading to
carcinogenesis.
• U
 VA (wavelength: 320–400 nm): causes formation of free radicals, and therefore altered collagen
and elastin synthesis, leading to skin wrinkles.
SPF (sun protection factor) is the ratio of the minimal dose of sunlight to cause redness of sun-
protected skin divided by the minimal dose of sunlight to cause redness of unprotected skin. So,
using an SPF of 15, it takes 15 times more time to achieve the same redness.

Kaposi Sarcoma
Kaposi sarcoma (KS) is most frequently seen in homosexual men with AIDS. It is associated
with human herpes virus-8 (HHV-8). KS lesions appear in a widespread symmetrical distri-
bution on the skin and oral mucosa, with red, purple, or brown oval or elongated plaques
or nodules on the skin or mucosa, especially the oral mucosa. In most cases of AIDS-related
KS, antiretroviral is effective by itself. Other therapeutic options for unresponsive cases and
non-AIDS related KS are cryotherapy, intralesional vinblastine, radiotherapy, laser surgery,
and systemic chemotherapy.

Mycoses Fungoides
Mycoses fungoides is a cutaneous T-cell lymphoma. Look for localized (rarely generalized)
patches or plaques, usually on the trunk, that are associated with pruritus and that don’t
respond to emollients and topical steroids.

What is the prognosis of mycosis fungoides? It depends on the stage of the disease:
• Patch-and-plaque stage: 12 years
• Tumor stage: 5 years
• Nodal or visceral stage: 3 years

What is the treatment for mycosis fungoides?

• PUVA
• Interferon-alpha
• Oral retinoids
• Electron-beam therapy (radiotherapy)
• Extracorporeal photopheresis for erythrodermic manifestations

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INFECTIONS

Dermatophytes
All dermatophytes are diagnosed via a potassium hydroxide test on a scraping, and all require
culture for specific confirmation. Dermatophytes take weeks to grow, and seldom is a specific
organism identified at the time therapy is initiated. Hair and nail involvement need therapy
with terbinafine or itraconazole. Fluconazole does not work well for dermatophytes since it
is mostly a yeast (candida) medication. Beware of liver toxicity for terbinafine. If there is no
hair or nail involvement, you can use any topical antifungal.

Herpes Simplex and Herpes Zoster

When herpes simplex and herpes zoster vesicles are clearly present, your answer should be
“Therapy,” not a Tzanck prep or viral culture, although these are the “best initial” and “most
accurate” tests. Acyclovir, famciclovir, and valacyclovir are all equal for both infections. Oral
therapy is the answer for zoster, unless it is disseminated or there is ophthalmic involvement.
Use foscarnet for acyclovir-resistant herpes.

Scabies and Pediculosis

For diagnosis, scrape the smaller Sarcoptes scabies, and use direct visual identification for the
larger pediculosis (or crabs). Both are treated with permethrin or lindane solution.

Impetigo and Erysipelas

Impetigo and erysipelas are very superficial infections of the epidermis, so there is weeping,
crusting, and draining. Although oral dicloxacillin and cloxacillin are good, your first answer
should be topicals, such as mupirocin or bacitracin. Erysipelas is often very hard to identify
for certain. One clue is a bright red lesion that is swollen and raised. Use penicillin G or VK
to treat because it is Strep pyogenes.

Cellulitis and Necrotizing Fasciitis

Cellulitis is treated with oxacillin or nafcillin. First-generation cephalosporins, such as cefazo-
lin, are used if the patient has a mild penicillin allergy. Use macrolides or vancomycin where
there is a severe penicillin allergy, such as anaphylaxis. Necrotizing fasciitis needs anaerobic
coverage in addition, with clindamycin and surgical debridement.

SYPHILIS
A primary syphilitic chancre is firm and indurated. It is best diagnosed with a darkfield exam
in addition to an RPR and FTA. Treat with single-dose penicillin.

Secondary syphilis is a generalized, copper-colored, maculopapular rash in which the RPR is

100% sensitive. Treat with single-dose penicillin.

CONDYLOMATA ACUMINATA
Remove condylomata acuminata any way you want: freeze, burn, cut, melt with podophyllin,
apply imiquimod. No specific test is necessary. Visual appearance is sufficient. Do not rou-
tinely subtype the papilloma virus.

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10
CASE 1
35-year-old woman comes to your office with stiffness in her hands that is worse
in the morning that lasts approximately 45 minutes. She has symptoms for the
last 6 months. Exam significant for joint swelling, redness, and warmth over the
PIP and MCP joints in both hands. No nodules found.

1. Most likely diagnosis?

A. Osteoarthritis
B. Polymyalgia rheumatica
C. Systemic lupus erythematosis (SLE)
D. Reactive arthritis
E. Rheumatoid arthritis

Answer:

2. Which is the most common extra-articular manifestation?

A. Episcleritis
B. Pleural effusion
C. Pericarditis
D. Vasculitis
E. Skin nodules
F. Carpal tunnel syndrome

Answer:

3. What test is the most specific?

A. Anti–cyclic citrullinated peptide (Anti-CCP)
B. Anti-dsDNA
C. Antinuclear antibodies (ANA)
D. Erythrocyte sedimentation rate (ESR)
E. Rheumatoid factor
F. C-reactive protein (CRP)

Answer:

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4. Best initial therapy?

5. What is the disease-modifying antirheumatic drug (DMARD)?

Methotrexate Monitor
A. Methotrexate
• Liver Function Test B. Sulfasalazine
• CBC C. Hydroxychloroquine
D. Etanercept (anti-TNF medication)
• Add Folinic Acid
E. Anakinra

Answer:

6. Major side effects of this medication? The major contraindications?

A. Lung and liver fibrosis
B. Rash and oligospermia
C. Ocular
D. TB reactivation
E. Bacterial infection

Answer:

7. When methotrexate fails, what is the next best DMARD to be used?

Felty’s Syndrome
A. Abatacept
• RA B. Anti-TNF
• Neutropenia C. Hydroxychloroquine
• Splenomegaly D. Anakinra

Answer:

8. Which of the following is most consistent with severe disease?

A. Nodules, high ESR, fatigue
B. Knee, hip, shoulder involvement
C. Ulcer development
D. Mononeuritis multiplex and lung involvement
E. High RF or anti-CCP, abnormal x-ray, anemia and >20 joints involved

Answer:

9. What is the most common eye problem in RA?

A. Dry eye (sicca)
B. Uveitis
C. Optic neuritis

Answer:

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Summary: Case 1
This patient presents with bilateral, symmetrical, and inflammatory arthritis that has lasted
more than 6 weeks. Pain swelling and impaired function are typically located in distal joints
(PIPs, MCPs, wrists knees ankles, and cervical spine). In the absence of systemic complaints or
manifestations of disease, the most likely diagnosis is rheumatoid arthritis (RA). Initial diag-
nostic workup includes RF, ANA, and hand x-rays to evaluate for the presence of erosions. Anti-
CCP is more specific for RA than rheumatoid factor. The presence of nodules also strengthens
the diagnosis as RA. The best initial therapy is an NSAID for the arthritis. NSAIDs and steroids
provide symptomatic relief but do not alter the progression of disease in RA. DMARDs are the
class of medications that have been shown to retard progression. The decision to start DMARD
therapy is based upon the prognosis of the patient at presentation. Patients with involvement
of more than 3 joints, persistently high levels of acute phase reactants (CRP), high RF titers
and anti-CCP, joint erosions, rheumatoid nodules, and functional disability have a poorer
prognosis. Early initiation of DMARD therapy is important in management. With poor prog-
nostic signs, the initial DMARD of choice is methotrexate because of its relatively short onset
of action (6–8 weeks). After methotrexate, you can add leflunomide, infliximab, etanercept, or
the combination of hydroxychloroquine and sulfasalazine. Patients with a better prognosis can
be treated with a combination of an NSAID, steroids, and either hydroxychloroquine or leflu-
nomide. X-ray abnormalities are not necessary to establish a diagnosis of RA. That lowers the
threshold for early diagnosis and allows earlier treatment with DMARDs.

1. 52-year-old woman with neck pain and stiffness of 4 weeks’ duration. She has a history
Note
of rheumatoid arthritis. Taking methotrexate therapy for 10 years. On exam, she has
weakness of her lower extremities. Tendon reflexes are increased with extensor plantar Tocilizumab is an IL-6
responses. Which is most appropriate in management? antagonist. Use when resistant
A. Discontinue methotrexate to MTX and TNF drug.
B. Recommend NSAIDs
C. Initiate anti-TNF therapy
D. Increase dose of methotrexate
E. Spinal fusion

Answer:

2. Which test is essential in diagnosing rheumatoid arthritis? Symptoms of C1/C2

Compression
A. Anti-CCP or rheumatoid factor
B. Normocytic anemia • Altered consciousness
C. Abnormal x-ray • Loss of bladder control
D. Joint fluid aspiration
• Dysphagia
Answer:
• Vertigo
3. Which is most appropriate on a patient with rheumatoid arthritis who becomes • “Drop attacks”
pregnant?
A. Add methotrexate
B. Add leflunomide
C. Use hydroxychloroquine or sulfasalazine
D. Anti-TNF inhibitors

Answer:
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4. Which is most characteristic of pleural effusion from Rheumatoid arthritis (RA)?

A. High protein
B. Low glucose
C. High Lymphocyte count

Answer:

5. Which is the most common cause of death in RA?

A. Renal failure
B. Hip fracture
C. Stroke
D. Accelerated coronary disease

Answer:

6. Why does RA cause hoarseness?

A. Recurrent laryngeal nerve paralysis
B. Infection
C. Cricoarytenoid joint involvement
D. Brainstem stroke

Answer:

7. When is leflunomide the answer?

A. Add on to methotrexate
B. Add to Anti-TNF if they fail
C. Those intolerant of methotrexate

Answer:

8. Which of the following is NOT a cardiac manifestation of RA?

A. Acute pericarditis
B. Constrictive pericarditis
C. Myocarditis
D. Coronary Disease
E. Infective Endocarditis

Answer:

9. Patient with RA, sudden calf pain, Doppler negative.

What is it?
How to treat?

10. RA + Nodules + Pneumoconiosis = ??

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11. 32 year old. Recurrent pain in PIP, then MCP and then the knee. A few hours later,
joints are swollen. Within 24 hours, pain resolves. In between attacks, all is well. What
is this?
A. Reactive arthritis
B. Behcet’s
C. Palindromic Rheumatism

Answer:

12. Pregnancy will make RA:

A. Better
B. Worse
C. Unchanged

Answer:

CASE 2
24-year-old woman comes with joint pain and swelling that started 2 weeks ago
after a mild febrile illness. She denies rashes, back pain, or diarrhea. The patient
works in a daycare center taking care of young children and has experienced
multiple upper respiratory tract infections over the last several months. Exam
shows symmetrical tenderness of the PIP and MCP joints.

1. Most likely diagnosis?

A. Rheumatoid arthritis
B. Disseminated gonorrhea
C. Reactive arthritis
D. Parvovirus
E. SLE

Answer:

2. Best initial test?

A. Bone marrow with giant pronormoblasts
B. Decreased reticulocyte count
C. IgM and IgG
D. Rheumatoid factor
E. ANA

Answer:

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3. How is this disease differentiated from rheumatoid arthritis?

A. Absence of skin lesions
B. Duration
C. Joint involvement
D. Anemia

Answer:

4. What is the most specific diagnostic test?

A. PCR for DNA
B. Biopsy
C. Viral culture
D. Response to therapy

Answer:

5. What would you find on x-rays of hands?

6. What other systemic complication is associated with this disease?

A. Anemia
B. Stroke
C. Pericarditis
D. Meningitis

Answer:

7. Treatment?

Summary: Case 2
Viral arthropathy is the most likely diagnosis in this case. Many times, the symptoms of dry
or itchy eyes may be falsely attributed to conjunctivitis or allergic symptoms. Parvovirus B19
is a common cause of acute arthritis in child care workers. The pattern of arthritis is identical
to that of RA. The distinguishing factor between RA and any viral arthropathy is the duration
of symptoms. It is impossible to make the diagnosis of RA, if the patient’s symptoms have
lasted less than 6 weeks. The diagnostic test of choice is serology for parvovirus B19 IgM.
Hand x-rays will be negative for any erosions or joint space narrowing. Patients with chronic
hemolytic anemias, such as sickle-cell anemia, may experience red-cell aplasia when infected
with parvovirus B19. Aplastic anemia is also a complication of parvovirus B19 infection.
Treatment is with NSAIDs for symptomatic relief. In many patients, ESR and RF are also
elevated.

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CASE 3
45-year-old woman comes with itching and burning of her eyes that began
3 weeks ago. The patient thought that she was experiencing seasonal allergies
but became concerned when, at a funeral, she was unable to tear when she was
crying. She has also been having some difficulty speaking. Exam is significant for
bilateral parotid gland enlargement.

1. Most likely diagnosis?

A. Conjunctivitis
B. Mixed connective tissue disease
C. Mumps
D. Scleroderma
E. Sjögren syndrome

Answer:

2. Best initial test?

A. Erythrocyte sedimentation rate
B. Schirmer test
C. Serology
D. Slit lamp evaluation
E. Tonometry

Answer:

3. Most specific blood test?

A. ANA
B. RF
C. SSA and SSB
D. Anti-centromere
E. Anti-histones

Answer:

4. Most accurate test?

A. Rose Bengal test
B. Parotid sialography
C. Salivary gland biopsy
D. Tear break-up time

Answer:

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5. Which is most likely to be positive?

A. ANA
B. Anti-Ro/SSA
C. Low complement
D. Anti-smooth muscle

Answer:

6. Which is the most common manifestation of this disease?

A. Joint pain
B. Lung disease
C. Peripheral neuropathy
D. CNS
E. Heart/pericardial

Answer:

7. What malignancy is associated with this disease?

A. Colon
B. Salivary
C. Lymphoma
D. Liver

Answer:

8. Treatment?

Summary: Case 3
The most likely diagnosis of any patient presenting with bilateral parotid gland enlargement
associated with reduced tearing and a dry mouth is Sjögren syndrome. The Schirmer test to
quantify the amount of tearing is the best initial test. Anti-SSA (Ro) and anti-SSB (La) are
the most specific serologic markers for the disease.

Minor salivary gland biopsy is the most accurate test for the diagnosis. Parotid gland biopsy is
indicated when there is asymmetric enlargement of the parotid gland. Pancreatitis, RTA Type I,
and dysphagia are systemic complications of this disease. Rheumatoid arthritis, primary biliary
cirrhosis, scleroderma, and pulmonary fibrosis are associated with Sjögren syndrome. B-cell
lymphomas and Waldenstrom macroglobulinemia are associated with Sjögren syndrome.
Treatment involves artificial tears; frequent mouth hydration with pilocarpine is rarely used for
refractory symptoms.

The most common cancer is lymphoma and the most common serologic test is an ANA,
although it is nonspecific. The single most accurate test is a salivary gland biopsy. Although
there are many extra-glandular manifestations, the most common is joint pain which is in
50% of patients. Cevimeline is an agonist of acetylcholine which stimulates gland function.

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CASE 4
57-year-old woman comes complaining of “aches and pains” that seem to be
localized to her wrists and shoulders. She has been feeling “under the weather”
for the last several weeks. Swelling of her fingers and states that her hands turn
blue when she walks out in the cold without gloves. Exam ulcerations on fingers
bilaterally.

1. Most likely diagnosis?

A. SLE
B. Rheumatoid arthritis
C. CREST syndrome
D. Scleroderma (progressive systemic sclerosis)
E. Eosinophilic fasciitis

Answer:

2. Best initial test?

3. What is the hand manifestation in this patient and how is it treated?

4. How can we distinguish between diffuse and limited scleroderma (CREST)?

A. Sclerodactyly
B. Gastrointestinal manifestation
C. Renal and cardiac involvement
D. Telangiectasia
E. Raynaud’s

Answer:

5. Treatment for systemic sclerosis?

A. Prednisone
B. Penicillamine
C. Cyclophosphamide
D. Mycophenolate
E. No effective therapy

Answer:

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6. What is eosinophilic fasciitis, and how does its diagnosis and treatment differ from that
of systemic sclerosis?

7. What disease is associated with taking tryptophan?

8. Which is indicated to treat renal disease?

A. ACE inhibitor
B. Penicillamine
C. Calcium channel blocker
D. Beta blocker

Answer:

9. Patient with GERD symptoms, color changes of hands with cold and positive anti-
centromere antibodies. He has many fingers with telangiectasias. He has dyspnea most
likely secondary to which of the following?
A. Pulmonary hypertension
B. Interstitial fibrosis
C. Cardiomyopathy
D. Anemia

Answer:

10. 62-year-old woman presents with progressive gastroesophageal reflux symptoms and
chest pain. Shortness of breath on exertion. BP 168/102 mm Hg. Exam reveals diffuse
skin thickening and limited range of motion of hands. Echocardiogram shows ejection
fraction is 58%; and mild mitral regurgitation. Pulmonary artery pressure is 58 mm Hg.
Which of the following is the next step in diagnosis?
A. Esophagram
B. High resolution CT scan of chest
C. Renal arteriogram
D. Right heart catheterization
E. Upper endoscopy

Answer:

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Summary: Case 4
In a patient who presents with chronic hand swelling over several months, the most likely
diagnosis is scleroderma. The best initial test for the diagnosis of scleroderma is the ANA,
which has a sensitivity of 80–95%. Systemic sclerosis is associated with renal involvement, pul-
monary fibrosis, and more proximal soft-tissue swelling involving the trunk in addition to the
extremities. The specific antibody for systemic scleroderma is anti-Scl 70. Limited scleroderma
will never involve the trunk or the kidney. The pulmonary manifestation of the limited form
of the disease is pulmonary hypertension. The specific antibody is anticentromere antibody.

Raynaud phenomenon is described as pain in the fingertips induced by cold secondary to

arterial spasm, followed by relaxation. Treatment for this disorder consists of a calcium chan-
nel blocker. Systemic scleroderma is not treated with steroids; the treatment is supportive. In
hypertensive emergencies associated with renal involvement, the antihypertensive of choice
is an ACE inhibitor.

Eosinophilic fasciitis presents as an orange peel–like thickening of the skin that can be
induced by physical activity. Eosinophilia will be present in the peripheral blood count. The
treatment is systemic steroids. Eosinophilia-myalgia syndrome is associated with tryptophan
ingestion. Treatment is with systemic steroids.

Scleroderma patients are at risk of pulmonary hypertension. The standard for diagnosing
pulmonary hypertension remains right heart catheterization and is performed after an ele-
vated pulmonary artery pressure is found on echocardiographic screening. High resolution
CT scanning is performed to evaluate for pulmonary fibrosis.

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CASE 5
25-year-old woman with bald spots on her scalp progressively worsened over
past 4 months. She also has a rash which seems to be worse in the summer
time. She reports feeling more tired than usual. She has had generalized joint
and muscle aches over the past 4 weeks. Physical Exam shows symmetric
swelling of the bilateral PIP and MCP joints. Laboratory workup shows anemia,
thrombocytopenia. Urine pregnancy test is negative. Urine is negative for
leukocyte esterase, 2+ proteinuria.

1. Most likely diagnosis?

2. Best initial diagnostic test?

A. ANA
B. Double-stranded DNA
C. Complement levels
D. ESR

Answer:

3. Most specific serologic test?

A. ANA
B. Double-stranded DNA
C. Complement levels
D. ESR
Answer:

4. What are the major clinical manifestations of the disease?

5. What medication for this patient’s joint and cutaneous symptoms?

A. Hydroxychloroquine
B. Etanercept
C. Methotrexate
D. Cyclosporine
E. Anakinra

Answer:

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6. What are the major indications for treatment with corticosteroids?

7. What is the main difference between drug-induced disease and routine disease?
A. Positive ANA and increased ESR
B. C-reactive protein
C. CNS and renal involvement
D. Rash
E. Joint pain

Answer:

8. She is found to have elevated PTT and normal PT. Which of the following is the next
step?
A. Antiphospholipid antibodies
B. D-dimer
C. Fibrin split products
D. Fibrinogen

Answer:

9. What is the best initial test?

A. Mixing study
B. Russell viper venom test
C. Anti-dsDNA
D. Ristocetin testing

Answer:

10. A woman with a long history of lupus maintained on hydroxychloroquine has just
become pregnant. What do you test for?
A. Anti-Ro
B. Complement levels
C. Anti-Sm
D. ANA levels

Answer:

11. What is the only antibody MORE specific than DS-DNA?

A. Ro
B. Single stranded DNA
C. Anti-Sm

Answer:

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Summary: Case 5
Systemic manifestations unique to SLE involve the skin, kidney, and central nervous system.
The treatment of SLE depends on the severity of the systemic manifestations. Systemic mani-
festations including thrombocytopenia, hemolysis, myocarditis, pericarditis, convulsions,
and nephritis warrant therapy with corticosteroids. Pulse cyclophosphamide and steroids are
the treatment of choice for Stage III and IV lupus nephritis. For isolated rash and arthritis,
hydroxychloroquine is the treatment of choice. Drug-induced lupus can be distinguished
from systemic lupus on a clinical and serological basis. Drug-induced lupus is associated with
chlorpromazine, hydralazine, isoniazid methyldopa, procainamide, quinidine, and mino-
cycline. Antihistone antibodies are the specific way to make the diagnosis. Clinically, drug-
induced lupus does not have systemic manifestations, it affects males and females equally,
and it resolves when the offending drug is withdrawn. Antihistone antibody is specific for
the diagnosis of drug-induced lupus. SLE is associated with the development of the lupus
anticoagulant (an immunoglobulin, which binds to phospholipids and prevents coagulation
reactions from taking place on the platelet surface). It is associated with arterial and venous
thrombosis and recurrent spontaneous abortions, and should also be suspected in patients
with elevated PTT and normal PT without bleeding.

1. 36-year-old woman with SLE comes to hospital complaining of shortness of breath and
chest pain that began 2 weeks ago. She also complains of low-grade fever and general-
ized weakness. Exam reveals decreased breath sounds bilaterally. Chest x-ray shows
bilateral infiltrates. What would you advise regarding her pulmonary disease?
A. Likely to develop obstructive pulmonary disease.
B. Likely to develop restrictive pulmonary disease.
C. Arthritis will worsen.
D. Prognosis is very poor.

Answer:

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CASE 6
27-year-old man is admitted because of fever to 40ºC (104ºF) for a couple of
weeks. Blood cultures, UA, urine culture, CXR, and multiple imaging studies
have been negative. CBC came back with a leukocytosis of 34,200 with
neutrophilic predominance and mild anemia. He has a sore throat, cervical
lymphadenopathy, and hepatosplenomegaly. Through day 3 of his hospital
stay, the patient continues to have high fevers at night, and an intern notices a
salmon-colored rash that appears with the patient’s rise in temperature.

1. Most likely diagnosis?

2. Best initial treatment?

A. Ibuprofen
B. Methotrexate
C. Adalimumab
D. Prednisone
E. Cyclosporine

Answer:

3. Most effective treatment?

A. Ibuprofen
B. Methotrexate
C. Adalimumab
D. Prednisone
E. Cyclosporine

Answer:

4. How is this disease differentiated from Epstein-Barr infection?

A. Blood cultures
B. Viral cultures
C. CBC and ferritin levels
D. Bone marrow
E. ANA

Answer:

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Summary: Case 6
In a young man who presents with relapsing fever and the absence of infection associated
with a salmon-colored rash, the most likely diagnosis is adult Still disease. The best initial
treatment for adult Still disease is NSAID therapy. The most effective therapy is steroids. In
Epstein-Barr infection, the peripheral smear will characteristically have atypical lymphocytes
with an associated lymphocytosis. In Still disease, the WBC count is elevated, but there is
primarily a granulocytosis rather than a lymphocytosis.

S Splenomegaly
T Temperature
I Ill patient
L Lymphadenopathy
L Leukocytosis
S Salmon rash

Case 1
22-year-old woman presents to ED with swelling of ears and over the bridge of
nose. She has noticed progressive deformity of her nose and ears over the last
several months. This is her 5th visit to the hospital for this problem.

1. Most likely diagnosis?

A. Polyarteritis nodosa
B. Relapsing polychondritis
C. Systemic lupus eythematosis
D. Syphilis
E. Wegener granulomatosis

Answer:

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Case 2
31-year-old man with oral and genital ulcers, blurry vision, and joint pain
involving both knees and ankles. You find right-sided third-nerve palsy with
uveitis of left eye.

1. Most likely diagnosis?

A. Behçet syndrome
B. Crohn disease
C. Multiple sclerosis
D. Reiter syndrome
E. Syphilis

Answer:

Case 3
37-year-old man burning and aching pain in his right hand. 6 months ago, while Note
horseback riding, he fell on his right hand and sprained his right wrist. The pain
worsens when he goes out in the cold. Exam warm and slightly swollen right HLA B5 in Behcet’s.
hand and forearm. The swelling has been spreading up his arm over the last
several weeks. Hand radiograph reveals severe osteopenia.

1. Most likely diagnosis?

A. Median nerve injury
B. Osteonecrosis
C. Raynaud disease
D. Reflex sympathetic dystrophy
E. Septic arthritis

Answer:

2. Most accurate diagnostic test?

A. Bone image (scintigraphy)
B. Nerve conduction studies
C. Joint aspiration
D. Biopsy
E. ESR

Answer:

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Summary: Cases 1–3

Chronic episodic swelling of cartilage, especially on the nose and ears, is consistent with
relapsing polychondritis. Patients may also complain of fever or deafness, and the course of
the disease is rarely complicated by glomerulonephritis and aortic stenosis. The treatment for
this disease is oral steroids.

Oral and genital ulcers associated with cranial nerve palsies, arthritis, and uveitis is consistent
with the diagnosis of Behçet syndrome. The clinical course is characterized by a relapsing
and remitting pattern. A common complication of this disease is thrombophlebitis with deep
vein thrombosis. The treatment is with immunosuppressive therapy (steroids, Colchicine,
thalidomide cyclophosphamide, and cyclosporine have all been effective).

Burning or aching pain that is more severe and longer in duration than expected after trauma
to an extremity is consistent with the diagnosis of reflex sympathetic dystrophy. The find-
ing of severe osteopenia is also consistent with the disease. The best test for the diagnosis is
a bone scan. X-ray, MRI, and CT scanning may also be useful. Patients may also complain
of worsening symptoms when the extremity is exposed to cold. Initially, patients should be
treated with physical therapy and a mild anxiolytic. Gabapentin and opioid analgesics have
also been shown to be effective.

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CASE 7
Right hip pain. With difficulty sleeping because of hip pain, and states that the pain
began suddenly 2 days ago. Normal internal rotation of the right hip. Range of
motion of the affected joint is normal with some focal tenderness at the right hip.

1. Most likely diagnosis?

A. Hip fracture
B. Aseptic necrosis of femoral head
C. Bursitis
D. Septic arthritis

Answer:

2. Main causes of this condition?

3. What is the treatment for these patients?

A. NSAIDs and physical therapy
B. Methotrexate
C. Anti-TNF
D. Hydroxychloroquine
E. Steroid injection

Answer:

4. When would you suspect infection?

Summary: Case 7
The most likely diagnosis in this case is acute bursitis of the hip. Bursitis can be differentiated
from arthritis by its relatively abrupt onset associated with focal tenderness and swelling. The
range of motion is relatively unaffected. The main causes of any bursitis are trauma, infection,
rheumatoid arthritis, and osteoarthritis. Infection of an inflamed bursa should be suspected
when there is fever, swelling, and redness. If infection is suspected, the next step is to aspirate
the affected joint and analyze the joint fluid. To rule out infection, the most useful test is the
cell count of the synovial fluid. WBC counts >50,000 speak strongly for an acute infectious
process. Staph aureus is the most commonly identified pathogen. When the WBC count lies
between 3,000 and 50,000, the bursitis is caused by any infectious arthritis. For uncomplicated
bursitis, the treatment of choice is an NSAID. If pain persists, steroid injections can be given.
For bursitis complicated by infection, appropriate antibiotic therapy is indicated.

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1. A 47-year-old obese woman comes to your clinic with pain on the medial aspect of her
knee. Her pain is worsened when she climbs up stairs. You note exquisite tenderness
over the medial (inner) knee region over the upper tibia.
A. Jumper’s knee (patellar tendonitis)
B. Plantar fasciitis
C. Prepattelar bursitis
D. Anserine bursitis
E. Achilles tear

Answer:

2. Housemaid for a local hotel comes with right knee swelling. Redness and swelling
superficial to the kneecap.
A. Jumper’s knee (patellar tendonitis)
B. Plantar fasciitis
C. Prepattelar bursitis
D. Anserine bursitis
E. Achilles tear

Answer:

3. A 17-year-old high-jumper comes with pain and tenderness of her right knee. You note
tenderness over patellar tendon.
A. Jumper’s knee (patellar tendonitis)
B. Plantar fasciitis
C. Prepattelar bursitis
D. Anserine bursitis
E. Achilles tear

Answer:

4. 45-year-old man with right heel pain. Pain is worse upon early rising and improves after
a couple of steps. Your patient is a postal worker and stands up for most of the day. You
note tenderness upon palpation of the medial calcaneal tubercle.
A. Jumper’s knee (patellar tendonitis)
B. Plantar fasciitis
C. Prepattelar bursitis
D. Anserine bursitis
E. Achilles tear

Answer:

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CASE 8
32-year-old man with low back pain that began 7 months prior. Pain is dull,
constant, and radiates down his thighs bilaterally. The pain is associated with
stiffness that is worse in the morning and gets better after he walks to the
bus stop. More recently, the patient has noticed that it’s more difficult to play
basketball. Exam shows blowing diastolic murmur and reduced lung expansion.
Decreased forward flexion of the spine. No rash, or ocular changes.

1. Most likely diagnosis?

A. Ankylosing spondylitis
B. Herniated disk
C. Psoriatic arthritis
D. Reactive arthritis
E. Spinal stenosis

Answer:

2. Best initial test?

A. HLA B27
B. X-ray
C. MRI
D. CT

Answer:

3. Most accurate radiologic exam?

A. Bone scan
B. CT scan
C. MRI
D. Plain radiograph

Answer:

4. What laboratory test is specific for diagnosing this disease?

A. HLA B27
B. ESR
C. RF
D. CRP
E. None

Answer:

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5. What are the systemic manifestations of this disease?

6. What is the Schober test?

7. Best therapy if NSAIDs do not control pain?

A. Anti-TNF
B. Methotrexate
C. Leflunomide
D. Sulfasalazine
E. Steroids

Answer:

8. Which is most accurate to follow response to treatment?

A. ESR
B. Clinical symptoms
C. MRI

Answer:

Summary: Case 8
Ankylosing spondylitis is the most likely diagnosis. An inflammatory arthritis that involves
the axial skeleton points to a seronegative spondylarthropathy. The 4 seronegative spondylar-
thropathies are ankylosing spondylitis, psoriatic arthritis, Reiter syndrome, and the arthritis
associated with inflammatory bowel disease. In the present patient, his age and lack of other
extra-articular manifestations speak strongly for the diagnosis of ankylosing spondylitis.

Radiographs of the lower spine and sacrum are the best initial test for the diagnosis of anky-
losing spondylitis. The classic “bamboo spine” is a late finding after several years of disease.
The most accurate test for the diagnosis is an MRI of the lower spine and sacroiliac joints.
Seronegative spondylarthropathies are seronegative. The rheumatoid factor and ANA are
negative. These arthritides are inflammatory and an elevated ESR is expected. About 20 to
25% of patients will have uveitis. Aortic insufficiency occurs in another 3 to 5% of patients.
Other complications include atrioventricular conduction delays and pulmonary fibrosis.

To perform the Schober test you make two marks, one 10 cm above S1 and one 5 cm below S1.
You then ask the patient to bend forward as much as possible. Normally the distance between
the two marks should increase by more than 5 cm. If this does not occur, then the patient has
significant spinal immobility. There is a clear association between HLA-B27 and the seronega-
tive spondylarthropathies (90% sensitive), but these associations are not considered specific
enough to be used for diagnosis (they occur in approximately 8% of the normal population).
The best initial therapy for ankylosing spondylitis is NSAIDs and physical therapy. In patients
who continue to have pain, the TNF-α receptor blocker can be used for therapy.

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1. A 24-year-old man comes to your office complaining of back pain that started 1 week
ago. Pain is located in lower back; does not radiate; and is not associated with fever,
weight loss, or diarrhea. Exam normal. What is the next step?
A. Spinal x-ray
B. Electromyography (EMG)
C. Begin ibuprofen
D. Begin carisoprodol

Answer:

2. Man with history of ankylosing spondylitis presents with sharp back pain in mid-
thoracic region which began suddenly. Blood pressure is 185/96 mm Hg. Examination
reveals a decrescendo diastolic murmur. Marked tenderness over 5th thoracic vertebra.
Which is the most likely diagnosis?
A. Acute mitral insufficiency
B. Aortic dissection
C. Atlantoaxial subluxation
D. Septic arthritis of the thoracic spine
E. Vertebral compression fracture

Answer:

3. The patient’s brother wishes to be tested with HLA-B27. Which of the following is cor-
rect regarding HLA-B27 testing?
A. HLA-B27 testing is never indicated.
B. All first-degree relatives of patients with a confirmed diagnosis of ankylosing
spondylitis should be screened for possible disease with HLA-B27 testing.
C. Any male patient under the age of 35 years with chronic back pain should be tested
for HLA-B27 as part of the routine workup.
D. HLA-B27 is indicated in patients with negative radiographs but with chronic low
back pain and decreased lumbar spinal flexion.
E. HLA-B27 positivity in first-degree relatives predicts a worse disease.

Answer:

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CASE 9
57-year-old man comes to office complaining of pain and swelling in his joints. He
has also noticed swelling of his fingers and toes. 1 year ago he began noticing a
scaly rash on his scalp. Exam is significant for sausage-like fingers, pitting of nails,
and swelling of DIP.

1. Diagnosis?

2. Typical radiographic finding?

3. Typical pattern of joint involvement?

4. What blood test is specific for the diagnosis?

A. HLA-B27
B. ESR
C. Rheumatoid factor
D. Serum psoriasis antibody
E. None

Answer:

Note 5. The “sausage” appearance of this patient’s fingers is typical of which class of rheumato-
logic diseases?
Hydroxychloroquine makes it
worse.

6. Best initial therapy?

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Summary: Case 9
In a patient who presents with complaints of finger swelling and a scaly rash, the diagnosis
is likely to be psoriatic arthritis. The arthritis can be similar in joint pattern to osteoarthritis
but is differentiated by the presence of inflammation in the form of morning stiffness lasting
longer than 45 minutes. The “pencil in cup” deformity is the typical radiographic finding.
The sacroiliac joints are frequently involved asymmetrically. The typical joint pattern is an
asymmetric, inflammatory arthritis involving the distal joints of the fingers. Axial skeleton
involvement is also common.

There is no serologic test that can be used for the diagnosis. The RF and ANA are always
negative. The seronegative spondylarthropathies are characterized by enthesopathy, which is
defined as inflammation of the tendon as it inserts into the bone. The major manifestations
of enthesopathy are dactylitis (“sausage fingers”), Achilles tendon tendinitis, and plantar
fasciitis.

CASE 10
22-year-old Caucasian man with pain and swelling of his right knee that began
gradually. He has low-grade fevers and weight loss over last 2 months. He had an
episode of gastroenteritis 3 months ago. Exam significant for oral ulcers, redness
in both eyes, and tender, swollen erythematous right knee joint.

1. Most likely diagnosis?

A. Gonococcal arthritis
B. Gout
C. Keratoderma blenorrhagicum
D. Reactive arthritis
E. Septic arthritis

Answer:

2. Next step in the management?

A. Antibiotics
B. Joint aspiration
C. Physical therapy
D. Radiograph
E. Steroid injection

Answer:

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3. What infectious diseases precede this syndrome? How will that change your manage-
ment of the case?

4. Best initial treatment for this disease?

Summary: Case 10
Gradual onset of pain and swelling in one isolated joint should prompt you to think of
Reiter syndrome or reactive arthritis as the most likely diagnosis. Unlike gonococcal arthritis,
Reiter syndrome is associated with oral ulcers, urethritis, and uveitis. Keratoderma blenor-
rhagicum, a rash that affects the soles of the feet, is a characteristic finding associated with
Reiter syndrome.

Other causes of reactive arthritis are:

• Yersinia
• Salmonella
• Shigella
• Ureaplasma
• Campylobacter

E.coli does NOT cause reactive arthritis.

Gonococcal arthritis typically responds dramatically to antibiotic therapy. The history of a

previous gastroenteritis or sexually transmitted disease preceding the onset of symptoms is
consistent with Reiter syndrome. In any monoarticular arthritis the next step in manage-
ment is always to aspirate the joint, to rule out an acute infectious process (where the WBC
>50,000). In any seronegative spondylarthropathy, HLA-B27 is the gene associated with
the disease. Gastroenteritis and chlamydial infections can precede this syndrome. If a chla-
mydial infection is implicated in the development of disease, then doxycycline can be used
for treatment of Reiter syndrome. The best initial therapy for Reiter syndrome is an NSAID.
Etanercept may be added if initial therapy with an NSAID fails.

Reactive arthritis
• Oligoarthritis of lower extremities
• Enthesitis (sausage)
• Dactylitis
• Eye problems (conjunctivitis, uveitis)
• Genital problems

Treat with NSAIDs, steroids, or sulfasalazine.

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CASE 11
67-year-old man comes into the emergency department complaining of
headache, jaw pain when he is eating, and nonspecific throat pain. Also
describes tenderness when he combs his hair. Denies fever or malaise. He has
been experiencing shoulder and hip stiffness over last several days. Exam is
unremarkable.

1. Diagnosis?
A. Dermatomyositis
B. Fibromyalgia
C. Giant cell arteritis
D. Lambert-Eaton syndrome
E. Myasthenia gravis

Answer:

2. Initial diagnostic test?

A. Antinuclear antibodies
B. Erythrocyte sedimentation rate
C. C-ANCA
D. C-reactive protein
E. P-ANCA

Answer:

3. Most sensitive test?

4. Next step in management?

5. How can you differentiate clinically between giant cell arteritis and polymyalgia
rheumatica (PMR)? How does their treatment differ?

6. What cardiovascular complication occurs?

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Summary: Case 11
The best initial test for the diagnosis of giant cell arteritis is to obtain an ESR. The ESR is elevated
in more than 90% of patients with giant cell arteritis. Forty percent of patients with giant cell
arteritis present with atypical symptoms such as dry cough, painful shoulder paralysis (mono-
neuritis multiplex), or fever of unknown origin. Therefore, in elderly patients with an elevated
ESR, FUO, and a normal WBC, the diagnosis should be considered even if they do not complain
of typical symptoms such as jaw, scalp, or throat pain. The most sensitive test for the diagnosis
is a temporal artery biopsy. When you suspect the diagnosis of giant cell arteritis, the next step
is to give high dose steroids immediately. Polymyalgia rheumatica (PMR) usually presents with
shoulder and hip pain without any evidence of temporal artery involvement (no headache, jaw
claudication, blurry vision, throat pain, or scalp tenderness on history). Both PMR and giant cell
arteritis are treated with steroids. PMR is treated with a lower dose of a systemic steroid. Any
large-vessel vasculitis can be complicated by aortitis resulting in aortic regurgitation. Thoracic
aneurysms of the aorta occur in 15% of patients with giant cell arteritis.

CASE 12
31-year-old man comes with fever and weight loss over last several months.
He has difficulty walking, with frequent tripping and falling. Started gradually
4 months ago. Along with his fever and weight loss is right lower calf pain. He
has abdominal pain that begins 40 minutes after a meal and is associated with
nausea and vomiting. History for intravenous drug use (IDU). BP 176/102 mm Hg;
a mottled, net-like rash located on the patient’s lower extremities bilaterally,
gangrene of the left big toe, and right foot drop with 3/5 strength on dorsiflexion
of right foot.

1. Most likely diagnosis?

A. Alcoholic neuropathy
B. Guillain-Barré syndrome
C. Mononeuritis multiplex
D. Reflex sympathetic dystrophy

Answer:

2. Best initial test?

A. Nerve conduction studies
B. P-ANCA
C. Renal biopsy
D. Sural nerve biopsy
E. Angiogram

Answer:

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3. If the blood tests are all negative, what test would you order next?

4. Can you differentiate between PAN and microscopic polyangiitis?

A. GI involvement
B. Skin lesion
C. Arthralgia
D. Lung involvement
E. Uveitis

Answer:

5. What is the significance of IDU in this case?

A. HIV causes this presentation
B. Staphylococcus toxin leads to this
C. Focal segmental glomerulosclerosis is common here
D. Hepatitis B or C is found in 15–30% of these patients

Answer:

6. Best initial treatment?

Summary: Case 12
Foot drop, as a presentation of mononeuritis multiplex, and evidence of a systemic illness
with fever and weight loss, suggest vasculitis as the diagnosis. The presence of a mottled,
“net-like” rash, also known as livedo reticularis, suggests a vasculitis. The best initial test is a
p-ANCA. It is sometimes helpful in making the diagnosis. In order to make the diagnosis of
polyarteritis nodosa (PAN), either an angiogram demonstrating microaneurysms or a renal
biopsy demonstrating vasculitis has to be obtained. Both are extremely sensitive and accurate
in confirming the diagnosis. When a vasculitis involves medium-sized arteries, including the
renal artery, hypertension will usually be a manifestation of the disease.

In PAN, which is a vasculitis of medium-sized arteries, manifestations include livedo reticu-

laris, skin ulcers with gangrene, and hypertension. What is interesting about PAN is the
absence of pulmonary involvement. Microscopic polyangiitis manifests with purpura, hema-
turia, and proteinuria, and does involve the lung. Abdominal pain can be seen in both PAN
and microscopic polyangiitis. PAN is associated with hepatitis B and hepatitis C in 10–30%
of all cases. The best initial therapy is high-dose corticosteroids.

Patients whose disease is refractory to steroid therapy or who have major organ involvement
should have cyclophosphamide added to their treatment regimen.

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CASE 13
34-year-old woman with rash and joint/abdominal pain that began 2 days ago.
The rash is associated with abdominal pain and her urine “looked like tea.” Also
has muscle pain. She had just recovered from an upper respiratory tract infection
2 days prior to her first episode of hematuria. You note a nonblanching rash
located over the lower extremities and the buttocks. Platelet count is normal and
creatinine is 2.1 (elevated).

1. Next step in management?

A. ANA
B. Skin biopsy
C. Urinalysis
D. Protein:creatinine ratio in the urine
E. Antistreptolysin O

Answer:

2. Diagnosis?
A. Cryoglobulinemia
B. Henoch-Schonlein purpura
C. Post-streptococcal glomerulonephritis
D. Systemic lupus erythematosis
E. IgA nephropathy

Answer:

3. Most accurate test?

4. Treatment?
A. ACE inhibitors
B. Fish oil
C. Angiotensin receptor blockers
D. Cyclophosphamide
E. None

Answer:

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5. The patient develops a urine protein-to-creatinine ratio of 7 to 1. Creatinine rises to

3.8 mg%. What would you do?
A. Enalapril and prednisone
B. Prednisone
C. Cyclophosphamide
D. Prednisone and cyclophosphamide
E. Plasmapheresis

Answer:

Summary: Case 13
In any patient who presents with hematuria and renal insufficiency the next step is to obtain a
urinalysis. Urinalysis is to ascertain whether there are RBC casts. Casts suggest acute glomeru-
lonephritis as the cause of the renal insufficiency. Unlike poststreptococcal glomerulonephritis,
where there is a latent period before the onset of the hematuria, in IgA nephropathy the latent
period is 1–2 days in length. Nonblanching rash can be secondary either to a vasculitis or to
thrombocytopenia. A normal platelet count rules out idiopathic thrombocytopenic purpura
(ITP). A normal platelet count points toward a vasculitis as the cause of the rash. IgA levels in
the serum will be elevated in 50% of cases. The most accurate test is a renal biopsy where immu-
nofluorescence shows IgA deposits in the mesangium. Patients with proteinuria >5 g/24 hours
should be started on an ACE inhibitor. Steroids are used to treat the underlying disease only if
there is no spontaneous resolution, severe proteinuria is present, or renal failure is progressing.

CASE 14
56-year-old man comes to ED complaining of right knee pain that began
suddenly and awoke him from sleep. This is his first episode of arthritis. Temp
38.8ºC (102ºF). Right knee is warm, erythematous, swollen, and extremely
tender to touch.

1. What is the next step?

A. Antibiotics
B. Joint aspiration
C. Physical therapy
D. Radiograph
E. Steroid injection

Answer:

2. What would an x-ray show?

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Arthrocentesis shows negatively birefringent, needle-shaped crystals. White cell

count returns as 23,000.

3. Diagnosis?
A. Gout
B. Osteoarthritis
C. Osteochondritis dissecans
D. Pseudogout
E. Septic arthritis

Answer:

4. Initial treatment?
A. Allopurinol
B. Oral steroids
C. Colchicine
D. Ibuprofen
E. Intra-articular steroids
F. Febuxostat

Answer:

5. When is intra-articular steroids the answer?

6. When are oral steroids the answer?

7. When is colchicine the answer?

Note
Pegloticase and rasburicase
are used if allopurinol or 8. What nonpharmacologic measures are recommended to prevent further attacks?
febuxostat do not control
disease.

9. What are the indications for using drugs that reduce uric acid levels? When would you
use allopurinol? probenecid/sulfinpyrazone?

10. When is febuxostat the answer?

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Summary: Case 14
In any acute monoarticular arthritis the next step in management is to aspirate the joint for
analysis of the joint fluid. It is impossible to distinguish between a crystal-induced arthropa-
thy and a septic arthritis on the basis of clinical criteria alone. Both will give a warm, inflamed
joint on exam, associated fever, and an elevated WBC count. Analysis of the joint fluid will
identify the presence of crystals, and a cell count of >50,000 white blood cells implies an
infectious arthritis. Non-specific soft-tissue swelling is often found. Linear radiodense cal-
cium deposits are seen in pseudogout along the menisci or articular cartilage. The presence
of negatively birefringent, needle-shaped crystals on joint fluid analysis is extremely specific
for the diagnosis of gout. In pseudogout, the joint fluid would show positively birefringent,
rhomboid-shaped crystals.

The best initial therapy for the treatment of acute gouty arthritis is an NSAID. Any NSAID
is equally effective for the treatment of gouty arthritis, but indomethacin is the traditional
choice. Patients who have a history of PUD, use alcohol, and/or are currently taking steroids Note
or warfarin have a higher risk of NSAID-associated GI bleeding. An alternate approach is to
add a proton pump inhibitor to the NSAID therapy to prevent the development of a signifi- Uric acid level can drop to
cant bleed. Misoprostol has been used in the past for this purpose, but because of its poor normal during acute attacks.
tolerability (diarrhea in 10–20% of patients) it is no longer considered first-line therapy for
prophylaxis of NSAID-associated GI bleeding.

Both standard NSAID therapy and COX-2 inhibitors can induce fluid retention and acute
renal failure in all patients, but especially in patients with congestive heart failure. Use of
these therapies is relatively contraindicated in the setting of renal insufficiency and/or con-
gestive heart failure. Colchicine is rarely used for the treatment of acute gouty arthritis. Use
is limited by its poor tolerability (abdominal cramping, nausea, vomiting, and diarrhea).
In older patients or patients with renal insufficiency or liver disease, the dose of colchicine
should be adjusted. Patients who cannot take an NSAID should receive steroids for treatment
of acute gouty arthritis. One joint is injected; multiple joints get systemic steroids.

Nonpharmacologic measures that can reduce the number of gouty attacks include decreas-
ing consumption of meat and alcohol, and losing weight. Low-dose aspirin, loop/thiazide
diuretics, and niacin may exacerbate gouty arthritis. Indications for reducing uric acid levels
in gouty arthritis include renal insufficiency, nephrolithiasis, tophaceous gout. and recurrent
attacks. Patients who require diuretic therapy for other comorbid conditions should be con-
sidered for prophylactic therapy.

Use allopurinol or febuxostat for prophylaxis. Probenecid and sulfinpyrazone rarely, if ever,
correct. There is no need to measure 24-hour urine for uric acid any longer. The concept of
overproducer or underexcretor does not matter.

Pseudogout occurs when calcium-containing salts become present in articular cartilage. It is

associated with hemochromatosis, hyperparathyroidism, diabetes mellitus, hypothyroidism,
and Wilson disease. Pseudogout is treated acutely with NSAIDs and prophylactically with
colchicine.

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Note 1. 56-year-old man with history of gout, PUD, and chronic renal insufficiency undergoes
CABG and complains of right toe pain that began suddenly 2 days postoperatively.
CPPD caused by Physical exam reveals a red, swollen right big toe. What is the best initial therapy?
• Hyperparathyroid A. Celecoxib
• Hemochromatosis B. Indomethacin
C. Prednisone
• Hypercalcemia
D. Arthrocentesis and intra-articular injection of triamcinolone
• Hypophosphatemia
Answer:
• Wilson’s disease
2. A man is found to have a markedly elevated uric acid level on a blood test by his urolo-
gist for a reason he can’t remember. He feels well. What to do?
A. Sulfinpyrazone
B. Allopurinol
C. Febuxostat
D. 24-hour urine uric acid level
E. Nothing

Answer:

3. 43 year old man comes with pain in the knees and wrist. He is fatigued and has erectile
dysfunction. Exam: Swelling of wrists and knees X-ray: Calcification of menisci AST/
AST elevated HgA1c 8%. What is the diagnosis?
A. Diabetic joint disease
B. Hyperparathyroidism
C. CPPD from hemochromatosis

Answer:

Summary:
Patients with acute gout and a contraindication to NSAIDS such as ulcer disease and renal
insufficiency should be treated with intra-articular or systemic steroids. There is no meaning-
ful use for routine testing of urine uric acid excretion to guide therapy. You can have a high
uric acid without gout and you can have a low uric acid level during an acute attack of gout.

Calcium pyrophosphate deposition disease (CPPD) or pseudogout is managed acutely in the

same way at gout with NSAIDs and steroids. The cell count in the joint fluid is the same in
range of 2,000 to 20,000 with some occasionally going up to 50,000 WBCs.

Synovial Fluid Cell Count

<200: Normal

200–2,000: Non-Inflammatory

2,000–50,000: Inflammatory (gout, CPPD)

>50,000: Infectious/Septic

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CASE 15
58-year-old woman comes with joint stiffness that began slowly 1 year ago.
Stiffness lasts less than 20 minutes and occurs in the morning. Her right DIPs and
right knee are affected. Symptoms have gradually worsened. Now experiences
pain when she crochets and when walking for longer than 20 minutes. Pain is
relieved by rest. Exam is significant for crepitus on passive motion of right knee.

1. Diagnosis?
A. Charcot joint
B. Osteoarthritis
C. Rheumatoid arthritis
D. Psoriatic arthritis
E. Septic arthritis

Answer:

2. What would you expect on x-ray?

3. What are characteristic laboratory findings?

A. Elevated C-reactive protein
B. Elevated ESR
C. Rheumatoid factor
D. Ferritin level
E. None
Answer:

4. Are there any nonpharmacologic interventions?

5. Best initial treatment?

A. Acetaminophen
B. Celecoxib
C. Colchicine
D. Indomethacin
E. Oxycodone

Answer:

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Summary: Case 15
Patients who present with an asymmetric, distal arthritis involving the DIP joints of the
hands are very likely to have osteoarthritis (OA). The absence of significant morning stiff-
ness helps to differentiate OA from the asymmetric distal arthritis of psoriatic arthritis.
Radiographic findings on x-ray would show osteophytes and joint space narrowing. The ESR,
ANA, and CRP are typically normal, as the arthritis is a noninflammatory one.

Nonpharmacologic treatment for OA consists of patient education, physical and occupa-

tional therapy, and weight loss. While most patients will ultimately require pharmacologic
therapy, nonpharmacologic treatment reduces the intensity of pharmacologic intervention.
After nonpharmacologic therapy, the best initial therapy for OA is acetaminophen. If patients
fail therapy with acetaminophen, then a trial of NSAIDs/COX-2 inhibitors may be attempted.
If patients have persistent pain despite all these interventions, the next step is intra-articular
steroids.

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CASE 16
Man in ED with right knee pain. Began suddenly and is dull in character. Patient
denies weight loss, fever, or malaise over the last several months. Past medical
history consists of asthma, for which he has been taking prednisone off and on
for the last 6 years. Exam: no pain on passive movement of knee but significant
pain on internal rotation of the hip.

1. Most likely diagnosis?

A. Avascular necrosis of the hip
B. Hip fracture
C. Osteoporosis
D. Ischial bursitis
E. Trochanteric bursitis

Answer:

2. Initial test?

3. Most sensitive test?

4. Best initial treatment?

5. Why is this patient’s history of asthma important?

Summary: Case 16
This patient’s most likely diagnosis is avascular necrosis of the hip. He complains of knee
pain, but movement of the knee joint does not produce any pain. Rather, internal rotation
of the hip is the maneuver that elicits symptoms. The best initial test for the diagnosis of
avascular necrosis bone is radiographic evaluation of the joint in question. The most accurate
test for the diagnosis is a CT or MRI scan. Nonoperative treatment usually results in a poor
prognosis. Protected weight-bearing is associated with a greater than 85% rate of femoral
head collapse. For this reason surgical decompression is indicated in most lesions. Severe
joint dysfunction associated with cortical collapse is an indication for surgical intervention.
Chronic steroid therapy is an important cause of avascular necrosis of bone. Other causes
include SLE, alcoholism, gout, sickle-cell anemia, and Gaucher disease.

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Tendon, Ligament, Cartilage and Bursa disorders

1. Patient with swelling of ears, hoarseness and nose. Laryngoscopy shows edema and
inflammation. Earlobe is normal. Biopsy of cartilage is abnormal.
What is it?
Treatment?
2. What is it?

Pain on abduction of shoulder mainly anterior tenderness over

bicipital groove.
What is it?
Treatment
3. Patient in motor vehicle accident. Fell on outstretched hand. Couple of hours later with
shoulder pain, swelling and unable to abduct, but he can shrug his shoulder
What is it?
Treatment

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4. Pain the medial (inside) aspect of the elbow. It is tender as well

Humerus

Lateral epicondyle
Medial
Radius
epicondyle

Ulna

What is it?
Treatment
5. Stiffness of the ulnar aspect of the hand. Unable to extend 3rd and 4th fingers.
Thickening and shortening of palmar fascia. Often with diabetes and alcoholism.

Cord

What is it?
Treatment
6. Patient falls on outstretched hand. Tender at anatomical snuff box.

What is it?
Treatment

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7. Football player. Knee pain. Heard “popping sound”

Drawer sign and Lachman sign positive

Anterior drawer

Lachman

What is it?
Treatment

Summary:
The ears are made of cartilage, but not the earlobe. Relapsing polychondritis will give hoarse-
ness as well. Treat with steroids. When the anterior shoulder over the bicipital groove is pain-
ful and tender, bicipital tendonitis is treated with NSAIDs and a steroid injection

Rotator cuff tear makes it impossible for the patient to abduct the arm past 40 degrees or to
comb hair. Look for phrase “unable to lift arms above head”. Only surgery can ultimately fix
the rotator cuff. Tennis elbow is the lateral epicondyle. Golfer’s elbow is the medial epicon-
dyle. Both are treated with NSAIDs and physical therapy.

Dupuytren’s contracture is treated with collagenase to try to dissolve the contracture. If this
does not work, surgical release is needed.

Tenderness at the anatomical snuff box after a fall/injury is a scaphoid fracture. Do an x-ray
or MRI. Treat with splints and NSAIDs. Football injury of the knee with a “pop” and the lax-
ity of increased mobility of the drawer and Lehman sign are from anterior cruciate ligament
tear.

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CASE 17
A 57-year-old, African-American woman presents to office with difficulty rising
from a seated position. Denies double vision but chokes when eating. The
patient explains that it is difficult for her to “start swallowing.” Exam is noteworthy
for scaly patches over the PIPs and MCPs bilaterally. You also note proximal
muscle weakness of the lower extremities bilaterally.

1. Diagnosis?
A. Dermatomyositis
B. Eaten-Lambert syndrome
C. Guillain-Barré syndrome
D. Hypothyroidism
E. Myasthenia gravis

Answer:

2. Best initial test?

A. Acetylcholinesterase antibodies
B. Antinuclear antibodies
C. Serum creatinine phosphokinase and aldolase
D. TSH
E. Tensilon (edrophonium) test

Answer:

3. Most accurate test?

4. What other testing

5. Significance of anti-Jo-1 antibodies?

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6. Initial treatment?
A. Prednisone
B. Azathioprine
C. Cyclophosphamide
D. Anti-TNF
E. Colchicine

Answer:

7. What would you give if this fails?

A. Prednisone
B. Azathioprine
C. Cyclophosphamide
D. Anti-TNF
E. Colchicine

Answer:

Summary: Case 17
In a patient who presents with proximal muscle weakness associated with scaly patches found
symmetrically over her finger joints (Gottron papules), the most likely diagnosis is dermato-
myositis. The best initial test for the diagnosis is to measure serum creatinine phosphokinase or
aldolase. The most accurate test for the diagnosis is muscle biopsy that typically shows various
stages of muscle fiber necrosis and regeneration. In any patient who presents with dermato-
myositis, the presence of an underlying malignancy must be considered. The next step after the
diagnosis is confirmed is to search for an occult malignancy. Anti-Jo-1 antibodies are associated
with pulmonary fibrosis. The best initial therapy is steroids. Patients with dermatomyositis who
fail to respond to steroids can be treated with methotrexate, azathioprine, or IV Ig.

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CASE 18
A 25-year-old woman comes to your clinic with fatigue, headaches. Aching and
pain in shoulder, neck, and both hips. Denies morning stiffness, low-grade fever, or
weight loss over recent months. Exam is significant for tender points on the trapezius
muscle but is otherwise normal.

1. What lab abnormalities would you expect?

A. ESR
B. C-reactive protein
C. Aldolase
D. CPK
E. None

Answer:

2. What diseases are associated with this disorder?

3. How are these patients managed?

Summary: Case 18
This patient has a history consistent with fibromyalgia. The presence of trigger points asso-
ciated with diffuse muscle pain is consistent with the disorder. Chronic fatigue syndrome
and fibromyalgia are different extremes along the same spectrum of disease. Essential to the
diagnosis of fibromyalgia is the absence of any objective measures of inflammation. The ESR,
CBC, and thyroid function are all expected to be normal. Sleep disorders, hypothyroidism,
anxiety, and depression are associated with fibromyalgia.

Treatment for fibromyalgia is with antidepressant medications, such as amitriptyline or

fluoxetine.

1. 37-year-old woman complaining of “aches and pains.” Symptoms started several

months ago and have been progressively worsening. She also complains of fatigue and
frequent headaches. Pain produced upon palpation of medial fat pad of knee. CBC,
ESR, and electrolytes are normal. Therapy?
A. Ibuprofen
B. Codeine
C. Amitriptyline
D. Prednisone

Answer:

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CASE 19
Receptionist with numbness and tingling in her right hand. Symptoms get worse
after she spends the day typing. Pain worsens at night and radiates to her forearm
and shoulder. Exam significant for positive Phalen and Tinel signs. No thenar
muscle atrophy and no weakness in the right hand.

1. Diagnosis?

Note
2. Test?
Causes of Carpal Tunnel A. Cervical CT scan
• Rheumatoid Arthritis B. Nerve biopsy
• Diabetes C. Nerve conduction study
D. Radiograph of the hand
• Pregnancy
E. Ultrasound
• Hypothyroidism
Answer:
• Amyloid
• Acromegaly 3. Therapy?

4. If initial therapy fails, what would you do next?

Note

Do not use MRI to assess

carpal tunnel.
5. When would your answer be surgery?

6. What systemic disorders are associated with this disorder?

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Summary: Case 19
In a patient with a history of repetitive hand motion who complains of numbness and tingling
over the distribution of the median nerve, the most likely diagnosis is carpal tunnel syndrome.
Atrophy of the thenar muscle also implies compression of the median nerve. The diagnostic
test of choice is nerve conduction studies to assess the presence of conduction delay in the
median nerve. The best initial therapy is hand splinting. In patients who fail physical therapy,
the next step in management is to inject steroids into the carpal tunnel. Surgical decompres-
sion is the most effective therapy for the treatment of carpal tunnel syndrome. Patients with
severe pain refractory to medical therapy or with significant thenar muscle atrophy should be
treated with surgical decompression. Rheumatoid arthritis, myxedema, amyloidosis, sarcoid-
osis, leukemia, acromegaly, diabetes mellitus, and hyperparathyroidism are all associated with
carpal tunnel syndrome.

CASE 20
20-year-old man with abdominal pain worse on eating. Better on empty stomach.
Also with Joint pain in hands and feet. There is 20 pounds of weight loss. Leg
ulcers for years. Exam: purpuric rash on lower extremities. ESR 100 mm/hour BUN
and creatinine are elevated. Chest x-ray is normal.

1. What is the diagnosis?

A. Churg-Strauss
B. Polyarteritis nodosa
C. SLE
D. Microscopic polyangiitis

Answer:

2. What test should you do first?

A. C-reactive protein
B. Rheumatoid factor
C. CPK
D. Abdominal Angiogram
E. Colonoscopy

Answer:

3. Which of the following is most likely to be associated with this?

A. Mononeuritis multiplex
B. MI
C. Lung cancer

Answer:

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4. If angiography is not diagnostic, what test to do?

A. Renal biopsy
B. Skin biopsy
C. Sural nerve biopsy

Answer:

5. 28-year-old person of Middle Eastern origin with recurrent abdominal pain every two
months lasting for 1-2 days. Attacks of periumbilical pain become generalized and are
associated with fever and joint pain. Imaging studies normal. Father has the same. What
is the diagnosis?
Treatment?
Complication?

6. Patient with asthma and allergic rhinitis comes with worsening shortness of breath as
steroids are tapered. He takes zafirlukast for asthma. CBC shows eosinophils. Urinalysis
shows red cells and red cell casts.
What is the diagnosis?
Treatment?

7. Chronic cough with abnormal x-ray. Shortness of breath. No upper respiratory prob-
lems. Hematuria and red cell casts.
Eosinophils: Normal
C-ANCA: Negative
P-ANCA: Positive
Biopsy shows vasculitis, but no granulomas.
What is it?
Treatment?

8. Swelling of skin of arms and legs after exercise with an orange peel type quality. Spares
the hands and feet.
CBC: increased eosinophils
What is it?
Treatment?

Summary: Case 20
Abdominal pain with joint pain, weight loss and renal insufficiency in the absence of lung
findings is polyarteritis nodosa. Once the ESR is elevated, there is no added benefit of testing
for CRP. Abdominal angiography spares the need for biopsy. If this is not clear, it is better to
biopsy the sural nerve in the leg, rather than the more invasive renal biopsy. Treat with steroids
and cyclophosphamide. Don’t forget to test for chronic hepatitis B and C.

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Recurrent attacks of fever and abdominal pain particularly with a family history in someone
of Middle Eastern origin is familial Mediterranean fever. Treat with colchicine. They can
develop amyloidosis.

Churg-Strauss syndrome is a vasculitis affecting the kidneys with asthma and eosinophilia.
It is associated with the use of the leukotriene inhibitor zafirlukast. Treat with steroids and
cyclophosphamide.

Microscopic polyangiitis is a lung and renal syndrome that can easily be confused with
Wegener’s but there are no upper respiratory issues (no otitis, no sinusitis) and no granulomas
on biopsy. Treat with steroids and cyclophosphamide.

Eosinophilic fasciitis has orange skin, worse after exercise and is treated with steroids.

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 Infectious Diseases
11
CENTRAL NERVOUS SYSTEM INFECTIONS

Case 1
32-year-old, generally healthy woman comes to ED with 1 day of fever and a
headache. She has photophobia. With her knees flexed against her abdomen,
there is pain when you try to passively extend them. No focal deficits, and she
is in pain but alert. When she is supine and you passively flex her neck, there is
spontaneous flexion of her hips and knees.

1. When do you need to do a CT scan of the patient’s head?

2. Most specific test on lumbar puncture?

The culture

3. Most likely organism?

Strep. pneumoniae

The CSF reveals 2,400 WBCs with 92% neutrophils. Gram stain is normal.

4. Best initial therapy?

5. When will your answer be to use steroids?

6. When do steroids lower mortality?

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Summary: Case 1
All central nervous system infections will be characterized by a fever and a headache. There
is an advantage to a Board question as compared with ordinary patient care, because if a
Board question asks you for the most likely diagnosis, it must give you a definite clue as to the
answer. If the patient has only a stiff neck, it must be meningitis. If the stiff neck is combined
with confusion and a seizure, then you cannot tell if the diagnosis is encephalitis. Although
a lumbar puncture (LP) is definitely the first test to perform, there are certain circumstances
in which your answer must be a CT scan of the head as the best next step prior to an LP. CT
scan is required before an LP if the patient has focal neurologic abnormalities, severe confu-
sion that makes the neurologic examination inaccurate, papilledema, and/or a recent seizure.
Immunocompromised patients, such as those with HIV and <200 CD4 cells, should also have
a CT prior to an LP. When you are asked “What is the most accurate test?” on an infectious
disease question, then the answer in general is a culture. You rarely, if ever, wait for the results
of the culture before initiating therapy. In all age groups outside of the neonatal period, the
most common cause of bacterial meningitis is Streptococcus pneumoniae. For immunocom-
petent patients, the best initial therapy for bacterial meningitis is ceftriaxone (or cefotaxime)
and vancomycin. Steroids are most useful for tuberculous meningitis to decrease the risks
of neurologic deficits, and they may decrease mortality in pneumococcal meningitis. Your
answer will be to use steroids when bacteria are found on Gram stain and/or when the CSF
white cell count is >1,000.

Case 2
48-year-old man with 3 days fever, headache, and stiff neck. Lumbar puncture
shows 158 white cells, 95% lymphocytes. Gram stain negative, latex agglutination
antigens for Streptococcus Haemophilus, and Neisseria are negative.

1. Diagnosis?

If THIS is in the history …then THIS is Answer THIS as the best Answer THIS as the best
and physical… the most likely initial and most specific initial therapy
diagnosis tests
HIV with <100 CD4 Cryptococcus India ink and cryptococcal Amphotericin and 5-FC,
cells; milder symptoms antigen test followed by lifelong
fluconazole
Lyme Specific IgM and IgG Ceftriaxone or penicillin
antibodies by ELISA and
Western blot
Rocky Mountain
spotted fever
Pulmonary TB; very Tuberculosis AFB stain and culture INH, rifampin, PZA,
high CSF protein ethambutol, steroids
Specific PCR
Viral meningitis

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Summary: Case 2
The point of the patient description here is to show that a mild meningitis with a moderate
lymphocytic pleocytosis can be consistent with a number of etiologies and, in fact, excludes
only bacterial causes. Viral meningitis can be confirmed with polymerase chain reaction (PCR)
methods; however, it is generally a diagnosis of exclusion. Viral meningitis is the most common
cause of this type of presentation and is the answer to the question “What is the most likely
diagnosis?” in a case such as this one if there are no other points of history or physical attached
to the case. If the history includes HIV with <100 CD4 cells, then the most likely diagnosis is
cryptococcosis and the best initial test is an India ink. The India ink is like a Gram stain: it is
extremely specific if it is positive, but it only has a 50–70% sensitivity. The most accurate test
for cryptococcosis is an antigen test, and the best initial therapy is amphotericin followed by
fluconazole.

Lyme is the “most likely diagnosis” when there is a history of a tick bite, target (erythema
migrans) rash, joint pains, or facial palsy. Because Borrelia and Rickettsia cannot be seen on a
Gram stain or grown in regular culture media, then the “most accurate diagnostic tests” for each
of these is a specific serologic test such as PCR or IgM by enzyme-linked immunosorbent assay
(ELISA) and Western blot. The “best initial therapy” for Lyme is ceftriaxone, and for Rocky
Mountain spotted fever is doxycycline. TB meningitis is treated with the same four-drug regi-
men as pulmonary TB except that steroids are added. There is no therapy for viral meningitis.

Case 3
72-year-old man with fever, headache, photophobia, and stiff neck for last
24 hours. Past history is significant for AIDS, CD4 count 34. History of PCP and
CMV retinitis. He is also pregnant. LP shows 3,800 WBCs, 92% neutrophils,
elevated protein, and a normal Gram stain. Latex agglutination tests for bacteria
are negative.

1. Most likely organism?

2. Best empiric therapy?

3. Best therapy if patient is penicillin-allergic?

Summary: Case 3
Immunocompromise is the most common risk for an increased incidence of Listeria mono-
cytogenes. Although neutrophil defects do predispose to Listeria, it is more common with
T-lymphocyte defects. Although Listeria is more common in these patients than in the gen-
eral population, Strep pneumonia is still the most common. Therefore, although we have to
start ampicillin for Listeria, we still have to cover for Pneumococcus with ceftriaxone and van-
comycin. Listeria is resistant to all cephalosporins. When a diagnosis of Listeria is confirmed,
gentamicin is added for synergy and to increase the rate of clearance of the bacteremia.
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Case 4
17-year-old boy brought by girlfriend to emergency department because he had
a seizure while they were kissing. His other friends arrive and state they are sure
he was well yesterday because they were sharing cigarettes just yesterday. Patient
complains of severe headache and is severely confused and febrile. Pulse 55.
BP 145/95 with irregular respirations. Petechial rash on trunk.

1. Diagnosis?

2. What would you do first?

A. CT
B. Ceftriaxone and vancomycin
C. C5-C9 levels
D. Lumbar puncture

Answer:

Eventually the boy’s CSF cell count shows 2,500 neutrophils.

3. What additional measures are necessary in this case?

4. If patient has recurrent disease of this type, for what will you test?
A. Neutrophil function
B. HIV
C. C5-C9 levels
D. IgG levels
E. Serum protein electrophoresis (SPEP)

Answer:

Summary: Case 4
The most common cause of bacterial meningitis in an adolescent or college-age patient, espe-
cially with a rash, is Neisseria meningitis. Prophylaxis for meningococcus is indicated only
for intimate contacts, such as household contacts or day care center or nursery school con-
tacts, where there can be salivary contact. There must be a reason to suspect salivary contact.
You do not routinely have to give prophylaxis to health care worker contacts. The method
of transmission of meningococcus requires direct fluid contact between the patient’s saliva
and the contact’s mucous membranes. Meningococcus is not spread via the airborne route,
as would be considered for close contacts of patients with TB. The most common wrong

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answer is to say that the doctors and nurses who attend patients with meningococcus need
prophylaxis with rifampin or ciprofloxacin.

This patient has severe confusion and a recent seizure. This means he must have a CT scan
prior to the LP. When a CT scan must be performed, then the first thing to do is to give a dose
of empiric antibiotics; this is the answer to “What is the best next step in the management of
this patient?” Whenever there is a delay to the LP, then your answer should be “Antibiotics next.”

Patients with terminal complement deficiency are predisposed to recurrent Neisseria infections.

1. After bone-marrow transplantation, a 72-year-old patient comes with meningitis. CSF

shows high protein and elevated white cell count. Stain shows gram-positive rods.
Which is the most likely causative organism?
A. Strep. pneumoniae
B. Listeria
C. Nocardia
D. Anthrax
E. Bacillus species

Answer:

Case 5
42-year-old, HIV-positive man comes with fever and worsening headache for last
2 weeks. No photophobia. Minimal neck stiffness. CT head is normal. CSF shows
an elevated protein, negative Gram stain. Cell count 4. All lymphocytes. Opening
pressure 240 mm H2O.

1. What is the single most important thing to know about this patient’s history?
A. Viral load
B. Whether his CD4 count is <100
C. History of pneumonia
D. Current prophylactic medication use
E. Current antiretroviral medication use

Answer:

2. What would you do for him at this time (as in, now)?
A. Intrathecal amphotericin
B. Caspofungin
C. Oral fluconazole
D. Intravenous amphotericin and flucytosine
E. Intravenous 5-flucytosine

Answer:

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3. Most accurate diagnostic tests?

4. Would you start this patient on HIV medications (anti-retrovirals)?

Summary: Case 5
In evaluating a patient with meningitis and HIV, the most important clue to the diagnosis is
the patient’s CD4 count. It is very rare with >100 CD4 cells and essentially impossible to have
cryptococcal meningitis as an opportunistic infection if the CD4 is >200. India ink is like a
Gram stain for bacterial meningitis in that it is a cheap, easy, immediately available test with
results obtainable within an hour after the lumbar puncture. Like the Gram stain, the test
lacks sensitivity and is positive in only 60–80% of cases at most. Cryptococcal antigen testing
is extremely sensitive and specific at rates >95% for each.

A low CSF white cell count does not exclude cryptococcal meningitis. In fact, the worst
prognostic factors for cryptococcal meningitis are a high opening pressure, a high cryptococ-
cal antigen titer, and a low CSF white cell count. Amphotericin should be started first in all
patients with acute CNS cryptococcosis. As the choice of “best initial therapy,” amphotericin
has a far greater mortality benefit compared with fluconazole.

Case 6
48-year-old man with a week of progressive confusion, disorientation, and fever.
You can’t find specific neck stiffness. Too confused to determine if he has focal
neurologic deficits.

1. Diagnosis?

2. Initial test, and what might you find?

A. MRI with contrast with cerebellar involvement
B. CT with contrast
C. CT without contrast with temporal lobe involvement
D. Urethral swab with multinucleated giant cells

Answer:

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3. Most accurate diagnostic test?

A. EEG
B. CSF with increased white and red cells
C. PCR for DNA
D. Brain biopsy

Answer:

4. Therapy?
A. Acyclovir
B. Ganciclovir
C. Valganciclovir
D. Interferon and steroids

Answer:

Summary: Case 6
The most common cause of acute febrile confusion is herpes simplex. Although there are
many viruses that can cause encephalitis, herpes is so much more overwhelmingly common
that you should just answer herpes for any cause of encephalitis. Although a head CT may
show some temporal lobe involvement, this is relatively nonspecific and insensitive. PCR is at
least as accurate as a brain biopsy. Acyclovir is considered the drug of choice, mostly on the
basis of administration issues. Famciclovir and valacyclovir are not available intravenously.

1. Man placed on acyclovir and develops high BUN and creatinine. What is most appro-
priate?
A. Stop acyclovir
B. Stop acyclovir and begin foscarnet
C. Reduce the dose of acyclovir
D. Reduce the dose of acyclovir and increase hydration and urine flow

Answer:

2. Patient comes with fever, headache, stiff neck and photophobia. CSF shows elevated
lymphocyte count. This is the fifth episode in six years. The last time he stayed home
and it resolved spontaneously.
What is the diagnosis?
What treatment?

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RESPIRATORY TRACT INFECTIONS

OTITIS MEDIA

Case 1
34-year-old man with pain in ear and decreased hearing over several days.
1. Most sensitive physical finding for otitis media?
A. Redness
B. Loss of light reflex
C. Immobility of the tympanic membrane
D. Bulging
E. Dullness

Answer:

2. Most accurate diagnostic test?

3. Initial therapy?

4. What will you treat with if this fails?

Summary: Case 1
Otitis media commonly presents with a bulging tympanic membrane, pain, decreased hear-
ing, and loss of the light reflex; however, it can be present even in the absence of these find-
ings. The most sensitive finding for the presence of otitis media is an immobile tympanic
membrane on insufflation of the ear. Although it is rarely done, tympanocentesis is the most
accurate diagnostic test for otitis media. Tympanic membrane puncture is the only way to
identify a specific bacteriologic etiology. Tympanocentesis is your answer to “What is the
next step in the management of this patient?” when the patient has been repeatedly treated
and has shown either no response or frequent recurrences. Amoxicillin is your answer as the
“best initial therapy.” If there is no response to amoxicillin, then coverage is broadened with
the addition of clavulanic acid or a switch to oral second- or third-generation cephalosporins,
such as cefixime, ceftibuten, loracarbef, or cefdinir.

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Case 2
34-year-old man with headache, fever, discolored nasal discharge, and a bad taste
in his mouth. On exam he has tenderness over maxillary sinuses.

1. What would you do next?

2. Most accurate test?

A. X-ray
B. CT scan
C. Biopsy
D. MRI scan
E. Culture of the discharge

Answer:

3. What is the Best initial therapy?

A. Amoxicillin/clavulanic acid and decongestants
B. Erythromycin
C. Nitrofurantoin
D. Linezolid

Answer:

Summary: Case 2
When your case gives such obvious findings as a discolored nasal discharge, facial pain and
tenderness, and headache, then there is no point in answering a radiologic study as the “best
next step in management.” You should go straight to treatment. Although a CT scan is an
accurate test for sinusitis, it still cannot give you a specific microbiologic diagnosis.

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INFLUENZA

Case 3
A 46-year-old secretary comes to see you because of 1 day of fevers, myalgias,
arthralgias, sore throat, headache, nasal stuffiness, and dry cough. Physical
examination is normal except for fever.

1. What would you do next?

A. Acetaminophen and fluids
B. Oseltamivir
C. Amantadine
D. Nothing

Answer:

2. Best initial diagnostic test?

3. Who should be vaccinated?

A. > 50 yearly
B. Chronic heart, lung, and liver diseases
C. Everyone

Answer:

Summary: Case 3
Patients with influenza can be treated with neuraminidase inhibitors, such as oseltamivir and
zanamivir, if they present within the first 48 hours. Neuraminidase inhibitors are active against
both influenzas, A and B. Older agents, such as amantadine and rimantadine, are effective only
against influenza A and should never be used as empiric therapy. The influenza vaccine should
be administered to all patients if they are generally healthy. In addition, the following groups
derive the greatest benefit from vaccine: all health care workers, regardless of age, to prevent
spread of the virus to patients; patients with chronic cardiopulmonary disorders, diabetes,
and/or hemoglobinopathies; and pregnant women in their second and third trimesters.

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HEAD AND NECK INFECTIONS

Case 5
Patient with low-grade fever, sore throat, fatigue, and posterior cervical adenopathy.
(Question may be accompanied by a picture of red spots on the palate.)

1. With which complication would you use steroids?

A. Tonsillar enlargement
B. Guillain-Barré syndrome
C. Airway obstruction
D. Myocarditis

Answer:

Case 6
17-year-old comes with abrupt onset of high fever, sore throat, and dysphagia. Also
drooling and pooled saliva in the back of the throat.

1. Treatment?
A. Albuterol
B. Oral amoxicillin
C. Ceftriaxone and clindamycin
D. Erythromycin
E. Vancomycin

Answer:

Case 7
18-year-old high fever, extremely enlarged tonsils, dysphagia, neck pain, and
drooling of saliva. He is speaking with a “hot potato” voice. There is swelling of
the neck, and the uvula is deviated to one side.

1. Diagnosis?
A. Epiglottitis
B. Tonsillitis
C. Mononucleosis
D. Peritonsillar abscess

Answer:

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Case 8
40-year-old, healthy woman with pain in right ear with itching. She has tenderness
on manipulation of the lobule. The tympanic membrane cannot be seen because
of swelling of the canal. What do you recommend?

A. Otic steroids
B. Amoxicillin/clavulanic acid orally
C. Irrigation of the canal with saline
D. Otic solution of neomycin and topical steroids

Answer:

Case 9
25-year-old man comes with fever and worsening throat and neck pain of 2 days’
duration. He has inflamed tonsils and exquisite tenderness and swelling along
the left sternocleidomastoid muscle. WBC is 18,000; and the chest x-ray shows a
pleural effusion. Diagnosis?

A. Diphtheria
B. Epiglottitis
C. Streptococcal pharyngitis
D. Septic thrombophlebitis of the internal jugular vein

Answer:

Case 10
A patient had nasal packing several days ago for severe epistaxis. You are called for:

• BP 84/50, Pulse 126, 102F

• Confusion
• Elevated Creatinine
• Low calcium, low sodium

There is a diffuse red rash “like a sunburn” and desquamation

What is the treatment?

A. Piperacillin/Tazobactam
B. Clindamycin and Vancomycin
C. Ampicillin and gentamicin
D. Oxacillin

Answer:

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Which of the following is the LEAST common cause of toxic shock syndrome?
A. Tampons
B. Skilled nursing facility wearing diapers
C. Nasal packing
D. Sutures in wound
Answer:

Case 11
Which of the following is a first line indication for amphotericin?

A. Candidemia in neutropenic cancer patient

B. Pulmonary aspergillosis after bone marrow transplantation
C. Mucormycosis with black necrosis in an uncontrolled diabetic

Answer:

PNEUMONIA

Case 1
62-year-old man comes to the ED because of fever, cough, and sputum. Sputum
is yellowish and thick. Uses no medications. His temperature is 38.8ºC (102ºF) and
respiratory rate is 28. Lung exam reveals rales at the right base.

1. What would you do first?

Obtain pulse oximetry and blood gas or give oxygen

2. What test first?

Chest x-ray is always first to evaluate respiratory infections

3. Most accurate diagnostic test?

Sputum Gram stain and culture

4. What criteria will you use to determine when your answer is admission to the hospital?

5. What is the most appropriate therapy?

Empiric therapy of community-acquired pneumonia

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Outpatient (Nonhospitalized) Inpatient (Hospitalized)

First choice: All as a single agent. First choice: Second- or third-generation
Macrolides: azithromycin, clarithromycin cephalosporins: ceftriaxone, cefotaxime,
cefuroxime
Alternative: Doxycycline
Combined with either: Doxycycline or a
macrolide
Or, for patients with a history of Alternative: New fluoroquinolones alone,
cardiopulmonary disease: New levofloxacin, gemifloxacin, moxifloxacin
fluoroquinolones, levofloxacin,
gemifloxacin, moxifloxacin
Or, for immunocompromised or critically
ill patients: Beta-lactam/Beta-lactamase
combination (ticarcillin/clavulanate;
piperacillin/tazobactam);
Or: Carbapenems
Or: Ceftazidime, cefepime
Combined with either: A macrolide or a
fluoroquinolone

6. Who receives the pneumococcal vaccine?

People over age 65; people with chronic disease such as diabetes, cirrhosis, COPD, or CHF;
asplenic patients; people with hematologic malignancies; steroid users; people with renal
failure; HIV-positive people; organ transplant recipients; and those on immunosuppressive
medications

7. Who should be re-vaccinated after 5 years?

8. Who should receive more than one re-vaccination?

Summary: Case 1
Pneumonia is the most common infectious cause of death in the United States and therefore is
guaranteed to be found on the Board exam. The first question is to determine which patients
should be admitted to the hospital. The most important criteria are the presence of hypoxia,
hypotension, tachycardia, tachypnea, altered mental status, hyponatremia, and an elevated
BUN. The older and sicker a patient is, the more likely your answer should be to admit the
patient. Underlying comorbid conditions are important criteria as well, such as cancer or heart,
liver, or kidney disease. Although the best initial test for any respiratory infection is a chest x-ray,
this is not actually one of the criteria for admission. Even if just one lobe is involved, a patient
who is hypoxic and hypotensive should be admitted. Even if several lobes are involved, a patient
who does not have the serious conditions described above does not need to be admitted.

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Case 2
Man admitted for elective abdominal surgery and receives perioperative cefoxitin.
He develops a severe pneumonia on the seventh hospital day, requiring intubation
and mechanical ventilation.

1. Most likely organisms?

A. Gram-negative rods
B. Gram-positive cocci
C. Mixed anaerobic flora
D. Candida

Answer:

Case 3
A patient is admitted with gram-negative sepsis and abdominal pain. He is placed
on imipenem. Creatinine elevated at 2.5 mg/dL. He has his first seizure.

1. Most likely cause of his seizure?

A. Hypocalcemia
B. Hypomagnesemia
C. Meningitis
D. Imipenem toxicity
E. Uremia

Answer:

Summary: Case 3
Hospital-acquired pneumonia is most often from gram-negative bacilli. This is particularly
true for those who develop their pneumonia while intubated and on mechanical ventilation
in an intensive care unit. The best drugs for pneumonia that develops in this setting are car-
bapenems (imipenem, meropenem), piperacillin-tazobactam, and third- or fourth-generation
cephalosporins such as ceftazidime or cefepime.

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Case 4
47-year-old Australian farmer comes with several days of fever, cough, and chest
soreness. Cough is dry, and his chest hurts worse with inspiration. He has been
having some soft stools and headaches. He is healthy and on no medications. He
is occasionally intimate with the animals. Besides fever and a respiratory rate of 20,
his physical examination is normal. Chest x-ray shows bilateral interstitial infiltrates.

If THIS is in the . . . Then THIS is Answer THIS as the best Answer THIS as the best
history and physical . . . the most likely initial and most specific initial therapy
diagnosis tests
HIV CD4 <200, no Pneumocystis LDH TMP/SMZ,* pentamidine,
prophylaxis Bronchoscopy steroids if pO2 <70 or A-a
gradient is >35
Young, healthy, anemia, Mycoplasma
hemolysis
Legionella

Coxiella burnetii
(Q fever)
Very little, hoarseness, Chlamydia Specific serology Macrolides, quinolones
laryngitis pneumoniae
Alternative: Doxycycline
*Trimethoprim-sulfamethoxazole

Summary: Case 4
The point of this case is to show that the presentation of dry cough, nondiagnostic sputum
Gram stain, fever, and shortness of breath alone is too nonspecific to allow for a specific diag-
nosis. You are dependent upon other words in the history, such as “HIV with <200 CD4 cells,”
to answer PCP as the most likely diagnosis. Although the LDH can be elevated in any cause
of pneumonia, an elevated LDH is particularly associated with PCP on board questions. The
same is true of hyponatremia and Legionella pneumonia. Although any form of pneumonia
can give you syndrome of inappropriate antidiuretic hormone secretion (SIADH), hypona-
tremia is especially associated with Legionella in board questions. Legionella is most accurately
diagnosed by sputum culture on specialized charcoal yeast extract agar. The best initial test for
Legionella is a urinary antigen, which is 99% sensitive and specific for Legionella. Most of the
“atypical” pneumonias, such as Legionella, Mycoplasma, and Chlamydia, are best treated with
a macrolide or a new fluoroquinolone such as levofloxacin, gatifloxacin, and moxifloxacin.
Although hoarseness is suggestive of Chlamydia pneumonia, there are very few specific asso-
ciations with this organism to allow an answer to “the most likely diagnosis” question until the
serologic test for Chlamydia is obtained. If you do not see animal exposure in the history, there
is no reason to answer Coxiella burnetii as the most likely diagnosis. Coxiella can be effectively
treated with doxycycline or a macrolide.

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1. Aerosolized transmission is most common in which of the following?

A. Rickettsia typhi
B. Rickettsia prowazekii
C. Coxiella burnetti
D. Brucellosis

Answer:

2. Veterinarian comes with a papule on his hand. He has muscle pains and conjunctival
suffusion and his neck is stiff and he is confused. He has thrombocytopenia, and eleva-
tions of CPK, AST, ALT and his creatinine. Most likely diagnosis?

A. Coxiella burnetti
B. Tularemia
C. Ehrlichia
D. Leptospirosis
E. Trichinosis

Answer:

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Case 5
Construction worker has recently returned to New York after a job in Arizona for
the last month. Fever, dry cough, and joint pains, which have started to improve.
Erythema nodosum lesions on legs. The chest x-ray shows scattered nodular
infiltrates.

1. Diagnosis?

2. Diagnostic test?
Serology with complement fixation titers

3. Therapy?

Summary: Case 5
“Desert rheumatism” is the term applied to Coccidioidomycosis. The organism starts as a
nonspecific respiratory infection and is clinically indistinguishable from viral pneumonia.
When you see the word “Arizona” in the history, you should suspect coccidioidomycosis. This
organism is frequently associated with joint pain and erythema nodosum. Do not answer
“fungal culture” as the best method of establishing a diagnosis. Serology with complement
fixation titers is the answer to “most accurate diagnostic test.” No treatment is necessary for
mild respiratory symptoms. Disseminated disease is best treated with amphotericin.

1. Most common bacteria that causes lung infection in 75 year old patient after viral
infection?
A. Streptococcus pneumonia
B. Pseudomonas
C. Legionella
D. Staphylococcus

Answer:

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TUBERCULOSIS

Case 1
42-year-old man emigrated from Russia and was recently released from prison has
been experiencing cough, fever, and weight loss. He is an HIV-positive health care
worker who used intravenous drugs and was homeless. Chest x-ray is abnormal
for an apical infiltrate.

1. What would you do next?

Sputum AFB stain and culture; respiratory isolation; never PPD first in acutely symptom-
atic patients

2. Initial therapy, and for how long?

3. What if the patient were a pregnant female?

4. What if he had a CD4 <500 and needed antiretroviral medications?

5. When do you treat for longer than 6 months?

6. When do you use steroids to treat tuberculosis?

A. Pericardial and CNS
B. Bone and CNS
C. Pleural
D. CNS
E. Gastrointestinal

Answer:

Summary: Case 1
Tuberculosis is best tested for initially with a sputum stain for acid-fast bacilli (AFB). Do not
answer “a PPD” as the best initial test on any acutely symptomatic patient. Six months is the
standard length of therapy. This is true if the patient has extrapulmonary TB as well, as long
as the organism does not involve the bone (osteomyelitis) or brain (meningitis), occurs dur-
ing pregnancy, or is military. Four drugs are used in the first 2 months, then isoniazid (INH)

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and rifampin are used for another 4 months, making treatment a total of 6 months. In HIV-
positive patients who must be treated immediately for their HIV, you cannot use rifampin.
Instead, substitute rifabutin for rifampin and use either the protease inhibitors nelfinavir or
indinavir or the non-nucleoside efavirenz.

1. Vietnamese man comes to the public health clinic for newly diagnosed tuberculosis
(TB). He was started on 4 tuberculosis medications exactly 4 weeks ago. His organism
is sensitive to all 4 TB medications. What do you recommend?
A. Isoniazid and rifampin for a total of 6 months, pyrazinamide for another month,
and stop the ethambutol now
B. Isoniazid, rifampin, and pyrazinamide for 5 more months and stop the ethambutol
now
C. Continue all 4 drugs for 6 total months of therapy
D. Isoniazid and rifampin for a total of 6 months and stop both the pyrazinamide and
ethambutol now

Answer:

Case 2
23-year-old medical student is admitted with a pleural effusion and a PPD that is
positive at 14 mm.

1. What is the single most accurate test?

A. Pleural biopsy
B. Pleural fluid acid fast stain
C. Sputum acid fast stain
D. Adenosine deaminase level
E. PCR of pleural fluid

Answer:

Case 3
2% increase in the yearly rate of positive PPDs is noted at a place of employment.
What is the reason?

A. New case of tuberculosis in the workplace.

B. Test has a booster effect.
C. Batch of PPD is defective.
D. Increased incidence of tuberculosis in the community.

Answer:

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Case 4
1. Mycobacterium marinum is the answer for:
A. Injury occurring in a pool
B. After exposure to water in a fish tank
C. Nosocomial sternal wound infection after coronary artery bypass
D. All of the above

Answer:

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PPD TESTING

Case 1
1. Who should be tested, in general?
A. Acute symptomatic patients
B. General population
C. High risk groups
D. High risk groups if never vaccinated with BCG
E. School children

Answer:

2. What is a positive test?

>5 mm
>10 mm
>15 mm

Answer:

3. What is the “booster effect”?

4. What is treatment for a positive test?

A. Isoniazid (INH) for 6 months
B. INH for 9 months
C. Rifampin for 6 months
D. INH and rifampin for 6 months

Answer:

5. What is the difference between interferon gamma release assays and the PPD?

6. What is the risk of developing TB with a positive test?

A. 1% a year
B. 10% a year
C. 10% in a lifetime
D. 90% in a lifetime

Answer:

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7. What is the effect of previous BCG vaccination on these recommendations?

8. Who should receive anergy testing?

A. Steroid user
B. AIDS patient
C. Immigrant
D. Bone marrow transplant recipient
E. No one

Answer:

All of the following patients are asymptomatic with NORMAL CHEST X-RAYS.

1. 27-year-old resident has 8 mm of induration. Last year’s PPD was negative.

2. 34-year-old nurse, who has never been tested, has a PPD of 14 mm.

3. 20-year-old college student from Wyoming has a PPD of 14 mm.

4. 48-year-old, HIV-positive male inmate has 7 mm of induration.

5. 92-year-old nursing home resident had 3 mm of induration last year and has 14 mm
this year.

6. You are a 24-year-old medical student who has 6 mm of induration. You have been
taking care of a patient with active tuberculosis for the last 3 days.

7. An ancient Egyptian priest is 4,500-years-old and has recently been brought back to life
in the Temple of Dendur at the Metropolitan Museum of Art. A BCG vaccine was given
immediately before his mummification, and he was given a booster just before leaving
Egypt last year. He has never been PPD tested before and now has 17 mm of induration.

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Summary: Case 1
PPD testing is one of the most complex tests in all of medicine. The PPD is not a general screen-
ing test. PPDs should be performed only on populations at an increased risk of tuberculosis, such
as immunocompromised patients (diabetes, chronic renal failure, silicosis, post-gastrectomy),
recent immigrants, health care workers, close contacts of those with TB, and residents of home-
less shelters, chronic care facilities, and prisons. If a patient with none of these risk factors is
tested, then the test is not considered positive unless there is >15 mm of induration. The general
cutoff for a positive test is 10 mm. Use a cutoff of 5 mm if the patient is a close contact of some-
one with TB, is on steroids, has an abnormal chest x-ray consistent with old TB, or is an organ
transplant recipient. Anyone who has not been tested in the last 2 years should undergo two-
stage testing to look for a “booster effect.” This is to make sure that the first test is truly negative.
There is no point in doing a second test if the first test is positive. Isoniazid for 9 months is the
standard therapy for all PPD-positive patients. There is no effect to be taken into consideration if
the patient has had previous BCG vaccination. There is no routine recommendation to perform
anergy panel testing. If a patient has been exposed to drug-resistant TB, an attempt should be
made to give a drug to which the patient’s TB is not resistant. The interferon gamma release assay
is more specific for TB than a PPD. It does not become falsely positive in those who have been
vaccinated with BCG.

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TROPICAL DISEASES AND ANIMAL-BOURNE ILLNESSES

Case 1
43-year-old Haitian woman has nonresolving pneumonia with cough. No
improvement with azithromycin or moxifloxacin. CBC shows eosinophilia. What
is the etiology?

A. Chlamydia pneumoniae
B. Strongyloides
C. Paragonimus westermani
D. Cycloplasma
E. Pneumocystis

Answer:

Case 2
50-year-old pig farmer from Arkansas has fever, muscle pain, and facial edema.
He has hyperlipidemia and is on atorvastatin. He has periorbital edema,
subconjunctival hemorrhages. CBC shows eosinophilia. He has high LDL and high
CPK. Diagnosis?

A. Drug-induced
B. Polymyositis
C. Dermatomyositis
D. Trichinosis
E. Allergic reaction
F. Lymphoma

Answer:

Case 3
Rabbit hunter from Arkansas is admitted with conjunctivitis, adenopathy, myalgia,
and arthralgia, and later develops pneumonia. Has an ulcer on his hand.

1. Diagnosis?

2. Diagnostic test?

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Summary: Case 3
The rabbit hunter from Arkansas gives a classic presentation for tularemia. The organism
passes through the skin in people who have contact with infected rodents and rabbits. The
infected person will have conjunctivitis and an ulcer at the original site of the inoculation.
In addition, later hematogenous spread brings the organism to the lungs. The test for the
organism is serologic. This is because it is difficult to cultivate the organism in vitro as well
as because it is a highly transmissible laboratory hazard.

Tropical Diseases and Animal-Bourne Illnesses Questions

1. After returning from a trip to Africa, a patient undergoes the development of abrupt
fever, severe headache, chills, and severe pain in the back, legs, and bones. The white cell
count and platelets drop with the development of a petechial rash. Most likely diagnosis?
A. Dengue
B. Malaria
C. Lyme
D. Ehrlichia
Answer:

2. A patient without a spleen is bitten by an Ixodes tick and develops hemolysis. There is fever
and shaking chills. The red cells show small ring forms. What is the most likely diagnosis?
A. Malaria
B. Ehrlichia
C. Babesia
D. Lyme
E. Coxiella burnetti
Answer:
What is the treatment?

3. A man from Long Island, New York comes in with fever, chills, and headache. Platelet
count and white count are low. Transaminases are high. He remembers being bitten by
a tick. There are ‘morulae’ visible in the white cells. What is the most likely diagnosis?
A. Coxiella
B. Babesia
C. Lyme
D. Ehrlichia/Anaplasma phagocytophila
Answer:

4. Mexican man on vacation from his job there as a butcher comes in with 2 weeks of
chills, headache, enlarged lymph nodes, and tiredness. He has an enlarged spleen. What
is the most likely diagnosis?
A. Brucellosis
B. Tularemia
C. Plague
D. Salmonella/typhoid
E. Campylobacter
Answer:

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5. A young man is camping in Connecticut. He denies tick bite. He comes for facial palsy.
He has clusters of vesicular eruptions over his external auditory canal. What is the
diagnosis?
A. Lyme
B. Ehrlichia
C. Babesia
D. Varicella
Answer:

6. Patient with splenectomy for ITP gets scratched/injured by a pet dog. The following day
he is hypotensive. What is the organism?
A. Streptococcus
B. Eikenella
C. Pasteurella
D. Capnocytophagia
Answer:

7. Cat bites give you:

8. HIV patient with increased AST/ALT.

CT: liver with cystic spaces filled with blood (peliosis hepatis).
Nodular lesions on extremities. Biopsy: silver stain/Warthin-Starry positive
What is the diagnosis?
What is the organism?

9. Homeless man, living in shelters and on street. Itching. Flea bitten. Has endocarditis
What organism?

10. A man has been inhaling white spores derived from a dead deer found on a highway.
He becomes short of breath. The chest x-ray shows a widened mediastinum. Skin shows
ulceration with dark, black eschar. What is the diagnosis? What is the therapy?
A. Cipro or Doxycycline
B. Cefazolin
C. Amphotericin
D. Pentamidine
Answer:

11. Itchy folliculitis in a person recently using hot tub/Jacuzzi (“Hot tub rash”) What is the
organism?

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FOOD POISONING/INFECTIOUS DIARRHEA

Case 1
At 8:30 a.m. today 28-year-old medical resident had breakfast consisting of eggs,
juice, and a chicken burrito. Last night she had hamburgers at Jack in the Box. At
12:10 p.m. today she begins to have explosive, bloody, diarrheal bowel movements.
She has 3 watery stools before she is able to make it to the bathroom. She has a
fever of 38.8ºC (102ºF) and crampy abdominal pain with flatus and bloating. She
works in a daycare center at the Fulton Fish Market.

1. Most likely organism?

2. What test first?

A. Stool culture
B. Fecal leukocytes
C. Gram stain
D. Ova and parasite exam
E. Sigmoidoscopy

Answer:

3. Most accurate test?

A. Stool culture
B. Fecal leukocytes
C. Gram stain
D. Ova and parasite exam
E. Sigmoidoscopy

Answer:

4. Best therapy?

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If THIS is in the history … Then THIS is the Answer THIS as the Answer THIS as the
and physical … most likely diagnosis most specific test best initial therapy
Chinese food, fried rice

Campers, hikers

Nursery schools

HIV-positive with <50

CD4 cells

Seafood, shellfish, clams,

mussels

Liver disease,
hemoglobinopathy

5. What cause of food poisoning gives symptoms fastest?

Summary: Case 1
The most important feature in diagnosing a patient with food poisoning or infectious
diarrhea is whether or not there is blood in the stool. Blood in the stool tells you that the
case described is secondary to an invasive organism such as Salmonella, Shigellosis, Yersinia,
E. coli, or Campylobacter. No matter what exposure there may be in the history, Bacillus
cereus, Staphylococcus, viruses, Giardiasis, and cryptosporidium do not give blood in the
stool. Crypotsporidia is detected with a modified acid-fast stain. B. cereus and Staphylococcus
should present predominantly with vomiting. If blood is not described in the history, you
can use occult blood or the presence of white cells in the stool to give the same information.
Viruses and the other noninvasive forms of diarrhea do not present with white cells in the
stool.

When a patient has an invasive diarrhea severe enough to warrant antibiotic therapy, cip-
rofloxacin is the best empiric therapy until a specific microbiologic agent can be identified.
Your answer should be ciprofloxacin when the disease is very severe and bacteremia is pos-
sible. Clues to the presence of very severe disease are hypotension, blood in the stool, tachy-
cardia, abdominal pain, and tenesmus. The most accurate test for giardia is a stool ELISA
antigen. This is more accurate than an ova and parasite examination. The treatment for
cryptosporidia is nitazoxanide.

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1. Which indicate, the highest risk of transmission?

A. Hepatitis B surface antigen and e-antibody
B. Hepatitis B core antigen
C. Hepatitis B IgM core antibody
D. Hepatitis B surface antigen and e-antigen

Answer:

2. 56-year-old alcoholic man has been walking along the beach in New Orleans. He has
a history of liver disease and he develops cellulitis of the leg. Which of the following is
most likely to be associated with this patient’s problem?
A. Naegleria
B. Vibrio parahaemolyticus
C. Vibrio vulnificus
D. Strep agalactiae

Answer:

Summary: Case 1
All forms of acute hepatitis have the same initial presentation. You cannot tell which organ-
ism the patient has when there is jaundice, elevated ALT, elevated bilirubin, dark urine, and
light stool. The only way to distinguish the organism is by serology. There is no therapy for
any form of acute hepatitis. Hepatitis C is seen almost exclusively in patients with chronic
hepatitis. Fewer than 20% of patients with hepatitis C ever remember having the acute syn-
drome, which includes jaundice and the other symptoms just described. Chronic hepatitis C
cannot be diagnosed with just the hepatitis C antibody test. You need a hepatitis C viral load
by PCR, and especially a liver biopsy to assess disease activity.

Chronic hepatitis C is treated with a combination of interferon, ribavirin and either telapre-
vir or boceprevir. Ribavirin increases the response to interferon alone from 15% to almost
50% when it is used in combination. Chronic hepatitis B is treated only when there is a posi-
tive hepatitis B e antigen. Chronic hepatitis B is treated with either interferon, lamivudine,
or adefovir.

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POSTEXPOSURE PROPHYLAXIS
Case 1
1. 28-year-old physician gets stuck by a needle of a hepatitis B surface antigen–positive
patient. The idiot is a board-certified idiot and has never been vaccinated for hepatitis B.
What is risk of transmission, and what do you do? How would this answer change if the
person getting stuck were pregnant?
A. Vaccine A. 0.3%
B. Immunoglobulin B. 3–6%
C. Interferon C. 10–30%
D. Immunoglobulin and vaccination
E. No treatment
Answer:
2. Intern gets stuck on same day by the needle of a hepatitis C antibody–positive patient
who has extremely active disease and is dying of cirrhosis from his chronic hepatitis C.
Risk of transmission? What do you do?
A. Vaccine A. 0.3%
B. Immunoglobulin B. 3–6%
C. Interferon C. 10–30%
D. Immunoglobulin and vaccination
E. No treatment
Answer:
3. 31-year-old resident gets stuck by a needle used on an HIV-positive patient with a high
viral load and low CD4 count. What is the risk of transmission, and what do you do?
A. 0.3%
B. 3–6%
C. 10–30%
Answer:
4. A 32-year-old pregnant nurse has just given a bed-bath to a patient with varicella zos-
ter (shingles). Over 3–4 dermatome. The nurse has never been vaccinated for varicella
zoster. What do you do?
A. Vaccine
B. Immunoglobulin
C. Interferon
D. Immunoglobulin and vaccination
E. No treatment
Answer:
5. What type of isolation should this patient have been on?
A. Droplet
B. Contact
C. Contact and respiratory
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6. A 40-year-old gastroenterology attending physician is having sex in the great outdoors

when he is bitten on the buttocks by a bat, a raccoon, and a skunk. What do you do?
A. Vaccine
B. Immunoglobulin
C. Interferon
D. Immunoglobulin and vaccination
E. No treatment

Answer:

7. Can rats transmit rabies?

8. 72-year-old woman outside working in her garden when she steps on a dirty rake. What
do you do?

Summary: Case 1
In general, postexposure prophylaxis consists of giving disease-specific immunoglobulin, fol-
lowed by vaccination later. This is only for those who have never been vaccinated. Immune
globulin does absolutely nothing for the acute therapy of a disease that has already established
itself; it is only preventive. You are considered to be protected from tetanus for up to 10 years if
the wound you obtain is relatively clean, such as that occurring in a kitchen. If the case describes
a soil- or dirt-contaminated wound, there is a far greater volume of spore delivery and the
injured person is considered protected for only 5 years from the time of the last vaccination. If
previous vaccination with tetanus has occurred, then you give only a booster injection.

1. Pregnant phlebotomist sustains needle-stick with needle positive for hepatitis B surface
antigen. She completed her third injection for hepatitis B vaccine series. Currently, her
hepatitis B surface antibody is not detected. What do you do?
A. Do nothing secondary to the pregnancy
B. Do nothing. She has already received the three vaccine injections.
C. Immune globulin and a vaccine
D. Hepatitis B vaccine booster

Answer:

2. 48-year-old man has undergone splenectomy for persistent severe thrombocytopenia.

What immunizations would you recommend?
A. Varicella
B. Pneumococcus
C. Varicella, Pneumococcus, hepatitis B
D. Pneumococcus, Meningococcus, Haemophilus influenzae
E. Pneumococcus and influenza

Answer:
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MALARIA

Case 1
1. 28-year-old resident is planning a trip to India. What do you recommend as prophylaxis
for malaria?

2. He forgets to take the medication you prescribed, and has a fever with a blood smear
positive for malaria. Most effective therapy for him?

3. What malaria drug is avoided in heart block?

Summary: Case 2
The majority of the world’s malaria is now resistant to chloroquine. Hence, the standard of
care for most travelers is mefloquine. This therapy should start the week before travel and
continue for 4 weeks after leaving the endemic area. There is a small but significant risk of
neuropsychiatric manifestations, and therefore this drug should be used with caution in
those with a history of seizures or psychiatric disorders. The treatment for malaria is with
oral quinine and doxycycline in the majority of cases.

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SEXUALLY TRANSMITTED DISEASES

Case 1
42-year-old man comes to clinic with several days of urinary urgency, burning, and
frequency. He has a urethral discharge.

1. Diagnosis?

2. Best initial and most accurate diagnostic tests?

Urethral swab or voided urine for PCR DNA genetic (nucleic acid amplification) testing is
the most accurate test for gonorrhea and chlamydia.

3. Best initial therapy?

One drug for chlamydia (azithromycin or doxycycline) and one drug for gonorrhea (cef-
triaxone). All of these are given as a single dose except for the doxycycline, which is taken
twice a day for a week.

4. How would your answers differ if patient were pregnant?

5. What will you test for if the patient continues to have recurrent Neisseria infections,
particularly bacteremia?

Summary: Case 1
Both cystitis and urethritis can present with symptoms of dysuria such as frequency, burning,
and urgency. Urethritis gives a urethral discharge, and cystitis doesn’t. In addition, cystitis can
give suprapubic tenderness, and urethritis doesn’t. It is impossible to distinguish chlamydia
from Neisseria by symptoms alone. The urethral swab for a gonorrhea culture and DNA gene
probe are the most accurate diagnostic tests. Treatment is with one agent for the gonorrhea
and one agent for the chlamydia. Ligase chain reaction testing on the urine is extremely sensi-
tive and specific for chlamydia infection. Single-dose therapy with ceftriaxone or cefixime is
used for the gonorrhea. Single-dose oral fluoroquinolones such as ciprofloxacin are no longer
recommended, secondary to the development of resistance. Single-dose therapy with azithro-
mycin has the same efficacy as a week of doxycycline. Patients with a deficiency of terminal
complement are at risk of recurrent Neisseria infections.

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1. 16-year-old girl for treatment of combined gonococcal and chlamydial cervicitis. She is
not accompanied by her parents. What should you do?
A. Do not treat unless her parents are notified
B. Make a “good faith” effort to contact the parents; if you cannot find them, treat her
anyway.
C. Refuse to administer therapy unless she is physically accompanied by her parents.
D. Treat her without parental consent, as if she were an adult

Answer:

2. Young man with yellowish urethral discharge is found to have a Gram stain positive
for multiple neutrophils with intracellular gram-negative diplococci. He is treated with
ceftriaxone. He returns in 7 days and has persistence of the urethral discharge. What is
the most likely cause?
A. Resistant gonorrhea
B. Re-infection with gonorrhea
C. Chlamydia trachomatis
D. Ureaplasma urealyticum

Answer:

3. Man comes for follow-up of chlamydia treatment. He took a gram of azithromycin in

your office a week ago. He has no symptoms. PCR of the urethra done today on the
return visit is positive for chlamydia. What do you tell him?
A. He must be retreated with azithromycin.
B. He must be treated for a longer period of time with doxycycline.
C. His girlfriend re-infected him.
D. “Don’t worry. Do nothing. You shouldn’t have done the repeat test.”
E. It is punishment for his sins.

Answer:

4. Which of the following it NOT an accurate method of diagnosis?

A. Urine nucleic acid amplification test (NAAT) for urethritis in men
B. Self administered swab for cervicitis in women
C. Gram stain of urethral discharge in men
D. Blood Chlamydia testing for PID in women

Answer:

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BACTERIAL VAGINOSIS AND TRICHOMONIASIS

Case 2
32-year-old woman comes to see you because of an uncomfortable vaginal
discharge.

1. Best initial test?

Wet mount and KOH preparation

What is the best test You should answer …And THIS is the How would the
for this disorder? THIS diagnosis… treatment treatment differ in
pregnancy?
Clue cells Bacterial vaginosis Metronidazole or
clindamycin
Motile forms on wet Trichomoniasis Metronidazole
mount
Fungal hyphae on Fungal vaginitis Any topical
KOH antifungal or oral
fluconazole

Summary: Case 2
It is very difficult to determine a specific organism from signs and symptoms of vaginal dis-
charge alone. Bacterial vaginosis with an overgrowth of Gardnerella is the most likely to have
a fishy odor, but 30% of patients have two infections. Never culture the vagina. A wet mount
can identify clue cells for bacterial vaginosis and motile ciliated forms for Trichomoniasis.
Fungal hyphae become visible on a potassium hydroxide (KOH) test in fungal vaginitis.
Bacterial vaginosis is treated with metronidazole in general, or clindamycin in pregnancy.
Metronidazole is safe in the first trimester of pregnancy. Local antifungal agents such as
clotrimazole, miconazole, nystatin, butoconazole, and terconazole can be used in fungal
vaginitis, including during pregnancy. There is no good alternative to metronidazole for
trichomoniasis. It is okay to use metronidazole for trichomonas in pregnancy.

1. Which is most likely to be associated with a low vaginal pH?

A. Bacterial vaginosis
B. Trichomoniasis
C. Fungal vaginitis

Answer:

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2. 19-year-old sexually active woman comes to clinic with mild vaginal discharge. No pain
or itching. KOH and wet mount are normal. pH is 4.0. What do you do?
A. Colposcopy
B. Vaginal culture
C. Prescribe metronidazole
D. Prescribe fluconazole
E. Observation

Answer:

Case 3
32-year-old woman comes to your clinic. She has recurrent vaginal yeast infections
and has been given topical antifungal treatment five times over the past year.

1. Best method of preventing further recurrences?

Summary
In patients with four or more recurrences of fungal vaginitis per year, consideration should
be given to administering chronic suppressive therapy. The best option is to use oral flucon-
azole on a weekly basis.

Case 4
27-year-old sexually active woman comes with bilateral lower abdominal pain.
Temperature of 38.8ºC (102ºF) orally and a WBC count of 17,000. She has cervical
motion tenderness and adnexal tenderness.

1. Diagnosis?
Pelvic inflammatory disease

2. Most accurate diagnostic test?

A. Ultrasound
B. CT scan
C. Cervical culture
D. HCG
E. Laparoscopy

Answer:

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3. Best initial diagnostic test?

A. Ultrasound
B. HCG
C. Cervical culture

Answer:

4. How will you determine whether she has to be admitted?

5. Best therapy?

Outpatient Therapy Inpatient Therapy

Ceftriaxone and doxycycline Cefoxitin (or cefotetan) and doxycycline
Ofloxacin (or levofloxacin) and Penicillin-allergic clindamycin and
metronidazole gentamicin

Summary: Case 4
Any woman with lower abdominal pain, particularly when combined with adnexal tender-
ness, should be considered to have an ectopic pregnancy until it is excluded. Cervical motion
tenderness can also occur when there is an ectopic pregnancy. This is why a urine pregnancy
test is considered the “best initial step” in the management of the patient described in this
case. Laparoscopy is the most accurate diagnostic test for pelvic inflammatory disease (PID),
even though it is rarely necessary. As a matter of test-taking strategy, a question asking
“what is the most accurate test?” is not the same thing as asking “what will you DO next?”
Laparoscopy is performed in those who have numerous recurrences of PID, or those in
whom the diagnosis is indeterminate. The presence of lower abdominal pain, tenderness,
and cervical motion tenderness virtually assures a diagnosis of PID. These findings are even
more important than finding a fever. The treatment of PID is similar to the treatment of ure-
thritis in that you want to give one drug for gonorrhea and one drug for chlamydia. Severely
penicillin-allergic patients should be treated with clindamycin and gentamicin.

1. A woman comes to the clinic for advice. Her sexual partner has just been treated for
gonorrhea. She has no symptoms. What will you recommend?
A. Cervical DNA probe for gonorrhea, and treat only if it is positive
B. Ceftriaxone and azithromycin
C. Ceftriaxone
D. Azithromycin
E. Doxycycline

Answer:

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Case 5
37-year old man comes to clinic with several days of a genital ulcer and inguinal
adenopathy.

If THIS is in the history and …Then THIS is the Answer THIS as the Answer THIS as the
physical… most likely diagnosis best initial and most best initial therapy
specific tests
Vesicles Herpes simplex

Soft and painful chancre

Prominent, huge, tender

lymph nodes; painless lesion

Firm and painless chancre Syphilis

Terrible looking like cancer Granuloma inguinale

Summary: Case 5
A genital ulcer with adenopathy is a relatively nonspecific presentation and could occur with
any STD except gonorrhea. In real patients you often cannot determine a specific diagnosis,
but on the Boards, if they want you to answer “which of the following is the most likely diag-
nosis?” they have to give you a clue. Syphilis is more often nontender and firm (or indurated).
The Venereal Disease Research Laboratory test/rapid plasma reagin (VDRL/RPR) has only a
65–75% sensitivity in primary syphilis. The darkfield exam is more sensitive in early-stage
disease. Chancroid is soft and painful. It is diagnosed with a smear looking for pleomorphic
gram-negative bacilli, and most specifically confirmed with culture on media specific for
Haemophilus ducreyi. The Tzanck preparation is relatively nonspecific, and the presence of
multinucleated giant cells can be found in any form of herpes family virus infection. Viral
culture for herpes simplex will grow in as little as 2 days. Acyclovir, famciclovir, and valacy-
clovir are all equally effective for both herpes simplex and herpes zoster. Because lympho-
granuloma venereum is caused by Chlamydia trachomatis, it is diagnosed by serologic tests on
the blood or on aspiration of the buboes. As with many forms of chlamydial and rickettsial
disease, it is treated with doxycycline.

Granuloma inguinale is caused by Klebsiella granulomatis. The lesions can look like cancer.
Dark staining “Donovan bodies” are seen on biopsy or crush prep of tissue. These lesions are
usually painless. Treat with doxycycline, Azithromycin or TMP/SMZ.

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1. A 25-year-old sexually active woman comes to the clinic with second episode of cer-
vicitis found to be from chlamydia. She had a similar episode 2 months ago that was
definitively identified as being secondary to chlamydia. She received treatment with
azithromycin and ceftriaxone at the time, and symptoms resolved for several weeks. She
has a single sexual partner. What is the most likely reason for her problem?
A. Inadequate dose of the azithromycin
B. Inadequate duration of therapy
C. Occult promiscuity
D. Her partner was not treated, and he re-infected her.
E. HIV infection in the patient

Answer:

2. A 33-year-old man presents with genital lesions for one week following an episode
of unprotected sex. You find two small non-tender ulcers of 1 cm diameter on the
penile shaft. There is bilateral tender inguinal adenopathy. Organism?
A. Herpes simplex
B. Treponema pallidum
C. Chlamydia
D. Hemophilus ducreyi

Answer:

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 Infectious Diseases

SYPHILIS

Case 6
32-year-old man in clinic for rash consistent with secondary syphilis. He receives
intramuscular injection of benzathine penicillin. Four hours later he returns with
fever, myalgias, and a headache.

1. Diagnosis?

2. What will you do?

A. Just reassurance
B. Aspirin
C. Recheck VDRL and RPR
D. Switch to doxycycline

Answer:

Stage Clinical Manifestations Tests Treatment (and if Note

penicillin-allergic)
Syphilis can cause elevated
Primary syphilis Chancre Darkfield, LFTs.
VDRL/RPR,
FTA-ABS
Secondary syphilis Skin lesions, rash, VDRL/RPR,
condylomata lata, mucous FTA-ABS
patches, alopecia
(rarely hepatitis, meningitis,
nephrotic syndrome)
Tertiary syphilis Neurologic, aortitis VDRL/RPR,
FTA

Summary: Case 6
Primary syphilis is characterized by ulcerative genital lesions and is treated with a single intra-
muscular dose of penicillin. Penicillin-allergic patients are treated with doxycycline. Secondary
syphilis is characterized by various forms of skin lesions such as a rash, condylomata lata,
mucous patches, and alopecia. The treatment is the same as that for primary syphilis. Tertiary
syphilis is essentially only a neurological disease in the United States. Gummas and aortitis are
quite rare. The treatment is with intravenous penicillin. The Jarisch-Herxheimer reaction is
not dangerous and is managed with just aspirin as a mild anti-inflammatory agent.

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Case 7
Panicked couple comes because of a positive RPR at a titer of 1:4 found on testing
by the state prior to marriage. MHA-TP is negative.
1. What is your response?
A. Treat them for syphilis
B. Tell them they may have been exposed
C. Repeat the test
D. Do nothing and tell them to get married
E. It is tertiary syphilis.
Answer:

Summary: Case 7
Routine syphilis testing prior to marriage has been discontinued in many states because of
a markedly decreased incidence of syphilis. Hence, with a very low pre-test probability of
disease, the majority of positive VDRL and RPR tests are false-positives. False-positive tests
generally occur with titers that are <1:8. The specific treponemal tests such as the MHA-TP
and FTA-ABS are negative in these cases, excluding the disease.

1. Man comes to clinic with painless penile ulcer with heaped-up, indurated edges. There
is inguinal adenopathy, which is painless and nontender. Serum RPR is nonreactive.
Most likely etiology?
A. Herpes
B. Syphilis
C. Chancroid
D. Chlamydia
E. Behçet syndrome
Answer:

2. A man presents to the emergency department with painful, necrotic penile ulcers with
tender lymph nodes. On Gram stain, you see gram-negative bacilli with a “boxcar-like”
or “school-of-fish” appearance. Which is the most likely organism?
A. Anthrax
B. Chlamydia trachomatis
C. Haemophilus ducreyi
D. Calymmatobacterium granulomatis
Answer:

3. Which of the following is the most sensitive test of CSF for Neurosyphilis?
A. Protein
B. WBC count
C. VDRL
D. RPR
E. FTA
Answer:
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 Infectious Diseases

WARTS

Case 8
35-year-old woman comes to see you with warts on her vulva. The lesions are
firm, nonvesicular, non–fluid filled, not pustular, and not tender.

1. Diagnostic test?

2. Treatment?
• Removal
• Freeze, burn, cut, laser, shave, and melt with podofilox or trichloroacetic acid
• Gradually remove with imiquimod

Summary: Case 8
There are several different types of warts that are managed in basically the same way. The diagno-
sis does not need stains, cultures, biopsies, serology, or smears. The management is the same for
molluscum contagiosum, common verrucous warts, and condylomata acuminata. You remove
the superficial accumulation of keratinized epithelium by various mechanical methods.

Case 9
A 17-year-old boy with itchy lesions at his elbows and his hands. Short, thin trails
or burrows are visible in the skin folds.

1. Diagnosis?

2. Test?

3. Therapy?

Summary: Case 9
Both scabies and crabs, or pediculosis, are treated with permethrin or lindane. You do not need
to do a scraping to diagnose crabs, however, because the bugs are larger and are on the surface.

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URINARY INFECTIONS

Case 1
27-year-old woman with 2 days of urinary frequency, urgency, and burning.
Dipstick in the office shows 3+ leukocytes and positive nitrites.

1. What to do next?
A. Repeat urinalysis
B. Wait for culture results
C. Renal ultrasound
D. Trimethoprim/sulfamethoxazole (TMP/SMX) for 3 days
E. TMP/SMX for 7 days

Answer:

2. What would you differently if she had a fever and flank tenderness?
Extend the length of therapy to 10–14 days with the same drugs

3. When will you give 7 days of therapy?

4. How does prostatitis change your management?

Extend the length of therapy to 2 weeks for acute prostatitis

5. Which of the following should be treated for asymptomatic bacteriuria?

A. Catheterized patients
B. Elderly patients
C. Pregnant patients
D. Diabetics
E. Incontinent patients

Answer:

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 Infectious Diseases

Summary: Case 1
Patients with recurrent urinary tract infections or more than three episodes a year of cys-
titis should be considered for chronic suppressive therapy with TMP/SMZ. A single tablet
of TMP/SMZ can also be tried postcoitally. If a patient has clear symptoms of dysuria and
white cells in the urine, a urine culture is not necessary for an uncomplicated cystitis and you
should answer TMP/SMZ or ciprofloxacin as the “best next step in management.”

The presence of pyelonephritis is determined primarily by the presence of flank pain and ten-
derness. The same organisms that cause cystitis also cause pyelonephritis, so the therapy just
has to be lengthened—although there are additional choices of medications for gram-negative
bacilli that can easily be given intravenously, such as ampicillin/gentamicin, third-generation
cephalosporins, or aztreonam. All beta-lactam antibiotics are safe in pregnancy. These are the
penicillins, cephalosporins, carbapenems, and aztreonam. Erythromycin and azithromycin are
also safe for use in pregnancy, as is nitrofurantoin. Drugs that are dangerous in pregnancy are
quinolones, doxycycline, and metronidazole in the first trimester.

1. 34-week-pregnant woman is seen in the office with cystitis with a pan-sensitive

E. coli. Most appropriate management for her?
A. Ciprofloxacin
B. TMP/SMX
C. Amoxicillin
D. No treatment is necessary
Answer:

2. Which of following is most likely to have Staphylococcus saprophyticus?

A. Catheterized patients
B. Young healthy, sexually active women
C. Diabetics
Answer:

3. For Uncomplicated Cystitis, which of these is NOT a good choice?

A. Nitrofurantoin
B. TMP/SMZ
C. Fosfomycin
D. Erythromycin
Answer:

Case 2
78-year-old woman admitted to intensive care unit. Urine culture shows
vancomycin-resistant enterococcus.
1. Which is the most appropriate precaution to take?
A. Hand-washing by personnel
B. Private room with contact isolation
C. Private room with airborne isolation
D. Semiprivate room with negative pressure

Answer:
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 IM Board Review Course Book

Case 3
You are asked to evaluate a 63-year-old woman who is planned for elective
cholecystectomy. Urine sample grows yeast. She has no symptoms. Most
appropriate management?

Note A. Amphotericin bladder wash

Treat asymptomatic bacteruria B. Fluconazole orally
with urologic procedures C. Cancel the surgery
(cystoscopy). D. Proceed with the surgery

Answer:

FOURNIER GANGRENE

Case 4
60-year-old diabetic man has recently undergone a vasectomy. He develops pain
in his groin, with a lesion at the base of the penis. Crepitus is present?

1. What the therapy?

A. Zosyn (piperacillin/tazobactam)
B. Clindamycin
C. Gentamicin
D. Metronidazole
E. Cefazolin

Answer:

2. Patient comes with Fournier gangrene and his surgeon is out of state. What would
you do?
A. Topical and oral antibiotics
B. Hospitalize for IV antibiotics and consult with another surgeon
C. Hospitalize for observation
D. Prescribe antibiotics and refer the patient to his surgeon when he returns from
vacation

Answer:

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 Infectious Diseases

Summary: Case 4
Fournier gangrene is a mixed infection of the perineum that happens more often in dia-
betic men. It is a mix of streptococci, anaerobes, and gram-negative bacilli. Urgent surgical
debridement is essential in addition to the use of antibiotics. Clindamycin does not cover aer-
obic gram-negative rods. Gentamicin will not cover streptococci or anaerobes. Metronidazole
is predominantly for anaerobic gram-negative bacilli. Cefazolin will not cover anaerobes.

1. 24-year-old college student comes with acute right testicular pain. While on vacation
in San Francisco, he had numerous sexual contacts. Right testicle is enlarged and very
tender. Most likely organism involved?
A. Enterobacter
B. Chlamydia
C. Ureaplasma urealyticum
D. Mycoplasma hominis
E. E. coli
Answer:

Epididymitis: You will recognize the diagnosis of epididymitis when the case gives a chief
complaint of “testicular pain.” There will also be swelling and tenderness. There are two basic
questions generally found on the Board exam: they ask for the causative organism and/or the
treatment. In patients younger than 35 years of age the answers are, respectively, Chlamydia,
and to give the same treatment that you would for urethritis—i.e., either ceftriaxone com-
bined with either azithromycin, or doxycycline. Ofloxacin alone is an alternative to the com-
bination. In patients over 35 the answers are E. coli as the most common organism, and either
TMP/SMZ or ciprofloxacin as the treatment. If the question asks for the diagnosis, remember
that testicular torsion does not give fever, and is very sudden. The pain of epididymitis is
relieved by slightly elevating the testicle, however the testicle is riding high all the time with a
torsion. In addition, the cremasteric reflex is always absent with torsion.

Case 5
You are treating Fournier gangrene and blood cultures grow enterococci. Which of
these will NOT cover enterococci?
A. Ampicillin and Gentamicin
B. Vancomycin
C. Piperacillin/Tazobactam
D. Linezolid
E. Ceftriaxone
Answer:

Case 6
What it the BK virus most often associated with?
A. Pneumonia
B. Encephalitis
C. Overwhelming urge to eat hamburgers
D. Renal transplant rejection
Answer:
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 IM Board Review Course Book

BONE AND JOINT INFECTIONS

Case 1
70-year-old man with a history of peripheral arterial disease and diabetes is
admitted to the hospital with an ulceration on the distal tibia for last 5 days. He
has pain and swelling of the lower extremity around the area of the ulcer. Afebrile.
There is a small amount of necrotic material in the ulcer and a draining sinus tract
with a small amount of purulent material coming out of the draining sinus. Tibia
is red and tender.

1. What test(s) would you do first?

A. X-ray
B. CT scan
C. MRI
D. Swab the draining sinus tract for culture
E. ESR
F. Nuclear bone scan
G. Biopsy

Answer:

2. Most accurate test?

A. Biopsy
B. CT scan
C. MRI
D. Swab the draining sinus tract
E. ESR
F. Nuclear bone scan

Answer:

3. Therapy?

4. When will your answer be ESR?

5. When will your answer be to culture the draining sinus tract?

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 Infectious Diseases

Summary: Case 1
The majority of cases of osteomyelitis in adults spread from a contiguous infection of the
skin, such as an ulceration in diabetic patients and in people with peripheral vascular disease.
Culturing the ulcer is never sufficiently accurate to allow prolonged therapy to be guided
by the results. The ulcers are often colonized, as are draining sinus tracts. Therapy should
always be guided by a bone biopsy. Although Staphylococcus aureus is still the most common
organism, you cannot tell if it is sensitive to oxacillin or nafcillin, or whether the patient
needs therapy with vancomycin or linezolid for methicillin-resistant Staphylococcus aureus
(MRSA). In addition, diabetics are predisposed to gram-negative bacilli such as E. coli. Gram-
negative osteomyelitis can also be treated with oral ciprofloxacin, and you cannot tell what
the patient has for sure until you have bone culture results.

Case 2
59-year-old Catholic nun is admitted to the hospital with swelling of her right
knee for the last week. She has a temperature of 38.8ºC (102ºF ). The knee is red,
swollen, and tender, with decreased mobility.

1. What test(s) first?

A. X-ray of joint
B. Arthrocentesis
C. MRI of joint
D. Arthroscopy

Answer:

2. Most accurate diagnostic test?

A. Gram stain
B. Crystal analysis
C. Protein level
D. Cell count
E. Culture of synovial fluid
F. Blood cultures

Answer:

3. Best therapy?

4. How do you know when to culture multiple sites, such as the cervix, urethra, rectum,
and throat, looking for disseminated gonorrhea?
A. Sexual history
B. HIV testing
C. History of syphilis in the past
D. Polyarticular involvement, rash, and tenosynovitis

Answer:
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Summary: Case 2
Septic arthritis from any cause presents with a swollen, tender, immobile, and warm joint
with the presence of an effusion. In all cases, the arthrocentesis is the single most accurate
test. The difficulty is in the diagnosis of disseminated gonorrhea. Although the arthrocentesis
is still the single most accurate test, an elevated white cell count above 30,000 to 50,000 can
be found in all causes of septic arthritis. When you combine culture of the throat, urethra,
rectum, blood, and cervix for gonorrhea, you actually get a greater yield than for culture of
the joint alone. The key is how to recognize the person with suspected disseminated gonor-
rhea who needs all of these cultures. Disseminated gonorrhea is more often associated with
migratory polyarthralgias, a petechial rash, and inflammation of the tendon sheaths of the
hands and feet (known as tenosynovitis). The treatment of disseminated gonorrhea is with
ceftriaxone, which does not reliably cover staphylococcus. The most common organism in
septic arthritis, when gonorrhea is not the causative organism, is Staphylococcus.

1. A landscaper, who is a part-time rose gardener, comes for evaluation of a skin lesion.
She has a painless nodule on the wrist with an enlarged lymph node in her axilla. Most
appropriate therapy?
A. Saturated solution of potassium iodide
B. Itraconazole
C. Amphotericin
D. Fluconazole
E. Surgery

Answer:

2. Your own cat at home has scratched you on the hand. Today you are developing swell-
ing of the hand. What is the most likely organism?
A. Pasturella multocida
B. Capnocytophagia canimorsus (DF2)
C. Bartonella henselae
D. Bartonella quintana
E. Eikenella corrodens

Answer:

3. A 42-year-old man gets involved in a fistfight at a bar. He strikes several people in the
mouth and sustains several lacerations to his hand. Which is the most appropriate
antibiotic choice?
A. Trimethoprim–sulfamethoxazole
B. Amoxicillin
C. Ciprofloxacin
D. Doxycycline
E. Cefadroxyl
F. Amoxicillin/clavulanic acid

Answer:

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 Infectious Diseases

LYME DISEASE

Case 1
48-year-old man has recently been camping in woods of New England. Now
comes to see you because of a 5-cm rash on his back. Lesion is oval in shape, with
an erythematous border and a pale center. He denies a tick bite.

1. What would you do next?

A. Reassurance
B. Serology (Western blot and ELISA)
C. PCR for Borrelia burgdorferi
D. Oral doxycycline

Answer:

Case 2
Man comes to visit his friends in Connecticut and finds a tick attached to his ankles.
No symptoms. He just got off a plane from Los Angeles 2 hours before coming
to see you.

1. What will you do?

A. Reassurance
B. Serology (Western blot and ELISA)
C. PCR for Borrelia burgdorferi
D. Oral doxycycline

Answer:

2. How do you remove the tick, and should you send it for analysis?

Summary: Cases 1–2

The diagnosis of Lyme disease is based on the presence of clinical manifestations and is sup-
ported by a positive serologic test. When there is a characteristic erythema migrans rash—which
should be at least 5 cm in diameter, red on the outside, and pale on the inside—then the diag-
nosis is sufficiently confirmed in order for you to answer doxycycline or amoxicillin as the best
next step in management. In other words, a classic target rash is more important than a positive
ELISA or Western blot. There is still no conclusive proof that there is efficacy in treating patients
who have sustained an asymptomatic tick bite. No further therapy is required in the case of an
asymptomatic tick bite. In addition, the tick must be attached for at least 24 hours in order to

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transmit the disease Because the diagnosis of Lyme is based more on clinical manifestations than
on a positive serologic test, there is no point in doing serology on an asymptomatic tick bite.
Serology is useful when there are manifestations such as a seventh cranial-nerve palsy, menin-
gitis, or joint pains that could have other etiologies. The rash, seventh cranial-nerve palsy, and
joint pains are best treated with oral doxycycline or amoxicillin. More serious manifestations,
such as meningitis, pericarditis, and heart block, should be treated with intravenous ceftriaxone.

Case 3
68-year-old man has a bilateral seventh cranial-nerve palsy. He denies a tick bite
but does spend every weekend at a country house in Connecticut.

1. What will you do first?

2. With what will you treat the patient?

3. What treatment will be your answer if there is joint involvement as well?

Summary: Case 3
Tick-borne illnesses: The other tick-borne illnesses that are possible but less likely as a source
of questions are Rocky Mountain spotted fever, Babesiosis, and Ehrlichia. Rocky Mountain
spotted fever presents with fever, headache, and myalgias for 3–4 days before the onset of
a petechial rash on the wrists and ankles that moves toward the trunk. It is diagnosed with
serology and treated with doxycycline. Babesiosis and Ehrlichia are carried by the same
Ixodes tick that carries Lyme disease and therefore have the same geographic distribution.
Babesiosis occurs more frequently in the patient described as having had a splenectomy in
the past. There is hemolysis like that in a patient with malaria, but milder, and the patient
will be described as having been camping near Long Island or Massachusetts with no travel
outside the United States. The diagnosis is made by finding characteristic ring forms in the
red cell. Babesiosis is treated with quinine plus clindamycin or atovaquone plus azithromycin.
Ehrlichia rarely causes a rash and does not produce hemolysis. Answer Ehrlichia if the ques-
tion gives you a Long Island camper with low platelets and a low white count with elevated
transaminases. Ehrlichia is diagnosed by seeing inclusions in white cells on a smear, and is
confirmed by serology. Ehrlichia is treated with doxycycline.

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Case 4
A 24-year-old medical student is walking the halls with an IV in her arm. “What’s
it for?” you ask. “Six weeks of IV ceftriaxone. I finished four weeks already. I took
three months of Doxycycline earlier this year for chronic lyme disease but I still
have joint pain”

1. What do you tell her?

A. Continue ceftriaxone
B. Go back to oral doxycycline
C. Stop antibiotics

Answer:

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 IM Board Review Course Book

FEVER AND NEUTROPENIA

Case 1
47-year-old woman is admitted to the hospital after having recently undergone
combination chemotherapy for lymphoma. Her temperature is 38ºC (100.3ºF)
repeatedly, and the absolute neutrophil count is <500.

1. Which of the following is NOT appropriate for initial therapy for this patient?
A. Ceftriaxone
B. Cefepime
C. Imipenem
D. Piperacillin/tazobactam and gentamicin
E. Meropenem

Answer:

2. What do you do if she is still febrile after 3 days?

3. 55-year-old man is admitted for fever and chills after chemotherapy for lymphoma. He
was placed on cefepime and had vancomycin added after three days of persistent fever.
Now, several days later, he is still febrile. What should you change in therapy?
A. Add gentamicin
B. Switch both antibiotics to meropenem
C. Add caspofungin
D. Add metronidazole
E. Add amphotericin

Answer:

Case 2
A 58-year-old man with neutropenia and persistent fever despite the use of cefepime
has been started on vancomycin. He develops flushing and generalized pruritus.

1. What is your next step in management?

A. Stop the vancomycin
B. Switch the vancomycin to nafcillin
C. Add hydrocortisone
D. Measure urine for 24-hour 5-HIAA
E. Slow the rate of infusion of vancomycin

Answer:
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 Infectious Diseases

2. A 50-year-old man is transferred to the intensive care unit for fever and hypotension.
He has new infiltrates on chest x-ray. His central line is changed over a guidewire. He
markedly improves on ceftriaxone and azithromycin; and the sputum grows pneumo-
coccus. Blood culture shows no growth. The catheter tip culture grows coagulase nega-
tive staphylococcus. What is the best management?
A. Continue the same antibiotics and retain the catheter
B. Same antibiotics but place a new central line at a new site
C. Add vancomycin
D. Add vancomycin and place a new central line.

Answer:

3. A patient had chemotherapy leading to profound neutropenia. As the cell count rises
and recovers, he develops new fevers. Abdominal CT shows new nodules in the liver.
Chest x-ray is normal. What is the diagnosis?
A. Nocardia
B. Aspergillus
C. Hepatosplenic candidiasis

Answer:

4. Which of the following is the best therapy for pulmonary aspergillus with a Halo sign?
A. Amphotericin
B. Itraconazole
C. Voriconazoles
C. Fluconazole

Answer:

Summary: Cases 1–2

Any patient with an absolute neutrophil count <500 and a temperature above 100.3ºF (38ºC)
should have blood cultures drawn and be started on antibiotics. Appropriate choices include
cefepime, ceftazidime, imipenem, or meropenem as single agents, or a combination of ticar-
cillin/clavulanate and an aminoglycoside in combination. Vancomycin does not need to be
automatically included with the initial therapy unless there is mucositis, hypotension, a defi-
nite catheter-related infection, or a clear infection with methicillin-resistant Staphylococcus
aureus. The simple presence of an intravenous central line is not the same thing as saying
there is a definite catheter infection. If the patient is still febrile after 3 days, you should then
add vancomycin. If vancomycin was already part of the original treatment and the fever per-
sists, then you should start antifungal coverage with amphotericin. Voriconazole can be used
as an alternative to amphotericin, but amphotericin still has the best evidence for mortality
benefit. The only reason to use a liposomal formulation of amphotericin is if renal insuf-
ficiency is present.

Chronic granulomatous disease (CGD): CGD is a hereditary disorder of a defect in the ability to
fight off catalase-positive organisms. You will recognize the case because a patient with a normal
white cell count will have recurrent infections with Staphylococcus, Pseudomonas, and Aspergillus.
The case will show extensive inflammation of the lymph nodes, to the point of suppuration.

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Look for recurrent lymphadenitis. It is like a patient who has neutropenia and fever, but the
white count is normal and there are big lymph nodes. The granulomas can even obstruct the gas-
trointestinal tract or urinary system. The diagnostic test is the nitroblue tetrazolium dye test. In
addition to treating acute infections, chronic therapy is with chronic TMP/SMZ and interferon.
Steroids are sometimes used for gastrointestinal or urinary tract obstruction.

Case 3
18-year-old man comes with recurrent episodes of fever and multiple skin
abscesses requiring antibiotics. He has a history of episodes of cervical lymph node
enlargement that has worsened to the point of suppuration and drainage since
childhood. They have grown staphylococcus and pseudomonas. Normal white cell
count. Brother has a similar problem. Most appropriate test for this patient?

A. Serum protein electrophoresis

B. Complement levels
C. HIV testing
D. Skin testing for T cell function
E. Nitroblue tetrazolium testing

Answer:

Summary: Case 3
Toxic shock syndrome (TSS): Originally, most TSS cases were associated with the use of
superabsorbent menstrual tampons, which subsequently have been removed from the mar-
ket. Currently, the most common causes are from infection of surgically placed material,
such as sutures, breast implants, or surgical mesh, or from nasal packing. There is no specific
diagnostic test for TSS. You must recognize the clinical presentation and the potential for
colonized or infected foreign material. Patients present with hypotension, tachycardia, fever,
confusion, and a diffuse rash described as a “sunburn.” The rash occurs from the staphylococ-
cal- or streptococcal-mediated toxin and is followed by sloughing of the skin. In addition, the
toxin affects most organs in the body diffusely and there is elevation of the BUN, creatinine,
and transaminases. Treatment consists of fluid resuscitation; antistaphylococcal and anti-
streptococcal antibiotics such as oxacillin, nafcillin, or cefazolin; and removal of the infected
material that is producing the toxin.

Case 4

Which of the following is NOT true about pseudomonas?

A. Causes malignant otitis externa

B. Endocarditis in injection drug users
C. Sepsis in neutropenia
D. Treated with ertapenem or ampicillin/sulbactam
E. Hot tub folliculitis
F. Causes ecthyma gangrenosum

Answer:

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 Infectious Diseases

CATHETER INFECTIONS, BACTEREMIA AND INFECTION CONTROL

Case 1
You have a patient with ESRD transferred from the dialysis center because of a
temperature of 103F. There is no clear source. The patient is being dialysed through
a temporary large lumen catheter that is tunneled. The tunnel is not red, warm or
tender. Chest x-ray and urinalysis are normal.
What is the next step?
A. Obtain blood culture and treat based on result
B. Draw cultures through catheter and peripherally and start Vancomycin and gentamicin
C. Echocardiogram
D. Oxacillin
Answer:

What is the route of antibiotics in a patient such as this?

A. Peripheral line
B. Current central line
C. Both peripheral and central line
Answer:

Two days later the cultures are confirmed as growing MRSA. Gentamicin is stopped.
This is the third episode this year. After five days of therapy, MRSA is still growing.
The MIC to Vancomycin is >4.
What to do?
A. Increase dose of Vancomycin
B. Switch to linezolid
C. Add gentamicin back
D. Switch to tigecycline
Answer:

Case 2
What is the best way to prevent hospital infection?
A. Handwashing
B. Respiratory isolation
C. Reverse Isolation
Answer:

What is the best way to prevent hospital acquired pneumonia?

A. Rotate antibiotics
B. Stop proton pump inhibitors
C. Elevate head of bed 45 degrees
Answer:
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What is the best therapy for Stenotrophomonas maltophilia?

A. TMP/SMZ
B. Cefazolin
C. Vancomycin
D. Voriconazole

Answer:

1. 34-year-old man was admitted for Pneumocystis pneumonia 4 days ago and was placed
on TMP/SMZ, prednisone, efavirenz, lamivudine, and didanosine. He develops a rap-
idly dropping white cell count. What is the most appropriate management?
A. Switch the TMP/SMZ to pentamidine
B. Bone marrow biopsy
C. Increase the prednisone dose
D. Stop the efavirenz
E. Stop the didanosine

Answer:

2. A 47-year-old man is admitted for PCP and is placed on TMP/SMZ. He develops a high
BUN and hyperkalemia. What is your next step?
A. Change to pentamidine
B. Decrease the dose of TMP/SMZ and give saline
C. Stop the TMP/SMZ
D. Stop the TMP/SMZ and hydrate with saline

Answer:

3. Which of these is most likely a true pathogen on Bronchoalveolar lavage in HIV?

A. Candida
B. Herpes
C. CMV
D. Nocardia

Answer:

4. What is the treatment?

5. Besides the lung, what other organ is most likely to be involved?

A. CNS
B. Skin
C. Kidney

Answer:

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 Infectious Diseases

Any patient with HIV and <200 CD4 cells should be on prophylaxis for Pneumocystis pneu-
monia (PCP) with TMP/SMZ. If the patient is allergic to TMP/SMZ, then oral dapsone or
atovaquone should be used. Aerosolized pentamidine has the worst efficacy with the greatest
number of adverse effects. For active therapy in a sulfa-allergic person, intravenous pentami-
dine should be used. If the patient is severely hypoxic (pO2 <70, or A–a gradient >35), then
oral prednisone should be given for 3 weeks in addition to the TMP/SMZ. Prednisone will
reduce mortality with severe PCP.

Case 1
48-year-old woman with AIDS seen because of blurry vision. She had 35 CD4 cells
and a viral load of 418,000 several months ago and was prescribed zidovudine,
didanosine, and indinavir 3 times a day. Whenever you ask, she always says she is
taking her medications.

1. Diagnosis?

2. What would you do next?

A. Dilated ophthalmologic exam
B. Head CT
C. Serum CMV antibody test
D. Serum PCR for CMV

Answer:

3. Therapy?

4. Most likely complications of therapy?

5. Is there routine prophylaxis? If so, what?

Summary: Case 1
Abnormal vision of any kind in an HIV-positive person with <50 CD4 cells should raise the
suspicion of cytomegalovirus (CMV) retinitis. The diagnosis is confirmed with a dilated oph-
thalmologic examination. Serology is useless because 50% of the general population is always
seropositive for CMV. Treatment for CMV is with intravenous ganciclovir or foscarnet. This
is followed later with an intravitreal implant of ganciclovir and oral valganciclovir. There is
no recommendation for routine primary prophylaxis for CMV.

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Case 2
Man who is HIV-positive several weeks of fevers to 39.4–40.0ºC (103.0–104.0ºF),
fatigue, weight loss, and wasting. He has 23 CD4 cells. On no HIV medications
since diagnosis was made several years ago. Liver and spleen are normal. Chest
x-ray is normal. Hematocrit 30, MCV (mean corpuscular volume) of 85.

1. What is the diagnosis?

2. What would you expect to find on liver function tests?

Elevated alkaline phosphatase and gamma glutamyl transpeptidase (GGTP) with a nor-
mal bilirubin

3. What is the best therapy?

4. Is there routine prophylaxis? If so, what?

Summary: Case 2
Disseminated Mycobacterium avium intracellulare (MAI) presents as a cause of fever and wasting
syndrome in HIV-positive persons with low CD4 cell counts. The least sensitive test, but the one
that is easiest to obtain, is a blood culture for mycobacteria. The liver biopsy is the most accurate
diagnostic test. Treatment for acute MAI is with clarithromycin and ethambutol.

1. A man with HIV has Mycobacterium avium complex (MAC/MAI) growing from his
sputum. The chest x-ray is normal. What is the management?
A. Rifabutin/ethambutol/clarithromycin
B. Clarithromycin/ethambutol
C. Azithromycin
D. None of the above

Answer:

Case 3
52-year-old man is brought to the emergency department because of a seizure.
CT scan of the head reveals a 3-cm, contrast-enhancing lesion of the parietal area.
There is minimal edema and no midline shift. He is HIV-positive but was refusing
all HIV medications prior to the event.

1. Diagnosis?

2. Next step in management of this patient?

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 Infectious Diseases

3. Most specific test?

4. Duration of therapy?

Summary: Case 3
Contrast, or “ring”-enhancing, lesions in the brain found with a CT scan are secondary to
two main groups of diseases: cancers and infections. In patients with HIV and a CD4 count
<50, contrast-enhancing lesions of the brain have a much more limited differential. As much
as 90% of the time, it will be from either lymphoma or toxoplasmosis. The best way to con-
firm the diagnosis at first is to treat empirically with pyrimethamine and sulfadiazine and
repeat the head CT in 2 weeks. If there is no change in the lesion, then you should perform a
brain biopsy to confirm the diagnosis. In HIV-negative persons you are much more likely to
do a brain biopsy at the beginning to guide therapy.

1. An HIV positive patient is asymptomatic with a CD4 count of 600. What infection is he
at risk for?
A. Pneumocystis pneumonia
B. Cytomegalovirus
C. Toxoplasmosis
D. Tuberculosis
E. Mycobacterium avium

Answer:

Case 4

Nucleoside Adverse Non- Adverse Protease Adverse

Reverse Effects Nucleoside Effects Inhibitors Effects
Transcriptase Reverse
Inhibitors Transcriptase
Inhibitors
Zidovudine Efavirenz Nelfinavir

Lamivudine Atazanavir

Stavudine Ritonavir

Didanosine Indinavir

Abacavir Saquinavir

Tenofovir Darunavir

Emtricitabine Amprenavir

Tipranavir

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1. When should antiretroviral medication be started?

2. What should be started?

Additional Cases
1. A 37-year-old woman comes to your office having recently been diagnosed as HIV-
positive. She has been healthy, with no opportunistic infections. Her CD4 count is
395 and her viral load is 7,000 by PCR-RNA measurement. What is appropriate in her
management?

2. A 23-year-old woman has been HIV-positive for several years and is asymptomatic. She
is maintained on zidovudine, lamivudine, and darunavir. Her last CD4 was 385 and her
last viral load was <20 (undetectable) 3 months ago. The total cholesterol was 295 and
triglycerides were 425 at that time. A repeat measurement in your clinic now reveals a
CD4 count of 392 and a viral load that is still undetectable. The lipid levels are essen-
tially the same. What would you do for her?

3. During routine testing in her second month of pregnancy, a 19-year-old woman finds
that she is HIV-positive. Her CD4 count is 42 and her PCR-RNA viral load is 271,000.
What would you do for her?

OR

A 24-year-old, HIV-positive female has been on zidovudine, lamivudine, and nelfinavir

for the last 6 months. Her CD4 has risen from 270 to 450, and the viral load has become
undetectable. She has just learned that she is pregnant. Which of the following is the
most appropriate management?
A. Stop the HIV medications until after the delivery
B. Stop the HIV medications except for zidovudine (AZT)
C. Stop all the medications except the protease inhibitors
D. Continue the same medications

Answer:

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4. A 38-year-old woman who knew she was HIV-positive becomes pregnant and comes to see
you for advice in her third month of pregnancy. She has never used antiretroviral medica-
tions before. Her CD4 is 859 and her viral load is 570. What is appropriate for her?

5. A 34-year-old man comes to see you for management of his antiretroviral medications.
He was started on zidovudine, lamivudine, and efavirenz 4 months ago. There was ini-
tially a decrease in the viral load from 140,000 to 50,000, but now it has risen back to
120,000. How would you best manage this patient?

6. A 29-year-old medical resident gets stuck in the thumb with the needle he just used to
test for a blood gas in an HIV-positive patient. What should you do next?
A. Observe until the results of the resident’s HIV test come back
B. Zidovudine alone
C. Immunoglobulin
D. Zidovudine, lamivudine, nelfinavir

Answer:

Summary: Case 5
The indication to start antiretroviral medications is a CD4 count <500. The primary reason
to start therapy is not based on the viral load. The main idea is to keep the person’s CD4
count above 200 cells so he will not be at risk of developing an opportunistic infection. The
best initial combination is two nucleoside reverse transcriptase inhibitors and a protease
inhibitor or efavirenz. The most common adverse effects of zidovudine (AZT) are bone mar-
row suppression and anemia. You cannot use rifampin in combination with protease inhibi-
tors. If a patient who has tuberculosis requires medications to treat the HIV disease, then use
rifabutin instead of rifampin. You should use either efavirenz or nelfinavir in combination
with antituberculosis medications such as rifabutin. The most common adverse effects of the
protease inhibitors are hyperglycemia and hyperlipidemia. Pregnant women who need the
HIV medications for their own health should be continued or started on therapy as needed.
The only one of the HIV medications that may be teratogenic is efavirenz. All patients with
significant HIV needle-sticks should be given a combination of three antiretrovirals for a
month without waiting for the results of the stuck person’s HIV test.

Pregnant patients with viral load >1,000 on the day of delivery need a C-section.

1. In a patient who has just been started on antiretrovirals for HIV, which of the following
is the first indication of treatment failure?
A. Cough, fever, adenopathy
B. Weight loss
C. CD4 count
D. HIV by PCR viral load

Answer:
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2. A 37-year-old woman with HIV and 395 CD4 cells comes in for routine evaluation.
Which of the following cannot be given to this person?
A. DPT vaccine
B. MMR
C. Hepatitis B
D. Varicella
E. Oral polio

Answer:

3. A man with HIV and 20 CD4 cells has been started on TMP/SMZ, azithromycin, zid-
ovudine, stavudine, and ritonavir/lopinavir. He is admitted for weakness and is found
to have a serum bicarbonate of 12 and an elevated lactic acid level. Which of the follow-
ing should you discontinue?
A. Ritonavir
B. Stavudine and zidovudine
C. TMP/SMZ
D. Azithromycin

Answer:

4. 24-year-old, HIV-positive patient has a CD4 count of 25 with a negative VDRL and a
6mm positive PPD. What is the most appropriate therapy for him?
A. Antiretroviral therapy and isoniazid
B. Antiretroviral therapy and TMP-SMZ and azithromycin and isoniazid
C. Antiretroviral therapy and TMP-SMZ
D. Antiretroviral therapy and azithromycin

Answer:

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 Statistics/
Preventive Medicine/Ethics 12
Test Discrimination

Diseased Disease-Free

Test Positive A = TP B = FP

Test Negative C = FN D = TN

TP = True Positive, FP = False Positive, FN = False Negative, TN = True Negative

UNDERSTANDING 2 × 2 THEORY

Boxes
A: Individuals who are diseased and positive

B: Individuals who are disease-free and positive

C: Individuals who are diseased and negative

D: Individuals who are disease-free and negative

A + C = Total number of diseased individuals

B + D = Total number of disease-free individuals

Sensitivity and Specificity

Sensitivity = A/(A + C) = proportion of diseased individuals labeled as positive by test (abil-
ity to detect when disease present) = true positive rate.

Specificity = D/(B + D) = proportion of the disease-free individuals labeled as negative by the

test (ability to detect when disease not present) = true negative rate.

Positive Predictive Value (PPV): proportion of individuals with a positive test who have the
disease = A/(A + B).

Negative Predictive Value (NPV): proportion of individuals with a negative test who do not
have the disease = D/(C + D).

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Case 1: Application of a New Test

Assume that a new test is applied to a population of 1,000 persons, 500 with and 500 without
the disease . . . 50% prevalence! The results of the studies are that 400 patients with the disease
were correctly identified as having the disease. Unfortunately, 50 healthy persons were inap-
propriately identified as having the disease when no disease was present.

What are the sensitivity, specificity, PPV, and NPV?

Diseased Disease-Free

Test Positive 400 50

Test Negative 100 450

Sensitivity = A/(A + C)
= 400/(400 + 100) = 400/500 = 80%

Specificity = D/(B + D)
= 450/(50 + 450) = 450/500 = 90%

PPV = A/(A + B)
= 400/(400 + 50) = 400/450 = 89%

NPV = D/(C + D)
= 450/(450 + 100) = 450/550 = 82%

Case 2: What If the Prevalence of Disease Is 10%?

Make the 2 × 2
Sensitivity = A/(A + C) = 80/(80 + 20) = 80/100 = 80%

Specificity = D/(B + D) = 810/(90 + 810) = 810/900 = 90%

PPV = A/(A + B) = 80/80 + 90 = 80/170 = 47%

NPV = D/(C + D) = 810/20 + 810 = 810/830 = 97%

Why are the sensitivity and specificity the same? Why did the PPV decrease?
Sensitivity and specificity are not affected by prevalence. However, the prevalence of disease
affects the number of individuals who are falsely labeled as positive and falsely labeled as
negative.

Note: The lower the prevalence of disease, the lower the positive predictive value (PPV).

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SCREENING TESTS AND BIAS

Case 3
A healthy, 22-year-old woman undergoes numerous laboratory tests for a
physical exam. Accidentally, the nurse orders a CA 19-9. The level is over 700
(normal less than 50). The test is repeated and the level is confirmed. A CT is
performed with fine cuts through the pancreas. The CT is normal.

Knowing the sensitivity and specificity for pancreatic cancer of this test to be over 80%, what
is the best next step?
A. The test should be repeated annually
B. Reassure the patient that the test is not accurate in patients such as her
C. Submit a CEA level
D. MRI/MRCP

PPV

0 100 500 1000

CA 19-9

PPV CA19-9 Pancreatic Cancer

Case 4
The University Study Group performed a long-term study on the effect of
prostate-specific antigen (PSA) in decreasing mortality from prostate cancer.
Patients were randomized to two groups. The first group underwent PSA testing
annually. The second group had no PSA screening. After 10 years, more people
in the PSA screening group were found to have survived prostate cancer. The
authors of the study concluded that PSA screening prolongs life due to early
detection of prostate cancer.

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The study’s conclusions may be affected by

A. lead-time bias.
B. confounding variable.
C. length bias.
D. reporting error.
E. beta-error.

Screening for Prostate Cancer

Results in Increased Survival

Time of
Diagnosis with Test Death

Symptoms
or time of diagnosis (no test)

Study conclusion: Life prolonged if test applied

Lead Time Bias

Case 5
An Italian investigator shows that people who drink coffee are more likely to
develop pancreatic cancer. He concludes that coffee consumption is associated
with pancreatic cancer. Reviewing the manuscript, you notice that patients who
consumed coffee also smoked more cigarettes.

The conclusion of the investigator may thus have been affected by

A. lead-time bias.
B. confounding variable.
C. length bias.
D. reporting error.
E. beta-error.

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Case 6
An investigator claims that his study of 32 patients failed to show any benefit of
using thrombolytics in treating patients with myocardial infarction (p = 0.22). The
investigator submits his paper to an academic journal.

The journal editor refuses to accept the paper on the basis of which of the following?
A. Type I error
B. Type II error (beta-error)
C. Confounding variable
D. Lack of significance

Case 7
Which of the following is a true statement?
A. Type I error occurs when an investigator declares a benefit but there is none
B. In an intention-to-treat analysis, the investigators ignore noncompliance and use
all patients in the final analysis
C. A study that uses statistics to combine many randomized trials is referred to as a
meta-analysis
D. All of the above are true

Case 8
In a study to evaluate a drug, 200 patients are randomized equally to two arms;
one receives the drug, the other a placebo. The mortality in the placebo group
is 80%. Mortality is 60% in the drug treatment group.

How many patients need to be treated with the new drug to save one life (i.e., what is the
number needed to treat)?
A. 10
B. 7
C. 5
D. Cannot be calculated

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Case 9
What is the most accurate method of determining the prevalence of a disease in a geographic
area?
A. Physicians’ office records
B. Local incidence over the past month
C. Random selection of persons within a defined population
D. Hospital records

Explanation: Case 9
Sensitivity and specificity are defined by a specific test and refer to accuracy in relationship to
a gold standard. The most important factor in interpreting an abnormal test result in a patient
is the prevalence of the disease in the population being tested. Prevalence is the proportion of
persons in the population who are affected by the disease. Thus, a positive test is more likely to
be true if the disease is widespread than if the disease is rare. The positive and negative predic-
tive values are thus a more important parameter for clinicians. The p value is a way of express-
ing the significance of a study. It measures the probability of the test result being due to chance.
The null hypothesis implies that the results are by chance. The smaller the p value, the greater
the likelihood that the results occurred as a “surprise”—not by chance. Confidence intervals
are another method of determining whether the results occurred by chance. The numbers in
the confidence interval represent the range that results could fall within to be by chance. A
meta-analysis is defined as the quantitative analysis of two or more independent studies to
integrate the findings and describe features of the studies that contribute to variation in their
results. Odds ratios compare the affected population with the unaffected population; this
comparison is expressed as a ratio. The odds ratio gives the odds of having a risk factor if the
condition is present as compared with having a risk factor if the condition is not present.

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ETHICS

Case 1
Colonoscopy reveals that a 78-year-old man is found to have colon cancer. His
children insist that he will “die of a heart attack” if you tell him the diagnosis. They
request that you tell their father nothing. They will arrange surgery.

You should:

Case 2
The HMO that you work for insists that only flexible sigmoidoscopy be
recommended for colon-cancer screening. They insist that you not discuss other
options with patients.

You should:

Case 3
A 44-year-old woman is in a car accident with her 9-year-old child. The family
members are devout Jehovah’s Witnesses. The woman and her child both need
blood due to splenic ruptures, HCT 20 preoperatively. The mother and father
refuse blood for both the mother and child.

You should:

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Case 4
A 55-year-old man developed severe confusion while being treated for AML. His
care decisions have been given to his wife via a “living will” document signed by
the patient, his lawyer, and witnesses. Now, the patient is no longer confused.
Due to excess blasts in the smear, a relapse is suspected. You want to give the
man a trial of chemotherapy with a research protocol. His wife says no. She
instructs you not to discuss the matter with her husband and claims that as his
designated health care proxy, she has the authority to make that decision.

You should:

Questions
1. Which of the following has the highest priority in a patient’s health care management?
A. Physician of record
B. Patient’s spouse
C. Durable power of attorney
D. The hospital’s Ethics Committee

2. A 94-year-old man, who is clearly alert and oriented, presents with lung cancer. The
patient is realistic about his expectations of treatment. He has been living alone at
home, caring for himself. His daughter comes to the hospital and has her father’s health
care proxy. The patient refuses to have chemotherapy and requests comfort care only.
His daughter demands that he receive treatment. You should
A. comply with the patient’s requests without reservation.
B. order a mental health evaluation for the patient.
C. present the case to the hospital’s Ethics Committee.
D. review the proxy document.

3. A 71-year-old patient presents to the ER with respiratory failure. He is intubated and

put on the respirator. He is unconscious and likely to die without mechanical ventila-
tion. Later that evening, his family brings to you his advance directive, which clearly
states that he never wanted to have life-sustaining interventions. What is the most
appropriate next step?
A. Take him off the ventilator
B. Tell the family that it is too late for the living will
C. Obtain an Ethics Committee consult
D. Ask to speak to the lawyer who constructed the document

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4. A 66-year-old man with small cell carcinoma of the lungs and severe COPD refuses
intubation. He is dyspneic, and oxygen/nebulizers are unable to raise his saturation
above 88%. What is the best course of action?
A. Administer morphine
B. Administer meperidine
C. Ask family members for permission to use mechanical ventilation
D. Pressure the patient for a trial of mechanical ventilation

5. A confused patient with severe spinal stenosis that requires surgery is brought to you
by family members for evaluation. The patient’s son has durable power of attorney. The
son and other family members demand that the father be referred to a chiropractor.
What should you do?
A. Comply without reservation
B. Refuse, because such a referral is futile
C. Present the case to the hospital’s Ethics Committee
D. Review the proxy document

6. A 16-year-old girl comes to the office with a thick vaginal discharge. She admits to having
unprotected sex. What is your next step?
A. Contact her parents
B. Examine and treat the patient without parental consent
C. First establish the diagnosis, then discuss it with the parents
D. Ask to speak to the family lawyer

7. A 72-year-old man has had two traffic accidents in the past month because he failed to
stop at a red light and at a stop sign. He denies that the accidents were his fault. He has
a mildly impaired mental status and a slow, broad-based gait. What is your next step?
A. Refer him to an ophthalmologist for possible diagnosis
B. Report the findings to the state’s Department of Motor Vehicles
C. Tell the patient to be more careful
D. Neurology consult

8. A 94-year-old man has colon cancer. He requests that he receive no treatment. How will
you know if he is capable of making that decision?
A. If he can tell you what condition he has and the risks of not treating the tumor
B. If he can recognize family members
C. If he is alert and oriented to place and time
D. If he has a living will

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Summary: Case Studies 1–4

Autonomy requires that you, the physician, discuss all issues with the patient regardless of
family wishes. A competent patient may tell you that she/he does not want that information;
however, the decision belongs to the patient. Informed consent requires complete disclosure
for the benefit of the patient (beneficence). Case 1 clearly requires discussion with the patient
regardless of the family’s concerns. Comforting the family about the extremely low risk of MI
is recommended. Case 2 requires disclosure of all important information, including the best
test available. Case 3 allows the mother to maintain her autonomy, but she does not control
that of the child, who should receive blood. The child’s fate should not be decided on the
basis of a mother’s religious beliefs when life-saving care is needed. Lastly, in Case 4, legal
documents do not supersede informed consent and a patient’s autonomy. The patient must
be allowed to hear the options.

PREVENTATIVE MEDICINE
1. A 48-year-old woman with diabetes should be seen by an ophthalmologist
A. at the time of diagnosis.
B. 1 year after the diagnosis.
C. 5 years after the diagnosis.
D. when symptoms develop.
E. when the primary care physician identifies retinal abnormalities.

2. A 58-year-old man after myocardial infarction with a history of diabetes mellitus,

hypertension, hyperlipidemia, and a history of peptic ulcer disease complicated by a
massive upper GI bleed should
A. take aspirin daily.
B. take only enteric coated aspirin.
C. take only buffered aspirin.
D. take aspirin with lansoprazole.
E. never take aspirin, clopidogrel, or warfarin.

3. A 50-year-old woman presents with concerns of developing ovarian cancer. She wants
to be screened. Although a friend recently developed the disease (Stage III), no family
members have ever had ovarian cancer. You should
A. order a CA 125.
B. order a CA 125 and pelvic ultrasound.
C. instruct the woman to have a CA 125 and pelvic ultrasound annually.
D. instruct the woman to have a CA 125 and pelvic ultrasound at 2-year intervals.
E. explain that there are no accurate screening tests available.

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4. A 61-year-old man with a 40-pack-year history of smoking presents to you for a routine
evaluation. He denies cough, wheezing, and shortness of breath. His lungs are clear.
Aside from explaining to him the importance of discontinuing tobacco use, you should
A. obtain a chest radiograph, PA only.
B. obtain a chest radiograph, PA and lateral.
C. schedule a non-contrast CT of the chest.
D. perform pulmonary function testing.
E. refuse to perform a screening chest radiograph if requested for screening.

5. A 44-year-old man presents for evaluation. He has hepatitis C, and he failed treatment
with pegylated interferon and ribavirin. His spleen is enlarged, and his platelet count
is 77,000. He is currently on no medications. An upper endoscopy report from his
gastroenterologist reveals Grade II esophageal varices. He has no prior GI bleeding. For
primary prophylaxis, you should
A. inform him that if bleeding occurs, a beta blocker such as metoprolol should be
given.
B. if no contraindications, begin nadolol.
C. if no contraindications, begin metoprolol.
D. refer him for liver transplantation evaluation.
E. start him on omeprazole immediately.

6. A 54-year-old woman with mitral valve prolapse presents for discussion regarding the
need for a colonoscopy. On physical examination, there is a Grade II systolic murmur
radiating to the left axilla. You should
A. start her on oral amoxicillin prior to the colonoscopy.
B. give a dose of cefazolin intravenously prior to the colonoscopy.
C. give a dose of vancomycin and gentamicin intravenously prior to the colonoscopy.
D. inform her that only patients with a history of rheumatic heart disease need anti-
biotics for colonoscopy.
E. comfort the patient. Antibiotics are not necessary.

7. The only oral substance shown to prevent breast cancer in persons at increased risk is
A. vitamin E (alpha tocopherol).
B. folic acid.
C. tamoxifen.
D. calcium.
E. aspirin.

8. The most important risk factor for the development of colon cancer and breast cancer is
A. family history.
B. age.
C. diet.
D. history of radiation exposure.

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9. A 55-year-old man presents for discussion of the need for an exercise treadmill test. He
has not seen a physician in 5 years. At work, many of his colleagues have had this test
performed to rule out coronary artery disease. After your evaluation, you find no risk
factors for heart disease and no symptoms. You should
A. perform an exercise treadmill test.
B. obtain an electrocardiogram and serum cholesterol panel.
C. obtain a serum cholesterol panel only.
D. schedule an electron beam CT.
E. schedule a stress thallium.

10. A 50-year-old woman presents for an annual routine evaluation. You should
A. obtain a screening TSH.
B. schedule a mammogram and a screening TSH.
C. schedule a mammogram, and order a TSH only if multiple symptoms develop.
D. schedule a mammogram, and order a TSH if one or more symptoms exist.
E. schedule a mammogram, and order a thyroid panel.

11. Which of the following statements is true regarding vitamins?

A. Vitamin E helps to prevent colon cancer
B. Vitamin A helps to prevent leukemia
C. Calcium helps to prevent lung cancer
D. Folic acid helps to prevent certain birth defects
E. Vitamin C helps to prevent the common cold

12. A 45-year-old man with no significant past medical history presents for a routine evalu-
ation. He has no complaints. There were no prior hospitalizations or surgeries. He takes
no medications, no drug allergies. His family history is remarkable for a father and
mother who had myocardial infarctions before the age of 50. A grandfather had colon
cancer. An uncle had prostate cancer. The patient smokes 1 pack of cigarettes per day
and does not exercise. In addition to a lipid panel, he should be screened for which of
the following diseases?
A. Diabetes
B. Prostate cancer
C. Colon cancer
D. Thyroid disease
E. None

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 Statistics/Preventive Medicine/Ethics

13. A 45-year-old woman presents for discussion regarding the need for a screening mam-
mogram. Which of the following is true?
A. The palpation of the breast is more important than the mammogram itself
B. Palpation of the breast has no role in the setting of the mammogram (the mam-
mogram includes all pathology that would be found on palpation)
C. Self-palpation is more important than physician palpation for screening
D. There is clear evidence that a baseline mammogram should be performed between
ages 35 and 40
E. Women should have mammograms every 1–2 years after the age of 50

14. Which of the following is a risk factor for the development of pancreatic cancer?
A. Alcohol abuse
B. Smoking
C. History of acute pancreatitis
D. Family history of pancreatic cancer
E. History of Hodgkin disease treated with mantle irradiation

15. A 35-year-old woman is found to have hepatitis C, genotype 1B, with elevated trans-
aminases. She should receive which of the following vaccines?
A. Hepatitis B
B. Hepatitis A
C. Pneumococcal
D. Influenza
E. All of the above

16. Cervical cancer screening with a PAP smear is best considered

A. primary prevention.
B. secondary prevention.
C. tertiary prevention.
D. just good medical care.

17. Which of the following statements concerning practice guidelines is correct?

A. Guidelines should be used only for managed care
B. Guidelines protect patients from substandard care
C. Following guidelines decreases malpractice cases
D. Guidelines are based on cost-cutting

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 IM Board Review Course Book

18. A 29-year-old woman with HIV calls you regarding her recent Pap smear, which was
normal. She has had an annual Pap smear for the last three consecutive years. She
should have her next Pap
A. in 1 year.
B. in 2 years.
C. in 3 years.
D. in 4 years.
E. only if she has symptoms.

19. A 55-year-old woman who had right knee replacement one year ago calls you the night
before her colonoscopy. She inquires about the need for antibiotics during the proce-
dure. What do you tell her?
A. The colonoscopy scheduled for the next day should be canceled because the artificial
knee requires 3 days of antibiotics prior to the procedure
B. Vancomycin and gentamicin should be given as single doses during the procedure
C. Cephalexin will be given during the procedure
D. Oral amoxicillin will be given before and after the procedure
E. No antibiotics are needed

20. A 32-year-old obese woman presents to you for advice regarding weight loss. She has
no significant medical problems at this time. She requests a pill to help her lose weight.
You should do which of the following?
A. Tell her that exercise and a low-calorie diet are the best ways to lose weight
B. Prescribe phentermine
C. Prescribe fenfluramine
D. Prescribe L-thyroxine
E. Refer her for gastric bypass surgery

21. Which of the following should be done?

A. DEXA bone scan for anyone above age 65
B. Varicella zoster vaccine above age 65 and aortic ultrasound in men who were ever
smokers
C. Pneumococcal vaccines in everyone above age 50
D. Offer mastectomy if BRCA is positive above age 50.

22. A 45-year-old woman presents with anemia. She has celiac disease. On physical exami-
nation, what finding would be characteristic?
A. Seborrheic dermatitis
B. Atrophic tongue
C. Glossitis
D. Corneal abrasions

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 Statistics/Preventive Medicine/Ethics

23. In a patient who is on long-term TPN, which micronutrient deficiency is associated

with diabetes (glucose intolerance)?
A. Selenium
B. Copper
C. Chromium
D. Zinc

24. A 72-year-old man is admitted to the hospital with a complicated gangrenous cholecys-
titis. After cholecystectomy, he is kept NPO and placed on antibiotics for an extended
period of time. Twelve days later, he develops ecchymosis and mild anemia. Hgb is 10,
PT 16 sec, and PTT 40 sec. What is the most common cause of these findings?
A. Vitamin C deficiency
B. Vitamin K deficiency
C. Factor X deficiency
D. Antiphospholipid syndrome

25. Endocarditis prophylaxis is indicated for a patient in which of the following circum-
stances?
A. Bicuspid valve, undergoing dental cleaning
B. Ostium secundum defect, undergoing vaginal hysterectomy
C. Bioprosthetic valve, undergoing dental extraction
D. Mitral valve prolapse with murmur, undergoing colonoscopy
E. Metal valve with transesophageal echo

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Cardio answers:
Case 1: 1. please see table
1. HTN 2. atropine
2. Diabetes Case 7 continued:
3. Positional, pleuritic, painful 1. A - right ventricular leads
4. D - Atypical pain + EKG normal --> do stress 2. C - ventricular septal rupture
test 3. C - tricuspid regurgitation
5. when can't read EKG (LBBB, LVH, pacemaker) Case 8:
6. when can't exercise 1. D - exercise tolerance test
7. angiogram 2. If ischemia is found, the patient should be
Case 1 continued: offered angiography
1. B - asthma 3. C – RCA 90% or LAD 90% in diabetic
2. yes 4. A - tobacco cessation
Case 2: 5. Wait for 2-6 weeks or if stress is negative
1. A - aspirin - treat first! Case 8 continued:
2. B - statin for all (abnormal stress test - RF's 1. A - ASA, clopi, ACE-I, statins
don't matter = CAD, give statins for any LDL!) 2. A - hepatopulmonary syndrome
3. PAD, DM, aortic stenosis 3. D - cholestyramine
4. most common AE of statins is liver disease 4. C - decrease digoxin and warfarin doses
Case 2 continued: Case 9:
1. B - <100 1. D - steroids and antihistamines
2. D - Fibric acid derivatives 2. C - neurocognitive dysfunction
Case 3: Congestive Heart Failure:
1. C - aspirin LOWERS mortality! Case 1:
2. ASA, tPA, BB/ACE/statin, cath 1. Oxygen, diuretics. nitrogen, morphine
3. can't use BB, prinzmetal's, cocaine use 2. C - dobutamine
4. have arrhythmia now 3. MUGA scan
5. C - myoglobin 4. do the same thing - a creatinine as low as 2.3
6. C - accelerated idioventricular rhythm in particular is not a contraindication to any of
7. C - 3 vessels and >70% these medications
Case 3 continued: 5. When the ACE inhibitors cannot be tolerated
1. D - statins because of adverse effects, such as cough
Case 4: 6. D - EKG
1. A - prasugrel 7. A - ACE-I, BB, spironolactone, diuretic
Case 4 continued: Case 1 continued:
1. B - transfer for angioplasty 1. C - RBBB
Case 5: 2. A - LBBB
1. A - thrombolytics Case 2:
2. A – retreat with thrombolytics 1. CHF
Case 5 continued: 2. C - spironolactone, lisinopril, carvedilol,
1. A - propranolol furosemide
2. A - diltiazem 3. C - EF worsens --> heart transplant
Case 2 continued:
3. A - hypertensive 1. D
Case 6: 2. C - septic shock
1. C - Low molecular weight heparin 3. E – all of the above
2. C - angiography Valvular heart disease:
Case 7: Case 1:
1. mitral stenosis 2. A - AICD
2. echo 1st, cardiac cath best 3. C - HOCM
3. diuretics Case 5:
4. C - opening snap earlier 1. atrial myxoma
5. C - mitral balloon valvotomy now 2. echo
6. B - increased thoracic pressure decreases 3. echo, then surgery to replace/remove
return Congenital cardiac anomalies:
Case 1 continued: Case 1:
1. A - ASD 1. B – can lead to aortic regurgitation
2. B - mitral stenosis (really bad also - 2. A –coarctation
peripartum CM, Eisenmenger's) Case 1 continued:
3. C – wide splitting of S2 1. E - refer for valve replacement
Case 2: 2. A - valve replacement
1. Mitral valve prolapse 3. E - previous peripartum cardiomyopathy
2. Echo 4. B - okay to become pregnant
3. increase murmur 5. C - Selenium
4. B - verapamil Pericardial disease:
5. C - nothing Case 1:
Case 2 continued: 1. Pericarditis
1. D - aortic regurg 2. Any infection (viral most common), trauma or
2. D - aortic regurg (phenylephrine is same as cancer near heart, connective tissue disease
hand grip) (SLE most common)
Case 3: 3. C - PR depression
1. LVH 4. NSAID's
2. echo first, cath best Case 1 continued:
3. valsalva decreases, hand grip decreases, leg 1. D - Kussmaul's sign
raise increases 2. C - pericardiocentesis
4. D - CHF 3. A – constrictive pericarditis
5. replace valve in aortic stenosis, balloon in Case 2:
mitral stenosis 1. Pericardial tamponade
6. repeat echo in 6-12 months 2. D - decrease of 10 mmHg when inhale
7. A - ACE-I 3. C - electrical alternans
8. no ballooning for aortic stenosis unless refuse 4. C -pericardiocentesis
or cannot tolerate surgery Case 2 continued:
Case 3 continued: 1. E - equalization of diastolic pressures
1. B - no viagra with nitrates, no viagra in aortic Rhythm disturbances:
stenosis Case 1:
2. E - LBBB 1. Electrical defibrillation
3. D - angiography 2. Never
4. E – nothing 3. Never
5. B – fix heart 4. Epinephrine or vasopressin prior to next
Case 4: attempt at defibrillation
1. HOCM 5. E - Beta blockers
2. A - dyspnea Case 1 continued:
3. BB, AICD 1. C - unsynchronized defibrillation
4. nothing Case 2:
Case 4 continued: 1. AICD
1. A - MR
2. Electrophysiological testing to find inducible 1. blood cultures (if positive --> vanco
VT/VF +gentamcin, then echo)
3. Recurrence of the dysrhythmia while on 2. staph
medications 3. D - vanco + gent
4. C - Decreased ejection fraction under 35% 4. A - CHF
5. ACID Case 2:
Ventricular tachycardia: 1. Penicillin or Ampicillin
Case 1: 2. Penicillin or Ampicillin + Gentamicin for 1st 2
1. CP, dyspnea, hypotension weeks
2. C - amiodarone 3. Amp + Gent for 6 weeks
3. Torsades de pointes or hypomagnesemia 4. Same as above
Case 2: 5. Add rifampin
1. C - AICD (because dilated CM) 6. A - HACEK
Case 2 continued: 7. A - Switch to vancomycin
1. D - implantable defibrillator Case 3:
2. Brugada's syndrome 1. E - continue present therapy
Atrial arrhythmias: Case 4:
Case 1: 1. Stop warfarin (only for a fib)
1. A - carotid sinus massage Case 6:
2. vagal, adenosine, BB/CCB/digoxin 1. None
3. BB/CCB/digoxin 2. None
4. Ablation 3. None
5. Same for all atrial tachyrcardias 4. None
Case 2: 5. None
1. WPW 6. None
2. Loud S1, early depolarization Peripheral artery disease:
3. Procainamide Case 1:
4. Ablation 1. Ankle/brachial ratio
5. CCB and digoxin 2. Angiography
Case 3: 3. Aspirin, stop smoking, statins, cilostazol, and
1. Control the rate with beta blockers exercise
2. Anticoagulation with dabigatran, warfarin, or 4. Cilostazol
rivaroxaban 5. Combination of aspirin and clopidogrel
3. Dofetilide and ibutilide 6. B - metoprolol
Case 4: Case 1 continued:
1. C - aspirin 1. B - Diabetes
Bradycardia: 2. C - Angiography
Case 1: Aortic disease:
1. C - Pacer Case 1:
Case 2: 1. TEE = CTA = MRA
1. 1st degree AV block 2. Angiogram
2. Nothing 3. Chest x-ray, CTA or TEE or MRA, propranolol,
Case 3: nitroprusside
1.3rd degree AV block 4. Surgical repair of the defect, if necessary (if >
2. Paced rhythm 5 cm)
Endocarditis: 5. Repeat ultrasonography annually, surgical
Case 1: repair if gets bigger than 5 cm
Pre-op:
Case 1: 8) E - h pylori
1. E - no further eval 9) C - resp failure
Case 2: 10) D - nuclear med scint
1. C - Atenolol Diarrhea:
Case 3: 1) B - norovirus
1. E - no further eval 2) A - norovirus
Case 4: 3) D - c. jejuni
1. E - no further eval 4) E - staph toxin
Case 5: 5) B - vibrio
1. 6) C - e coli
Peri-op: 7) C - e. coli
1. C - S3 gallop 8) A - b. cereus
9) E - yersinia
GI Answers: 10) A - celiac
Esophageal disease: 11) E - all
1) B 12) B - ischemic colitis
2) B - 10% 13) D - reassurance
3) E 14) D - c. diff
4) E - PPI 15) D - clear liquids/observation
5) C - 24 hr pH 16) if sx’s recur once re-treat with
6) B - DES/nifedipine metronidazole, if sx’s occur twice use
7) A - peptic/Schatzski ring vancomycin, if toxic megacolon remove colon
8) D - manometry/motility 17) prevent c. diff with probiotics
9) B - manometry 18) D – strongyloides (treat with ivermectin or
10) B - Barrett's esophagus abendazole)
11) C - fluconoazole 19) A – cryptosporidium (treat with
12) C - 24 hr amb pH nitazoxanide)
13) C - 24 hr pH 20) C - e histolytica (treat with metronidazole)
14) D - anticholinergics 21) strongyloides
15) B - endoscopy 22) irritable bowel syndrome, diarrhea use
16) A - squamous cell loperamide, constipation use linaclotide or
17) lubiprostone
18) 23) laxative use, stop laxatives
19) 24) yersinia, treat with quinolones if needed
20) (fever + hypotension + tachycardia)
21) 25) bacterial overgrowth syndrome from small
22) bowel diverticuli, treat with cipro +
23) metronidazole
24) 26) celiac disease, small bowel biopsy, anti
endomysial ab, anti-tissue transglutaminase
Stomach diseases: 27) bowel wall lymphoma, biopsy,
1) B - endoscopy noncompliance
2) D - both A and C true 28) strongyloides, stool for worms
3) B - gastrin levels 29) whipple’s disease, PCR, treat with doxy
4) C - lanso, amox, clarith Colon cancer screening
5) A - secretin stim 1) B - colonoscopy every 10 years
6) A - EUS/octreoscan 2) E - all acceptable
7) B - duodenal ulcers 3) E - nothing like colonoscopy
 4) C - 3-5 years Hepatitis:
5) A - every 5 yrs, start at 40 Case 1: Chronic Hepatitis
6) A - colonoscopy every 1-2 yrs 2) C
7) C - every 1-2 yrs, starting now 3) C
8) B - flex sig at age 12 4) D
Inflammatory Bowel Disease: 5) B
1) D 6) A
2) A 7) E
3) B - pyoderma gangrenosum 8) A
4) B - mesalamine 9) B
5) C - prednisone 10) E
6) B - 6-MP 11) A
7) B - sclerosing cholangitis 12) A
8) A - sclerosing cholangitis 13) A
9) E - all of the above 14) B
10) A - infliximab 15) B
11) B - frequency similar Case 2: Acute Hepatitis
12) B - iron def anemia 1) A
13) C - corticosteroids 2) B
14) C - NSAID's 3) A
15) A - surveill colonoscopy 4) E
Bleeding and Liver: Case 2 continued:
Case 1: 1) D
1) B 2) A
2) E - B and D correct 3) D
3) A - low risk of rebleeding 4) B
4) D - prednisone 5) E
5) E - all of the above 6) C
6) D – discharge 7) B
Pancreas 8) B
Case 1: 9) C
1) C - lab testing 10) E
2) B 11) E
3) B 12) D
4) C 13) alcoholic hepatitis, folate/thiamine/vit K,
5) A >32 points on DF  start steroids
6) C 14) primary biliary cirrhosis, ursodeoxycholic
7) B acid
8) B 15) autoimmune hepatitis, steroids +
9) D azathioprine
10) B 16) primary sclerosing cholangitis, liver
11) B transplant is only proven treatment
12) B
14) if painful and > 4 cm  drain Neuro answers
Case 2: Case 1:
1) D 1) stroke (L MCA)
2) A 2) CT (contrast neg)
3) B 3) A - Echo, Holter, carotid Doppler
4) tPA - 1st 3 hrs thymectomy (if pyr/neo not working & <60 yo),
5) D IVIG for acute myasthenic crisis
Case 2: 6) gentamicin (can block NMJ)
1) TIA 7) B - small cell lung cancer (Eaton-Lambert)
2) A - ASA Case 6:
3) add dipyr or switch to clopi 1) peripheral vertigo – likely labyrinthitis
4) no indication 2) central if focal symptoms, no hearing loss or
5) A - Anterior cerebral artery tinnitus
6) A - MRI 3) steroids, meclizine
7) C - clopidogrel 4) focal signs to look at posterior fossa
Case 2 continued: 5) Meniere's - chronic
1) A – neurocardiogenic syncope BPV - positional
2) A - statin Labyrinthitis - acute
3) C – carotid doppler VB TIA -
Case 3: Perilymphatic fistula – barotrauma
1) MS - symptoms indicate 6) C
2) carbamazepine, oxcarbazepine Case 7:
3) MRI 1) subarachnoid hemorrhage (sudden onset
4) MRI severe headache, stiff neck, photophobia)
5) C - steroids 2) C - lumbar puncture
6) A - slow progression of MS - glatiramer, IFN, 3) to get location of SAH so know where to
mitoxantrone, natalizumab, fingolimod (MCC Q embolize/clip
about MS - know this!) 4) Cerebral vein thrombosis (DVT in the head!)
7) walking (dalfampridine), fatigue 5) Mag resonance venography
(amantadine), urge incontinence (oxybutynin), 6) A
spasticity (baclofen, tizanidine) 7) B – angiogram to figure out where it is so you
8) D (internuclear opthalmoplegia) can fix it
9) E - CSF to look for oligoclonal bands 8) C – catheter embolization
Case 3 continued: 9) A – nimodipine (CCB decrease risk of stroke)
1) PML Case 8:
2) C - natalizumab 1) D
Case 4: 2) listed below question
1) Guillain-Barre Syndrome 3) D - xray of spine
2) E - FVC (check if need to intubate) 4) C - MRI
3) A - IVIG OR plasmapheresis (but NO combos) 5) C – B12 deficiency
4) A - IVIG alone OR plasmapheresis ALONE 6) C - syringomyelia
5) C - nerve conduction velocity 7) A – Brown-Sequard syndrome
6) NEVER Case 9:
7) C - IVIG 1) C - restless leg syndrome
8) C - campylobacter 2) B - pramipexole or ropinirole
Case 5: 3) C – iron
1) D - myasthenia gravis 4) benzos, opiate, dopamine agonists
2) C - ACh receptor antibodies Case 10:
3) single fiber EMG 1) D - essential tremor
4) C - chest CT (look for thymoma) 2) D - all of the above
5) pyridostigmine/neostigmine (for symptoms), 3) A – propranolol
prednisone/plasmapheresis (acutely), 4) when medical therapy does not work
Case 11:
1) C - nalaxone, thiamine, dextrose 2) triptans
2) bilateral pinpoint pupils 3) if headaches more than 4 times per month
3) unilateral dilation 4) propranolol for > 3 months
4) bilateral dilation 5) A - foods
5) will see skin lesions 6) A – pregnancy and cardiac disease
6) A – thiamine, then glucose Case 17 continued:
Case 12: 1) oxygen, verapamil
1) A - vitamin B12 deficiency 2) C
2) F - MMA levels 3) C
Case 13: 4) C - migraine
1) C - ALS 5) A - migraine
2) E - Riluzole 6) C
3) D - CPAP Case 18:
Case 14: 1) pseudotumor cerebri
1) Parkinson's disease 2) A - diplopia
2) mild, >60-70 yo, if can't use anticholinergics 3) CT scan (rule out a space-occupying lesion)
3) severe disease 3) LP (shows high pressure)
4) added to levo/carbi to extend duration of 4) weight loss, check for vit A toxicity, stop
therapy OCP's
5) alternative to levi/carbidopa, but less efficacy 5) acetazolamide, VP shunt, cut optic nerve
and less side effects 6) when med therapy doesn’t work
6) when max out meds 7) A – visual field loss
7) never Case 19:
8) C - quetiapine 1) delirium
9) progressive supranuclear palsy, 2) check sodium, oxygen, glucose, calcium,
antidepressants may help (no real treatment infections – exclude all correctable causes
though) 3) haloperidol NOT benzos
Case 15: 4) C – optic neuritis
1) benzodiazepine 5) D – prednisone and eye lubrication
2) fosphenytoin 6) B – laser photocoagulation
3) phenobarbital Case 20
4) midazolam, then anaesthesia 1) somatization
5) 2 years with no seizures 2) reassurance, freq doctor meetings
6) B – observation 3) A – education and relaxation techniques
7) C - temporal lobe 4) D
8) A - serology for cysticercosis 5) C - citalopram
Case 16: 6) C - fluoxetine
1) see chart on p. 147 7) E - haloperidol
2) donepezil 8) A - discontinue clozapine
3) D - memory 9) D - fluoxetine
4) B - NPH 10) A – 2 months
5) C - memantine 11) A - supportive therapy
6) E - stop lorazepam 12) A – somatoform disorder
7) CJD 13) B – borderline personality disorder
8) EEG, but brain biopsy most definitive test 14) A – bipolar disorder
9) supportive Case 21:
Case 17: 1) D
1) migraine 2) C
3) A Case 5:
4) B - radial nerve palsy 1) Serum GGT level
5) F 2) Paget's disease
6) D 3) Bone survey
7) C 4) A - Bone scan
8) E 5) Bisphosphonates
9) C 6) Almost never (used for bone pain only)
10) D 7) C - bone biopsy
11) D Case 6:
12) B 1) osteoporosis
13) C 2) DEXA scan - most accurate test
14) A 3) C - vit D and TSH
4) steroids, thyroid, vit D def
Endocrine answers 5) bisphosphonates
Case 1: 6) Never alone for OA, can be used within 7 yrs
1) B - HCG of menopause
2) E - panhypopituitarism 7) bisphosphonates
3) C - pituitary adenoma 8) SERM - 2nd line agent
4) B - Sheehan syndrome 9) A - calcitonin (best tx for pain)
5) D - sarcoidosis 10) D - calcium and vit D
6) acute hemorrhagic necrosis of pituitary 11) C - HRT increases fibroid size
7) A - histiocytosis X 12) C - testosterone
Case 2: 13) A - alendronate
1) acromegaly 14) E - d/c alendronate and start PTH
2) B - IGF1 15) D - Raloxifene
3) 2 hr glucose tolerance test Case 7:
4) TSH 1) E - calcium gluconate
5) after biochemical confirmation 2) calcium, PTH, vitamin D
6) osteoarthritis, HTN, DM, 2x mortality 3) QT prolongation
7) surgical resection 4) activated vitamin D
8) B – lanreotide (or octreotide) 5) loop diuretics
9) Add on (adjuvant) to somatostatin analogue 6) A - TSH
10) 3rd line agent Case 8:
Case 3: 1) hyperparathyroid, malignancy
1. Kallman's syndrome (hypogonadotropic 2) stones, moans, groans, psychic overtones
hypogonadism) (renal stones, hypertension, polyuria, fatigue,
2) Testosterone. If fertility is a concern, replace mental status changes, bone pain, osteoporosis,
LH/FSH and pathologic fractures.)
Case 4: 3) B - normal saline
1) Prolactinoma 4) hypercalcemia due to malignancy
2) Serum prolactin level 5) hypercalcemia secondary to granulomatous
3) D - MRI disease
4) see table p. 222 6) parathyroid adenoma and hyperplasia, most
5) Visual field deficit, osteoporosis, common complication of surgery is
reproductive concerns, and macroadenoma hypoparathyroidism
6) B - Cabergoline 7) C - calcitonin
7) Rarely; in the case of resistance to meds 8) D - check sister's calcium
8) A - repeat prolactin with hemodilution Case 9:
1) Cushings syndrome 8) A - factitious hyperthyroidism
2) ACTH 9) A - increased uptake
3) ACTH - to distinguish central vs adrenal vs. 10) B – wait three months
extra-adrenal Case 15:
4) E - serum and urinary cortisol 1) hyperthyroid --> not FNA and no biopsy
5) if adenoma needed, euthyroid --> FNA
6) adrenal cancer or resistant cushings 2) A - sonogram-guided FNA
Case 10: Case 16:
1) B - hyperaldosteronism 1) hypothyroid
2) D - renin and aldo 2) TSH
3) C - aldo supp test 3) levothyroxine
4) CT (if adenoma --> take it out, if bilateral --> 4) C - IV glucocorticoids
no surgery) 5) Steroids plus IV T4/T3, sepsis
5) A - increased plasma renin activity 6) Thyroid lymphoma
6) adrenal vein sampling 7) KI
7) adenoma only, NOT hyperplasia Case 17:
8) C – spironolactone 1) Sick euthyroid syndrome
9) measuring aldosterone in samples of adrenal Case 18:
venous blood 1) fasting sugar > 126, random 2 hr > 200, A1C >
Case 11: 6.5, impaired glucose tolerance 5.7-6.4
1) pheochromocytoma 2) HbA1c (only one value needed)
2) D - urina metanephrines 3) C – 24-26 weeks gestation
3) adrenal CT 4) always (except if Cr > 1.4)
4) always! 5) 3rd line
5) preop - to prepare for surgery 6) add on
6) B - after phenoxybenzamine 7) HbA1c > 9.5
7) A - thyroid sonogram 8) D - insulin
Case 12: 9) A
1) A - 24 hr urinary cortisol 10) A - metformin
2) skip - cut out if functional 11) B – diet and exercise
3) A - ACTH level 12) A - glipizide (shorter half-life because renal
Case 13: insufficiency)
1) primary adrenal insufficiency Case 19:
2) B - AM cortisol 1) B - lisinopril
3) if low AM cortisol 2) cough, hyperkalemia
4) when primary insufficiency (tell if it's atrophy 3) urine microalbumin/cr > 30:1 (yearly)
or hemorrhage) 4) exercise, UTI, menstruation
5) secondary adrenal insufficiency 5) BP control and lipids
6) hydrocortisone. prednisone 6) yearly ophtho checkups
7) E - anti-CYP21 antibodies 7) quarterly podiatry exams, microfilament
Case 14: 8) D - duloxetine (gabapentin, tricyclics okay
1) D - Grave's disease too)
2) post-viral, tender thyroid 9) DM gastropathy --> add erythromycin
3) beta blockers 10) erectile dysfuction
4) thyroid storm 11) EKG - because diabetics at increased risk for
5) toxic nodule the development of coronary artery disease
6) D - propranolol Case 20:
7) B - free T4 1) diabetic ketoacidosis
2) IVF + IV insulin 2) Rifampin (Penicillin, Allopurinol, Quinidine,
3) C - glucose < 200 Lamotrigine)
4) K+ < 5, monitor PO4- < 1.5 3) UA
5) 24 hour rate x 0.7 = basal dose = glargine 4) Eosinophils in urine (Hansel stain)
dose 5) Stop medication and wait, if progress --> use
6) normal AG, very high glucose steroids
7) eliminated (said was stupid question) 6) Ethambutol caused visual disturbances
8) C - decrease pre-dinner aspartate (somogyi) 7) Not based on BUN/Creatinine
9) Increase glargine (not one of the answer 8) Refractory hyperkalemia, metabolic acidosis,
choices, typo here) pericarditis, fluid overload, encephalopathy
10) D - increase pre-breakfast aspartate Case 3:
Case 21: 1) K+ levels/EKG
1) meds (insulin, sulfonylureas), liver failure, 2) B - urinanalysis
cachexia, chronic kidney disease, insulinoma 3) Mannitol, IV fluids, if K is up --> Bicarbonate
Case 22: Case 4:
1) high testosterone 1) Hydration with normal saline
2) B - CT scan of adrenal 2) Cholesterol emboli
4) D - metformin 3) Biopsy skin (Cholesterol Crystals)
Case 23: 4) Nothing
1) Rule out pregnancy with beta-hCG 5) A - contrast
2) Prolactin and TSH levels Case 5:
3) C - MRI 1) Papillary necrosis
4) D - observation 2) B - CT scan
5) B - pelvic US (endometriosis) 3) No specific therapy
6) B - fluoxetine 4) E - aminoglycosides
Case 24: End-stage renal disease:
1) D - testosterone 1) C - coronary disease
2) FSH/LH 2) C – cultures, vanco
3) no testosterone indicated Glomerular Disease:
4) Case 1:
5) SSRI’s 1) All
6) total testosterone, LH, FSH 2) All
7) pump to assist in erection 3) B
8) sex without protection 2-3 times per week 4) Alport’s syndrome
9) and 10) sildenafil after 24 hours, tadalafil Case 1 continued:
after 48 hours 1) D - amyloidosis
12) adrenal CT scan 2) B - cryoglobulinemia
Case 2:
Nephro answers: 1) B – repeat sample in 2-3 weeks
Acute Renal Failure: 2) A – no further eval needed
Case 1: 3) D - split urine
1) E - no clear diagnosis 4) B
2) chart Case 2 continued:
3) if prerenal, give octreotide 1) B - Goodpasture's syndrome
4) no test 2) D - Bartter syndrome
5) no specific therapy Case 3:
Case 2: 1) Nephrotic syndrome
1) AIN - Allergic Interstitial nephritis 2) Lymphoma with Membranous
3) Protein:Creatinine Ratio Potassium disorders:
4) B – kidney biopsy Case 1:
5) Prednisone 1) Diarrhea--> vomiting --> inhaled beta
Case 3 continued: agonists drove potassium into cells
1) A -Focal segmental glomerulosclerosis 2) Very low: <20 mEq/L
2) B – Berger disease 3) EKG - U wave
3) B - Goodpasture's disease 4) no maximum
4) C – loss of urine carrier protein 5) < 20 mEq/hr
Case 4: 6) B - D5W
1) E - antiretroviral therapy 7) 5 mg/kg per point increase in potassium
Case 4 continued: 8) Mg
1) D - light chains Case 2:
2) D - immediate hemodialysis 1) Tumor lysis syndrome
3) A - calcium acetate with meals 2) EKG
4) C - decrease efferent arteriolar pressure 3) Calcium chloride or gluconate if peaked T
5) B - continue waves
Sodium Disorders: Phosphate disorders:
Case 1: 1) E – Fanconi syndrome
1) DI (Lithium) or Psychogenic Polydipsia 2) E – renal failure
2) Both Metabolic acidosis:
3) Both Case 1:
4) E - Nocturia 1) Ethylene glycol causes renal problems,
5) Urine Na and Urine osmolarity Methanol causes eye problems
6) ADH administration 2) All three will
7) Central - ADH replacement, nephrogenic - 3) All three will
thiazides, NSAID's 4) UA for ethanol, eye exam for methanol
Case 1 continued: 5) Fomepazole (stops toxic metabolite from
1) C – psychogenic polydipsia forming)
Case 2: 6) Normal anion gap
1) non-ketotic hyperosmolar syndrome Renal tubular acidosis:
2) normal saline and insulin Case 1:
3) 357 1) A - Proximal RTA
Case 3: 2) Bicarb
1) SIADH 3) Diuretics
2) Mets, Tumor Case 2:
3) TB, pneumonia 1) B - Distal
4) Una and Uosm - both high 2) Give acid
5) Hypertonic saline or Saline+Diuretic 3) Bicarb
6) C - Tolvaptan (V2 receptor antagonist) Case 3:
7) central pontine myelinosis 1) Type IV
Case 4: 2) Na restriction
1) Nephrogenic DI 3) Fludrocortisone
2) 8.57 Continued questions:
3) High Ca and Low K 1) B – low anion gap
4) Thiazides, NSAID's 2) E – chronic renal failure
NSAID’s: Metabolic Alkalosis:
1) D – constricts afferent arteriole Case 1:
2) E - hypokalemia 1) Licorice
2) In Conn - high Urine K 1) Kegel exercises
3) In Bartters – low or normal BP 2) Surgery
4) Urine chloride Hypertension:
5) "U" waves Case 1:
6) Stop licorice 1) Lifestyle modifications
Tubular disorders: 2) Weight loss
1) A – Barrters (like taking furosemide all the 3) Relaxation
time) 4) Diuretics - thiazides
2) B 5) Add beta blocker, calcium blocker, ACE
3) C inhibitor, or ARB
4) C 6) Never minoxidil, methyldopa in pregnancy,
5) D - all clonidine if opiate/alcohol use
6) D - all 7) Reserpine, then clonidine, then beta blockers
Acid-base disorders Case 2:
1) B – licorice ingestion (like eating aldosterone) 1) Beta blocker
2) B - diuretics Case 3:
3) A – gapped metabolic acidosis 1) Beta blocker
4) B – metabolic acidosis and alkalosis 2) <130/80
Nephrolithiasis: Case 4:
Case 1: 1) Beta blocker
1) B - analgesics 2) Beta blocker
2) Spiral CT Case 5:
3) Calcium oxalate 1) Labetalol, nitroprusside, nicardipine
4) If > 20 mm --> surgery, if 5-20 mm --> shock 2) see table
wave, if < 5 mm --> wait/hydrate Case 6:
Case 2: 1) Young (<30), told (>60), hard to control HTN
1) Ca oversecretion in urine Case 6 continued:
2) Thiazides 1) B - primary hyperaldo
3) C - Uric acid stones, citrate to alkalinize Case 7:
Case 2 continued: 1) Ultrasound
1) B - increased oxalate absorption 2) Angiogram
2) C - tamsulosin 3) Balloon and stent
Polycystic kidney disease: Case 8:
Case 1: 1) Phenoxybenzamine
1) Liver, ovary, pancreas, diverticuli 2) Alpha blocker
2) Mitral valve prolapse, diverticular disease,
and circle of Willis aneurysms Pulmonary answers:
3) End stage renal disease Case 1:
4) Not routine 1) FiO2 x 4-5 = pO2
Urinary Incontinence: 2) are alveoli healthy & allowing gas exchange
Case 1: 3) B - Guillain-Barre syndrome
1) Urge incontinence 4) respiratory acidosis (pH/pCO2/pO2/HCO3)
2) Urodynamic studies (manometry) Case 2:
3) Oxybutynin, tolterodine, trospium, 1) metabolic acidosis
darifenacin - dry mouth 2) additional resp acidosis - Winter's formula
4) B – observation (expected CO2)
5) A – neurogenic bladder 3) ignore
Case 2: 4) AG MA - MUDPILES, Non-AG MA - RTA 1 & 2
Case 2 continued: Case 7:
1) D - dialysis 1) B
2) B - measure serum osmolarity 2) C
Case 3: 3) yes, cover atypicals (quinolones, azithro)
1) metabolic alkalosis 4) C
2) no additional resp disorder 5) B - LV failure
3) urine chloride 6) B - home oxygen
4) B - Gitelman syndrome 7) E - smoking cessation
Case 4: 8) E - recommend lung resection surgery
1) metabolic acidosis Case 8:
2) EtOH 1) GERD, post-nasal drip, cough-variant asthma
3) 2Na + BUN/2.8 + glucose/18 2) nasal steroids
4) met acidosis of unknown etiology 3) A - methacholine challenge test
Case 5: 4) not necessarily
1) C- normal saline 5) B - fluticasone
2) septic shock 6) B - peak flow (FEV1)
3) norepinephrine, dopamine 7) albuterol, steroids
4) 8) add on to albuterol
5) 2D echo 9) steroids
Case 6: 10) D - stat MICU consult and intubation
1) ARDS (bilateral infiltrate, Fi02/PO2 < 200, 11) find and get rid of trigger
normal LV normal EF, wedge < 18) 12) add omalizumab
2) D - 2D echo Case 8 continued.....
3) maintain FiO2 < 40%, low TV 1) B - chest xray
4) plateau - pulm tissue and airways, peak - 2) D - inhaled steroid and salmeterol
large airways 3) D - skin test for aspergillus
5) A- COPD 4) C - corticosteroids
6) drops 5) E - CT scan
7) C - bronchodilators 6) D - stop aspirin
8) reducing tidal volume and thus allowing the 7) E - short-acting beta agonist
levels of carbon dioxide to rise. By reducing Case 9:
tidal volume, the amount of barotrauma is 1) B - bronchiectasis
reduced 2) chest x-ray
9) B – decrease in BP 3) CT chest - shows dilated airways
10) D - mucus plug 4) obstructive pattern
Case 6 continued..... 5) D - alpha-1-antitrypsin
1) D - lobar atelectasis Case 10:
2) C - occluded ET tube 1) sarcoidosis
3) D - pulmonary embolism 2) A - lymph node biopsy to look for
4) D – semi-recumbent position noncaseating granulomas of unknown origin
Pulm function testing: 3) serious organ involvement
1) FEV1 low, FVC high, ratio < 80% 4) restrictive pattern
2) FEV1 low Case 11:
Flow Volume Loops: 1) cough, SOB associated with work
1) A5, B6, C3, D2, E1, F4 2) fever, increased WBC
2) A - DLCO 3) high neutrophil count in CAP
3) D - phrenic nerve paralysis 4) B - pleural biopsy
4) D - pulmonary edema 5) oral steroids
Case 12: 7) D
1) chest x-ray (idiopathic pulmonary fibrosis) Case 17:
2) CT chest 1) chest x-ray
3) biopsy 2) C - mediastinoscopy
4) restrictive 3) staging
5) D - refer for heart-lung transplant 4) no surgery - chemo and radiation
Case 12 additional cases: 5) stages 1-3A
1) bronchiolitis obliterans organizing 6) PFT's
pneumonia (BOOP) Case 18:
2) alveolar proteinosis 1) pulmonary hypertension
3) pulmonary lymphangioleiomyomatosis 2) A - 2D echo
Case 13: 3) right heart cath with nitrate challenge
1) narcolepsy 4) class I symptoms
2) sleep studies (polymsomnography) - see 5) class IV symptoms
increased REM sleep 6) if vasculature responds to adenosine,
3) stimulants - methylphenidate prostacyclin or nitrous oxide
Case 14: 7) right heart failure on prostacyclin or cannot
1) obstructive sleep apnea tolerate medical therapy
2) nighttime pulse oximetry Case 19:
3) sleep studies with at least 30 apneic episodes 1) pulmonary embolism
4) D - CPAP 2) hemodynamically unstable PE without
5) obesity hypoventilation contraindications to thrombolytics
6) acetazolamide (CA inhibitor) 3) E - VQ scan (high resolution CT lungs ideal)
Case 15: 4) D - dalteparin (heparin)
1) pleural effusion 5) 6 months (3 months - 1 year)
2) lateral decubitis film 6) immobility, malignancy, nephrotic syndrome,
3) if fluid layers > 10 mm thick, order serum to oral contraceptives, pregnancy, obesity,
fluid ratio of LDH > 60%, protein > 50% hormone replacement therapy, heart failure
4) exudate 7) B - CT
5) E - cirrhosis Case 19 continued.....
6) infections, rheumatic, cancers, pancreatitis 1) A
7) B - rheumatoid arthritis 2) C
8) pleurodesis 3) D
9) palliative measures 4) B
10) empyema (pH < 7.19, gross pus, or lg WBC), Case 20:
must insert chest tube 1) A - alveolar hemorrhage
Case 15 continued..... 2) B - bronchoalveolar lavage
1) A - open pleural biopsy 3) A - anti-GBM antibodies
2) C - pleurodesis 4) A - crack cocaine
3) A- antibiotic therapy Case 21:
4) C - decortication 1) A - acetazolamide
Case 16: 2) B - descent to lower altitudes
1) history &radiographic appearance of nodule
2) compare current x-ray with previous ones
3) A Heme/Onc:
4) popcorn calcifications Microcytic anemia:
5) “onion-skin” or “bull’s-eye” calcification Case 1:
6) A 1) C - reticulocytes
2) B – vitamin C 5) transfuse GCSF, infection, 5q deletion -
3) C - PPI azacytadine
Case 1 continued: Case 2:
1) D 1) AML
2) C – iron deficiency anemia 2) smear with blasts
3) C - hypothyroidism 3) cytogenetic abnormalities
4) D - colonoscopy 4) M3
5) A – sideroblastic anemia 5) relapsed disease
Case 2: Case 3:
1) B 1) differential WBC, 91% neutrophils
2) C – peripheral smear 2) CML
3) D – MMA level 3) increased basophils
4) B12 or folate replacement 4) B - BCR/ABL
5) C - hypokalemia 5) C - imitanib
6) F – MMA level 6) leukopheresis
7) E 7) hyroxyurea
8) C - both 8) lung fibrosis
9) metformin Case 4:
Hemolysis: 1) CLL
Case 1: 2) A - no treatment necessary
1) D – peripheral smear Case 5:
2) A - hemoglobin electrophoresis 1) B - fludarabine
3) A - oxygen, fluids, and analgesics Case 6:
4) high WBC or fever 1) hairy cell leukemia
5) ceftriaxone, levofloxacin, moxifloxacin 2) immunophenotyping/cell sorter
6) C – exchange transfusion 3) cladribine
7) parvovirus B19 CLL Review questions:
8) B – reticulocyte count 1) A - observation
9) E – PCR for DNA 2) C - hypogammaglobulinemia
10) IVIG 3) D - prednisone
11) hydroxyurea Myeloproliferative disorders:
12) D – aseptic necrosis Case 1:
Case 2: 1) polycythemia
1) normal 2) C - splenomegaly (hypoxia can raise HCT)
2) C - renal involvement 3) ABG's, renal US
3) more than SS trait, less than SS disease (SC in 4) B - JAK-2
middle) 5) D - none
4) A - visual disturbance 6) phlebotomy
2) D – cold agglutinin disease 7) hydroxyurea to drop cell count
3) B – autoimmune, cold IgM antibodies 8) thrombosis
4) C - parvovirus Case 2:
Leukemias: 1) B - myelofibrosis
Case 1: 2) C - lenalidomide
1) myelodysplastic syndrome Case 3:
2) Pelger-Huet cell, bi-lobed, pancytopenia 1) essential thrombocythemia
3) D - % of blasts 2) C – both bleeding and thrombosis
4) A - bleeding and infection 3) ASA, hydroxyurea, anagrelide
4) C - CML
5) C - observation 3) CD20 antiboduy
Case 3 continued: 4) add rituximab
1) B – leukemoid reaction 5) BM transplant
Plasma cell disorders: Transfusion reactions:
Case 1: Case 1:
1) Myeloma 1) major blood group ABO incompatibility
2) C - bone marrow biopsy 2) Stop the transfusion. Recheck the blood type.
3) B-J protein, hyperuricemia, increased Hydrate the patient and consider forced
ESR, low anion gap, rouleau formation, diuresis with mannitol. Possible use of
suppression of other Ig lines bicarbonate.
4) D (auto stem cell can be used up to 70 years 3) D - filter and irradiated
old) (B would have been the most effective 4) delayed development of jaundice
*medication*, but that wasn't the question!) 5) C - hypocalcemia
Case 2: 6) A – IgA deficiency reaction (anaphylaxis)
1) MGUS Case 2:
2) A - no treatment 1) C - leukoagglutination
Case 3: Case 3:
1) Waldenstroms Macro IgM 1) D - urticarial reaction
2) B - plasmapheresis Case 4:
Aplastic Anemia: 1) B - anaphylaxis
Case 1: Case 5:
1) HIV, EBV, CMV, hepatitis B and C serology, 1) A - febrile nonhemolytic reaction
ANA, B12, folate levels 2) E - febrile nonhemolytic reaction
2) none - diagnosis of exclusion Coagulation:
3) B - allogenic bone marrow transplant Case 1:
4) C - cyclosporine and antithymocyte globulin 1) von willebrand's disease
5) D - oral polio 2) superficial
6) A - B12 3) A - bleeding time
7) A - cobalamin deficiency 4) A - desmopressin
Lymphoma: 5) B - factor VII replacement
Case 1: 6) B – Bernard-Soulier syndrome
1) excisional biopsy 7) C – Glanzmann’s
2) chest x-ray, chest/abdomen/pelvic CT scans, 9) post-transfusion purpura, tx with IVIG
bone marrow biopsy and LDH level Case 2:
3) A - lymphocyte predominant 1) ITP
4) B - lymphocyte depleted 2) none
5) A - radiation therapy 3) steroids
6) B - ABVD chemotherapy 4) splenectomy
7) B - ABVD chemotherapy Case 3:
8) D - bone marrow transplant 1) IVIG
9) filter out antigens - don't alloimmunize 2) D - e. coli O157: H7
Case 1 continued..... Case 4:
1) B - lung cancer 1) factor XIII deficiency
2) C - acute leukemia 2) euglobulin clot lysis time
Case 2: 3) E - fresh frozen plasma
1) start treatment; more tests won’t change Case 5:
therapy 1) factor XI deficiency
2) radiation local, chemo widespread, CHOP 2) E - mixing time
3) F – specific factor level 1. A – perform PSA
4) B - fresh frozen plasma 2. B - transrectal ultrasound with biopsy
Coagulation Review Questions: Case 2:
1) A – acquired VW factor antibody 1. local - Gleason scoring (low - do nothing, high
2) A – fibrin low, D dimers high, fibrin split - surgery/radiation)
products high 2. A - radiation or surgery (use flutamide first)
3) B - DIC Case 3:
4) C – fresh frozen plasma 1. flutamide, GnRH agonist
5) B - hypocalcemia Testicular cancer:
6) A - VIII, IX, XI, XII 1. B - inguinal orchiectomy
7) A - nothing 2. A - regular follow up, no chemotherapy
8) A – lower dose unless it recurs
9) C – proceed with surgery Breast Cancer:
10) C - FFP Case 1:
11) A – nothing until echo done 1. mammogram, pap smear, colonoscopy
12) C – smear to look for clumping 2. neg = no axillary dissection
Case 6: 3. positive nodes = chemotherapy
1) heparin-induced thrombocytopenia 4. ER or PR + = tamoxifene, raloxifine,
2) C – venous thrombosis 5. B - HER2/Neu positive
3) stop heparin 6. alternative to tamoxifen
4) argatroban, lepirudin (direct-acting thrombin 7. B - yearly mammogram
inhibitors) Case 1 continued....
5) platelet factor IV antibodies, serotonin 1. A - lymph node involvement
release assay 2. E - lumpectomy with adjuvant chemotherapy
6) A – increased aPTT (lupus anticoagulant) and local radiation
Case 7: 3. A - radiation followed by tamoxifen and
1) D – antiphospholipid syndrome chemotherapy
2) mixing study Case 2:
3) Rusell viper venom test 1. D - tamoxifen
4) E – ASA and heparin 2. DVT
Case 8: 3. B
1) E – factor V Leiden 4. A
2) lifelong warfarin Cervical cancer:
3) skin necrosis 1. D
4) heparin resistance 2. D
Thrombophilia questions: 3. E
1) C – no treatment necessary Endometrial cancer:
2) C – advise not to use oral contraceptives 1. A
3) C – antiphospholipid syndrome 2. C
Case 9: Ovarian cancer:
1) uremia-induced platelet dysfunction 1. C
2) desmopression/DDAVP
Case 9 continued..... General Internal Med answers:
1) C - acquired factor VIII inhibitor Case 1:
Case 10: 1. gastric lavage
1) Wiscott-Aldrich syndrome 2. caustics
Prostate Cancer: 3. never dangerous
Case 1: 4. never
Case 2: 1. D - nicotinic acid deficiency (pellagra)
1. narcan, dextrose, thiamine Case 14:
2. intubate and lavage (empty stomach) 1. C - chromium
3. never Case 15:
4. seizures 1. D - zinc
Case 3: Case 16:
1. salicylate overdose 1. C
2. bicarbonate Case 17:
Case 3 continued..... 1. E - all of the above
1. B – ASA toxicity Acetaminophen overdose:
Case 4: Case 1:
1. D - EKG 1. nothing that can be done (too late for n-
2. if wide QRS --> give bicarbonate to protect acetylcysteine - only in 1st 24 hours)
heart Case 2:
Case 5: 1. n-acetylcysteine
1. give oxygen Case 3:
2. myocyte ischemia - EKG 1. n-acetylcysteine and charcoal
3. normal Adverse effects and toxicity:
hyperbaric oxygen indications - cardiac Case 1:
problems, metabolic acidosis, pregnancy 1. C
Case 5 alternate: Case 2:
1. C - call an ambulance 1. B
Case 6: Case 3:
1. nerve gas 1. C
2. C - atropine Case 3 alternate:
3. pralidoxime 1. B
Case 7: Case 4:
1. gastrointestinal disturbance 1.metabolic acidosis with normal AG
2. B Case 5:
3. vtach 1. B
4. EKG Case 6:
5. low potassium 1. E
6. life-threatening dysrhythmias or hypotension Case 7:
unresponsive to other measures 1. C - serotonin syndrome
Case 8: Case 8:
1. spider bite (black widow spider) 1. C - methadone (opiate withdrawal)
2. C - low calcium Case 9:
3. calcium, anti-venom antitoxin 1. C
Case 9: Case 10:
1. B - EKG 1. B - diphenhydramine
2. j waves on EKG (look like ST elevation) 2. B - calcium carbonate
Case 10: 3. D - amiodarone
1. scurvy 4. B - lactic acidosis
Case 11: 5. D – methemoglobinemia
1. vitamin A toxicity 6. B – malignant hyperthermia
Case 12: 7. C - glucagon
1. high altitude pulmonary edema - B 8. calcium
Case 13: Epidemiology:
1. D - >85 Case 9:
2. B - CHF 1) C - random selection across population
3. A - urine Ethics:
Immunology: Case 1:
Case 1: 1) tell father
1. D – desensitization (immunotherapy) Case 2:
Case 1 continued..... 1) don’t do it
1. B - stop propranolol Case 3:
Case 2: 1) no blood to mom, give blood to kid
1. enalapril Case 4:
Case 3: 1) C
1. C - glucagon 2) A
Case 4: 3) A
1. E - encase everything 4) A
Complement deficiency: 5) B
Case 1: 6) B
1. A - C2 deficiency 7) C
Case 2: 8) A
1. B - C1 inhibitor deficiency Preventative medicine:
Case 3: Case 1:
1. A - C2 and C4 levels 1) A
Case 4: 2) D – ASA with PPI
1. B - danazol 3) E
Case 5: 4) E
1. C - decreased C2 and C4 levels 5) B
Case 6: 6) E
1. A - nasal steroids 7) C
Case 7: 8) A – family history
1. C – pneumococcal sepsis 9) C – serum cholesterol only
Selective IgA deficiency: 10) D
1. D – common variable immunodeficiency 11) D
Hyper IgE syndrome: 12) E
1.C – IVIG 13) E
14) B
Stats/Ethics: 15) E
Stats: 16) B
Case 3: 17) B
1) B - reassurances 18) A
Case 4: 19) E
1) A – lead-time bias 20) A
Case 5: 21) B
1) B – confounding variable 22) C
Case 6: 23) C
1) B – type 2 error 24) B
Case 7: 25) C
1) all of the above
Case 8: Rheum answers:
1) C – 5 Case 1:
1. E - rheumatoid arthritis 8. A - ACE inihibitor
2. E - skin nodules 9. A - pulmonary hypertension
3. A – anti-CCP 10. D - right heart catheterization
4. NSAID's and prednisone Case 5:
5. A - methotrexate 1. SLE
6. A - liver and lung fibrosis 2. A - ANA
7. B - anti-TNF 3. B - dsDNA
8. E - high RF and most joints involved 4. 4 skin (malar, discoid, photosensitivity,
9. A – dry eye ulcers), joint, renal, CNS, chronic heme
Case 1 continued..... 5. A - hydroxychloroquine
1. E - spinal fusion 6. acute exaacerbations
2. A - anti-CCP or RF 7. C - CNA and renal involvement
3. C – hydroxychloroquine 8. B - antiphospholipid antibodies
4. B – low glucose 9. A - mixing study
5. D – accelerated coronary disease 10. A - anti-Ro (do EKG on baby for AV block)
6. C - cricoarytenoid joint involvement 11. C – anti-Sm ab’s
7. Case 5 continued:
8. E – infective endocarditis 1. B - restrictive pulmonary disease
9. Baker’s cyst, removal Case 6:
10. Caplan’s sydrome 1. Still disease
11. C – palindromic rheumatism 2. A - ibuprofen
12. A - better 3. D - prednisone
Case 2: 4. C - CBC and ferritin levels
1. D - parvovirus Case 1 p. 434:
2. C – decreased reticulocyte count 1. B - relapsing polychondritis
3. B - duration Case 2 p. 435:
4. A - PCR for DNA 1. A - Behcet syndrome
5. normal Case 3- p. 435:
6. A - anemia 1. D - reflex sympathetic dystrophy
7. NSAID's for joint pain, IVIG for anemia 2. A - bone image (scintigraphy)
Case 3: Case 7 - p. 437:
1. E - Sjogren syndrome 1. C – bursitis
2. B - Schirmer test 2. trauma
3. C - SSA and SSB 3. fever and increased WBC count
4. C - salivary gland biopsy 4. A – NSAID’s and physical therapy
5. A - ANA Case 7 continued.....
6. A - joint pain 1.D - anserine bursitis
7. C - lymphoma 2.C – prepatellar bursitis
8. artificial tears and saliva (no steroids), 3.A – Jumper’s knee (patellar tendonitis)
civemeline, pilocarpine 4.B - plantar fasciitis
Case 4: Case 8:
1. C - CREST syndrome 1.A
2. ANA 2.B
3. verapamil, nifedipine 3.C
4. C - renal and cardiac involvement 4.E
5. E - no effective therapy 5.aortic reg, uveitis
6. worse with exercise, treated with steroids 6.bent over test
7. eosinophiliic myositis, steroids work 7.A
8. B – clinical symptoms 8.stop eating organ meats, no beer
Case 8 continued: 9.after acute attacks
1.C 10.allopurinal alternative
2.E Case 14 continued.....
3.A 1. D
Case 9: 2. E
1.psoriatic arthritis 3. C
2.“pencil in cup” deformity Case 15:
3. asymmetric, inflammatory arthritis of distal 1.B
joints of fingers and axial skeleton 2.osteophytes
4.E 3.none
5.part of the sero negative, enthesitis 4.lose weight, exercise
6.NSAID’s, Methotrexate 5.A
Case 10: Case 16:
1.D 1.A
2.B 2.x-ray
3.GI campylobacter, chlamydia .....give 3.MRI
antibiotics 4.calcuim & Bisphosphonates
4.NSAID’s, antibiotics if infection 5.chronic steroid therapy can cause it
Case 11: Tendon, Ligament, Cartilage, Bursa disorders:
1.C 1. relapsing polychondritis, steroids
2.ESR 2. bicipital tendonitis
3.temporal artery biopsy 3. rotator cuff injury, surgical repair
4.high dose steroids immediately 4. medial epicondylitis, NSAID’s and PT
5.shoulder and hip pain with no temporal artery 5. Dupuytren’s contracture, inject collagenase
symptoms – PMR with lower dose steroids and steroids
6.thoracic aneurysm 6. scaphoid fracture, nothing
Case 12: 7. ACL tear, surgically reattach
1.C Case 17:
2.B 1. A - dermatomyositis
3.GI angiogram 2. C – CPK and aldolase
4.D 3. biopsy
5.D 4. must look for solid organ cancer
6.steroids 5. pulmonary HTN (if lung involvement)
Case 13: 6. A - prednisone
1.C 7. B - azathioprine
2.B Case 18:
3.Biopsy 1. E - none
4.A 2. depression/chronic fatigue, sleep disorders
5.E 3. TCA’s
Case 14: Case 18 continued:
1.B 1. C (fibromyalgia)
2. Linear radiodense calcium deposits Case 19:
3.A 1. carpel tunnel
4.D 2. C – nerve conduction study
5.patients who cannot take an NSAID 3. hand splinting
6.preventive 4. inject steroids
7.NSAIDs not working, multiple joints
5. severe pain refractory to medical therapy or 3. C - PCR for DNA
significant thenar muscle atrophy 4. A – acyclovir
6. DM, acromegaly Case 6 continued:
Case 20: 1. D - reduce the dose of acyclovir and increase
1. B hydration and urine flow
2. D 2. mollaret meningitis
3. A Otitis Media:
4. C Case 1:
5. familial mediterranean fever 1. C - immobility of the tympanic membrane
6. Churg-Strauss 2. tympanocentesis
7. microscopic polyangiitis, steroids and 3. amoxicillin
cyclophosphamide 4. add clavulic acid or 2nd/3rd gen cephalosporin
8. eosinophilic fasciitis, steroids Case 2:
1. treat
Infectious diseases answers: 2. C - biopsy
CNS infections 3. amoxicillin
Case 1: Influenza:
1. focal neurologic abnormalities, severe Case 3:
confusion that makes the neurologic 1. neuraminidase inhibitor
examination inaccurate, papilledema, and/or a 2. antigen detection on nasopharyngeal wash
recent seizure 3. everybody every year
2. culture Head and Neck infections:
3. strep pneumoniae Case 5:
4. ceftriaxone 1.C - airway obstruction
5. bacteria on Gram stain, CSF WBC >1,000 Case 6:
6. in pneumococcal meningitis 1.C - ceftriaxone and clindamycin (epiglottitis)
Case 2: Case 7:
1.viral meningitis 1.D - peritonsillar abscess (moved uvula)
Case 3: Case 8:
1. Listeria monocytogenes 1.D - otic solution of neomycin and topical
2. ampicillin, add gentamicin when listeria steroids (otitis externa)
confirmed Case 9:
3. TMP-SMX 1.D - septic thrombophlebitis of internal jugular
Case 4: vein
1. Neisseria meningitis Case 10:
2. Antibiotics and CT 1. B – clinda and vanco
3. LP 2. B – skilled nursing facility wearing diapers
4. C - C5-C9 levels Case 11:
Case 4 continued: 1. mucormycosis
1. B - listeria Pneumonia:
Case 5: Case 1:
1. B - CD4 1. pulse ox or blood gases, give oxygen
2. D - ampho, 5FC 2. chest x-ray
3. cryptococcal antigen testing 3. sputum gram stain and culture
4.CD4<500 4. hypoxia, hypotension, tachycardia,tachypnea,
Case 6: altered mental status, hyponatremia, elevated BUN
1. herpes simplex encephalitis 5. empirirc therapy of CAP
2. C - CT without contrast
6. >65; diabetes, cirrhosis, COPD, CHF, asplenic, 3. people who've never been tested or not
hematologic malignancies; steroid users, renal recently - test twice to make sure 1st one
failure, HIV, organ transplant recipients, wasn't false
immunosuppressive medications 4. B – INH
7. >65; diabetes, cirrhosis, COPD, CHF, asplenic, 5. no cross-reaction with BCG
hematologic malignancies; steroid users, renal 6. C - 10% in a lifetime
failure, HIV, organ transplant recipients, 7. no effect
immunosuppressive medications 8. E - no one
8. nobody (only one revaccination needed!) Case 1 continued
Case 2: 1. nothing
1. A – gram negative rods 2. INH for 9 months
Case 3: 3. nothing
1. D – imipenem toxicity 4. INH for 9 months
Case 4: 5. INH for 9 months
1. C – coxiella burnetti 6. nothing
2. D - leptospirosis 7. INH
Case 5: Tropical diseases/animal-borne stuff
1. coccidioidomycosis Case 1:
2. serology with complement fixation titers 1. B - strongyloides (assoc w/ eosinophilia)
3. amphotericin Case 2:
Case 5 continued: 1. D - trichinosis
1. D - staphylococcus Case 3:
Tuberculosis: 1. tularemia (ulceroglandular syndrome)
Case 1: 2. serology
1. sputum AFB stain/culture, respiratory Case 3 continued.....
isolation, never PPD 1st in acutely symptomatic 1. A - dengue
patients 2. C - babesia
2. 6 months (2 months of RIPE  then 4 3. D - ehrlichia/anaplasma
months INH + rifampin) 4. A – brucellosis
3. no pyrazinamide or streptomycin 5. D - varicella
4. substitute rifabutin for rifampin and use 6. D – capnocytophagia
efavirenz 7. pasteurella
5. bone, brain, military TB, pregnancy 8. bacillary angiomatosis, bartonella henselae
6. A – pericardial and CNS 9. bartonella quintana
Case 1 continued….. 10. anthrax, A – cipro or doxy
1. A 11. pseudomonas
Case 2:
1.A - pleural biopsy Food poisoning and diarrhea
Case 3: Case 1:
1.A - new case of tuberculosis in workplace 1. campylobacter (most common), shigella,
Case 4: salmonella, yersinia
1.D - all 2. B – fecal leukocytes
PPD testing: 3. A – stool culture
Case 1: 4. quinolones when severe (low BP, tachycardia,
1. C - high risk groups fever, metabolic acidosis)
2. >5 (close contacts, HIV, steroids), >15 (no 5. scombroid (histamine fish poisoning)
tests) Case 1 continued
1. D
2. C – vibrio vulnificus 1. B – ceftriaxone and azithro
Postexposure prophylaxis Case 5:
Case 1: 1. D - partner not treated and he reinfected her
1. D - Ig and vaccine 2. D - hemophilus ducreyi
2. E - no treatment, A - 0.3% Syphilis:
3. B - immunoglobulin (VZIG) Case 6:
4. D - Ig and vaccine 1. jarisch reaction
5. C - contact and resp isolation 2. B - aspirin
6. Ig and booster vaccine Case 7:
7. no 1. D - do nothing and tell to get married
8. TIG only for never vaccinated, Tdap for at Case 7 continued.....
least one booster 1. B - syphilis
Case 1 continued..... 2. C - haemophilis ducreyi
1. C – Ig and vaccine 3. E - FTA
2. D - pneumococcus, meningococcus, etc. Warts:
Malaria: Case 8:
1. prophylaxis - mefloquine, 1. no test
atovoquone/proguanil 2. removal, freeze, burn, cut, laser, shave, and
2. treat with atovoquone/proguanil, oral melt with podofilox or trichloroacetic acid,
quinine and doxyclycine, artemisinin gradually remove with imiquimod
3. quinidine Case 9:
Sexually transmitted diseases: 1. scabies/pediculosis
Case 1: 2. oil scrape
1. Urethritis 3. permethrin, lindane
2. Urethral swab for PCR DNA genetic testing Urinary infections:
3. One drug for chlamydia (azithromycin or Case 1:
doxycycline) and one drug for gonorrhea 1. D - TMP-SMX for 3 days
(ceftriaxone, cefixime) 2. extend the length of therapy to 10–14 days
4. single-dose therapy with azithromycin with the same drugs
5. check for deficiency of terminal complement 3. stones, strictures, tumors, obstruction,
Case 1 continued..... diabetes
1. D - treat without parental consent 4. extend therapy to 2 weeks for acute prostatitis
2. C - chlamydia 5. C - pregnant patients
3. D - do nothing Case 1 continued:
4. D – blood test 1. C - amoxicillin
Case 2: 2. B
1. urinary wet mount and KOH preparation 3. D
Case 2 continued: Case 2:
1. B - trichomoniasis 1. B - private room with contact isolation
Case 3: Case 3:
1. suppression with fluconazole 1. D - proceed with surgery
Case 4: Fournier gangrene:
1. PID Case 4:
2. E - laparoscopy 1. A - Zosyn (pip/tazo)
3. pregnancy test 2. B - hospitalize for IV abx and consult with
4. high fever and WBC another surgeon
5. 2 weeks of doxycycline Case 4 continued.....
Case 4 continued..... 1. B - chlamydia
Case 5: 1. E - nitroblue tetrazolium disease (chronic
1. E - ceftriaxone granulomatous disease - missing NADPH burst)
Case 6: Case 4:
1. D – renal transplant rejection 1. D – treat with irtrapenem
Bone and joint infections: Catheter infections, etc:
Case 1: Case 1:
1. A - x-ray 1. B
2. A - biopsy 2. B
3. staph - ox, naf, MRSA - vanco, linezolid, 3. B
dapto, gram neg - cipro Case 2:
4. to detemine duration of treatment 1. A
5. never 2. C
Case 2: 3. A
1. B - arthrocentesis HIV disease:
2. E - culture of synovial fluid 1. A
3. ceftriaxone and vanco 2. D
4. D - polyarticular involvement, rash, 3. D
tenosynovitis 4. TMP-SMX, meropenem, minocycline
Case 2 continued: Case 1:
1. B - itraconazole 1. CMV retinitis
2. C - bartonelle henselae 2. A - dilated ophtho exam
3. F - amoxicillin/clavulinic acid 3. IV ganciclovir or foscarnet
Lyme disease: 4. uveitis
Case 1: 5. no routine primary prophylaxis, oral valganciclovir
1. D - oral doxycycline Case 2:
Case 2: 1. D - tuberculosis
1. A - offer reassurance 2. elevated AP and GGTP with normal bilirubin
2. grab head, NOT body 3. clarithromycin and ethambutol
Case 3: 4. azithromycin
1. just treat Case 2 continued:
2. oral (joint, rash, facial) 1.D - none (treatment is clarithro and
3. oral (joint, rash, facial), IV ceftriaxone ethambutol)
(cardiac, CNS) Case 3:
Case 4: 1. CNS toxo
1. C – stop the antibiotics 2. pyrimethamine/sulfa
Fever and neutropenia: 3. brain biopsy
Case 1: 4. TMP-SMZ
1. A - ceftriaxone Case 3 continued:
2. add vanco, then caspofungin 1. D - tuberculosis
3. C - add caspofungin Case 4:
Case 2: 1. CD4 < 500, viral load > 1000
1. E - slow rate of vanco infusion (Red Man 2. 3 meds, 2 + PI or efavirenz
syndrome) Additional cases:
2. A - continue abx and retain catheter 1. 2 drugs + PI/efavirenz
3. C – hepatosplenic candidiasis 2. 2 drugs + switch to efavirenz
4. C - voriconazole 3. start HAART now! (answer to "OR" question
Case 3: is D - continue meds)
4. 2 drugs + PI
5. genotype for sensitivity
6. D - zidovudine, lamivudine, nelfinavir
Case 5:
1. D
2. E
3. B
4. B