2006 Lange Outline
2006 Lange Outline
2006 Lange Outline
Contents
Contributors / vii
Preface / ix
Acknowledgments / x
How to Use This Book / xi
1. CARDIOVASCULAR MEDICINE / 1
2. DERMATOLOGY / 39
3. ENDOCRINOLOGY / 71
9. MUSCULOSKELETAL AND
CONNECTIVE TISSUE DISEASE / 227
v
vi
CONTENTS
Index / 551
Contributors
vii
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viii
CONTRIBUTORS
ix
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Acknowledgments
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How to Use This Book
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Cardiovascular
Medicine 1
I. ISCHEMIC HEART DISEASE / 3
A. Acute / 3
B. Chronic / 5
V. CARDIAC ARRHYTHMIAS / 14
A. Supraventricular Arrhythmias / 14
B. Ventricular Arrhythmias / 16
Copyright © 2004, 2001 by The McGraw-Hill Companies, Inc. Click here for terms of use.
2 Cardiovascular Medicine
XI. HYPERTENSION / 25
A. Essential Hypertension / 25
B. Secondary Hypertension / 26
C. Malignant Hypertension / 27
BIBLIOGRAPHY / 37
3
A. Acute
1. Unstable Angina
H&P Keys
Anginal pain with accelerating pattern including new onset or rest
symptoms. Midsternal squeezing or heaviness that may radiate to the
left shoulder or arm, jaw, neck, etc. Symptoms may be similar to
those present previously; however, usual alleviating factors (e.g., ni-
troglycerin [NTG], rest) may no longer be effective. Diaphoresis,
nausea, dyspnea, are common.
With coexisting left ventricular (LV) dysfunction an S3 or S4 may
be heard. In the presence of global ischemia or LV dysfunction, a
dyskinetic cardiac impulse may be palpated, and papillary muscle
dysfunction may cause the murmur of mitral regurgitation (MR). Is-
chemia-induced elevations in cardiac filling pressures often cause
pulmonary congestion and rales.
Diagnosis
Electrocardiogram (ECG): ST depression or elevation or T wave in-
d
version. Exercise tolerance testing (ETT; not performed in patients
with unstable angina) or pharmacologic stress testing is combined diagnostic
with an imaging agent, such as thallium, that permits the visualiza- decisions
tion of myocardial perfusion. Coronary angiography is indicated for
patients with high-risk clinical features. ISCHEMIC HEART DISEASE
Disease Severity
Acute Myocardial
High-risk clinical features are advanced age, ST segment depression, Infarction
heart failure, and elevation of biomarkers of cell damage (tro- Sudden onset of typical
ponins). Response to therapy and duration of symptoms dictate eval- squeezing or crushing
uation. Patients not medically stabilized or whose symptoms substernal chest pain; ECG
reemerge on therapy and those with prominent ischemic ECG find- with ST segment elevation
in two or more leads;
ings usually undergo coronary angiography. Location and severity of
increased CPK-MB
stenoses (e.g., left main, three-vessel) dictate management. isoenzymes and troponins.
Concept and Application Angina Pectoris
The vast majority of patients with unstable angina have underlying Chest pain lasting 1–15
minutes, precipitated by
coronary atherosclerosis (CAD). Unstable symptoms are usually
exertion, relieved by rest;
caused by plaque rupture with superimposed thrombosis. Platelet associated ECG changes
aggregation at site of plaque rupture with release of vasoconstricting with ST segment
mediators plays an important role. depression. Abnormal
exercise stress test or
Treatment Steps abnormal perfusion on
1. Continuous ECG monitoring. nuclear scan.
2. Bed rest, mild sedation, and treatment of extracardiac precipi- Unstable Angina
tants of increased oxygen demand (e.g., hypoxia, sepsis, anemia, Angina that increases in
uncontrolled hypo- or hypertension, etc.). frequency or severity, is of
3. Intravenous nitrates, heparin, thienopyridines, and aspirin new onset or occurs at
(acetylsalicylic acid [ASA]) and IIB/IIIA inhibitors are of proven rest. Coronary angiography
defines the extent and
efficacy. Thienopyridines, especially clopidogrel, reduce adverse
severity of disease and
events including death, myocardial infarction (MI), and stroke. need for intervention;
Agents that block the platelet glycoprotein IIB/IIIA receptor sub- exercise testing with
stantially reduces mortality and the occurrence of acute MI pri- perfusion imaging useful for
marily in patients who undergo coronary intervention. Intra- risk stratification and
venous NTG often is successful when other routes fail. β-Blockers treatment decisions.
are useful and also reduce the incidence of acute MI (AMI).
4
CARDIOVASCULAR MEDICINE Ischemic Heart Disease
management
decisions d pulmonary rales.
Diagnosis
ECG: ST segment elevation MI (STEMI) and T wave inversions with
ISCHEMIC HEART DISEASE
subsequent evolution of Q waves. Non-STEMI: ST depression and T
wave inversions are seen. Elevations in cardiac enzymes: creatine kinase
Acute Myocardial (CK), specifically CK-MB isoforms, peaks at 24 hours; troponins I and
Infarction T, and myoglobin detect cell injury early in the course of MI. L-lactate
Open the occluded artery dehydrogenase (LDH) peaks at 3–5 days after MI. Two-dimensional (2-
to restore cardiac blood D) echocardiography and technetium nuclear scans can be valuable in
flow with thrombolytic detecting abnormalities in heart function and blood flow.
therapy or angioplasty;
monitor and treat serious Disease Severity
dysrhythmias, aspirin to Mortality increases with the number of ECG leads showing ST seg-
decrease clot formation,
ment elevation. Cardiac imaging with echocardiography (echo) or
β-blockers to decrease
myocardial oxygen needs,
radionuclide ventriculography (RVG) can help assess the extent and
oxygen and pain relief. prognosis of infarction by measuring LV systolic function and ejec-
Angina Pectoris tion fraction. Echo aids in diagnosis of MI complications, e.g., LV
Drug therapy with nitrates, thrombus or aneurysm, pericardial effusion, free wall and septal rup-
β-blockers, aspirin to ture, and MR.
prevent acute MI; calcium
antagonists are second-line Concept and Application
therapy. Intervention with MI results from the abrupt cessation of myocardial blood flow. The
bypass surgery or coronary vast majority of cases of MI are due to CAD. Plaque rupture, platelet
intervention for patients aggregation, and release of vasoactive mediators leads to thrombosis,
with refractory symptoms spasm, and coronary occlusion. Elevation of myocardial oxygen de-
or severe coronary disease.
mand (e.g., tachycardia) can increase myocardial cell damage. Irre-
Unstable Angina versible cell death occurs, usually within 6 hours, if therapy is not
Drug therapy for
given to restore blood flow or if spontaneous improvement does not
stabilization with
intravenous nitroglycerin, occur. Nonatherosclerotic causes of MI, such as embolism, trauma,
aspirin, heparin, vasculitis, or hypercoagulable states, are less common.
clopidogrel, glycoprotein
IIB/IIIA inhibitors, and β- Treatment Steps
blockers. Coronary 1. Supplemental oxygen, and continuous monitoring to detect po-
interventions and coronary tentially lethal dysrhythmias are important first steps.
bypass surgery for severe 2. Analgesia as necessary.
coronary disease. 3. Primary angioplasty of occluded artery optimum. Thrombolysis if
PTCA unavailable.
5
Treatment Steps
1. Avoid smoking and cocaine.
2. Nitrates and calcium channel blockers are usually effective; the
effect of β-blockers is unpredictable; in some they can precipitate
spasm.
3. α-Adrenergic β-blockers (e.g., prazosin) can be helpful.
B. Chronic
1. Stable Angina Pectoris
H&P Keys
Episodic chest discomfort, often described as heaviness or squeezing
lasting 1–15 minutes. Pain may radiate to the jaw, neck, shoulder, or
the left arm. Symptoms typically are precipitated by exertion, cold
weather, or emotional upset, and relieved by rest. Family history of
premature CAD, diabetes, hyperlipidemia, hypertension, cigarette
smoking. Exam may be normal, but if the patient is examined dur-
ing an ischemic episode, an S3 or S4 may be heard.
Diagnosis
ECG may be normal if patient is asymptomatic, but evidence of a
prior MI or ischemic ST and T wave changes may be noted. ETT,
pharmacologic stress testing, and exercise echo are useful.
Disease Severity
Global ECG changes suggest multivessel CAD. Quantitation of is-
chemic burden can be accomplished with perfusion imaging. Coro-
nary arteriography documents presence, extent, and severity of CAD
and suitability for revascularization.
6
CARDIOVASCULAR MEDICINE Heart Failure (HF)
A. Left-Sided
1. Low Output
H&P Keys
History may elicit cause, e.g., CAD, prior MI, hypertension, or valvu-
lar disease. Symptoms: fatigue, weakness, reduced exercise tolerance,
exertional or rest dyspnea, paroxysmal nocturnal dyspnea (PND), or-
thopnea, nocturia. Physical findings: displaced cardiac impulse; S3
murmurs, especially MR; rales; rarely Cheyne–Stokes respiration.
7
U
dominantly systolic dysfunction, activation of sympathetic nervous
system, renin–angiotensin–aldosterone axis, and hormonal (antidi-
uretic hormone [ADH]) elaboration occurs. cram facts
Treatment Steps
1. Search for precipitating causes of decompensation (infection, TREATMENT DECISIONS
MI, uncontrolled hypertension, dietary indiscretion, etc.). Cor-
rect underlying diseases, e.g., valvular disease, ischemia, arrhyth- Heart Failure
mia. Left-Sided Heart Failure
2. Pharmacologic therapy is tailored to the stage of the disease (A, Find and treat or correct
high risk to develop congestive heart failure [CHF]; B, struc- any precipitating or
tural disease without symptoms; C, prior or current symptoms; underlying cause, e.g.,
D, refractory symptoms requiring special intervention). valvular heart disease.
3. Most symptomatic patients should be managed with a combina- Medical treatment with
angiotensin-converting
tion of four drugs: diuretics, angiotensin-converting enzyme
enzyme inhibitors,
(ACE) inhibitors, β-blockers, and digitalis. diuretics, digoxin, and β-
4. Diuretics decrease symptoms related to volume overload. blockers. Anticoagulation
5. ACE inhibitors reduce afterload, increase cardiac output, and with severe dysfunction.
decrease mortality (Stages B–D). Severe decompensation
6. Digoxin augments contractility and reduces symptoms but does may require
not improve prognosis (Stages C and D). sympathomimetic agents,
a left ventricular assist
7. β-Blockers help modulate neurohumoral activation, blunt excess
device or cardiac
sympathetic tone, and are used in all stable, symptomatic pa- transplantation.
tients (Stages C and D).
High-Output Heart Failure
8. With severe decompensation, sympathomimetic amines (do- Find and treat the
butamine) or phosphodiesterase inhibitors (milrinone) are used. underlying cause, e.g.,
9. β-Natriuretic hormone therapy is also valuable to rapidly relieve thyrotoxicosis and/or
symptoms and improve hemodynamics. arteriovenous fistula;
10. Stage D patients may require cardiac transplantation or an LV diuretics.
assist device. Right-Sided Heart Failure
Correct underlying
2. High Output problems that resulted in
pulmonary obstruction or
H&P Keys
hypertension; diuretics and
Signs of hyperdynamic circulation, e.g., brisk pulses. Dyspnea, or- treatment similar to left-
thopnea often present. Angina in patients with CAD. sided failure.
Disease Severity
Assessed primarily on degree of disability.
Concept and Application
Patients often have an underlying syndrome causing elevation in
metabolic demands and/or cardiac output, e.g., arteriovenous fistu-
las, Paget’s disease, hyperthyroidism, anemia.
Treatment Steps
Treatment of underlying cause.
8
CARDIOVASCULAR MEDICINE Heart Failure (HF)
B. Right-Sided
H&P Keys
Seen with history of LV failure, RV infarction, lung disease, pul-
monic stenosis, pulmonary emboli, myocarditis, etc. Clinical find-
ings: fatigue, RV heave and gallops, JVD, hepatomegaly, ascites,
edema, atrial arrhythmias.
Diagnosis
Signs and symptoms of RV or biventricular failure in setting of pre-
disposing condition. ECG may show RV or right atrial (RA) hyper-
trophy. Echo may show RV dilatation and hypokinesis.
Disease Severity
Often suggested by degree of edema and JVD on exam. Ascites sug-
gests more severe decompensation. Echo and RVG can quantitate
the degree of functional impairment and dilatation
Concept and Application
RV outflow obstruction (e.g., pulmonic stenosis), pulmonary hyper-
tension resulting from chronic LV failure, obstructive lung disease,
chronic pulmonary emboli (cor pulmonale), etc., lead to chronic
overload of the RV. LV cardiac output is also reduced. Elevated pres-
sures in systemic vasculature lead to accumulation of fluid in the ex-
travascular space. See Figure 1–1.
Figure 1–1. Echocardiograms recorded in end-diastolic and end-systolic in a normal ventricle (upper panel) with
uniform systolic contraction and a normal ejection fraction and an abnormal ventricle with reduced contraction
during systole. The cross-hatched areas outline the left ventricular cavities.
9
B. Hypovolemic Shock
Inadequate circulatory volume caused by hemorrhage or dehydra-
tion. Common with trauma, surgery, vomiting, diarrhea, and some
skin disorders.
H&P Keys
Weakness, postural light-headedness in setting of blood loss or fluid
loss, e.g., history of hemorrhage, melena. Postural hypotension and
tachycardia, low jugular venous pressure.
Diagnosis
Measurement of postural blood pressure and heart rate changes, na-
sogastric aspiration, hemoglobin, blood urea nitrogen (BUN):creati-
nine ratio.
Disease Severity
Level of blood pressure, severity of impaired organ perfusion, men-
tation, hourly urine output.
Treatment Steps
1. Acute, rapid volume restoration with blood, crystalloid, or colloid
solutions.
2. Temporary maintenance of peripheral perfusion pressure with
vasoconstrictors.
3. Identification and treatment of the cause or source of blood loss
or fluid loss.
C. Obstructive Shock
Impairment of venous return to the right ventricle or left ventricle
occurs secondary to obstruction in the venous system, pulmonary
artery, or pericardium. Observed with acute pulmonary embolus or
tamponade with a pericardial effusion. Effusion is caused by trauma,
infections, malignancy, connective tissue disease, and renal failure.
H&P Keys
Dyspnea, orthopnea, elevated venous pressure, chest pain, hemopty-
sis, pulsus paradoxus, faint and distant heart sounds, or pulmonary
hypertension.
110
DISEASES AND DISORDERS OF THE DIGESTIVE SYSTEM Colon
D. Appendicitis
H&P Keys
Typically, the first symptom is upper midline or perimbilical pain.
Discomfort develops over several hours and is followed by anorexia,
nausea, and vomiting. Once the serosal surface of the appendix be-
comes inflamed, the pain and tenderness shifts to McBurney’s point.
The usual presentation of periumbilical pain shifting to the right
lower quadrant (RLQ) is seen less frequently in older patients, in
whom diffuse pain and nonlocalized tenderness are more common.
Perforation is suspected if the temperature is > 38°C or if the leuko-
cyte count is > 15,000 cells/mm.
Diagnosis
Appendicitis is a clinical diagnosis, supported by carefully selected
laboratory studies. Patients with historical features and physical ex-
aminations do not need additional diagnostic studies. Immediate
surgical exploration is indicated for the following:
• Abnormal gas pattern on abdominal radiographs: RLQ ileus or
diffuse small bowel air–fluid levels are seen in 62% of patients
with uncomplicated appendicitis, 71% of those with periappen-
diceal phlegmon, and 97% of those with gangrenous appendicitis
or perforation.
111
Figure 4–2. Ulcerative colitis: diagnosis. (Adapted, with permission, from Hanauer SB, Meyers S. Management of
Crohn’s Disease in Adults. ACG Practice Parameters. Am J Gastroenterol 1997;92:559–566.)
Treatment Steps
Goal: symptomatic improvement with tolerance of medical therapy.
See Figure 4–3.
1. Mild to moderate disease: Anticipate 50% response. Oral amino-
salicylates: sulfasalazine 3–6 g/day or mesalamine 3.2–4.8 g/day.
Antibiotics: metronidazole 10–20 mg/kg/day, ciprofloxacin 500
mg bid. Add PPI for gastroduodenal Crohn’s disease. Use topical
mesalamine or corticosteroids for distal colonic disease.
2. Moderate to severe disease: Prednisone, 40–60 mg/day until im-
provement, then taper; treat concomitant infection; nutritional
support; azathioprine/6-MP.
3. Severe/fulminant disease: hospitalization; IV corticosteroids; an-
tibiotics; surgical consultation for obstruction or progression to
114
DISEASES AND DISORDERS OF THE DIGESTIVE SYSTEM Colon
Figure 4–3. Crohn’s disease management. (Adapted, with permission, from Kornbluth A, Sachar DB. Ulcerative Colitis in Adults. ACG Practice Parameters. Am J
Gastroenterol 1997;92:204–211.)
115
G. Intestinal Obstruction
H&P Keys
Crampy, spasmodic abdominal pain, vomiting, borborygmi, abdomi-
nal distention, obstipation; can develop slowly (months or years) or
acutely (over hours). With mechanical obstruction, distress is appar-
ent by extreme restlessness. With ileus, pain is usually less severe.
Careful inspection of skin; palpation of umbilicus, lower-trunk in-
guinal, and femoral areas to detect hernias, intra-abdominal masses,
hepatosplenomegaly; rectal and vaginal exam for masses and occult
blood. Bowel sounds are infrequent and hypoactive in ileus. In ob-
struction, bowel sounds become loud, high-pitched, and hyperactive.
Diagnosis
Biochemical and hematologic tests including: CBC, levels of amy-
lase, alkaline phosphatase, aspartate aminotransferase, alanine
aminotransferase, lactate dehydrogenase; acid–base balance;
116
DISEASES AND DISORDERS OF THE DIGESTIVE SYSTEM Colon
H. Diverticulitis
H&P Keys
Acute diverticulitis commonly manifests with fever, localized left
lower quadrant abdominal pain, leukocytosis, and a palpable mass
seen, typically, in American patients above the age of 35.
Diagnosis
Leukocytosis, roentgenographic obstruction series, ultrasonographic
or CT imaging. As the process resolves, colonic evaluation (either
colonoscopy or flexible sigmoidoscopy and barium enema) to con-
firm the diagnosis and exclude other colonic pathology (e.g.,
Crohn’s disease, carcinoma). The risk of perforation in colonoscopy
is higher within 4 weeks of resolved diverticulitis.
Disease Severity
Complications include gross perforation and peritonitis: a surgical
emergency requiring aggressive resuscitation, broad-spectrum an-
tibiotics, laparotomy, and colonic resection with fecal diversion.
Elective resection is indicated after two documented episodes of
acute diverticulitis, depending on age, operative risk, and severity.
Immunocompromised patients should be treated surgically after
one attack due to the higher risks and higher mortality. Chronic di-
verticulitis is acute diverticulitis complicated by colonic fistula, large-
bowel obstruction, or stricture. Colovesical fistula is the most com-
mon fistula. All symptomatic manifestations of chronic diverticulitis
are treated surgically by resection and primary anastomosis after res-
olution.
Treatment Steps
1. Diet and lifestyle: acute uncomplicated diverticulitis––bowel rest
and antibiotic therapy. Outpatient treatment: clear liquids until
symptoms resolve. Inpatient: clear liquids until symptoms resolve;
NPO, IV hydration.
2. Antibiotic outpatient regime includes: ciprofloxicin and met-
117
I. Constipation
H&P Keys
Onset and duration of complaint: constipation present from birth or
neonatal period suggests congenital disorder; recent onset demands
a workup for organic disorders. Frequency of defecation, defecatory
difficulties such as excessive straining, discomfort, sense of incom-
plete evacuation. Drug history. Careful physical examination with ab-
dominal palpation for distention, retained stool, prior surgical proce-
dures, autonomic dysfunction; anorectal and perineal examination.
Diagnosis
Flexible sigmoidoscopy and barium enema; exclude metabolic and
endocrine disorders such as diabetes mellitus, hypothyroidism, hy-
percalcemia, hypokalemia; exclude muscular collagen vascular and
neurogenic disorders. Anal manometry and full-thickness rectal
biopsies when Hirschsprung’s disease is suspected.
Disease Severity
Colonic transit studies, defecography, and anal manometry for pa-
tients with severe constipation who have not responded to simple di-
etary measures.
Concept and Application
Impairment in large-bowel transit can be a primary motor disorder,
in association with a large number of diseases, and a side effect of
many drugs.
Treatment Steps
1. Adequate dietary fiber.
2. Behavioral approaches; biofeedback.
3. Discouragement of routine use of laxatives except bulk-forming
agents.
J. Peritonitis
H&P Keys
Severe abdominal pain and rigidity, intercostal breathing, fever,
tachycardia, hypovolemia, tenderness on direct and referred palpa-
tion, voluntary guarding, tenderness on rectal and pelvic exams, ten-
derness on percussion, loss of liver dullness, decrease or absence of
bowel sounds.
Diagnosis
Leukocytosis with left shift or leukopenia, hemoconcentration,
metabolic acidosis, hyperkalemia, paralytic ileus, or free air.
Disease Severity
Suppurative peritonitis has abrupt onset and relatively short course
with rapid progression. Mortality from fluid shifts, hypovolemia, and
septic shock with resultant renal, respiratory, cardiac, and hepatic
failure.
118
DISEASES AND DISORDERS OF THE DIGESTIVE SYSTEM Rectum
V. RECTUM
B. Hemorrhoids
H&P Keys
Anal discomfort, pruritus ani, fecal soiling, prolapse, bleeding, pain.
Diagnosis
Flexible sigmoidoscopy or anoscopy. Occult bleeding in the stool re-
quires a complete colonic evaluation, regardless of the presence of
hemorrhoids. Hemorrhoids are classified according to their degree
119
C. Anal Fissure
H&P Keys
Severe pain during or after defecation associated with scant, bright
red rectal bleeding. Most commonly found in young and middle-
aged adults.
Diagnosis
Inspection after spreading the buttocks. Anoscopy is difficult with-
out topical anesthesia. Linear tears are perpendicular to the dentate
line.
Disease Severity
Can progress to chronic fissure.
Concept and Application
Because elliptical anal sphincteric fibers offer less muscular support
posteriorly, 90% occur posterior midline. Lateral tears suggest un-
derlying disease (IBD, proctitis, leukemia, carcinoma).
Treatment Steps
1. High-fiber diet and adequate fluid intake.
2. Topical anesthetic preparations for symptomatic relief.
120
DISEASES AND DISORDERS OF THE DIGESTIVE SYSTEM Rectum
D. Anorectal Abscess
H&P Keys
Acute pain and swelling. Pain in absence of swelling with small inter-
sphincteric or pelvorectal abscesses. Sitting, movement, defecation
increase pain. Antecedent history of constipation, diarrhea, trauma.
Fever, malaise, and purulent foul-smelling drainage are common.
Associated medical diseases include diabetes, hypertension, heart
disease, IBD, and a neutropenic state as a result of hematologic ma-
lignancy.
Diagnosis
Inspection of the perineum reveals redness, heat, swelling and ten-
derness, drainage from infected crypt orifice. Rectal examination is
difficult without anesthesia.
Disease Severity
Delay in making the diagnosis can lead to necrotizing anorectal in-
fection and increases risk of overwhelming sepsis.
Concept and Application
Obstruction, stasis, and infection of anal glands is most common
cause. Obstruction may occur as a result of trauma, eroticism, diar-
rhea, hard stools, and foreign bodies.
Treatment Steps
1. Abscesses require drainage. Superficial abscesses can be drained
under local anesthetic in outpatient setting. All others require
drainage in the operating room with anesthesia and surgical in-
strumentation.
2. Antibiotics are not necessary for otherwise healthy patients. Peri-
operative antibiotics are used for patients with underlying disease
such as acute leukemia, valvular heart disease, diabetes.
3. Postoperative management: inspection to ensure proper healing,
sitz baths, analgesia, bulk-forming agents to soften stool.
E. Anorectal Fistula
H&P Keys
Chronic purulent drainage. Prior history of anorectal abscess. Pain
with defecation but not as severe as with anorectal abscess or anal fis-
sure. Perianal skin may be excoriated.
Diagnosis
Inspection of the perineum usually reveals a red, granular papule
from which pus is expressed. Anoscopy and sigmoidoscopy to iden-
tify primary orifice and proctocolitis.
Disease Severity
Multiple secondary openings suggest either Crohn’s disease or
hidradenitis suppurativa.
Concept and Application
Primary orifice is at level of dentate line. Secondary orifice is any-
where else on the perineum.
Treatment Steps
1. Anorectal fistula is approached surgically, with postoperative care
similar to anorectal abscess.
121
F. Pilonidal Disease
H&P Keys
Pain, swelling, drainage midline skin lesion of internatal or gluteal
cleft seen most commonly in young men.
Diagnosis
Inspection: characteristic midline location. Appearance and lack of
communication with anorectum distinguish pilonidal disease from
anorectal fistula and hidradenitis suppurativa.
Disease Severity
Acute abscess versus chronic drainage.
Concept and Application
Common acquired lesion of coccygeal skin, possibly induced by lo-
cal stretching forces. Small skin pits secondarily invaded by hair pre-
cede development of draining sinus or abscess.
Treatment Steps
1. Acute pilonidal abscess requires incision, drainage, and hair re-
moval.
2. Chronic draining pilonidal lesions require surgical closure.
VI. GALLBLADDER
A. Acute Cholecystitis
H&P Keys
Acute onset of right upper quadrant (RUQ) pain (typically radiating
to the right shoulder blade), nausea, vomiting, and fever. Murphy’s
sign: inspiratory arrest elicited when palpating under the liver.
Diagnosis
Laboratory leukocytosis with preponderance of polys and bands. Mi-
nor elevations in aminotransferase levels and bilirubin. Ultrasound
is the preferred initial screening study. Cholescintigraphy (hepato-
iminodiacetic acid [HIDA], diisopropyl iminodiacetic acid
[DISIDA]) when clinical suspicion is high but ultrasound is normal.
Disease Severity
Secondary bacterial infection can progress to empyema, gangrene,
and perforation.
Concept and Application
Most common cause of acute cholecystitis is obstruction of a dis-
tended gallbladder with concentrated bile. Acalculus (5–10%) oc-
curs in setting of major surgery, critical illness, extensive trauma,
and burns.
Treatment Steps
1. Initial: hospitalization, NPO, intravenous hydration, and intra-
venous antibiotics to cover enteric organisms.
122
DISEASES AND DISORDERS OF THE DIGESTIVE SYSTEM Gallbladder
B. Cholelithiasis
H&P Keys
Biliary pain, nausea, and vomiting. Predisposing factors include obe-
sity, oral contraceptives, pregnancy, clofibrate, ileal disease or resec-
tion, and genetic factors.
Diagnosis
Ultrasonography has > 90% sensitivity in diagnosing gallstones.
Disease Severity
Biliary pain lasting for more than 3 hours indicates progression to
cholecystitis.
Treatment Steps
1. Asymptomatic stones require no treatment.
2. Symptomatic gallbladder stones are treated with cholecystectomy.
3. Oral bile acid therapy (Ursodiol) is a treatment option for mildly
symptomatic patients with small (< 10 mm) cholesterol stones
within a functioning gallbladder.
4. Common bile duct stones, particularly in the elderly and those
with comorbid disease are best treated with endoscopic retro-
grade cholangiopancreatography (ERCP).
Diagnosis
Leukocytosis with a left shift, mild elevation of serum transaminases
and alkaline phosphatase, hyperbilirubinemia, serum amylase level.
Ductal dilatation documented by ultrasonography or CT. Lack of di-
latation does not exclude obstruction. Gold standard is ERCP for di-
agnosis and management. Magnetic resonance cholangiopancre-
atogram (MRCP) and spiral CT provide excellent images of
common duct stones and are among available noninvasive alterna-
tives to diagnostic ERCP.
Disease Severity
Progression leads to ascending cholangitis and endotoxemia with
shock and liver abscess.
VII. LIVER
A. Hepatitis
H&P Keys
Pertinent information: age, gender, race, preceding episodes, his-
tory of chronic liver disease or cirrhosis, alcohol consumption
(Table 4–14), drug and toxin exposure (include herbal as well as
“traditional” medications), occupation and work environment, sex-
ual history, immunologic and nutritional status, immunizations,
travel history, and family history.
Hepatitis may present with a flulike or serum sickness syndrome,
anorexia, malaise, fever, arthralgia, arthritis, rash, and/or an-
gionecrotic edema, with or without jaundice, dark urine, light stools,
abdominal discomfort. Nonspecific constitutional symptoms may be
present for a short or protracted time.
Physical findings may be subtle or nonexistent. Findings are of-
ten reflective of the duration and severity of liver disease and are
common to all forms of hepatitis and include jaundice,
hepatomegaly (typically mild), lymphadenopathy, ascites,
splenomegaly, encephalopathy. Some findings are typically associ-
ated with alcoholic liver disease: palmar erythema, Muehrcke’s lines
and white nails, Dupuytren’s contracture, parotid and lacrimal gland
enlargement.
1. Viral Hepatitis
Diagnosis
Use specific tests for viral antigens, viral antibodies, and/or nucleic
acids.
• Acute hepatitis A, B, C (see Tables 4–15, 4–16, and 4–17).
• Acute δ coinfection: presence of IgM anti-HBc and IgM anti–
hepatitis D virus (HDV) or HDV RNA.
• Acute δ superinfection: presence of IgM anti-HDV or HDV RNA.
• Acute hepatitis E: IgM anti–hepatitis C virus (HCV) and compati-
ble clinical features.
4-14
CAGE QUESTIONNAIRE
Scoring: 1 point for each yes answer. A total of 2 or more indicates a likelihood of underlying alcoholism.
124
DISEASES AND DISORDERS OF THE DIGESTIVE SYSTEM Liver
4-15
DIAGNOSTIC SEROLOGY FOR ACUTE VIRAL HEPATITIS
Serology
2. Chronic Hepatitis
• Hepatitis A: no chronic state.
• Hepatitis B: presence of hepatitis B surface antigen (HBsAg), hep-
atitis Be antigen (HBeAg), HBV DNA, IgG anti-HBc.
• Chronic C: presence of anti-HCV ELISA. False positives may be
seen in low-risk populations. Confirm with anti-HCV RIBA or HCV
RNA.
• Chronic D: HBsAg and HDV RNA.
3. Alcoholic Hepatitis
5. Autoimmune Hepatitis
Suspect among women ages 15–35 and again at menopause: 40%
present with the abrupt onset of illness that can resemble acute viral
or toxic hepatitis; 25% have cirrhosis at the time of presentation.
The disease is fatal without treatment. History of concurrent autoim-
mune disease: autoimmune thyroiditis, vasculitis, ulcerative colitis,
Graves’ disease, vitiligo, insulin-dependent diabetes. Symptoms: fa-
tigue, jaundice, bleeding, easy bruisibility, RUQ pain. Transaminases
range from 200 to 1,000 U/dL; total serum globulin, γ-globulin, or
immunoglobulin G level ≥ 1.5 normal; antinuclear antibodies
(ANAs), smooth muscle antibodies (SMAs), antibodies to liver/kid-
ney microsome type 1 (anti-LKM1) titers ≥ 1:80 in adults or ≥ 1:20 in
children, antibodies to soluble liver antigen (anti-SLA), antimito-
chondrial antibodies (AMAs).
Concept and Application
Hepatitis, a necroinflammatory process of the liver parenchyma, can be
either acute (< 6-month duration) or chronic (> 6-month duration).
Acute hepatitis can resolve, progress to hepatic failure, or continue as
chronic hepatitis leading to cirrhosis and hepatocellular carcinoma.
4-16
CLINICAL SITUATIONS/SYNDROMES ASSOCIATED WITH HBV INFECTION
IgM
HBs Anti- Anti- anti- HBe HBV Anti-
Ag HBs HBc HBC Ag DNA HDV
Acute hepatitis B + − + + + + −
Chronic hepatitis B + +/− + −+/− + −
Healthy carrier + +/− + − − − −
Vaccinated − + − − − − −
Recovered HVB − + + − − − −
Acute hepatitis D + − + + +/− +/− +
Chronic hepatitis D + − + − +/− +/− +
125
Anti-HCV Anti-HCV
(ELISA) (RIBA) ALT HCV RNA
4-18
PATTERNS OF HEPATOTOXICITY
Treatment Steps
Treatment for acute hepatitis is primarily supportive care. Basic prin-
ciples include:
1. Avoidance of potentially liver-damaging circumstances, fluid
and electrolyte replacement, ambulation within the bounds of
fatigue, a regular or high-protein diet (in the absence of en-
cephalopathy).
2. Follow physical exam and biochemical tests: prothrombin time
is the best biochemical indicator of prognosis; also follow biliru-
bin, ALT, AST twice weekly while values are rising, weekly during
the plateau, then at lesser intervals until normalized.
3. Consideration for hospitalization is indicated for severe
anorexia, vomiting, changes in mentation and biochemical
changes, including a bilirubin value > 15 or 20 mg/dL, persis-
tence hyperbilirubinemia, rapidly falling aminotransferase activ-
ity with a rising bilirubin, increasing protrombin time, and other
evidence of hepatic failure.
4. Acute viral hepatitis. Most individuals with adequate family and
medical support are treated in the outpatient setting. Hospital-
4-19
SOME IMPORTANT INDUSTRIAL AND ENVIRONMENTAL TOXIC CAUSES
OF HEPATITIS
Chemical Uses
4-21
PERSONS RECOMMENDED FOR HBV PROPHYLAXIS (VACCINE
OR HBIG OR BOTH)
Preexposure prophylaxis
Persons with occupational risk, including clients and staff of institutions
Clients/staff of institutions for developmentally disabled
Patients on hemodialysis
Sexually active homosexual men
Sexually active heterosexual men and women
Users of illicit injectable drugs
Recipients of certain blood products, e.g., clotting factors
Household and sexual contacts of HBV carriers
Adoptees from countries of high HBV endemicity
Populations with high endemicity
Inmates of long-term correctional facilities
International travelers to HBV-endemic areas for > 6 months
Postexposure immunoprophylaxis for hepatitis B
Type of Exposure Immunoprophylaxis
Perinatal exposure Vaccination + HBIG
Sexual, acute infection HBIG ± vaccination
Sexual, carrier Vaccination
Household contact, carrier Vaccination
Household contact, known exposure HBIG ± vaccination
Infant < 12 months, acute case, primary caregiver HBIG ± vaccination
Inadvertent percutaneous or permucosal Vaccination + HBIG
128
DISEASES AND DISORDERS OF THE DIGESTIVE SYSTEM Liver
4-22
ALGORITHM FOR THERAPY OF HEPATITIS B
Initial evaluation
• Serial ALT, HBsAg, HBeAg, HBV DNA
• Liver biopsy
• Review side effects and expected results
• Verify lack of contraindications
Initial therapy
• α-interferon, 5 µ daily or 10 µ three times weekly for 16–24 weeks
Monitor during therapy
• Every 2–4 weeks:
Signs and symptoms
ALT, AST, bili, albumin, CBC + differential
• At 2 and 4 months
HBeAg, HBsAg, protime, TSH
• Follow up after therapy
• Every 2–3 months
Signs and symptoms
ALT, AST, bili, albumin, CBC
• At 6 months
HBsAg, protime, TSH
4-23
SIDE EFFECTS OF INTERFERON
Early
Severe/life-threatening syndrome
None
Mild:
Influenza-like
Late
Severe/life-threatening
Severe depression/suicide
Acute renal failure
Cardiotoxicity
Seizures
Exacerbation of preexisting autoimmune disease
(e.g., inflammatory bowel disease)
Mild
Fatigue
Emotional lability
Bone marrow suppression
129
B. Hepatocellular Carcinoma
H&P Keys
Hepatocellular carcinoma (HCC) has a specific geographic distribu-
tion. HCC is common in sub-Sahara Africa, China, Japan, and the
Southeast, where it is strongly associated with chronic hepatitis HBV
infection and repeated heavy exposure to the mycotoxin aflatoxin B.
Intermediate risk for HCC in areas of southern Europe and Japan is
associated with HCV infection. In other parts of the world, HCC oc-
curs as a late complication of cirrhosis (particularly alcoholic liver
disease and hemachromatosis).
Clinical Presentation—Among southern black Africans and Chinese
patients, HCC occurs in relatively young (mean age 33 years)
and apparently healthy individuals. Typically, the disease is silent
in its early stages. Onset of symptoms: abdominal pain, fullness,
early satiety, anorexia, and weight loss corresponds to advanced
disease. Physical findings include hepatomegaly, hepatic arterial
bruit, ascites, splenomegaly, jaundice, fever. Among individuals
with cirrhosis, an unexplained deterioration in liver function
may be the only clue.
Diagnosis
matic individuals (> 75%) from these regions which will have a
diagnostic level (> 500 ng/mL) at presentation. In low-incidence
regions, the AFP test is far less useful as a single tumor marker. A
combination of tumor markers: tumor-associated isoenzymes of
γ-glutamyl transferase (elevated total GGT leads to isoenzyme
fractionation) and the abnormal prothrombin, des-γ-carboxy
prothrombin may be abnormal when the AFP is nondiagnostic.
Newer molecular techniques amplify small amounts of tumor-
specific gene-transcripts for albumin and α-fetoprotein mRNA
promise to enhance detection of malignant hepatocytes in circula-
tion.
Imaging—In patients with suspected HCC, the combination of ar-
terial phase and portal venous phase CT imaging will detect the
majority of tumors. Magnetic resonance (MR) is useful for de-
tecting small (< 5 cm) HCC, differentiating HCC from heman-
giomas and evaluating the proximity of HCC to adjacent blood
vessels. Dynamic MR is superior to hepatic arteriography. Ultra-
sound is less useful. Hepatic scintigraphy has surpassed other
imaging modalities.
Pathology—A definitive diagnosis depends on histologic appear-
ance. Ultrasound or CT-guided percutaneous biopsy carries the
risk of systemic dissemination or seeding of the tumor but is of-
ten necessary.
Disease Severity
Symptomatic HCC carries a poor prognosis. In Africa and China, av-
erage survival is less than 4 months from the onset of symptoms. In
other geographic regions the course may be somewhat more indo-
lent. Prognosis is more favorable when tumors are detected prior to
the onset of symptoms. A high incidence of extrahepatic recurrence
and reappearance of tumor in the donor liver after transplantation
reflects early hematogenous spread of micrometastases. The use of
reverse transcriptase polymerase chain reactions to amplify small
amounts of tumor-specific gene transcripts offer promising results to
detect small numbers of malignant hepatocytes in peripheral blood.
Concept and Application
HBV and HCV promote mutagenesis indirectly by stimulating
necroinflammatory activity in the liver. Emerging evidence indicates
that both viruses also have direct oncogenic potential. Geographic
differences in the incidence of HCC among populations with com-
parable dietary aflatoxin B1 exposure may be due to individuals’ ca-
pacity to detoxify mutagenic metabolites.
Treatment Steps
1. In areas where HBV-related HCC is common, there is some hope
that early vaccination will decrease the incidence of HCC. For
HCV-related HCC, vaccination remains a distant goal. Parenteral
drug abuse and the high incidence of sporadic HCV infection in
countries at intermediate risk for HCC offer little hope for reduc-
ing the incidence of HCV-related HCC.
2. Dismal results are obtained with all forms of treatment for symp-
tomatic tumors. Newer molecular markers and imaging modali-
ties may increase the detection of small, asymptomatic and poten-
tially resectable HCC in high-risk individuals or populations.
Small (< 5 cm) lesions may be resectable when the tumor appears
131
Treatment Steps
1. Cirrhotic ascites: dietary salt restriction, diuretic therapy. Consider
large-volume paracentesis, peritoneovenous shunting, transjugu-
lar intrahepatic portosystemic shunting (TIPS), and liver trans-
plantation for refractory ascites.
2. Spontaneous bacterial peritonitis (SBP): cefotaxime is effective for ini-
tial therapy.
3. Encephalopathy: correction of any precipitant factors (GI bleeding,
infection, electrolyte imbalance), reduction of dietary protein,
and decrease in intestinal ammonia absorption of nonabsorbable
carbohydrates, especially lactulose.
4. Acute variceal bleeding: variceal band ligation, sclerotherapy,
and/or pharmacologic therapy: somatostatin, octreotide, vaso-
4-24
CHILD–TURCOTTE–PUGH SCALE
Child–Turcotte–Pugh Score
1 2 3
Encephalopathy None 1, 2 3, 4
Ascites Absent Slight Moderate
Bilirubin (mg/dL) 1–2 2–3 >3
Albumin (g/dL) > 3.5 2.8–3.5 < 2.8
Prothrombin time (s. prolonged) 1–4 4–6 >6
Total score
1–6 = A
7–9 = B
10–15 = C
133
VIII. PANCREAS
A. Pancreatic Carcinoma
H&P Keys
Risk factors for the development of pancreatic cancer include:
chronic pancreatitis, hereditary pancreatitis, smoking, a diet high in
fat content, and exposure to various chemicals including
β-naphthylamine. Earlier studies that reported an association with
caffeine appear unfounded.
Most common presenting features are abdominal pain, weight
loss, jaundice, and anorexia. With pancreatic head tumors, dis-
tended gallbladder (Courvoisier gallbladder) may be palpable. Oc-
casionally, acute pancreatitis or hypergylcemia may be the present-
ing features. Thromboembolic phenomena occur in 10% of patients
with pancreatic adenocarcinoma.
Tumor markers: CEA, CA 19-9 can be helpful (although not di-
agnostic) when very high.
Diagnosis
In patients with suspected pancreatic cancer the goals of the evalua-
tion are (1) to establish the diagnosis and (2) to determine re-
sectability. Several imaging modalities are useful.
• Biphasic helical CT with thin cuts through the pancreas is the best
imaging study for both diagnosis and staging.
• ERCP: useful for evaluating biliary or pancreatic duct obstruction
in the absence of a suspicious mass on CT. ERCP and brush cytol-
ogy may support a diagnosis of malignancy.
• Transabdominal ultrasound provides little in the evaluation of the
pancreas. It is useful in the evaluation of obstructive jaundice
when there is a strong suspicion of choledocholithiasis.
• Endoscopic ultrasonography (EUS) with fine-needle aspiration
biopsy is the most accurate test to assess resectability.
• MR cholangiopancreatography is less sentitive than ERCP. How-
ever, because of its noninvasive nature, it may be useful for pa-
tients requiring repeat imaging of the pancreatic duct.
• Biopsy: limited indications.
A confirming biopsy prior to resection is unnecessary when:
• Clinical suspicion of pancreatic cancer is high.
• The cancer is surgically resectable.
• The patient is a satisfactory candidate for surgical resection.
A confirming biopsy may be needed prior to treatment when:
• The diagnosis of pancreatic cancer is doubtful.
• The cancer is not surgically resectable.
• The patient is a poor operative candidate.
134
DISEASES AND DISORDERS OF THE DIGESTIVE SYSTEM Pancreas
B. Acute Pancreatitis
H&P Keys
Abdominal pain, nausea, vomiting. Patients appear ill, anxious, and
restless. Hypotension, tachypnea, tachycardia, hyperthermia or hy-
pothermia, jaundice, mild distention, involuntary guarding in upper
abdomen, generalized abdominal tenderness, bowel sounds dimin-
ished, basilar atelectasis, pleural effusions, flank and periumbilical
ecchymosis (Grey Turner’s and Cullen’s signs).
Diagnosis
Elevated serum amylase (within 2–12 hours, declining over the next
3–5 days), elevated lipase (persists 5–7 days, can be detected after
135
cram facts U
CAUSES OF ACUTE PANCREATITIS
Treatment Steps
1. General supportive measures, with meticulous management of
fluid, electrolyte, ventilatory and hemodynamic alterations, and
parenteral alimentation.
2. Early (within 72 hours) intervention (ERCP) to remove stones
from the ductal system improves outcome.
3. Anticipate possible local complications: pseudocyst infection,
pancreatic ascites, abscess, blood vessel with or without rupture
or thrombosis, bowel necrosis or stricture, esophageal varices,
and fistulas.
C. Chronic Pancreatitis
H&P Keys
Recurrent or persistent abdominal pain, weight loss, steatorrhea,
glucose intolerance, epigastric tenderness.
Diagnosis
Plain x-ray of the abdomen may show pancreatic calcification. Ultra-
sonography or CT scan may show enlarged gland, dilated pancreatic
duct, or calculi. ERCP is most sensitive and specific test for chronic
pancreatitis. ERCP can differentiate chronic pancreatitis from pan-
creatic carcinoma. Tests of pancreatic exocrine function (secretin,
cholecystokinin [CCK], or bentiromide test) for the rare patient
with relatively minor ductal changes on ERCP in whom the diagno-
sis remains in doubt.
Disease Severity
Complications include pseudocyst, pancreatic ascites, fistula, and
splenic vein thrombosis. Suspect pseudocyst when a stable chronic
pancreatitis patient experiences worsening of abdominal pain.
Concept and Application
Alcohol in Western societies (70–80%) and malnutrition worldwide
represent major etiologies.
Treatment Steps
1. Avoidance of alcohol, analgesia, enzyme therapy for malabsorp-
tion and pain control, especially for patients with non–
alcohol-induced chronic pancreatitis.
2. Celiac plexus block.
3. Surgery if all other measures have failed.
D. Cystic Fibrosis
H&P Keys
Infants fail to gain weight despite vigorous appetite; watery stools.
Eighty percent have pancreatic exocrine insufficiency at diagnosis,
hypoproteinemia with edema, and “pot belly” on physical examina-
tion.
Diagnosis
Sweat electrolytes: sodium and chloride are elevated in sweat in
99%; pancreatic stimulation test with CCK and secretin may confirm
diagnosis when sweat test is equivocal; fetal screening and chromoso-
mal analysis to diagnose cystic fibrosis by DNA analysis in the future.
Disease Severity
Malnutrition and recurrent pulmonary infections are major con-
cerns. Associated conditions include chronic meconium ileus in
137
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141
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142 Hematology and Oncology
BIBLIOGRAPHY / 163
143
Diagnosis
Hypochromic, microcytic cells on peripheral smear. Mean corpuscu-
lar volume (MCV) and mean corpuscular hemoglobin (MCH) are
low; low serum ferritin, low serum iron, increased total iron-binding
capacity (TIBC), low percentage of saturation, increased red cell dis-
tribution width (RDW), thrombocytosis, and low reticulocyte count.
Disease Severity
Absence of iron on bone marrow aspiration (Prussian blue staining).
Treatment Steps
1. Determine etiology.
2. Begin supplemental oral iron if patient is mildly to moderately
symptomatic.
3. Transfuse if patient has any sign of cardiac compromise or is se-
verely symptomatic.
4. Reassess oral replacement in 14–21 days expecting to see a signifi-
cant reticulocytosis and the hemoglobin corrected by at least half.
If it has not, assess compliance and absorption issues.
5. Some patients do not absorb or cannot tolerate oral iron and
must receive either transfusion or intravenous supplementation.
Diagnosis
Low serum and red blood cell (RBC) folate levels (serum folate lev-
els quickly correct following one hospital meal; therefore, RBC fo-
late levels are more accurate). Macrocytic anemia and hyperseg-
mented neutrophils on peripheral smear. Megaloblastic bone
marrow cells. Normal B12 level. Increased lactic dehydrogenase
(LDH). Increased bilirubin.
144
HEMATOLOGY AND ONCOLOGY Anemia
Disease Severity
Pancytopenia; infertility, skin pigmentation abnormalities. Severe
malabsorption syndromes diagnosed by jejunal biopsy. Neural tube
defects if folate deficiency present during pregnancy.
Concept and Application
Folic acid absorbed in the proximal jejunum. Folate deficiency leads
to diminished thymidylate synthesis and abnormal DNA replication.
Treatment Steps
1. Determine etiology.
2. Replace with oral folic acid 1 mg/day.
C. α-Thalassemia
H&P Keys
An inherited disorder seen in American blacks and people of
Mediterranean or Southeast Asian background. Carrier state (lack of
one normal allele) is undetectable and very common. Patients who
lack two alleles (called α-thalassemia trait) are usually asymptomatic,
although they may have a mild microcytic anemia. Patients with
deletion of three alleles (hemoglobin [HbH]) have moderately se-
vere hemolytic anemia. Absence of α chains is incompatible with
life.
Diagnosis
Microcytosis on peripheral smear in patients with α-thalassemia trait.
Normal iron studies. Elevated RBC count. RBC inclusions in patients
with HbH and evidence of hemolysis (elevated LDH and bilirubin).
Coombs’ test is negative. Molecular studies can quantitate number
of copies.
Disease Severity
Hemolytic anemia and splenomegaly in patients with HbH. Hydrops
fetalis occurs in homozygotes, in whom no α-globin is produced. Af-
fected fetuses are either stillborn or die shortly after birth.
Concept and Application
Caused by deletion of one or more α-globin genes. Presence or ab-
sence of symptoms depends on number of alleles deleted (or mu-
tated). Inadequate production of α-globin leads to precipitation of
β-globin (in HbH) or to β4 (β chain tetramer) formation, which is
incompatible with life.
Treatment Steps
1. Establish diagnosis by molecular studies or staining of peripheral
blood smear with cresyl blue.
2. Rule out underlying iron deficiency.
3. Symptomatic patients may need periodic transfusions.
4. Prenatal diagnosis can identify fetus at risk for hydrops fetalis.
5. Splenectomy can be beneficial in patients with HbH.
D. β-Thalassemia
H&P Keys
Most common in patients of Mediterranean background or from
equatorial regions of Asia and Africa. Thalassemia minor (heterozy-
gotes) patients usually have asymptomatic mild anemia.
Thalassemia major (homozygotes) presents in childhood with symp-
toms of anemia. Splenomegaly occurs in thalassemia major. Frontal
145
Disease Severity
Serum B12 level is low. Neurologic symptoms consistent with long-
tract disease, then cerebral dysfunction. No correlation between
anemia and neurologic symptoms.
Treatment Steps
B12 1,000 µg given parenterally as a loading dose daily for 5 days,
then 1,000 µg/month for life.
F. Aplastic Anemia
H&P Keys
Weakness, fatigue, pallor, petechiae, bleeding, and evidence of infec-
tion. Etiology may be idiopathic, familial (e.g., Fanconi’s syndrome),
or acquired (e.g., secondary to radiation, drugs, hepatitis, or autoim-
mune mechanisms).
Diagnosis
Hypocellular bone marrow. Normal cytogenetics. Decreased reticu-
locyte count (< 5%). Normochromic and normocytic RBCs. Rule
out paroxysmal nocturnal hemoglobinuria with a Ham test (sucrose
hemolysis test).
Disease Severity
Severe aplastic anemia defined as two or more of the following: ab-
solute neutrophil count < 500, reticulocyte count < 1%, and platelets
< 20,000. Percentage of bone marrow cellularity < 25%.
Treatment Steps
1. Determine etiology if possible.
2. Human lymphocyte antigen (HLA)-matched bone marrow trans-
plant if patient has a donor.
3. If transplant is not an option, other treatment options include an-
tithymocyte globulin (ATG), cyclosporine, and corticosteroids.
4. Avoid transfusions as much as possible if bone marrow transplant
is planned.
Diagnosis
Low reticulocyte count. Normochromic, normocytic RBCs. In-
creased serum creatinine. Serum erythropoietin level decreased.
147
Diagnosis
Peripheral smear reveals sickle-shaped cells, Howell–Jolly bodies, nu-
cleated RBCs, reticulocytosis, thrombocytosis. HbS on electrophore-
sis. Prenatal diagnosis by amniotic fluid DNA analysis.
Disease Severity
Heterozygous state (sickle cell trait) usually asymptomatic, but a fre-
quent cause of hematuria secondary to papillary necrosis. Homozy-
gous state results in severe hemolytic anemia (Fig. 5–2).
Factors that induce sickling: infection, dehydration, low oxygen
tension, low pH. Microvascular occlusion can lead to ischemia and
tissue infarction known as a pain crisis. Aplastic crisis (abrupt halt in
erythropoiesis) caused by parvovirus B19 infection.
Treatment Steps
Chronic
1. Folate 1–2 mg PO daily.
2. Pneumovax.
3. Antibiotic prophylaxis in childhood.
4. Genetic counseling.
5. Hydroxyurea helpful in some patients.
6. Hypertransfusion to suppress Hb S levels.
149
Acute
1. Treat pain crises with hydration, adequate analgesia, and
treatment of inciting event if known.
2. Oxygen if patient is hypoxic.
3. Transfusion or exchange transfusion for CVA or acute chest
syndrome.
Diagnosis
CBC reveals anemia and thrombocytopenia. White blood count
(WBC) usually elevated with increased blasts or occasionally de-
pressed. Auer rods in some subtypes. Bone marrow is usually hyper-
cellular, positive peroxidase or Sudan black staining, negative peri-
odic acid-Schiff (PAS) staining. Flow cytometry to determine cell
surface markers. Cytogenetic abnormalities include: t(8;21) (M2),
t(15;17) (M3), inv16 (M4 with eosinophils). Central nervous system
(CNS) involvement can be seen in M4 and M5 subtypes. Spuriously
low PO2 and serum glucose can be seen with high WBC.
Disease Severity
Very high WBC can cause leukostasis with CNS symptoms or pul-
monary syndromes. AML in elderly patients, AML occurring after
chemotherapy for other malignancies, and AML developing in a pa-
tient with myelodysplastic syndrome (MDS) has a very poor progno-
sis.
French–American–British (FAB) Classification—Still in common use but
many modifications exist:
• M0: undifferentiated cells
• M1: minimal maturation
• M2: early differentiated cells, Auer rods, t(8;21)
• M3: acute promyelocytic leukemia (APL), large granules, Auer
rods, t(15;17)
150
HEMATOLOGY AND ONCOLOGY Malignant Diseases
Treatment Steps
1. Pathologic diagnosis based on bone marrow biopsy, cytogenetics,
and cell typing.
2. Antibiotics if infected.
3. Transfusion of blood products as needed.
4. All-trans retinoic acid (ATRA) for acute promyelocytic leukemia
(APML) (M3). Monitor for DIC. Conventional cytotoxics once in-
duction is complete.
5. Allopurinol to prevent urate nephropathy.
6. Monitor for tumor lysis with serum K+, creatinine, and PO4.
7. Treatment usually includes an induction phase followed by 2–4
consolidation cycles.
8. Bone marrow transplant later in appropriate patients.
Diagnosis
Hypercellular bone marrow with > 30% blasts. Blasts are often posi-
tive for terminal deoxynucleotidyltransferase (TdT), common acute
lymphoblastic leukemia antigen (CALLA) (CD10), and PAS; and
negative for peroxidase and Sudan black. Flow cytometry to deter-
mine cell surface markers. Elevated WBC, anemia, thrombocytope-
nia. Lumbar puncture to rule out CNS involvement. Elevated creati-
nine, LDH, PO4, K+ predict for tumor lysis syndrome. Philadelphia
(Ph) chromosome t(9;22), seen in 10% of children and 30% of
adults, confers a poor prognosis. Burkitt’s type of ALL accompanied
by t(8;14), t(2;8), or t(8;22) involving c-myc oncogene.
Disease Severity
Disease Severity
Several staging systems. Rai staging best known:
• Stage 0: lymphocytosis only
• Stage 1: lymphocytosis with lymphadenopathy
• Stage 2: lymphocytosis with splenomegaly
• Stage 3: lymphocytosis with anemia
• Stage 4: lymphocytosis with thrombocytopenia
Modified Rai staging:
• Low risk: stages 0 and 1
• Intermediate risk: stages 2 and 3
• High risk: stage 4
Aggressive transformation into large-cell lymphoma, Richter’s
transformation.
Concept and Application
Monoclonal proliferation of dysfunctional mature β lymphocytes.
More frequent infections due to lack of opsonization.
Treatment Steps
1. Observation for low-risk patients.
2. Treat higher-risk or symptomatic patients with chlorambucil and
prednisone or fludarabine.
E. Hodgkin’s Disease
H&P Keys
Frequently younger patients. Superficial, painless, enlarged “rub-
bery” lymph nodes (60–80% cervical or axillary, nontender),
splenomegaly. “B” symptoms: fevers, night sweats, weight loss. Infec-
tions associated with depressed cell-mediated immunity. Bimodal
peak incidence.
Diagnosis
Lymph node biopsy reveals Reed–Sternberg cells with reactive lym-
phocytes (Fig. 5–4). Disease spread by contiguous lymph node chains.
Initial staging tests include computed tomographic (CT) scans of the
chest, abdomen, and pelvis, bone marrow aspiration and biopsy.
Positron-emission tomography (PET) and gallium scans are useful in
bulky disease. Laparotomy and lymphangiogram are rarely per-
formed. Nodular sclerosing subtype most common. Lymphocyte-
depleted histologic specimens associated with poor prognosis. Mixed
cellularity and lymphocyte-predominant tissues offer better prognosis.
153
Disease Severity
F. Low-Grade Lymphoma
H&P Keys
Lymphadenopathy usually diffuse and not necessarily contiguous.
Splenomegaly can be seen. Extranodal (e.g., bone marrow) disease
common. Rarely, fever, weight loss, night sweats.
Diagnosis
Lymph node biopsy. Bone marrow aspiration and biopsy (bone mar-
row involvement common). κ/λ clonal excess. Immunoglobulin
gene rearrangement. Cytogenetic studies, e.g., t(14;18), associated
with follicular lymphoma (Fig. 5–5).
Disease Severity
Ann Arbor staging system (see Hodgkin’s disease staging). Chest
x-ray (CXR), CT scans of chest, abdomen, and pelvis. Often stage IV
154
HEMATOLOGY AND ONCOLOGY Malignant Diseases
Figure 5–5. (A) A benign lymphoid follicle compared to (B) lymphoma involving the marrow.
diagnostic
decisions d Treatment Steps
1. Watchful waiting acceptable if asymptomatic.
2. Treatment for bulky disease, obstruction of vital structures, cy-
topenias, or autoimmune complications.
LYMPHOMAS 3. Treatment options include alkylating agents plus corticosteroids,
radiation, fludarabine, Rituxan. Occasional patients require ag-
Low Grade gressive cytotoxics.
Patients are older, often
with stage III or IV disease; G. Intermediate- or High-Grade Lymphoma
disease is treatable and the
life expectancy is usually H&P Keys
many years. Types include: Lymphadenopathy, splenomegaly. Extranodal disease can be seen.
small lymphocytic (same as Fatigue, malaise. Rapidly enlarging lymphadenopathy can occur (es-
CLL), plasmacytoid,
pecially Burkitt’s lymphoma). Mediastinal mass can cause symptoms.
follicular small cell, and
follicular mixed. Diagnosis
Intermediate Grade Lymph node biopsy to make diagnosis. Needle biopsy not suf-
Lots of variability; some ficient. Bone marrow aspiration and biopsy. Spinal tap to rule
behave in more indolent
out meningeal spread especially in lymphoblastic lymphoma.
fashions and some are
more aggressive. Individual
Burkitt’s lymphoma associated with Epstein–Barr virus (EBV) and
type very important. Types translocation t(8;14), t(2;8) or t(8;22). Increased LDH in aggressive
include: follicular large cell, disease. CXR to evaluate mediastinum. CT scans of chest, abdomen,
diffuse small cleaved, and pelvis. PET can be helpful. Testing for human immunodefi-
diffuse mixed, and maybe ciency virus (HIV). Tumor lysis can be seen with rapidly growing
diffuse large cell. lymphomas (e.g., Burkitt’s).
High Grade
Often younger patients; Disease Severity
aggressive therapy can Ann Arbor staging system. Bone marrow involvement or CNS in-
result in cure. Types volvement have worse prognosis. Evidence of tumor lysis. Poor prog-
include: immunoblastic, nosis, increased age, large tumor masses, B symptoms, bone marrow
lymphoblastic, and
involvement, increased LDH, poor performance status, HIV associ-
Burkitt’s.
ated.
155
H. Multiple Myeloma
H&P Keys
Anemia, bone pain, increased susceptibility to infections, fatigue,
weight loss, renal insufficiency, altered mental status.
Diagnosis
Anemia, hypercalcemia, presence of a paraprotein (or M-compo-
nent) on serum protein electrophoresis (SPEP), Bence Jones pro-
teins on urine protein electrophoresis (UPEP), lytic bone lesions on
skeletal survey, bone marrow with > 30% plasma cells. Measure β2
microglobulin and serum creatinine. Quantitate amount of im-
munoglobulin present. Serum viscosity if altered mental status pres-
ent.
Disease Severity
• Stage I: Low tumor burden with Hb > 10 g/dL, normal calcium,
IgG < 5 g/dL, IgA < 3 g/dL, urine light chains < 4 g/24 hours,
and normal skeletal survey.
• Stage II: Intermediate tumor burden; patients who are neither
stage I nor stage III.
• Stage III: High tumor burden and any one of the following: Hb <
8.5 g/dL, serum calcium > 12 mg/dL, IgG > 7 g/dL, IgA > 5
g/dL, urine light chains > 12 g/24 hours, or extensive lytic bone
lesions. The following subclassification of stages is used:
A. Creatinine level > 2 mg/dL
B. Creatinine level ≥ 2 mg/dL
Elevated β2 microglobulin at diagnosis reflects a poor prognosis.
Concept and Application
Monoclonal proliferation of plasma cells in the bone marrow. Osteo-
clastic activating factors (possibly interleukins [IL-1, IL-6], tumor
necrosis factor [TNF]) responsible for bone lesions. Bone destruc-
tion leading to pathologic fractures, pain, hypercalcemia. Patients
can develop amyloid deposition over time.
Treatment Steps
1. Manage low stage disease conservatively.
2. Chemotherapy for symptomatic or more advanced disease—mel-
phalan and prednisone or VAD (vincristine, adriamycin,
156
HEMATOLOGY AND ONCOLOGY Other Disorders
A. Polycythemia (Erythrocytosis)
H&P Keys
Often asymptomatic, diagnosed on routine lab tests. Splenomegaly,
early satiety, hypertension, facial plethora, venous thromboses, hem-
orrhages. History of tobacco use, travel to high altitudes. Spurious
erythrocytosis results from decreased plasma volume. If a true ery-
throcytosis, consider if physiologically appropriate: chronic obstruc-
tive pulmonary disease (COPD), right-to-left cardiac shunt, high-
affinity Hb, carboxyhemoglobinemia. Physiologically inappropriate:
tumors producing erythropoietin (renal cell, hepatocellular), renal
disease, adrenal cortical hyperplasia, exogenous androgens.
Diagnosis
Increased RBC mass with normal plasma volume key to diagnosis. In
primary process, polycythemia rubra vera (PRV), erythropoietin lev-
els are normal or low. Hypercellular bone marrow. WBC and platelet
counts are normal or elevated, increased LAP, increased B12, low or
absent iron stores. Other causes (see above) must be ruled out.
157
B. Transfusion Reactions
H&P Keys
Occur minutes, hours, or days after transfusion. Most severe is im-
mune-mediated hemolytic reaction: fever, chest or back pain, hy-
potension, dyspnea, hemoglobinuria, shock. Most common are
febrile reactions caused by WBCs present in packed RBCs, now de-
creased secondary to common use of WBC filtering by blood banks.
Rarely, febrile reactions are due to bacterial contamination.
Patients with IgA deficiency can have anaphylactic reaction: sud-
den onset; no fever; may have cough, respiratory distress, hypoten-
sion, nausea, vomiting, abdominal pain, loss of consciousness, and
shock. Delayed hemolytic transfusion reaction results from minor
antigen mismatch (not detected by indirect Coombs’ test). Post-
transfusion purpura results in moderate to severe thrombocytopenia
2–10 days following transfusion of packed RBCs.
Diagnosis
Stop transfusion and return unit to blood bank. Monitor Hb and
Hct, hemoglobinuria, unconjugated bilirubin, haptoglobin, LDH,
coagulation profile, and renal profile. Repeat ABO, Rh, and compat-
ibility screens. Direct antiglobulin test on posttransfusion blood sam-
ple from patient. Check IgA level if anaphylaxis. Culture remainder
of blood product.
Disease Severity
Most severe reactions are major hemolytic transfusion reaction and
anaphylaxis.
Concept and Application
Major hemolytic transfusion reaction caused by ABO incompatibil-
ity. Most often a result of clinical error.
Treatment Steps
1. Stop transfusion.
2. Maintain blood pressure and urine output with IV fluids.
3. For anaphylaxis: epinephrine, diphenhydramine hydrochloride
(Benadryl), and glucocorticoids.
4. Antibiotics if bacterial contamination suspected.
5. Premedicate with acetaminophen and use WBC filters to prevent
febrile reactions.
158
HEMATOLOGY AND ONCOLOGY Other Disorders
C. Hemophilia A and B
H&P Keys
Hereditary bleeding disorders. Hemophilia A most common. A and
B are clinically indistinguishable. Frequent episodes of bleeding into
joints, muscles, and skin with minimal or no trauma. Can result in
chronic arthritis. Prolonged postoperative hemorrhage. Intracranial
hemorrhage can occur.
Diagnosis
Elevated partial thromboplastin time (PTT) and low factor VIII or
IX level. Normal bleeding time, normal prothrombin time (PT),
normal thromboplastin time (TT). Specific assays for factor VIII or
IX. Polymerase chain reaction (PCR) analysis for prenatal detection.
Disease Severity
Patients with < 1% factor VIII (or IX) level have severe disease;
> 5% factor VIII (or IX) results in mild disease. Clinical history very
important in predicting bleeding tendency.
Treatment Steps
Mild Hemophilia A
1. First-line therapy is desmopressin (DDAVP) (stimulates imme-
diate release of VIII:C and von Willebrand factor [vWF] from
endothelial cell stores).
2. Second-line therapy is recombinant human factor VIII.
Severe Hemophilia A
Recombinant human factor VIII or factor VIII concentrate.
Hemophilia B
1. Desmopressin not effective.
2. Recombinant human factor IX.
Diagnosis
(1) Prolonged bleeding time. PTT can be prolonged or normal. (2)
Decreased factor VIII:C activity. (3) Decreased ristocetin cofactor on
platelet aggregation studies. (4) Decreased vWF. (5) Multimeric
analysis.
Type I—Generalized decrease in all multimeric forms of plasma
vWF.
Type II—Selective deficiency of higher-molecular-weight forms.
Normal or near-normal levels but dysfunctional.
Type III—Markedly decreased plasma and platelet levels of vWF.
Autosomal recessive.
Pseudo von Willebrand Disease—Abnormal vWF platelet receptor.
159
Treatment Steps
1. Therapy not required for asymptomatic patients.
2. Treat thromboses and emboli with heparin acutely.
3. Consider prophylactic anticoagulation during hypercoaguable
periods (e.g., postop, pregnancy).
4. Consider chronic anticoagulation for repeated thrombotic events
or a single life-threatening event. Choice of agent will vary de-
pending on the underlying cause.
I. Thrombotic Thrombocytopenic
Purpura (TTP)
H&P Keys
Onset may be fulminant or subacute, single episode or recurring.
Mental status changes result of intermittent ischemia. Classic pen-
tad: fever, renal dysfunction, neurologic changes, thrombocytope-
nia, microangiopathic hemolytic anemia.
Diagnosis
Low platelet count, normal or increased number of megakaryocytes
in bone marrow, increased LDH, and schistocytes on smear. Elevated
blood urea nitrogen (BUN), creatinine, and proteinuria reflect re-
nal disease. DIC testing is negative.
Disease Severity
Severity of neurologic symptoms (coma) and hemolysis.
Treatment Steps
1. Plasmapheresis.
2. If plasmapheresis unavailable, infuse fresh frozen plasma.
3. Treat underlying condition.
4. Corticosteroids usually given unless contraindicated.
J. Essential Thrombocythemia
H&P Keys
Usually asymptomatic. Neurologic symptoms most common:
headache, visual changes. Episodes of thrombosis, embolism, or he-
morrhage can occur.
Diagnosis
Platelet count > 800,000. Bone marrow: increased number of
megakaryocytes. Normal Hb level. Mildly increased WBC. Platelet
function testing usually abnormal. Rule out chronic inflammation,
iron deficiency.
Disease Severity
Complications infrequent in younger patients. In elderly patients,
transient ischemic attack (TIA) is the most common complication.
CNS hemorrhage or CVA secondary to emboli.
Treatment Steps
1. Hydroxyurea.
2. Antiplatelet agents for ischemia.
162
HEMATOLOGY AND ONCOLOGY Other Disorders
K. Platelet Dysfunction
H&P Keys
Diagnosis
Anemia, nucleated RBCs, teardrop cells. Bone marrow biopsy shows
increased fibrosis, increased megakaryocytes.
Disease Severity
Extent of fibrosis in bone marrow and extramedullary hemato-
poiesis. Can evolve into acute leukemia.
Treatment Steps
1. Transfuse as needed.
2. Colony stimulating factors (erythropoietin, granulocyte colony-
stimulating factor [G-CSF], granulocyte macrophage colony-stim-
ulating factor [GM-CSF].
3. Splenectomy rarely for painful enlargement or refractory cytope-
nias caused by splenic sequestration.
M. Neutropenia
H&P Keys
Acquired or congenital. Autoimmune neutropenia (Felty’s syn-
drome) is seen as an isolated event and in rheumatoid arthritis and
other systemic autoimmune disorders. Cyclic neutropenia: fatigue,
decreased appetite, mouth sores that occur every 3 weeks and last
3–4 days. Evidence of infection: mouth sores, sore throat, skin infec-
tions, pneumonia, urinary tract infection.
163
BIBLIOGRAPHY
Armitage J (ed.). Atlas of Clinical Hematology. Philadelphia: Lippincot Williams &
Wilkins, 2004.
Lichtman MA, et al (eds.). Williams Hematology, 7th ed. New York: McGraw-Hill, 2006.
Colman RW (ed.). Hemostasis and Thrombosis: Basic Principles and Clinical Practice.
Philadelphia: J.B. Lippincott, 2000.
DeVita V, Hellman S, Rosenberg S (eds.). Cancer: Principles & Practice of Oncology, 6th
ed. Philadelphia: Lippincott-Raven, 2001.
Hoffman R, Benz E Jr., Shattil S, et al (eds.). Hematology: Basic Principles and Practice,
4th ed. New York: Churchill Livingstone, 2005.
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Immunology
and Allergy 6
I. BRONCHIAL ASTHMA / 166
V. IMMUNODEFICIENCY / 171
A. Humoral Immunodeficiency / 171
B. Cellular (T-Cell) Immunodeficiency / 172
BIBLIOGRAPHY / 180
165
Copyright © 2004, 2001 by The McGraw-Hill Companies, Inc. Click here for terms of use.
166
IMMUNOLOGY AND ALLERGY Bronchial Asthma
I. BRONCHIAL ASTHMA
H&P Keys
Chronic or recurrent cough, dyspnea, or wheezing. Prolonged expi-
ration and wheezing with or without use of accessory muscles. Af-
fects > 5% of the population. Incidence, mortality, and hospitaliza-
tion rates increasing. Allergic (extrinsic) asthma related to exposure
to allergens (seasonal pollens such as grasses, trees, and weeds; ani-
mal dander, dust mites, and mold). Nonallergic asthma (intrinsic)
associated with infection, stress, environmental pollution, exercise,
cold air, and in a small percentage of patients, aspirin use.
Diagnosis
Pulmonary function studies with a reversible obstructive pattern,
positive methacholine challenge, sputum rich in eosinophils, and re-
sponse to bronchodilator therapy. Chest x-ray (CXR), total
eosinophil count, total immunoglobulin E (IgE), and allergy skin
tests. Oximetry and arterial blood gases (ABGs) in acutely ill pa-
tients.
Disease Severity
management decisions d
ASTHMA
Disease Treatment
Exercise-induced asthma Short-acting β2-adrenergic (albuterol)—2 puffs 15 minutes prior to
exercise.
and/or
Cromolyn (Intal)—2 puffs prior to exercise.
Mild intermittent asthma Short-acting β2-adrenergic (albuterol)—2 puffs qid, prn.
Mild persistent asthma Add inhalded low- to medium-potency corticosteroid (Flovent 44
or 110—2 puffs bid).
Moderate persistent asthma Add leukotriene modifier (Singulair, 10 mg qd) or long-acting
β2-adrenergic (Serevent—2 puffs bid).
and/or
Increase the inhaled corticosteroid dose.
Severe persistent asthma Increase the inhaled corticosteroid dose (Flovent 220—2–4 puffs
bid).
Add theophylline, nedocromil (Tilade), leukotriene modifier, oral
long-acting β2-adrenergic or oral corticosteroids.
167
Treatment Steps
General
1. Avoidance of precipitating factors, patient education.
2. Use peak flow meters in moderate and severe persistent
asthma.
3. Immunotherapy (allergy injections) can be added as an anti-
inflammatory adjunct.
4. Quick-relief medicine is the first step.
5. Add a long-term controller if a quick-relief medicine is neces-
sary more than twice weekly.
Quick-Relief Medicines
1. Inhaled short-acting β2-adrenergic (Albuterol HFA or
Xopenex via nebulizer).
2. Inhaled ipratropium bromide (Atrovent).
3. Subcutaneous epinephrine.
4. Oral corticosteroids.
5. Intravenous corticosteroids.
6. Intravenous aminophylline.
7. Oxygen.
8. Combination inhaled short-acting β2-adrenergic and ipra-
tropium bromide (Combivent).
Long-Term-Control Medicines
1. Inhaled corticosteroid (Flovent 44, 110, or 220).
2. Inhaled long-acting β2-adrenergic (Serevent Diskus).
3. Inhaled cromolyn sodium (Intal).
4. Inhaled nedocromil sodium (Tilade).
5. Oral sustained-release theophylline (UniDur).
6. Oral long-acting β2-adrenergic (Volmax).
7. Oral leukotriene modifiers (Singulair, Accolate, Zyflo).
8. Combination inhaled corticosteroid and long-acting β2-adren-
ergic (Advair 100/50, 250/50, or 500/50).
Treatment of Mild Intermittent Asthma
1. Inhaled short-acting β2-adrenergic (Albuterol HFA), 2 puffs
qid, prn.
2. For exercise-induced asthma, treat with an inhaled short-
acting β2-adrenergic (as previously) 15–20 minutes prior to
exercise and/or Intal, 2 puffs, also prior to exercise.
Treatment of Mild Persistent Asthma
1. See the treatment of mild intermittent asthma.
2. Add an inhaled corticosteroid (Flovent 44, 2 puffs bid), fol-
lowed by rinsing of the mouth to decrease the incidence of
thrush.
168
IMMUNOLOGY AND ALLERGY Allergic Rhinitis and Conjunctivitis
Severity Treatment
Mild Avoidance techniques.
Oral antihistamine (Allegra, 180 mg qd) with or without a decongestant
(Sudafed).
Ocular antihistamines (Naphcon A) or ocular antihistamine/mast cell
stabilizer (Optivar).
Moderate Add nasal corticosteroids (Nasonex—2 sprays each nostril qd).
Add immunotherapy (allergy injections).
Moderate to severe Add nasal antihistamine (Astelin—2 sprays in each nostril bid prn)
or
Nasal ipratropium bromide (Atrovent 0.03%—2 sprays in each nostril bid
prn).
Severe Nasal decongestants (Afrin—1–2 sprays in each nostril bid for 2–3 days).
Oral corticosteroids (prednisone—1–2 mg/kg/day for 5–7 days).
Diagnosis
Positive allergy skin tests or RAST to offending allergen. IgE-specific
antibodies are not identified in anaphylactoid reactions. Other stud-
ies include complete blood count (CBC), total IgE, total eosinophil
count, elevated serum tryptase, and urine histamine.
Concept and Application
Mast cells and basophils rich in mediators (ECF-A, NCF-A, PAF, hist-
amine, leukotriene, prostaglandins, etc.) suddenly undergo degran-
ulation following exposure to an allergen as a result of specific IgE
directed against that allergen, or, in the case of anaphylactic reac-
tions, allergens cause mediator release through a non-IgE-mediated
mechanism. Mast cell mediator release leads to shifting of intravas-
cular fluid to interstitial tissues and hypotension, and may lead to
hives and angioedema. Asthmatic patient will become dyspneic and
wheeze.
Treatment Steps
1. Oxygen if cyanosis, dyspnea, or wheezing is present.
2. Trendelenburg position if hypotensive.
3. Epinephrine (1:1,000 0.30–0.50 mL SQ or 0.1 mL/kg in chil-
dren).
4. Tourniquet—place a tourniquet proximal to the site of injection
or sting on an extremity, if applicable.
5. Antihistamines (Benadryl IV 1–2 mg/kg up to 100 mg).
6. H2 blockers (famotidine 20 mg IV).
7. IV fluids through large-gauge line to maintain systolic blood
pressure ≥ 100 mm Hg in adults, 50 mm Hg in children.
8. Nebulized β2 agonist if there is bronchospasm (albuterol 0.5 cc
in 3 cc saline).
9. Corticosteroids (hydrocortisone IV 7–10 mg/kg).
10. Vasopressors (dopamine IV 0.3–1.2 mg/kg/hr).
11. Shock trousers.
12. Intubation and ventilation.
13. Tracheostomy if airway obstruction secondary to airway edema.
14. Prevention of future reaction by avoidance, hyposensitization in
cases of insect venom hypersensitivity.
IV. URTICARIA
H&P Keys
Generalized pruritus; hives vary in size from 3–4 mm to giant lesion
10–15 cm in diameter. Usually have central clearing with peripheral
erythema. Often associated with angioedema of soft tissues includ-
ing eyes, lips, and tongue. Lesions leave no permanent changes in
the skin and are transient. Acute urticaria: hives occur over a period
of < 6 weeks. Chronic urticaria: hives persist for a period > 6 weeks.
Hives may be induced by extrinsic agents (e.g., drugs such as antibi-
otics, nonsteroidal anti-inflammatory agents [NSAIDs], and opiates;
radiocontrast media; and foods such as shrimp, peanuts, and straw-
berries), physical agents (e.g., light, heat friction, pressure, cold, and
vibration), and underlying systemic disease (e.g., serum sickness, in-
fection, autoimmunity, malignancy).
Diagnosis
CBC, sedimentation rate, antinuclear antibodies (ANAs), serum pro-
tein electrophoresis (SPEP), urinalysis (UA), liver function tests
171
Severity Treatment
Mild One or two antihistamines from different classes (Allegra, 180 mg in A.M.,
Zyrtec, 10 mg in P.M.)
Moderate Add H2 blocker (Zantac, 150 mg bid).
Moderate to severe Add tricyclic antidepressant (Sinequan, 10 mg tid) and/or leukotriene
modifier (Singulair).
Severe Add oral β2-adrenergic (Volmax, 4–8 mg bid)
and/or
Oral corticosteroids (prednisone, 1–2 mg/kg/day).
V. IMMUNODEFICIENCY
A. Humoral Immunodeficiency
H&P Keys
Recurrent infections including pneumonia, sinusitis, otitis media,
and skin infections. Frequent complaints of diarrhea and arthralgia
or arthritis. Children may suffer from failure to thrive. Young males
may have X-linked agammaglobulinemia. Older children and adults
often suffer common variable hypogammaglobulinemia. Patients
may have absent lymph nodes, and the physical signs relate to the
site of infection. Patients with recurrent bacterial infections usually
lack immunoglobulins (humoral immunodeficiency); those with re-
current fungal or viral infection suffer from absent or defective T
cells (cellular immunodeficiency).
172
IMMUNOLOGY AND ALLERGY Hereditary Angioedema
Diagnosis
CBC, quantitative immunoglobulins (IgG, IgG subclass levels, IgM,
IgA), T- and B-cell enumeration, anergy panel, chest and sinus
x-rays, sweat test, human immunodeficiency virus (HIV) testing.
Concept and Application
Failure of B cells to differentiate into immunoglobulin-producing
cells or other defect in immunoglobulin production or secretion.
Patients with IgA deficiency often have few symptoms.
Treatment Steps
1. IV γ globulin (IVIG), 300–400 mg/kg/month to maintain trough
serum IgG levels 4 weeks after treatment at > 500 mg/dL.
2. Hyperimmune γ globulin preparations (e.g., zoster immune glob-
ulin obtained from immunized donors).
3. Early use of antibiotics in infections.
4. Prevent transfusion reactions. IgA-deficient patients may have
anti-IgA antibodies and require blood products depleted of IgA.
Similarly, IVIG may cause anaphylaxis and is not indicated in IgA-
deficient patients.
Diagnosis
C1-esterase inhibitor qualitative levels are depressed. Quantitative
levels may be present, but not functional.
C1 levels depressed in the acquired but not the hereditary form.
Skin biopsy reveals no inflammatory changes and lacks eosinophilia.
Disease Severity
Severity can vary from episode to episode in an individual patient.
Each episode involving the upper airway is potentially life threaten-
ing. Severely affected patients have attacks of angioedema regularly
every few weeks, and mildly affected patients may go many months
between attacks.
Treatment Steps
1. Supportive therapy during acute attacks (e.g., mild analgesics or
narcotics, IV fluids, intubation or tracheostomy before critical ob-
struction occurs).
2. Infusion of C1-esterase inhibitor concentrate has been effective,
but not currently available in the United States.
3. Fresh frozen plasma is not recommended. Some patients may
worsen due to the addition of complement components.
4. Attenuated androgens (e.g., stanozolol, 1–2 mg bid) contraindi-
cated in children who are not fully grown and during pregnancy.
5. ε-Aminocaproic acid (a fibrinolysis inhibitor) may be used in chil-
dren.
6. Prophylaxis before surgical procedures with fresh frozen plasma
(two units 12–24 hours before the procedure).
7. Epinephrine should not be relied on.
Diagnosis
Total IgE is significantly elevated (> 1,000 IU/mL); elevated total
eosinophil count (> 1,000/mm3); positive immediate-type hypersen-
sitivity reaction to A. fumigatus. X-rays often reveal transient infil-
trates. The presence of central or proximal bronchiectasis and ab-
sence of distal bronchiectasis is highly suggestive of ABPA.
High-resolution CT (HRCT) is more sensitive and specific in detect-
ing proximal bronchiectasis than plain films. Patients often have sig-
nificant titers of precipitating IgG antibodies to Aspergillus, and A.
fumigatus may be cultured from sputum of some patients, but a posi-
tive culture is not diagnostic. Examination of sputum may identify A.
fumigatus hyphae and intense eosinophilia.
Disease Severity
ABPA may vary from mild to moderate disease, with intermittent
flares of asthmatic symptoms that quickly respond to cortico-
steroids, to severe progressive asthma associated with pulmonary fi-
brosis, progressive hypoxemia, digital clubbing, and end-stage pul-
monary disease. ABPA can be staged as follows: acute, remission,
exacerbation, corticosteroid-dependent asthma, and fibrotic.
X. HYPERSENSITIVITY PNEUMONITIS
H&P Keys
Hypersensitivity pneumonitis, or extrinsic allergic alveolitis, is
caused by an immunopathologic reaction involving the alveoli, bron-
chioles, and surrounding pulmonary tissues resulting from exposure
to organic dusts. Allergens causing hypersensitivity pneumonitis may
be derived from bacterial (e.g., thermophilic actinomycetes in mush-
room compost causing mushroom worker’s lung), fungi (e.g., As-
pergillus species in moldy tobacco causing tobacco worker’s lung), in-
sect proteins (e.g., Sitophilus granarius in infested flour causing wheat
miller’s lung), organic chemicals (e.g., isocyanates in various indus-
tries causing chemical worker’s lung), or miscellaneous agents (e.g.,
avian proteins in pigeon droppings causing bird breeder’s lung). An
acute reaction or insidious chronic disease may occur depending on
the concentration and duration of exposure of the allergen. Acute
disease is associated with fever, chills, sweats, myalgia, dyspnea, and
coughing; symptoms last for hours to days following exposure.
Chronic disease usually lacks systemic symptoms, with patients com-
plaining of dyspnea and perhaps cough. Physical examination may
reveal bilateral fine rales and signs of hypoxemia. Patients with
chronic disease may lack clinical symptoms initially but over time de-
velop symptoms of dyspnea resulting from chronic pulmonary fibro-
sis and other signs of hypoxemia.
Diagnosis
In part, the diagnosis is based on evidence of the immunologic reac-
tion to the inhaled antigen. Affected patients reveal evidence of the
presence of precipitating usually IgG, antibody directed against the
offending antigen. Enzyme-linked immunosorbent assay (ELISA)
may be used to detect circulating serum antibodies. Pulmonary func-
tion tests identify the presence of a restrictive lung disease, de-
creased diffusing capacity for carbon monoxide (DLCO), and arter-
ial blood gases identify hypoxemia. CXR may be normal initially.
However, eventually repeated or prolonged exposure to the antigen
will be associated with roentgenologic evidence of interstitial fibro-
sis. HRCT is more sensitive and specific in chronic disease and re-
veals scattered, small, rounded opacities. Inhalation challenge is
rarely used today because of the potential for serious damage. Bron-
choalveolar lavage may reveal IgG and IgA antibodies.
178
IMMUNOLOGY AND ALLERGY Hypereosinophilic Syndrome
Disease Severity
Hypersensitivity pneumonitis may present as an acute disease associ-
ated with fever and elevated white count and sedimentation rates.
Within a short time, signs, symptoms, and findings resolve. Repeated
allergen exposure may lead to subacute episode in which pulmonary
functions do not return to normal, and patients with chronic expo-
sure to the allergen will develop irreversible restrictive lung disease
and, eventually, end-stage lung disease.
Concept and Application
Type III (immune-complex mediated) and type IV (cell-mediated
delayed hypersensitivity) reactions occur to the inhaled organic anti-
genic matter.
Treatment Steps
1. Avoid offending agent.
2. Inhaled short-acting β2-adrenergic (e.g., albuterol).
3. Oral corticosteroids (e.g., prednisone 60 mg/day for 1 week, ta-
pered slowly).
4. Immunotherapy with the offending antigens is not recom-
mended.
d
Concept and Application
diagnostic The drug itself or its metabolite is often a low-molecular-weight hap-
decisions ten, which joins with body proteins to become an allergen. IgE-medi-
ated drug reactions usually result in urticaria, angioedema, or ana-
ALLERGIC DRUG REACTIONS phylaxis. Autoimmune disease or vasculitis may be a result of a
reaction involving immune complexes containing antigen, antibody,
Anaphylactic (Type I) and complement, whereas fixed drug eruptions and contact der-
Immediate IgE-mediated matitis appear to be caused by cellular or delayed-hypersensitivity re-
anaphylaxis, angioedema, actions.
and urticaria. Penicillin is
the most common cause of Treatment Steps
drug-induced anaphylaxis. 1. Discontinue the offending medication.
Cytotoxic (Type II) 2. Antihistamines to treat pruritus and urticaria.
IgG or IgM antibody 3. Antipruritics applied topically for maculopapular rashes.
against cell surface 4. Oral corticosteroids are necessary if exfoliative dermatitis, vasculi-
antigens cause tis, or major organ involvement occurs.
hematologic reactions and
5. Supportive treatment may include transfusions if a hemolytic
nephritis. Drug-induced
Coombs’-positive drug reaction is present or IV fluids if hypotension is noted.
hemolytic anemia and 6. Epinephrine subcutaneously is often helpful in acute and severe
methicillin-induced IgE-mediated reactions.
nephritis are good
examples.
Serum Sickness (Type III) BIBLIOGRAPHY
Immune
complex–mediated Adkinson NF Jr., Middleton’s Allergy: Principles and Practice, 6th ed. Mosby, 2003.
reaction involving fever, Fireman P. Atlas of Allergies and Clinical Immunology, 3rd ed. Mosby, 2005.
Lichtenstein L. Current Therapy in Allergy, Immunology, and Rheumatology, 6th ed. Mosby,
large-joint arthralgias, and 2004.
multiorgan vasculitis. Drug-
induced SLE from
hydralazine and
procainamide are
examples.
Cell-Mediated (Type IV)
Delayed-type
hypersensitivity reactions
take days to occur.
Reactions can range from
contact dermatitis, if the
drug is topical, to drug-
induced interstitial
nephritis.
Injuries, Wounds,
Toxicology, and Burns 7
I. EPISTAXIS / 183
181
Copyright © 2004, 2001 by The McGraw-Hill Companies, Inc. Click here for terms of use.
182 Injuries, Wounds, Toxicology, and Burns
X. DROWNING / 201
BIBLIOGRAPHY / 207
183
C. Concussion
H&P Keys
Neurologic exam may be normal. History reveals a brief alteration of
consciousness. May also have headache, amnesia, nausea, and vomit-
ing.
Diagnosis
History and physical exam (x-ray, CT scan, and magnetic resonance
imaging [MRI] are all normal in concussions). Repeated neurologic
assessments are important.
Disease Severity
Determine neurologic status, degree of injury.
185
D. Subdural Hematoma
H&P Keys
Symptoms may present after brief or prolonged time from injury:
lethargy, headache, seizures, and coma. May have dilated ipsilateral
pupil.
Diagnosis
History and physical exam, CT or MRI.
Disease Severity
Severity determined by neurologic exam and rate of deterioration.
Concept and Application
Trauma resulting in vein or brain tear and hemorrhage under the
dura.
Treatment Steps
Surgical (especially in acute subdural), observation in some cases
(small amount of bleeding, high-risk patient).
E. Epidural Hematoma
H&P Keys
Symptoms usually present very shortly after injury: lethargy,
headache, seizures, and hemiplegia. May have brief loss of con-
sciousness, then return to normal prior to deterioration (lucid inter-
val).
Diagnosis
History and physical exam, CT or MRI.
Disease Severity
Evaluate neurologic status.
Concept and Application
Trauma-induced artery tear (middle meningeal artery common). As-
sociated temporal bone fractures are common.
Treatment Steps
Urgent surgery to avoid brain herniation.
F. Ocular Injury
H&P Keys
May have pain, vision loss, subconjunctival hemorrhage. If light
flashes noted, rule out retinal detachment.
Diagnosis
History and physical exam, ophthalmoscopic and slit-lamp exam.
Disease Severity
Evaluate vision. In chemical exposure, prognosis varies with type of
agent, duration of exposure, and emergency care provided.
186
INJURIES, WOUNDS, TOXICOLOGY, AND BURNS Chest and Abdominal Injury
Treatment Steps
G. Auditory Injury
H&P Keys
Swelling, pain, hearing loss, vertigo, and hemorrhage.
Diagnosis
History, physical exam, and audiometric exam, x-ray of skull and
temporal bone (rule out associated fracture).
Disease Severity
Evaluate trauma to the pinna, external ear canal, and tympanic
membrane.
Concept and Application
Trauma.
Treatment Steps
A. Rib Fracture
H&P Keys
Pain following trauma, increased with inspiration and palpation, ec-
chymosis. Rib x-rays may appear negative shortly after injury, yet
show a “healing fracture” several weeks later.
187
B. Pneumothorax
H&P Keys
Dyspnea, chest pain, absent breath sounds, decreased tactile fremi-
tus, hyperresonance. Tachycardia and hypotension may present in
tension pneumothorax.
Diagnosis
History and physical exam, chest x-ray (CXR).
Disease Severity
Evaluate cardiovascular status, mentation, coexisting problems, oxy-
genation. Reduced venous return resulting from tension pneumo-
thorax requires urgent treatment.
Concept and Application
Air in pleural space, as a result of blunt or penetrating trauma (in-
cluding iatrogenic trauma). May also be spontaneous, in patients
with pulmonary disease, or menses-associated (catamenial).
Treatment Steps
C. Hemothorax
H&P Keys
Dyspnea, chest pain.
Diagnosis
History and physical exam (absent breath sounds), CXR.
Disease Severity
Evaluate and monitor cardiac and pulmonary status. Prognosis
worse in patients with significant preexisting condition.
Concept and Application
Trauma or spontaneous. May be iatrogenic (central venous pressure
[CVP] monitor insertion).
Treatment Steps
Large chest tube (32–40 French) with 20-cm water suction.
188
INJURIES, WOUNDS, TOXICOLOGY, AND BURNS Chest and Abdominal Injury
D. Flail Chest
H&P Keys
Paradoxic chest wall motion, respiratory distress.
Diagnosis
History and physical exam (chest palpation), x-ray exam.
Disease Severity
Monitor for reduced vital capacity and respiratory distress secondary
to multiple fractures.
Concept and Application
Respiratory paradox with inspiration, secondary to multiple rib frac-
tures.
Treatment Steps
Intubation and positive pressure ventilation with positive end-
expiratory pressure (PEEP).
Additional Information
E. Perforation of Viscus
H&P Keys
Abdominal rigidity, pain, peritoneal irritation, reduced or absent
bowel sounds, shoulder pain.
Diagnosis
History and physical exam, x-rays, CT scan, diagnostic peritoneal
lavage, exploratory laparotomy.
Disease Severity
Determined by initial presentation, presence of coexisting medical
problems, and baseline condition.
Management
Laparotomy and surgical repair.
Spleen—Repair or splenectomy.
Colon—Repair (resection if severe injury).
Stomach—Repair.
Additional Information
Diagnosis
History and physical exam, x-ray studies, CT exam.
Disease Severity
Review x-rays. Prognosis worse in elderly and with coexisting medical
problems.
Treatment Steps
1. Depends on multiple factors, with open reduction and internal
fixation (ORIF), traction, and external fixation as choices.
2. Evaluation for coexisting injuries or trauma and treatment ac-
cordingly.
Fracture of Ilium—Rest.
Fracture of Anterior Superior Spine—Surgery.
Fracture of Sacrum—Rest and support.
Additional Information
Hemorrhage is the most important complication of pelvic fracture.
Angiography with possible embolization may be required for severe,
persisting hemorrhage.
IV. LACERATIONS
H&P Keys
Observation; consider both history and source of injury; ascertain
tetanus immunization status.
Diagnosis
History and physical exam.
Disease Severity
Evaluate neurovascular status and check for associated injuries, frac-
tures, and hypotension.
190
INJURIES, WOUNDS, TOXICOLOGY, AND BURNS Foreign Bodies
V. FOREIGN BODIES
Diagnosis
Physical exam.
Disease Severity
Treatment Steps
B. Aspiration
H&P Keys
Wheezing and dyspnea may be present. History of child with object
in mouth, reduced cough reflex secondary to anesthesia, disease,
etc. Often an abrupt onset of cough or wheezing, dyspnea, and voice
change. Most common in children under age 4.
Diagnosis
CXR (opaque foreign body, atelectasis, or unilateral hyperinflation
causing mediastinal shift).
Disease Severity
Evaluate degree of respiratory distress.
191
C. Swallowed
H&P Keys
Sudden onset of gagging, pain, and choking.
Diagnosis
Indirect laryngoscopy, x-rays, including barium swallow.
Disease Severity
Observation and evaluation for esophageal perforation.
Concept and Application
Increased frequency with motility disorders, stricture, and children
results in lodged foreign body. Most common location is at the
cricopharyngeus muscle.
Treatment Steps
Endoscopic removal. Identify location (above or below gastro-
esophageal sphincter). Observe. If perforation, antibiotics are given
to avoid mediastinitis. Do not give meat tenderizer for obstruction
by meat.
VI. BURNS
A. Eye Burns
See Ocular Injury section on page 185.
B. Thermal Burns
H&P Keys
Assess degree of burn depth, determine etiology (thermal, chemical,
etc.), duration of exposure, and emergency or home treatment ren-
dered.
Diagnosis
History and physical exam.
Disease Severity
Erythema minor (first degree). Blisters (split thickness; second de-
gree). Pain (first and second degree). No pain (third degree).
Concept and Application
Burns result in thermal skin and tissue injury. Total epidermis de-
struction with partial dermis destruction is typical of second-degree
192
INJURIES, WOUNDS, TOXICOLOGY, AND BURNS Poisoning
Treatment Steps
Removal of patient from source of burn, CPR, cooling burn, clean-
ing and debridement of burn, fluids (Ringer’s initially), determina-
tion of area of burn, full history and physical exam, antibiotics,
tetanus toxoid, grafting. For minor burn: loose gauze wrap on non-
adhering dressing. For severe burns: CPR and airway control, fluid
replacement (monitoring CVP and output), NG tube, pain and sep-
sis control (morphine), surgical treatment (grafting, etc.).
Additional Information
Rule of nines to estimate burn extent: Each leg is 18%, each arm
9%, body front 18%, back 18%, head 9%, groin 1%.
C. Electrical Burns
H&P Keys
Look for an entry or exit wound (high voltage, lightning). Massive
tissue and bone destruction may be noted. Patients may be comatose
and in cardiac arrest.
Diagnosis
History and physical exam, serial arterial blood gases (ABGs), and
hematocrit.
Disease Severity
Prognostic factors include duration of electrical contact, amount of
grounding present, path of the current, and amount of moisture
present (moisture lowers skin resistance).
Massive tissue necrosis may precede infection, rhabdomyolysis.
Assess and monitor cardiac and pulmonary status. Persisting myoglo-
binuria indicates significant muscle injury.
Treatment Steps
1. Safe removal of patient from source, CPR, fluids and electrolyte
treatment, cleaning and debridement of burns, surgical evalua-
tion (fasciotomy, amputation), tetanus toxoid.
2. Significant fluid replacement may be required.
3. Monitor for arrhythmias.
4. Silver sulfadiazine cream may be employed topically.
VII. POISONING
A. Acetaminophen
H&P Keys
Nausea and vomiting and diaphoresis.
Diagnosis
Serum acetaminophen level.
193
B. Tricyclic Antidepressants
H&P Keys
Transient hypertension, then hypotension, tachycardia, arrhythmias,
conduction blocks, seizures, anticholinergic symptoms (dry mucosa
or skin, urinary retention).
Diagnosis
History and physical exam, ECG (wide QRS). Blood levels not rou-
tinely available as stat test.
Disease Severity
Increasing serum levels correlated with increasing risk (seizures, ar-
rhythmias). Monitor mentation, cardiac status, respiratory rate and
exchange.
Treatment Steps
1. Gastric lavage, activated charcoal, NG tube suction.
2. Sodium bicarbonate to correct acidosis.
3. Physostigmine, phenytoin (Dilantin) for seizures.
C. Sedatives
H&P Keys
Lethargy, confusion, coma, hypotension, respiratory depression, dis-
conjugate eye motion.
Diagnosis
History and physical exam, urine drug screen, blood drug level avail-
able.
Disease Severity
Coma scale, respiration depression. Length of time since ingestion
and history of amount ingested may assist in severity determination.
Treatment Steps
1. Control of airway, activated charcoal (if patient awake), cautious
gastric lavage.
2. Supportive care.
D. Stimulants
H&P Keys
Euphoria, dilated pupils, hypertension, tremors, tachycardia, hyper-
activity, psychosis, hyperthermia, seizures, anxiety, nausea and vomit-
ing.
Diagnosis
History and physical exam (tremor, increased bowel sounds, etc.).
194
INJURIES, WOUNDS, TOXICOLOGY, AND BURNS Poisoning
Disease Severity
Determined by amount ingested (peak effects 1–2 hours after inges-
tion), coexisting medical problems, cardiac status.
Treatment Steps
1. Supportive (treatment of hypertension, seizures, arrhythmias),
charcoal.
2. Emesis may cause seizures.
E. Cocaine
H&P Keys
Agitation, hyperthermia, hypertension, cardiac arrhythmia, tachycar-
dia, seizures, pulmonary edema.
Diagnosis
History and physical exam, urine drug screen.
Disease Severity
Assess by history, cardiac status.
Management
Supportive treatment, control of airway, monitor core temperature.
F. PCP (Phencyclidine)
H&P Keys
Nystagmus, blank stare, psychosis, lethargy, incoordination, violent
behavior, self-destructive behavior.
Diagnosis
History and physical exam, urine drug screen. May have elevated
creatine phosphokinase and myoglobinuria.
Disease Severity
Physical exam.
Treatment Steps
Control of airway, activated charcoal, supportive therapy.
G. Alcohol
H&P Keys
Diagnosis
History and physical exam, blood alcohol level. May have elevated
triglycerides, uric acid, and γ-glutamyl transferase (GGT).
Disease Severity
Assess history, impact on the individual and the family, and clinical
picture (withdrawal, abnormal lab tests, hepatic function).
Treatment Steps
J. Carbon Monoxide
H&P Keys
Headache, confusion, nausea, dyspnea, clumsiness, cyanosis, or
cherry-red skin.
Diagnosis
History and physical exam (cyanosis), elevated blood carboxyhemo-
globin.
Disease Severity
Chronic exposure associated with parkinsonism.
Treatment Steps
1. Removal from source, 100% oxygen.
2. Hyperbaric oxygen (if available) for comatose patients.
K. Diethyl-m-toluamide (DEET)
H&P Keys
CNS symptoms, seizures, coma, hypotension, GI irritation.
Diagnosis
History and physical exam.
C
196
INJURIES, WOUNDS, TOXICOLOGY, AND BURNS Poisoning
on rounds
Disease Severity
By clinical exam (neurologic status, time since exposure, degree of
exposure).
Treatment Steps
Emesis, gastric lavage.
L. Additional Information
1. Other Poisonings
Iron—Symptoms include diarrhea, abdominal pain, and bloody
stools. Gastric lavage, parenteral deferoxamine.
Aspirin—Causes respiratory alkalosis and metabolic acidosis. Pa-
tient may be hyperventilating, diaphoretic, and report tinnitus.
Theophylline—Look for tremor, nausea and vomiting, and meta-
bolic acidosis. Blood drug level can be checked.
Narcotics—Pinpoint pupils, hypotension; administration of nalox-
one hydrochloride (Narcan).
Barbiturates—Respiratory depression, hypotension; supportive
care, charcoal, and alkalinization of urine.
2. Selected Antidotes
Folic acid for methyl alcohol poisoning. D-Penicillamine for copper
poisoning. Protamine sulfate for heparin overdose. Latrodectus an-
tivenin for black widow spider bites.
3. Drugs Visible on X-Ray
Heavy metals, phenothiazines, iodides, and chloral hydrate.
4. Poisoning Management Overview
1. Airway, breathing, circulation: history and physical exam; lab
studies; gastric lavage and emesis; antidote after lavage; support-
ive care; laboratory workup.
2. Avoid ipecac with caustic ingestion and in somnolent patient.
3. If antidote available, cautious use of charcoal in addition. Used
primarily in children.
197
A. Vertebral Column
H&P Keys
Pain, neurologic abnormalities.
Diagnosis
History and physical exam, x-ray exam, CT exam.
Disease Severity
Neurologic status, serial evaluations.
B. Extremities
1. Tibia
H&P Keys
Pain when pressure applied to the tibia.
Diagnosis
History and physical exam, x-ray.
Disease Severity
Determine severity via x-ray, coexisting medical problems.
Treatment Steps
2. Fibula
H&P Keys
Pain and swelling, with retained ability to walk.
Diagnosis
History and physical exam, x-ray exam.
198
INJURIES, WOUNDS, TOXICOLOGY, AND BURNS Fractures
Disease Severity
Rule out coexisting ankle injury.
Concept and Application
Trauma.
Treatment Steps
Walking cast or boot, followed by therapy.
3. Femur
H&P Keys
Pain, swelling, deformity.
Diagnosis
History and physical exam, x-ray studies.
Disease Severity
Evaluate for coexisting medical problems, hypotension.
Concept and Application
Usually significant trauma. With children, rule out child abuse.
Treatment Steps
Femoral Neck
1. ORIF vs. long-term traction (usually in immobile patients).
2. Complications include avascular necrosis and nonunion of
fracture.
4. Radius
H&P Keys
Pain, reduced elbow-joint motion.
Diagnosis
History and physical exam, roentgenographic studies.
Disease Severity
Check neurovascular status.
Concept and Application
Fall on hand common.
Colles’ Fracture—Fall on extended wrist, fracture of distal radius
and ulnar styloid (volar angulation and dorsal displacement).
Smith’s Fracture—Fall on flexed wrist (dorsal angulation and volar
displacement).
Treatment Steps
Radial Head
1. Hemarthrosis aspiration and mobilization if simple.
2. Surgical (ORIF) if complete or displaced fracture.
Distal Radius Undisplaced—Cast 4–6 weeks, therapy.
5. Ulna
H&P Keys
Pain, swelling, deformity.
Diagnosis
History and physical exam, x-ray.
199
6. Humerus
H&P Keys
Pain, swelling.
Diagnosis
History and physical exam, x-ray studies.
Disease Severity
Evaluate neurovascular status.
Treatment Steps
Additional Information
A. Hand
1. Distal Interphalangeal (DIP) Sprain
H&P Keys
Pain, difficulty with joint flexion or extension.
Diagnosis
History and physical exam, x-ray exam.
Disease Severity
Evaluate strength and range of motion.
Concept and Application
DIP joint injury or sprain, resulting in possible flexor–extensor ten-
don disruption.
200
INJURIES, WOUNDS, TOXICOLOGY, AND BURNS Sprains and Dislocations
Treatment Steps
Symptomatic (if able to bend or extend joint).
Additional Information
Diagnosis
History and physical exam. X-ray to rule out fracture.
Disease Severity
Examine for neurovascular status.
Treatment Steps
Flexion splint 2 weeks.
Additional Information
B. Ankle
1. Lateral Ankle Pain
Injury to anterior talofibular ligament. May also include injury to
fibulocalcaneal and posterior talofibular ligaments.
201
C. Elbow Dislocation
Check vascular and neurologic status; if urgent reduction indicated,
splint.
X. DROWNING
H&P Keys
Wheezing, tachypnea, vomiting, pulmonary edema, unconscious-
ness, shock, and cardiac arrest.
Diagnosis
History and physical exam, CXR, ABGs, ECG.
Disease Severity
Consider duration of immersion, patient’s baseline medical status,
water temperature, timing of rescue measures, cardiac and pul-
monary status, electroencephalogram (EEG).
Treatment Steps
1. Urgent CPR and 100% oxygen.
2. Remember to continue CPR in hypothermic or prolonged cold-
water submersion victims.
Diagnosis
History and physical exam. Leukocytosis and coagulation disorders.
Disease Severity
Assess cardiac and pulmonary status. Consider patient’s age and pre-
existing medical problems, time since envenomation, location of
bite (trunk has worse outcome than extremity), snake size (larger
worse), and emergency treatment received.
Treatment Steps
Additional Information
Diagnosis
History (onset of symptoms in seconds to minutes) and physical
exam.
Disease Severity
Assess cardiac and pulmonary systems. Prognosis worse without early
intervention.
Treatment Steps
1. CPR and control airway.
2. Epinephrine (1:1,000 0.3–0.5 mL SQ or 1:10,000 0.5–1 mg slow
IV administration if in shock), Benadryl, fluids, dopamine, in-
haled β-agonists, corticosteroids, oxygen.
203
A. Traumatic Injury
Administer CPR, control airway, treat urgent problems (large pneu-
mothorax, hemorrhage, etc.), administer oxygen, insert IV line, give
fluids and medications, obtain history and physical exam, x-ray and
lab studies.
B. Shock
Administer CPR, control airway, obtain history and physical exam,
administer fluids (caution with cardiogenic shock, check CVP and
output), administer vasopressors (dopamine), get lab studies, ad-
minister: corticosteroids, diuretic (protects kidneys), buffers, antibi-
otics (septic shock).
D. Thermal Injuries
1. Frostbite
H&P Keys
Tissue cold and hard without feeling.
Diagnosis
History and physical exam; affected area may be white (superficial
injury) or firm and frozen (deep injury).
Disease Severity
Duration of exposure, prior presence of peripheral vascular disease
or other medical problems.
Concept and Application
Tissue damage as a direct result of thermal trauma. Skin and tissue
damage from ice crystal formation. May be superficial or deep. Line
of demarcation may develop.
Treatment Steps
Rapid rewarming after body core temperature warming, tetanus tox-
oid, possibly antibiotics, surgical evaluation, amputation.
2. Hypothermia
H&P Keys
Reduced core temperature (under 35°C), lethargy, coma, hypoten-
sion, confusion, miotic pupils.
Diagnosis
History and physical exam, core temperature at or below 95°F
(35°C), ECG (Osborne wave, elevated J-point; bradycardia; arrhyth-
mias), flat EEG, metabolic acidosis.
Disease Severity
Assess duration of exposure, age (mortality much worse in the el-
derly), emergency treatment rendered, and preexisting medical
problems. Worse prognosis with lower temperatures.
204
INJURIES, WOUNDS, TOXICOLOGY, AND BURNS Epidemiology and Prevention of Selected Accidents
A. Home Accidents
Epidemiology—Involves all age groups and consists of a wide variety
of hazards (electrical, thermal, poisoning, and trauma).
Prevention—Includes education, preventive planning (bicycle hel-
mets, toy and playground equipment checks, removal of danger-
ous objects, obstacles, etc.).
B. Workplace Accidents
Epidemiology—Includes increased risk groups (meat cutters and
packers, steelworkers, etc.), along with all employees.
205
INJURIES, WOUNDS, TOXICOLOGY, AND BURNS Epidemiology and Prevention of Selected Accidents
Prevention—Includes both education and exercise of precautions
(eye shield, hearing protection, hard hats, steel-tip shoes, etc.) and
elimination of dangerous materials, practices, and procedures.
C. Athletic Accidents
Epidemiology—Includes home, school, recreational, and profes-
sional accidents. Impact and type of injuries are multiple, includ-
ing falls, trauma, thermal injury, sprains and strains, fractures,
concussions, contusions, and death.
Prevention—Includes education, correction of both training and
performance errors (spearing in football), providing protective
equipment of correct fit.
F. Drowning
Epidemiology—Involves children most often.
Prevention—Centers on education (parents and children), swim-
ming instruction, water and boating safety, dangers of hyperven-
tilation, dangers of drug and alcohol use, and CPR training.
Recognition of high-risk patients (epilepsy, syncope, divers, chil-
dren, etc.).
I. Thermal Injury
Prevention—Key points include education (increased heat disor-
ders with alcohol; cystic fibrosis patients; dehydration; dark, non-
breathable clothing; use of antipsychotics and diuretics; high-
humidity days) and need for increased fluid intake and gradual
heat acclimatization. Skin protection and early recognition of
symptoms in frostbite and hypothermia patients is critical. In-
creased awareness for high-risk patients (elderly and children, al-
cohol and drug abusers, CNS disease, and sepsis) is important.
L. Fire Prevention
Prevention—Includes education (not smoking in bed, proper stor-
age of flammables, etc.), home precautions (smoke detectors
and fire extinguisher, fireplace glass screen, fire safety plan, es-
cape route, upkeep of electrical systems, etc.).
M. Falls
Epidemiology—Affect children, elderly, and adults (workplace in-
jury, seizure-disorder patients, alcoholics) and are a frequent
207
INJURIES, WOUNDS, TOXICOLOGY, AND BURNS Epidemiology and Prevention of Selected Accidents
cause of accidental death. Significant morbidity and mortality as-
sociated with hip fractures.
Prevention—For children includes child-proofing the house, cover-
ing sharp corners, window locks, stair gates, and control of obsta-
cles. For the elderly, medical and family assessment for need of
cane, walker, or wheelchair; medical treatment or control of con-
tributing illness (Parkinson’s disease, visual impairment, anemia,
stroke, etc.).
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D’Ambrosia RD. Musculoskeletal Disorders, Regional Examination and Differential Diagno-
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Infectious Disease 8
I. HUMAN IMMUNODEFICIENCY VIRUS (HIV) INFECTION AND THE ACQUIRED
IMMUNE DEFICIENCY SYNDROME (AIDS) / 210
A. HIV Infection / 210
B. Pneumocystis jiroveci (formerly carinii ) Pneumonia (PCP) / 212
C. Cytomegalovirus (CMV) Infection / 213
D. Tuberculosis (TB) / 214
E. Disseminated Mycobacterium avium-intracellulare Complex (MAC) / 215
F. Toxoplasma Encephalitis / 216
G. Cryptococcal Infection / 217
H. Herpes Simplex Virus (HSV) / 217
BIBLIOGRAPHY / 225
209
Copyright © 2004, 2001 by The McGraw-Hill Companies, Inc. Click here for terms of use.
210
INFECTIOUS DISEASE Human Immunodeficiency Virus (HIV) Infection and the Acquired Immune Deficiency Syndrome (AIDS)
A. HIV Infection
diagnostic
decisions d H&P Keys
HIV infection is acquired by sexual transmission, direct blood con-
tact, or from mother to child. About 4–6 weeks after exposure to
HIV, a mononucleosis-like illness (fever, malaise, lymphadenopathy,
HIV DIAGNOSIS— rash, headache, arthralgias, or myalgias) occurs in most but not all
FACTORS LEADING people, lasting 1–2 weeks. Then there is an asymptomatic period
TO SUSPICION with active viral replication and declining immune function. Ulti-
mately, the patient may develop sweats, weight loss, diarrhea, and/or
Risk Factors an opportunistic infection (see individual descriptions of common
Sexual contact, sharing opportunistic infections [OIs] for clinical clues) or malignant dis-
needles, unscreened blood ease associated with HIV-related immunosuppression.
or blood products,
maternal/fetal.
Diagnosis
Clinical Features Before HIV testing (enzyme-linked immunosorbent assay [ELISA], Western
Developing Opportunistic
blot) is the first blood test. Further workup in the HIV-positive pa-
Infection
Weight loss/wasting,
tient includes: (1) tracking immune function (CD4+-positive T-lym-
fatigue, adenopathy, phocyte count [CD4+]); (2) following viral burden (quantitative vi-
diarrhea, oral lesions, ral RNA); (3) general tests such as complete blood count (CBC),
cognitive deficits. chemistry (including liver tests), VDRL (Venereal Disease Research
Opportunistic Infections Laboratory) or rapid plasma reagin (RPR), purified protein deriva-
Pneumocystis jiroveci tive (PPD), hepatitis B serology, cytomegalovirus (CMV) serology,
pneumonia, toxoplasmosis serology, Pap smear. Viral load measurements help to
Mycobacterium avium predict the initial rate of progression to AIDS and to select, main-
complex infection,
tain, and modify effective antiretroviral therapy.
toxoplasmosis,
cryptococcal meningitis,
Disease Severity
unexplained thrush or
esophageal candidal Association between development of OIs and absolute (normal =
infection. 800–1,200) or percentage (normal = 55–70%) CD4+. As CD4+
Unexplained “Normal declines, risk of OI increases substantially. With CD4+ > 800, risk of
Infections” OI very small; CD4+ 200–500, increasing risk for Mycobacterium tuber-
Tuberculosis, syphilis, culosis, Histoplasma, Cryptococcus; CD4+ < 200, risk for Pneumocystis
severe pneumococcal jiroveci pneumonia (PCP; formerly known as Pneumocystis carinii
disease, histoplasmosis. pneumonia); CD4+ < 100, increasing risk for Toxoplasma, CMV, My-
Malignancies cobacterium avium complex (MAC). OIs are associated with the ma-
Kaposi’s sarcoma, jority of AIDS deaths. High HIV viral load predicts rapid disease pro-
lymphoma, Hodgkin’s gression. Initiation or modification of antiretroviral therapy may be
disease.
dictated based on these results. (See Tables 8–1 and 8–2.)
INFECTIOUS DISEASE Human Immunodeficiency Virus (HIV) Infection and the Acquired Immune Deficiency Syndrome (AIDS)
8-1
1993 REVISED CLASSIFICATION SYSTEM FOR HIV INFECTION AND EXPANDED
AIDS SURVEILLANCE CASE DEFINITION FOR ADOLESCENTS AND ADULTSa
Clinical Categories
1. ≥ 500/µL A1 B1 C1
2. 200–499/µL A2 B2 C2
3. < 200/µL A3 B3 C3
AIDS-indicator
T-cell count
aPersons with the AIDS-indicator conditions (category C) as well as those with CD4+ T-lymphocyte counts < 200/µL are reportable
as AIDS cases.
bSee text AIDS case definition.
PGL, persistent generalized lymphadenopathy.
8-2
CONDITIONS INCLUDED IN THE 1993 AIDS SURVEILLANCE CASE DEFINITION
Diagnosis
Usually develops when CD4+ < 200. Arterial blood gas: hypoxemia,
increased A-a gradient. Chest x-ray (CXR) usually shows bilateral in-
213
INFECTIOUS DISEASE Human Immunodeficiency Virus (HIV) Infection and the Acquired Immune Deficiency Syndrome (AIDS)
filtrates. Definitive diagnosis by demonstrating organism in pul-
monary specimen (induced sputum, bronchoalveolar lavage [BAL],
biopsy).
Disease Severity
Mild disease (patient may be candidate for oral, outpatient therapy):
PO2 > 70 mm Hg, able to take oral medication, reliable follow-up.
More severe hypoxemia and tachypnea are poor prognostic features.
Concept and Application
P. jiroveci is a fungus. Infection can occur early in life but rarely
causes disease in the normal host. HIV-related immunosuppression,
severe malnutrition, lymphopoietic malignancy, and organ trans-
plantation allow P. jiroveci to cause disease.
Treatment Steps
1. Mild disease may be treated orally; severe disease is treated par-
enterally.
2. Drug of choice is considered to be trimethoprim–sulfamethoxa-
zole (TMP-SMZ).
3. Alternative agents include pentamidine, dapsone and trimetho-
diagnostic
decisions d
prim, clindamycin and primaquine, or atovaquone.
4. Duration of therapy is usually 21 days. For nonventilated patients ELEMENTS TO FOLLOW
with more severe disease (room air PO2 < 70, A-a gradient > 35 IN HIV-INFECTED ADULT
mm Hg), a short course of corticosteroids is recommended.
Clinical
Health Maintenance—Primary PCP prophylaxis is recommended for Weight loss, sense of well-
all HIV-infected individuals with a CD4+ < 200. Secondary pro- being, cognitive function,
phylaxis is indicated for all with a previous episode of PCP. Drug functional status, new
of choice is TMP-SMZ with alternatives, including dapsone with infection, new malignancy.
or without trimethoprim or pyrimethamine, aerosolized or IV Laboratory—Immune
pentamidine, or clindamycin and primaquine. People receiving CD4+ absolute count,
active treatment for toxoplasmosis need not take PCP prophy- CD4+/CD8+ ratio, skin test
laxis. For people with stable increase in CD4+ cells above reactivity.
250/mm3, prophylaxis can be discontinued. Laboratory—Virologic
Level of HIV RNA (by PCR
C. Cytomegalovirus (CMV) Infection of b-DNA), genotypic.
H&P Keys
CMV infection is the most common viral OI in advanced AIDS. The
most common infections are retinitis, gastrointestinal (GI) tract
(colitis, esophagitis), and systemic (viremia associated with wasting
syndrome). Ocular complaints include “floaters,” decreased vision,
or blindness. Ophthalmoscopic exam is diagnostic. Colitis is associ-
ated with persistent diarrhea and crampy abdominal pain. Esophagi-
tis presents with odynophagia. Wasting syndrome consists of signifi-
cant weight loss with fever or diarrhea. CMV encephalitis is
characterized by cognitive and focal neurological changes, periven-
tricular lesions on brain scan, and sometimes a polyradiculopathy.
Diagnosis
CMV retinitis diagnosed by ophthalmoscopic exam revealing exu-
dates and inflammatory changes following a vascular distribution.
Isolation of CMV from other body sites confirms the ophthalmo-
scopic impression. CMV colitis or esophagitis is diagnosed by en-
doscopy revealing edema, erythema, erosions, and hemorrhage. Cy-
tomegalic inclusions seen on histopathologic exam are diagnostic.
CMV may be recovered from buffy coat blood culture in some pa-
tients with the wasting syndrome.
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INFECTIOUS DISEASE Human Immunodeficiency Virus (HIV) Infection and the Acquired Immune Deficiency Syndrome (AIDS)
Disease Severity
The severity of CMV retinitis often is dictated by the anatomic loca-
tion of lesions. Macular involvement severely affects vision; optic
nerve involvement may cause blindness.
D. Tuberculosis (TB)
H&P Keys
Symptoms of TB are usually pulmonary or may reflect extrapul-
monary disease. The most common sites are peripheral lymph nodes
and bone marrow. Other extrapulmonary sites include bone and
joint, urine, liver, spleen, GI mucosa, and cerebrospinal fluid (CSF).
TB may present as wasting syndrome. TB in early HIV infection
tends to be similar to disease in non–HIV-infected patients. TB in
later-stage HIV disease is more commonly atypical.
Diagnosis
Pulmonary TB often occurs at a CD4+ of 250–500; extrapulmonary
TB more commonly occurs at a lower CD4+, often < 200. Skin test-
ing with PPD may be unreliable because of skin test anergy. CXR
may show typical nodular infiltrates, with or without cavitation (api-
cal lung fields most common), or more atypical lesions. Isolation of
M. tuberculosis from pulmonary or other sites is diagnostic.
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INFECTIOUS DISEASE Human Immunodeficiency Virus (HIV) Infection and the Acquired Immune Deficiency Syndrome (AIDS)
Disease Severity
Response to therapy is usually good. Mortality rates are higher and
median survival time shorter in the AIDS patient, although patients
with restored immune function do well. Disseminated or extrapul-
monary disease is more common with advanced immunodeficiency.
Concept and Application
HIV-related immunosuppression increases the frequency and sever-
ity of TB disease. The HIV-infected person is at risk for developing
active disease from a new exposure as well as from reactivation of
previously acquired, inactive disease.
Treatment Steps
1. A minimum treatment course of 9 months is recommended in
AIDS patients.
2. In patients without previous treatment for TB and living in an
area where drug resistance is low, a four-drug regimen (isoniazid
[INH], rifampin, pyrazinamide, and ethambutol) is recom-
mended for initial treatment.
3. In patients with a history of previous TB treatment, contact with
multidrug-resistant TB, or living in an area with frequent drug re-
sistance, five or more initial anti-TB drugs are indicated.
4. These regimens consist of the standard four drugs plus ofloxacin,
ciprofloxacin, or streptomycin or other second-line anti-TB
drugs.
5. Even with these regimens, the likelihood of response for docu-
mented multidrug-resistant TB is disappointing.
6. Direct observed therapy (DOT) should be used whenever possible.
Health Maintenance—Twelve months of INH is indicated for all
HIV-infected people with a positive PPD. A 2-month course of rif-
ampin and pyrazinamide is also effective but may be more toxic.
F. Toxoplasma Encephalitis
H&P Keys
Toxoplasma gondii is a common cause of latent central nervous system
(CNS) infection in AIDS patients. Toxoplasma encephalitis often in-
volves the cortex, brain stem, and/or basal ganglia, with associated
neurologic abnormalities that may include focal deficit, change in
reflexes or sensation, ataxia, or decreased cognition.
Diagnosis
Blood tests for Toxoplasma are usually positive but can be unreliable.
Brain imaging typically reveals multifocal disease. A presumptive di-
agnosis usually triggers empiric therapy. Definitive diagnosis re-
quires visualization of the organism in brain tissue. Polymerase
chain reaction (PCR) of CSF is promising.
Disease Severity
Brain biopsy for definitive diagnosis usually is reserved for patients
who are seronegative, have atypical radiologic imaging (single le-
sions), or do not improve on empiric therapy.
Concept and Application
Reactivation of a latent infection generally occurs when the CD4+ is
< 100. Up to one-third of seropositive AIDS patients may ultimately
develop Toxoplasma encephalitis.
Treatment Steps
1. The initial 6 weeks of therapy commonly consists of pyrimeth-
amine, sulfadiazine, and folinic acid.
2. Response rates (at least partial improvement) are quite high, ap-
proximately 85%. Response is usually fast—failure to respond in a
week casts significant doubt on the diagnosis.
3. Clindamycin may replace sulfadiazine in the sulfa-allergic patient.
4. Chronic maintenance, often at reduced dose, is required lifelong
to prevent relapse.
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INFECTIOUS DISEASE Human Immunodeficiency Virus (HIV) Infection and the Acquired Immune Deficiency Syndrome (AIDS)
Health Maintenance—Trimethoprim–sulfamethoxazole used for
PCP prophylaxis is also effective prophylaxis for Toxoplasma en-
cephalitis.
G. Cryptococcal Infection
H&P Keys
Cryptococcal infections are the most common disseminated fungal
infections in patients with AIDS. Cryptococcus may involve the lungs,
skin, blood, bone marrow, prostate, and genitourinary tract, but usu-
ally presents as meningitis with subacute and nonspecific symptoms.
The most common are fever and headache, with nausea, vomiting,
and mental status changes occurring less frequently. Classic symp-
toms of meningismus, neck stiffness, and photophobia are uncom-
mon.
Diagnosis
CSF cryptococcal antigen allows for the most rapid diagnosis of cryp-
tococcal meningitis, and culture is the standard confirmatory test.
The India ink exam is no longer needed.
Disease Severity
Mental status changes, very high serum or CSF cryptococcal antigen
titers, and high opening pressure of CSF are associated with poor
prognosis.
Concept and Application
Cryptococcus neoformans is an encapsulated yeast commonly found in
soil and associated with bird feces. Disease is acquired by inhalation,
so the first site of infection is the lungs.
Treatment Steps
1. Standard acute therapy includes an approximate 2-week course of
amphotericin B with or without flucytosine. Ideally, the CSF
should become culture negative.
2. Some patients with mild disease may be able to start with flucona-
zole.
3. All patients receive lifelong maintenance therapy to prevent re-
lapse unless they achieve immune recovery.
4. Fluconazole is preferred for maintenance therapy.
5. Patients with very high CSF pressures may need frequent lumbar
punctures to relieve headache and prevent serious sequelae such
as blindness and herniation.
Disease Severity
Severity depends on the site of infection and degree of immunosup-
pression.
Concept and Application
Most AIDS patients have previously been infected with HSV and
have latent infection in nerve root ganglia. Latent HSV often reacti-
vates in the immunocompromised patient and can cause severe, pro-
longed disease.
Treatment Steps
1. Usual therapy consists of oral or parenteral acyclovir until the le-
sions are healed.
2. Many patients will relapse after initial therapy and will require re-
peated therapy, followed by long-term maintenance.
3. Long-term maintenance therapy with acyclovir is associated with a
risk of development of acyclovir resistance, dictating therapy with
other agents such as foscarnet.
A. Gonorrhea
H&P Keys
Uncomplicated gonococcal infections include urethritis, cervicitis,
anoproctitis, and pharyngitis (pelvic inflammatory disease [PID] is
discussed separately). Urethritis in men causes discharge and dys-
uria, whereas women report only dysuria. Cervicitis is generally
asymptomatic. Pharyngitis generally causes no symptoms, but ery-
thema and exudate may be present on exam. Anoproctitis may be as-
sociated with pain, and discharge may also be present. Disseminated
gonococcal infection resulting from bacteremia may cause petechial
or pustular skin lesions, tenosynovitis, septic arthritis, and occasion-
ally, hepatitis, endocarditis, or meningitis.
Diagnosis
Specific diagnosis is made by recovery of Neisseria gonorrhoeae from
discharge, blood, or other body fluid. Therapy for uncomplicated
disease is often empiric (without benefit of culture).
Disease Severity
Of the uncomplicated gonococcal infections, pharyngitis is the most
difficult to cure and dictates more specific therapy than does anal or
genital infection (see Treatment Steps, below). Hospitalization may
be advisable for initial therapy of disseminated infection.
Concept and Application
Genital infections in women may produce minimal or no symptoms.
PID as a sequela of gonorrhea or chlamydia may cause tubal scar-
ring, resulting in infertility or ectopic pregnancy.
Treatment Steps
1. Coinfection with chlamydia is common in people with gonococ-
cal infections.
2. Treatment for gonorrhea should include therapy against C. tra-
chomatis (see chlamydia Treatment Steps recommendations).
3. Resistance to penicillin and tetracycline is common. Recom-
mended regimens for uncomplicated gonorrhea include ceftriax-
219
B. Chlamydia
H&P Keys
C. trachomatis causes urethritis and cervicitis (PID is discussed sepa-
rately). Urethritis is characterized by mucoid or purulent discharge
and dysuria. Cervicitis is characterized by yellow endocervical exu-
date.
Diagnosis
Nonculture tests for chlamydia are reliable and much more available
and sensitive than cultures. Combined tests, e.g., ligase chain reac-
tion, for gonorrhea and chlamydia.
Disease Severity
As with gonococcal disease, chlamydial infection may go untreated
in the female patient and result in tubal scarring, infertility, or ec-
topic pregnancy.
Concept and Application
C. trachomatis is an obligate intracellular parasite of columnar or
pseudostratified cells. The incidence of chlamydial infection is
higher than that of gonorrhea.
Treatment Steps
1. Coinfection with the gonococcus is common in patients treated
for chlamydial infection.
2. Treatment for chlamydia should include therapy effective for
gonococcal infection (see gonorrhea Treatment Steps recom-
mendations).
3. Recommended regimens for chlamydial urethritis or cervicitis in-
clude doxycycline or azithromycin.
4. Alternatives include ofloxacin or erythromycin.
Health Maintenance—Sex partners should be referred for evaluation
and treatment. Safer sex is encouraged.
C. Syphilis
H&P Keys
Patients may seek treatment for signs or symptoms of primary infec-
tion (ulcer at the site of infection), secondary infection (rash, muco-
220
INFECTIOUS DISEASE Sexually Transmitted Diseases (STDs)
Treatment Steps
d
1. Patients with primary, secondary, and early latent syphilis are
diagnostic treated with one 2.4-million-units IM injection of benzathine
decisions penicillin.
2. Nonpregnant penicillin-allergic patients can be treated with a
GENITAL ULCER DISEASE tetracycline for 2 weeks. This is less satisfactory than penicillin
and needs closer follow-up.
Syphilis 3. Patients with late latent and tertiary syphilis are treated with three
Clean, painless, usually weekly 2.4-million-units IM injections of benzathine penicillin G.
single ulcer; large nodes. 4. Four weeks of a tetracycline offers alternative therapy.
Chancroid 5. Neurosyphilis is treated for 10–14 days with high-dose penicillin.
Dirty, ragged, painful ulcer; Even patients with documented penicillin allergy should be con-
large nodes. sidered for penicillin desensitization.
Lymphogranuloma 6. All patients require follow-up serologic testing (CSF if appropri-
Venereum ate) to monitor response.
Small ulcer, large nodes, 7. Although data are incomplete, HIV-infected patients are gener-
“groove sign” between the
ally treated as above (although some recommend more intense
inguinal and femoral nodes.
treatment than is dictated by stage).
Herpes Simplex
Multiple painful, clustered Health Maintenance—Sex partners should be referred for evalua-
vesicles or ulcers; variable tion, although transmission occurs only when mucocutaneous le-
nodes. sions are present (uncommon after the first year). Patients
should be considered for HIV testing.
221
F. Epididymo-orchitis
H&P Keys
Unilateral testicular pain and tenderness and palpable swelling of
the epididymis are common.
Diagnosis
Culture for N. gonorrhoeae and C. trachomatis and urine culture.
Disease Severity
Failure to improve within 3 days requires reevaluation and consider-
ation of hospitalization.
Concept and Application
In men < 35 years of age, epididymo-orchitis is caused by gonococcal
or chlamydial infection. Nonsexually transmitted disease associated
with urinary tract infection caused by enteric bacilli is more com-
mon in men > 35 years of age.
Treatment Steps
1. Treatment of sexually transmitted epididymo-orchitis includes
therapy for both chlamydia and gonorrhea.
2. Single-dose ceftriaxone and 10 days of doxycycline or ofloxacin
are recommended.
Health Maintenance—Sex partners should be referred for evaluation
and treatment.
G. Genital Herpes
H&P Keys
Groups of painful vesicles that progress to shallow ulcerative lesions
prior to crusting and healing. Commonly seen on external genitalia
and on the cervix. Systemic symptoms such as fever, headache, and
myalgias are not uncommon with initial episodes.
Diagnosis
Viral culture for HSV.
Disease Severity
Most infected persons never recognize signs of genital herpes; some
have symptoms during the initial episode and then never again. A
minority of infected persons have recurrent genital lesions.
Concept and Application
Genital herpes, usually caused by herpes simplex virus type 2
(HSV-2), is a recurrent disease without cure. Serologic evidence sug-
gests that 30 million people in the United States have been infected.
Treatment Steps
1. Acyclovir and similar drugs such as valacyclovir and famciclovir
speed recovery from initial episodes or recurrences and can be
used to suppress recurrent disease.
2. Therapy cannot eradicate latent virus.
A. Infectious Mononucleosis
H&P Keys
Fever, sore throat, lymphadenopathy, splenomegaly. Headache,
myalgias, sweats, anorexia, and abdominal pain may also be present.
223
Disease Severity
Complications of infectious mononucleosis include hemolytic ane-
mia (positive Coombs’ test), thrombocytopenia, granulocytopenia,
splenic rupture rarely, neurologic problems (e.g., Guillain–Barré
syndrome), myocarditis, or pericarditis.
Treatment Steps
1. There is no specific therapy for EBV infection.
2. Corticosteroids are occasionally prescribed for severe tonsillitis
with airway compromise, severe hemolytic anemia or thrombocy-
topenia, neurologic complications, myocarditis, or pericarditis.
Diagnosis
Clinical clues supported by serologic tests are useful in diagnosing
these illnesses. While an astute clinician can usually diagnose them
easily, fewer and fewer doctors are seeing patients with these viral in-
fections.
Disease Severity
Varicella is occasionally complicated by pneumonia, encephalitis, or
Reye’s syndrome. Infection in the compromised host often includes
severe skin disease and dissemination to visceral organs. Measles can
be followed by pneumonia or encephalitis. Rubella is usually mild
except for persistent arthralgia and arthritis. The greatest risk is to
the fetus if a pregnant woman develops rubella. Mumps can lead to
orchitis in men.
Treatment Steps
1. The use of acyclovir in a usual episode of varicella, though ap-
proved, is controversial except in pregnancy.
2. Acyclovir therapy is routinely used for disseminated disease, CNS
involvement, and pneumonia (zoster is discussed in Chapter 2).
3. Acyclovir is not likely to be effective if use is delayed beyond 3–4
days following onset of symptoms.
4. There is no standard treatment for measles or rubella.
Health Maintenance—Vaccine for varicella zoster virus is indicated
for everyone older than 1 year without a history of clinical vari-
cella. The duration of protection remains to be determined.
Zoster immune globulin is given to seronegative immunosup-
pressed patients exposed to varicella.
224
INFECTIOUS DISEASE Other Infectious Diseases
C. Lyme Disease
H&P Keys
Lyme disease has been divided into three stages:
Stage 1––erythema migrans.
Stage 2––neurologic or cardiac involvement.
Stage 3––arthritis or subtle neurologic complaints.
Lyme disease does not progress in an orderly manner from stage to
stage, so there is greater clinical utility in determining whether the
infection is localized or disseminated and whether it is acute or
chronic. Current therapy is largely based on this determination. Ery-
thema migrans (EM) is an expanding, annular, erythematous skin
lesion at the site of tick attachment that clears centrally. Systemic
symptoms often occur with EM and correlate with systemic dissemi-
nation. There may be secondary skin lesions. Nervous system mani-
festations early in Lyme disease include meningitis, cranial neuritis,
and painful radiculopathy. Chronic nervous system manifestations
may include subtle changes in cognitive function or significant focal
abnormalities. The peripheral nervous system may also be involved.
Cardiac involvement is manifested as varying degrees of heart block,
myopericarditis, and cardiomyopathy. If untreated, nearly one-half
of patients will develop arthritis, mainly affecting large joints such as
the knee. Only a minority of patients progress to chronic arthritis.
Some people with late-stage Lyme disease can have persistent neuro-
logic complaints including forgetfulness, fatigue, and mild cognitive
impairment.
Diagnosis
The diagnosis is based on the clinical manifestations, with EM being
most useful. Serologic evidence is supportive but not standardized.
CSF (including serology) is examined with suspected CNS disease.
Disease Severity
Disease severity varies considerably among patients.
Concept and Application
Lyme disease is caused by the spirochete Borrelia burgdorferi. Ixodes
ticks are the principal vector throughout the world. Though re-
ported from most of the states, the majority of Lyme disease occurs
in three regions: the Northeast, upper Midwest, and the far West,
corresponding to the distribution of the tick vectors. Primary reser-
voirs for the tick include the white-footed mouse and other small ro-
dents. The larval, nymphal, and adult ticks feed on progressively
larger animals, with the adult tick favoring the white-tailed deer.
Transmission to humans occurs during feeding and requires attach-
ment for at least 24 hours.
Treatment Steps
1. EM, Bell’s palsy, mild cardiac disease, and early arthritis are
treated with oral doxycycline or amoxicillin.
2. More serious CNS disease, more serious cardiac disease, and
chronic arthritis are treated with parenteral ceftriaxone or peni-
cillin G.
3. A single 200-mg dose of doxycycline can abort infection but only
a small minority of tick bites transmit B. burgdorferi.
Health Maintenance—One may attempt to limit tick exposure and
the risk of Lyme disease by using repellents, reducing exposed
skin, and promptly removing any attached ticks.
225
227
Copyright © 2004, 2001 by The McGraw-Hill Companies, Inc. Click here for terms of use.
228 Musculoskeletal and Connective Tissue Disease
BIBLIOGRAPHY / 256
229
A. Osteomyelitis
H&P Keys
More common in children, may be triggered by trauma, usual seed-
ing is hematogenous. Presents with refusal to bear weight or move
joint or extremity. Fever usually present.
Diagnosis
White blood cell count (WBC), erythrocyte sedimentation rate
(ESR), blood cultures. Radiographs may show soft tissue swelling
early, followed by periosteal elevation and finally bone infarct and
involucrum. Bone scan shows focal increased activity. Magnetic reso-
nance imaging (MRI) shows marrow changes and soft tissue involve-
ment early in course of disease.
Disease Severity
Clinical evaluation following a change in ESR may be useful, and
C-reactive protein.
Management
Aspiration is useful to recover organisms for antibiotic selection.
Blood cultures may substitute if positive. IV antibiotics, followed by
oral medication after temperature is normalized for 6 weeks or until
ESR is normal. Immobilization for symptomatic relief. Surgical de-
bridement for refractory cases with involucrum and sequestra.
B. Septic Arthritis
H&P Keys
Pain, erythema, joint effusion, and soft tissue swelling. Refusal to
bend joint or bear weight in children.
Diagnosis
Joint aspirate, high white count > 50,000 suspicious, > 100,000 pre-
sumptive of infection pending culture results of infection. Polymor-
phonuclear leukocytes predominate. High peripheral WBC, elevated
ESR.
Disease Severity
Coexisting morbidity predisposes to infection (chronic disease, can-
cer, drug abuse, immune deficiency).
Treatment Steps
1. In children, hip joint requires early surgical drainage to prevent
joint destruction.
2. Adults may be treated with serial aspiration, following the WBC in
the fluid.
3. If no response (fall in WBC in synovial fluid), surgical debride-
ment, often arthroscopically.
4. IV antibiotics, followed by oral antibiotics.
C. Lyme Disease
H&P Keys
Variable presentation: joint effusions, arthralgia, myalgia, fatigue, oc-
casional cardiac arrhythmia, central nervous system (CNS) involve-
ment (Bell’s palsy, headaches). May have characteristic rash, ery-
thema chronicum migrans (“bull’s-eye” rash).
Diagnosis
Joint aspirates negative by culture, x-rays normal, serologic testing:
enzyme-linked immunosorbent assay (ELISA) screen, confirmatory
Western blot.
Disease Severity
Cardiac and CNS symptoms.
Treatment Steps
1. Treatment with oral doxycycline 100 mg bid or amoxicillin 2
g/day for 3–4 weeks.
2. Recalcitrant cases: IV ceftriaxone (Rocephin) 2 g/day for 6
weeks.
D. Gonococcal Tenosynovitis
H&P Keys
Acute loss of joint motion with fusiform swelling of digit. Erythema,
redness, fever, migratory polyarthritis, multiple arthralgia.
Diagnosis
Diagnosis confirmed by aspiration and culture (Gram stain showing
intracellular gram-negative diplococci). Radiographs normal.
Disease Severity
Multiple-location presentation.
Treatment Steps
Penicillin G or ceftriaxone.
231
Diagnosis
Characteristic radiographic findings are joint-space narrowing, sub-
chondral bony sclerosis, marginal osteophyte formation, subchon-
dral cyst formation. Laboratory findings are normal.
Differential Diagnosis—Roentgenographic findings of OA of the
hands are also seen with seronegative inflammatory arthritis. OA
of the hip may be avascular necrosis or pigmented villonodular
synovitis. OA of the knee may be meniscus pathology, osteochon-
dritis dessicans, or a sequela of septic arthritis, osteonecrosis.
Disease Severity
Disease severity is a clinical diagnosis with severe restriction of activi-
ties of daily living. Radiographs may be normal early, weight-bearing
films demonstrate joint-space narrowing. Osteophyte formation with
sclerosis and cyst formation occur late.
Treatment Steps
1. Protection of the joints from excessive force and chronic overuse.
Supportive splints, unloading braces, shoe wedges, and the use of
ambulatory aids (cane, crutches, walker) are protective.
2. Avoidance of repetitive impact loading (jumping, running) in
lower extremity OA. Weight loss is beneficial. Physical therapy to
increase joint motion and strength is beneficial.
3. Drug therapy consists of analgesics (acetaminophen), aspirin,
and nonsteroidal anti-inflammatory drugs (NSAIDs). Narcotics
should be used only as short-term measures. Intra-articular corti-
costeroid may be helpful in the management of acute flares. In-
232
MUSCULOSKELETAL AND CONNECTIVE TISSUE DISEASE Degenerative Disorders
Disease Severity
Physical evaluation, with loss of spine motion in all planes. Periverte-
bral muscle spasm. The presence of radicular pain below the knee
suggests neurologic involvement (e.g., disk disease). Pain with hy-
perextension and activity is suggestive of spinal stenosis (neurogenic
claudication).
Treatment Steps
1. Short period of rest (3–5 days).
2. Treatment of muscle spasm with ice massage and lumbar support.
3. Acetaminophen or NSAIDs for anti-inflammatory effect.
4. Major treatment is education in proper body mechanics to pre-
vent recurrence.
5. Muscle strengthening for the back and abdomen combined with
a flexibility program complete the treatment.
6. Aerobic fitness is beneficial in preventing recurrence.
Treatment Steps
1. Protective splinting to prevent contractures and physical therapy
to preserve joint motion may prevent surgical intervention.
2. For severe muscle imbalance, corrective surgery with release and
weakening of the flexors or augmentation of the extensors with
muscle transfers may be required.
3. Protection of insensate joints with bracing and full-contact or-
thotics may delay or prevent joint destruction.
4. Patient education in skin care.
Diagnosis
Radiographs are not helpful because the femoral head is not calci-
fied until at least 10 weeks of age. Ultrasonography of the hip is the
diagnostic procedure of choice in the first weeks of life.
Disease Severity
Delay in diagnosis gravely affects prognosis and treatment. If diagno-
sis delayed until 12–18 months as walking begins demonstrating a
limp and rolling gait, surgery will only achieve a useful hip but one
that will not be normal.
Management
Diagnosis
X-ray may be normal in early disease. Later x-rays will demonstrate
increased femoral head density or subarticular fracture line.
Disease Severity
Age is key to prognosis: Presentation after age 8 represents a poor
prognosis.
Treatment Steps
1. Maintenance of the sphericity of the femoral head is most impor-
tant factor for obtaining a good outcome.
2. Treatment is to first obtain normal range of motion with bed rest,
traction.
3. This is followed with bracing or surgery to contain the femoral
head in the acetabulum until revascularization and ossification
occurs.
Diagnosis
Radiographic images, especially lateral, are essential for showing dis-
placement.
Disease Severity
Degree of displacement determines residual problems. Significant
displacement may result in the development of avascular necrosis.
Chondrolysis may occur with the resultant osteoarthritis.
D. Intoeing
H&P Keys
Patient presents with an intoed gait. May complain of falling and
tripping over feet.
Diagnosis
No diagnostic studies are necessary.
Disease Severity
Clinical evaluation of degree of intoeing.
Concept and Application
There are three causes of intoeing: (1) anteversion of the femoral
neck, (2) metatarsus adductus, and (3) outward-curved tibiae (tibial
torsion). Femoral anteversion allows for greater internal rotation of
the hip than external rotation and is the major cause of intoeing.
The condition corrects itself as growth continues. Most instances are
corrected by age 10.
Treatment Steps
No treatment other than reassurance is needed.
A. Osteoporosis
H&P Keys
Progressive loss of height. Spontaneous, multiple vertebral body
compression fractures. Development of thoracic kyphosis. Fractures
of the hip and wrist. May complain of bone pain in the axial skeletal
or in the long bones of the lower extremity. History of multiple
stress fractures. Family history of osteoporosis. Risk factors: alcohol,
steroids, myeloma, postmenopausal, tobacco.
Diagnosis
Plain x-rays may show osteopenia. Increase in medullary/cortex ra-
tio of 2:1. Dual energy x-ray absorptiometry (DEXA) is the most sen-
sitive of the monitoring techniques. Complete lab evaluation should
include screens for thyroid, parathyroid, and renal disease; hemato-
logic disorders; and malignant disease.
Disease Severity
Loss of axial skeletal height, multiple compression fractures with
thoracic kyphosis. Multiple fractures of hips, wrists, ribs, ankles. Re-
current stress risks.
Treatment Steps
Best treated with prevention, low-dose estrogen replacement in
menopausal women. Calcitonin and bisphosphonates block bone re-
237
B. Gout
H&P Keys
Acute onset of painful, swollen, erythematous joints, deposition of
crystals in soft tissue, tophi. Attacks may be preceded by trauma, al-
cohol, drugs, surgical stress, or acute medical illness. Great toe com-
monly involved; ankle, knee, tarsal bone may be affected.
Diagnosis
Aspiration of joint fluid for birefringent crystals. Always send for cul-
ture and sensitivity.
Disease Severity
Polyarticular attacks, soft tissue tophi formation, joint destruction,
associated renal failure.
Concept and Application
Tissue deposition of monosodium urate crystals from supersaturated
extracellular fluids. Recurrent attacks of severe articular and periar-
ticular inflammation: gouty arthritis. Accumulation of crystalline de-
posits in the soft tissue: gouty tophi. Renal impairment: gouty
nephropathy. Causes: overproduction, 10%; underexcretion, 90%.
Dehydration as a result of trauma, surgery, diuretics may precipitate
attack.
Treatment Steps
1. Treatment with anti-inflammatory medications; indomethacin
(Indocin), 50 mg tid; or colchicine.
2. Aspiration and identification under polarized light microscopy
diagnostic with birefringent crystals.
3. If attacks reoccur, treatment with allopurinol is helpful.
4. Intra-articular steroids effective if oral medication is not tolerated
or contraindicated as in patients on anticoagulants or with
chronic renal insufficiency.
C. Rickets
H&P Keys
Brittle bones with ligamentous laxity, flattening of the skull, enlarge-
ment of the costal cartilages (rachitic rosary), dorsal kyphosis, bow-
ing of long bones.
Diagnosis
Radiographic evaluation: transverse radiolucent lines (“Looser’s
lines”), physeal cupping and widening. Laboratory evaluation: cal-
cium levels are normal, with low phosphate and high alkaline phos-
phatase levels.
238
MUSCULOSKELETAL AND CONNECTIVE TISSUE DISEASE Inflammatory or Immunologic Disorders
Disease Severity
Clinical evaluation: long-bone bowing, rachitic rosary.
Treatment Steps
1. Vitamin D produces rapid improvement.
2. Residual long-bone deformities may require surgical correction.
3. Vitamin D–resistant rickets requires treatment with phosphate re-
placement and vitamin D3.
A. Polymyalgia Rheumatica
H&P Keys
Occurs in men and women > 60, and occurs twice as often in
women. Patients complain of abrupt onset of pain affecting the
shoulders, neck, upper arms, lower back, and thighs. Morning stiff-
ness and gelling are predominant features. Physical examination re-
veals only tenderness and restriction of motion.
Diagnosis
Radiographs are normal. Rheumatoid factor and antinuclear anti-
bodies (ANAs) are normal, ESR is elevated. Diagnosis is by history
and response to treatment with prednisone.
Disease Severity
Elevated ESR, clinical impairment of activities.
Treatment Steps
1. Responds rapidly to low-dose prednisone.
2. If response is not seen in 1 week, diagnosis should be questioned.
3. Start prednisone, 10–20 mg/day, with taper to 5–7.5 mg/day.
4. Therapy may continue for > 1 year.
B. Lupus Arthritis
H&P Keys
Disease of young females ages 14–40, with a 5:1 female-to-male ratio.
The acute arthritis may involve any joint but usually involves the
239
Diagnosis
Clinical symptoms of morning stiffness, myalgia, arthralgia. Sero-
logic testing: ANA positive in 95%.
Disease Severity
Articular complaints are usually mild and reversible without ero-
sions. Disease activity, however, can severely impair renal function
and cause pulmonary and cardiac involvement. Neuropsychiatric
manifestations may also be present. Increased tendency for throm-
bosis.
Treatment Steps
Treatment consists of periods of rest, avoidance of sun exposure,
NSAIDs, hydroxychloroquine, and corticosteroids and other im-
munosuppressants such as cyclophosphamide, azathioprine, my-
cophenolate mofetil, and methotrexate. Splinting may preserve
function in weakened joints. Methotrexate is used in recalcitrant dis-
ease. Fertility and carcinogenesis issues need to be discussed prior to
immunosuppressant and immunomodulation therapy.
C. Polymyositis–Dermatomyositis
H&P Keys
Complaints of proximal hip weakness, with difficulty on stairs and
getting out of cars. Arm symptoms with difficulty lifting above the
horizontal and lack of endurance strength. Physical examination
may show diffuse symmetric muscle wasting and weakness. Affected
muscles may be sore to palpation. Gait may be slow and wide based.
Diagnosis
Clinical presentation of symmetric proximal muscle weakness. Ele-
vated serum creatine kinase, aldolase, lactic dehydrogenase, and
transaminase. Characteristic EMG abnormalities. Autoantibodies
may be present. Muscle biopsy.
Disease Severity
Involvement may extend to the heart, GI tract, lungs, peripheral
joints.
Treatment Steps
1. High-dose corticosteroid medication is the initial treatment.
2. Graded exercise after inflammation restores some strength and
range of motion.
3. Methotrexate or azothioprine is used in patients not responding
to corticosteroid.
Treatment Steps
1. Aimed at controlling the inflammation with acetaminophen,
NSAIDs, antimalarial immunosuppressives (such as methotrexate,
azathioprine, leflunomide, mycophenolate mofetil, and anti–tumor
necrosis factor [TNF] agents). Corticosteroids may be used to im-
prove patient functional levels. Remicade (infliximab) is a mono-
clonal antibody used in conjunction with methotrexate. Oppor-
tunistic infections are reported. Evaluate for latent tuberculosis
(purified protein derivative [PPD]) prior to using these agents.
2. Severely affected joints can be protected with splints and braces
to prevent destruction. Synovectomy prior to joint destruction, to-
tal arthroplasty for severe disease.
E. Ankylosing Spondylitis
H&P Keys
Common in young men aged 15–30. Presents with complaint of dif-
fuse low backache without radicular symptoms. Profound morning
stiffness. Usually a rapid response to anti-inflammatory medication.
May have painless or painful effusions of large joints. May have
uveitis and aortic valve disease, diminished spine motion in all three
planes.
Diagnosis
History and physical examination are most important. Restriction of
chest expansion and spine flexion. Elevated ESR. May be human
C
241
Polymyalgia Rheumatica
• Women over age 60, more common in women.
• Pain in the shoulders, neck, upper arms, low back, thighs; morning stiffness, gelling.
• X-rays are normal, RF and ANA are normal, ESR is elevated.
• Treat with prednisone
Ankylosing Spondylitis
• Common in men ages 15–30.
• Low back pain, profound morning stiffness.
• Elevated ESR, may be HLA-B27 positive, sacroiliitis on x-ray.
• Treat with anti-inflammatory medication.
Rheumatoid Arthritis
• Women ages 15–35.
• Symmetric painful swelling in the small joints, morning stiffness, fatigue; may affect elbows,
ankles, hips, spine, and shoulders.
• X-rays positive for erosions, positive RF, elevated ESR, anemia.
• Treat with acetaminophen, NSAIDs, antimalarial, gold, penicillamine, and immunosuppressives.
Lupus Arthritis
• Females 14–40 (5:1 female/male ratio).
• Acute arthritis most often affecting the small joints of the hand, wrists, and knee.
• ANA positive in 95%.
• Treatment includes NSAIDs and corticosteroids.
Polymyositis–Dermatomyositis
• Symmetric proximal hip weakness, symmetric muscle wasting, weakness, slow gait, muscle
tenderness to palpation.
• Elevated serum creatine kinase, aldolase, LDH, and transaminase; EMG abnormalities, may
have autoantibodies.
• Treat with corticosteroids or methotrexate.
Disease Severity
Restriction of spine motion, chin-on-chest deformity, restriction of
chest expansion with restrictive lung disease.
Treatment Steps
1. Initial flair is treated with rest and anti-inflammatory medication.
2. Mobilization and flexibility exercises are started as the inflamma-
tion is controlled. Etanercept for spondylitis.
242
MUSCULOSKELETAL AND CONNECTIVE TISSUE DISEASE Inflammatory or Immunologic Disorders
F. Bursitis
H&P Keys
Soft tissue swelling that may be either painful or nonpainful, usually
over a bony prominence. Swelling may occur spontaneously or as a
result of trauma or an inflammatory disease (gout, rheumatoid
arthritis).
Diagnosis
Clinical evaluation. Additional evaluation for underlying inflamma-
tory diseases. Radiographs to evaluate for bony prominences and
soft tissue calcifications.
Disease Severity
Clinical evaluation.
Concept and Application
Bursae form wherever two tissue planes move in opposite direction
or where tissues travel over a bony protuberance. Normal bursal lo-
cations are in the subacromial space of the shoulder, over the olecra-
non of the elbow, and over the tibial tubercle, and in the prepatellar
space. This normal structure allows skin and tendon and muscle to
slide over each other easily. If the bursa becomes infected, injured,
or inflamed, fluid will accumulate in the bursa and produce visible
swelling. Fibrinous loose bodies may also be formed in the bursa. Af-
ter the inflammation subsides, adhesions and fibrosis may occur in
the bursa, resulting in crepitation and, sometimes, pain.
Treatment Steps
1. Avoidance of repetitive motions and trauma will prevent bursal
inflammation.
2. Treatment of any underlying collagen vascular disease or crys-
talline arthritis will control the bursal swelling.
3. Acute treatment consists of rest, ice, anti-inflammatory medica-
tion. Aspiration to identify crystals or elevated WBC and bacteria
may be required. Steroid injection may be used.
4. The use of protective padding, elbow and knee pads, can prevent
recurrence. Steroid injection may be used.
5. Surgical excision is sometimes needed if the size or the location
interferes with activities of daily living.
G. Tendinitis
H&P Keys
Pain over tendon or at tendon insertion. May be acute in onset or as
a result of repetitive overload, often seen after a sudden change in
activity or sporting activity.
Diagnosis
Physical signs of localized inflammation with point tenderness over
the tendon. Weakness may be present secondary to pain.
Disease Severity
Functional assessment of impairment.
Concept and Application
Tendinitis is an inflammation of the tendon secondary to overload.
This may be due to acute overload with partial tearing of the tendon
or as a result of repetitive stress. A sudden change in activity or
sporting activity that exceeds the body’s reparative capabilities will
result in an “overuse” tendinitis.
243
H. Fibromyalgia
H&P Keys
Patients present with diffuse achiness, stiffness, fatigue, associated
with multiple areas of clinical tenderness. Seventy-five percent are fe-
male, with an age distribution of 20–60 years. Pain tends to be local-
ized to axial locations such as the neck and lower back. The upper
trapezius is a common location of pain. On physical examination
tenderness is present with moderate pressure. Common locations:
occiput, trapezius, supraspinatus at ridge of scapula, lower back, lat-
eral epicondyle.
Diagnosis
Clinical examination makes the diagnosis. Laboratory evaluation
(ESR, RF, ANA, complete blood count [CBC]) to exclude collagen
vascular diseases and infectious causes (Lyme disease). Thyroid
function tests are also valuable.
Disease Severity
Clinical examination.
Concept and Application
Etiology is unknown; fatigue is felt to be due to sleep disturbances,
with loss of rapid eye movement (REM) sleep patterns. Highly corre-
lated with abnormal sleep patterns and previous physical and sexual
abuse.
Treatment Steps
1. Patient education and reassurance.
2. Aerobic exercise is more beneficial than stretching alone.
3. NSAIDs are not effective as single agents but are effective in con-
junction with bedtime dose of amitriptyline or cyclobenzaprine.
VI. NEOPLASMS
A. Osteosarcoma
H&P Keys
Most common in second and third decade of life. Often presents as
a painless mass. Found after rather minor trauma on routine x-ray.
Diagnosis
Radiographs are diagnostic: demonstrate increased radiodensity with
areas of radiolucency and permeative destruction and soft tissue ex-
tension. Elevated periosteum (Codman’s triangle) may be present.
Bone scans and MRI will show extent of lesion and skip lesions.
Disease Severity
Extent of disease with involvement of multiple compartments.
244
MUSCULOSKELETAL AND CONNECTIVE TISSUE DISEASE Neoplasms
B. Metastases to Bone
H&P Keys
Presentation is usually with pain or with a pathologic fracture.
Diagnosis
Radiographs demonstrate lytic and blastic lesions. Lesions involving
> 50% of the cortex or that are > 2.5 cm in diameter are at risk for
spontaneous fracture. Bone scanning sensitive for detection of early
metastatic disease. MRI will accurately show extent of metastatic tu-
mor involvement of bone. Alkaline phosphatase is usually elevated.
Disease Severity
Lesions > 2.5 cm in diameter or > 50% of the cortex are at risk for
spontaneous fracture and should be prophylactically stabilized.
Concept and Application
Metastatic tumors are the most common malignancies of bone.
Spread to bone can be either arterial or venous. Classically, lytic le-
sions are found in the axial skeleton and long-bone diaphyses and
usually multiply. Most common lesions are breast, prostate, lung,
kidney, and thyroid. Prostate and breast metastases are typically blas-
tic. The most common metastases in children are Wilms’ tumor and
neuroblastoma.
Treatment Steps
1. Radiation therapy is often helpful to control the pain and
metastatic activity. Adenocarcinoma of lung does not respond to
radiation therapy.
2. For impending fractures, stabilization with load-sharing devices
(intramedullary rods) is preferred. For collapse of bony support
of joint surfaces, replacement arthroplasties may be useful proce-
dures if survival is expected beyond 6 months.
C. Pulmonary Osteoarthropathy
H&P Keys
Clubbing of the fingers is the characteristic feature. In some pa-
tients, especially those with malignant lung tumors, severe bone pain
may be present. Obtain family history of clubbing.
Diagnosis
Physical feature of bulbous deformity of the digits (clubbing).
Periostitis and thickening of the bones is present radiographically.
245
Diagnosis
Diagnosis is clinical; arthrogram will show decreased joint-space vol-
ume.
Disease Severity
Clinical evaluation of functional loss of shoulder motion.
Treatment Steps
1. Institution of early, aggressive range-of-motion exercises.
2. Adjunctive treatment with NSAIDs and ice.
3. May take 1 year to resolve. Surgical releases and manipulation are
reserved for nonresponders to physical therapy.
B. Dupuytren’s Contracture
H&P Keys
Patients are usually males older than 40, of northern European an-
cestry, with a family history of the condition. Alcohol, smoking, dia-
betes, and seizures are contributory. Forty percent are bilateral. Ul-
nar digits are more commonly involved.
Diagnosis
Clinical evaluation.
Disease Severity
Interference with hand function, inability to get the fingers out of
the palm of the hand.
Treatment Steps
Surgical intervention for deformities of > 30–45° of the MCP or of
any PIP involvement.
Diagnosis
Laboratory evaluation: increased alkaline phosphatase, increased
urinary hydroxyproline excretion. Radiographic appearance: initial
lesion is a focal area of radiolucency (osteoporosis circumscripta of
the skull). In the long bones, resorption is characterized by an ad-
vancing wedge of radiolucency. Attempts at repair create sclerotic-
appearing bone with thickening of the cortex.
Disease Severity
The major complication of Paget’s disease is sarcomatous transfor-
mation. This occurs in < 1% of cases. Presents with severe pain and
extremely elevated serum alkaline phosphatase activity. Increased lo-
cal circulation in hypervascular bone can create high-output conges-
tive heart failure. Paget’s fracture risk due to brittle bones. Increased
247
Treatment Steps
1. In most instances no treatment is necessary because of the
paucity of clinical symptoms.
2. In symptomatic patients, NSAIDs may suppress the discomfort.
Calcitonin administered subcutaneously may decrease pagetoid
bone activity. Etidronate will decrease bone resorption.
3. With irreversible joint destruction, total joint arthroplasty offers
relief of pain. Spinal decompression (laminectomy) for stenosis.
E. Eosinophil Granuloma
H&P Keys
May present as progressive back pain, more often in the thoracic
spine. Common locations: pelvis, femur, and spine. First and second
decade of life. Lytic-appearing lesion characteristic. Periosteal thick-
ening is common.
Diagnosis
Classically, may cause vertebral flattening (vertebra plana), lytic le-
sions in long bones.
Disease Severity
Lesions that compromise the structural integrity of long bones or
cause neurologic compromise.
Treatment Steps
1. Treatment consists of observation (many lesions heal sponta-
neously), low-dose radiation for neurologic deficits.
2. Curettage or excision may be indicated for persistent lesions.
A. Effusion of Joint
H&P Keys
Swelling of the joint, either spontaneous in onset or posttraumatic.
Physical examination reveals loss of the normal joint contours and
possibly a restriction in motion.
Diagnosis
Aspiration of the joint classifies the fluid as noninflammatory (WBC
< 2,000/mm3), inflammatory, or infectious (WBC > 100,000/mm3).
Evaluation for crystals is essential to differentiate gout and pseudo-
gout. Culture and Gram stain are needed to rule out infection. A
bloody effusion with the history of trauma is suggestive of a severe
248
MUSCULOSKELETAL AND CONNECTIVE TISSUE DISEASE Acute or Emergency Problems
Treatment Steps
1. Management depends on the etiology of the effusion.
2. Treatment of the inflammatory condition with oral anti-
inflammatory medications or intra-articular steroids.
3. Infectious effusions are treated with serial aspirations or surgical
drainage in combination with antibiotics.
4. Traumatic effusions resolve with rest, but treatment must address
the injury pattern.
B. Spinal Stenosis
H&P Keys
Elderly patients with complaints of back and buttock pain. Pain is
made worse with walking and descending stairs and relieved only
with prolonged rest. If activity continues, paresthesia and weakness
may develop. Pain is increased with hyperextension of the spine and
relieved with forward flexion of the spine. Sensory changes are de-
scribed as water or candle wax dripping down the leg.
Diagnosis
Radiographs often show degeneration of the facet joints. CT scan is
the best noninvasive test for bony stenosis. MRI underestimates the
degree of stenosis. Myelogram and postmyelogram CT are useful to
fully define the disease, especially stenosis of the lateral recesses of
the neural foramina.
Disease Severity
Clinical impairment of activities with neurogenic claudication. Pain
is increased with any activity that causes hyperextension of the spine.
Acute trauma in the presence of stenosis may cause catastrophic
neurologic symptoms with paraplegia and cauda equina symptoms
and demands prompt surgical decompression.
Concept and Application
The maturing of the skeleton results in degenerative changes involv-
ing the disk margins and facet joints. The bony overgrowth con-
stricts the nerve roots. This may be exacerbated by ligamentous
thickening and diskogenic protrusions.
Treatment Steps
1. Adjustment to the limitations of the disease in conjunction with
anti-inflammatory medications, ice or heat, and an exercise pro-
gram.
2. If symptoms are severely disruptive to the patient, surgery
(laminectomy) to decompress the nerve roots will give relief. Fu-
249
C. Contusions
H&P Keys
Contusions are a result of direct trauma. Localized erythema and
swelling are present with palpable tenderness. There may be loss of
adjacent joint motion if swelling is pronounced.
Diagnosis
Diagnosis is by history and clinical examination. Radiographs rule
out fracture or late myositis ossificans.
Disease Severity
Clinical loss of function of the joint and affected extremity define
severity. Large hematomas and contusions to a large area of the mus-
cle mass will produce greater disability. Myositis ossificans, or calcifi-
cation of the muscle, is a late sequela of a severe contusion or a re-
sult of repetitive contusions to the same muscle before primary
healing has occurred or early application of heat.
Concept and Application
Contusions are a result of direct trauma. Following the trauma there
is local damage to blood vessels and muscle. As bleeding and
swelling continue, there is increased muscle stiffness and loss of
joint motion. Secondary agents of inflammation produced as re-
sponse to the local tissue trauma produce the ache and stiffness that
characterize the early period after a contusion.
Treatment Steps
1. Treatment consists of ice and compression to control the swelling
and bleeding.
2. Adjunctive use of NSAIDs is useful in controlling the secondary
inflammation.
3. Early therapy is provided to restore painless range of motion, fol-
lowed by flexibility and strengthening exercises.
4. Return to activity is allowed when there is full, painless range of
motion and strength equal to the unaffected extremity.
Diagnosis
Radiographs: anteroposterior (AP), lateral, oblique, and open-
mouth odontoid are the standard views. Flexion and extension lat-
eral x-rays evaluate instability. CT scan and MRI are useful in deter-
mining the geometry of fracture fragments and evaluating the
degree of cord compression.
Disease Severity
Disease severity is based on clinical findings of neurologic compro-
mise.
Concept and Application
The pattern of injury is dependent on the mechanism of injury.
Treatment is based on decompression of the neurologic elements
and stabilization of the spine, either surgically or with bracing.
Treatment Steps
1. Treatment is stabilization of the unstable elements with surgery
or bracing.
2. For neurologic compromise, prompt intervention to decompress
the neural elements and stabilize the bony and ligamentous struc-
tures.
2. Thoracic Spine
H&P Keys
Usually, high energy is necessary to produce these injuries unless sig-
nificant osteoporosis is present. The pattern of injury depends on
the position of the axis of flexion and direction of the force at the
time of injury. These result in compression fractures, burst fractures,
flexion–distraction injuries (seat belt), and fracture dislocations.
Diagnosis
Radiographic evaluation with plain x-rays is usually adequate for tho-
racic fractures. CT scan is beneficial to define fracture geometry and
neurologic compromise.
Disease Severity
The degree of bony compression, displacement, and neurologic
compromise defines the severity of the injury.
Treatment Steps
1. Compression fractures of < 50% are usually treated with a short
period of rest with supportive bracing and restorative exercise.
2. For those > 50%, surgical stabilization may be indicated.
251
Diagnosis
Clinical examination correlated with biplanar x-rays is usually diag-
nostic.
Disease Severity
Intra-articular comminution is associated with poor function out-
comes. Spiral fractures may result in rotation residuals if great care
is not taken in the treatment of the fracture. Fractures of the distal
phalanx often result in injuries to the nail bed, which can result in
nail deformities if not anatomically repaired.
252
MUSCULOSKELETAL AND CONNECTIVE TISSUE DISEASE Acute or Emergency Problems
Diagnosis
AP and lateral x-rays usually confirm the diagnosis. In the elderly
with groin pain and osteopenia, CT and a bone scan may be neces-
sary to confirm the diagnosis.
Disease Severity
Displaced fractures of the femoral neck have a high rate of compli-
cations, with delayed unions and the development of avascular
necrosis of the femoral head from disruption of blood supply to the
femoral head by the circumflex vessels. In young patients with axial
loading fractures, posttraumatic chondrolysis may also be a compli-
cation. Even with minimal displacement, avascular necrosis and col-
lapse of the femoral head may occur.
Disease Severity
The residuals of joint dislocation depend on the joint injured. Hip
dislocations have a high rate of complication with avascular necrosis.
Dislocations of the knee may result in arterial injury to the popliteal
vessels and loss of perfusion to the lower leg. Shoulder dislocations
may result in injuries to the axillary or musculocutaneous nerves
and persistent instability of the shoulder. Dislocations of Lisfranc’s
joint may result in persistent pain, stiffness, and ambulatory dysfunc-
tion.
Concept and Application
Joint dislocations are serious injuries. They result when the forces
that are applied to the joint exceed the ligaments’ ability to with-
stand the stress. Instability occurs as a result of the loss of the passive
restraints provided by the ligaments. The displacement of the nor-
mal joint structures may result in secondary injury to adjacent struc-
tures. Chronic instability may be a result of the injury.
Treatment Steps
1. Rapid reduction of the dislocation and assessment of secondary
injury patterns are the mainstays of treatment.
2. Fracture bracing and early protected motion programs may be
useful in decreasing the morbidity associated with prolonged im-
mobilization with rigid casting.
3. Operative repair is indicated for irreducible dislocations and for
stabilization of grossly unstable joints.
Diagnosis
X-rays may be normal or may show superior migration of the
humeral head, impinging on the inferior surface of the acromion.
In an acute injury, the humeral head may be low in the glenoid fossa
secondary to intra-articular hematoma. Arthrography and MRI will
define the magnitude and location of the tear.
Disease Severity
The degree of symptoms depends on the magnitude of the tear. Par-
tial or small tears may result in only minimal dysfunction and pre-
sent with the predominant feature of pain. Larger tears will result in
loss of shoulder strength. Long-standing cuff tears will result in cuff
arthropathy, arthritis characterized by a high-riding humeral head
impinging on the acromion, with glenohumeral arthritis.
255
BIBLIOGRAPHY
Ball GV. Clinical Rheumatology. Philadelphia: W.B. Saunders, 1993.
Cailliet R. Neck and Arm Pain, 3rd ed. Philadelphia: F.A. Davis, 1991.
Callen JP. Cutaneous manifestations of collagen vascular disease and related condi-
tions. Med Clin North Am, September 1989.
Connolly JF. The Management of Fractures and Dislocations: An Atlas, 3rd ed. Philadel-
phia: W.B. Saunders, 1997.
D’Ambrosia RD. Musculoskeletal Disorders, 2nd ed. Philadelphia: J.B. Lippincott, 1986.
Dieppe PA. Atlas of Clinical Rheumatology. Philadelphia: Lea & Febiger, 1986.
Enneking WF. Musculoskeletal Tumor Surgery. New York: Churchill Livingstone, 1983.
Morrissy RT. Pediatric Orthopedics, 5th ed. Philadelphia: J.B. Lippincott, 2001; 1, 2.
Niwayama G, Resnick D. Diagnosis of Bone and Joint Disorders, 2nd ed. Philadelphia:
W.B. Saunders, 1988.
O’Donoghue DH. Treatment of Injuries to Athletes, 4th ed. Philadelphia: W.B. Saunders,
1984.
Pettid, FJ. Practical Orthopedics, 5th ed. Mosby, 2000.
Stone J. Current Rheumatology Diagnosis & Treatment. New York: McGraw-Hill, 2004.
Turek SL. Orthopedics: Principles and Their Application, 4th ed. Philadelphia: J.B. Lippin-
cott, 1984; 1, 2.
Neurology 10
I. INFECTIOUS DISEASES OF THE CENTRAL NERVOUS SYSTEM (CNS) / 259
A. Viruses / 259
B. Meningitis / 260
C. Abscesses / 263
D. Spirochetes / 263
257
Copyright © 2004, 2001 by The McGraw-Hill Companies, Inc. Click here for terms of use.
258 Neurology
X. DEMYELINATION / 283
A. Multiple Sclerosis / 283
BIBLIOGRAPHY / 285
259
A. Viruses
1. Human Immunodeficiency Virus (HIV)
H&P Keys
Risk factors include blood transfusion, needle sharing, unsafe sex;
presentations include: headache and fever (meningitis) 1%, pro-
gressive dementia with impaired saccadic eye movements (15–20%),
paraparesis (myelopathy) 20%, pain and numbness (neuropathy)
30%; focal deficits in CNS, lymphoma, toxoplasmosis, or progressive
multifocal leukoencephalitis (PML).
Diagnosis
HIV antibodies by enzyme-linked immunosorbent assay (ELISA) (if
positive, confirm with Western blot); magnetic resonance imaging
(MRI) of affected area to exclude mass lesion in myelopathy, demen-
tia, or focal deficits; cerebrospinal fluid (CSF) for pleocytosis; cul-
tures in meningitis; CSF protein and electromyography (EMG) and
nerve conduction velocities (NCV) in neuropathy; vitamin B12 level.
Disease Severity
CD4+ count < 200 associated with severe immunosuppression and
opportunistic infections; viral load > 100,000 copies/mL correlate
with CD4+ decline and clinical progression; dementia, myelopathy,
lymphoma, and PML carry poor prognosis.
Concept and Application
Etiology of dementia and myelopathy unknown; direct invasion in
meningitis; reduced immunocompetence in toxoplasmosis, lym-
phoma, neuropathy, or PML.
Treatment Steps
1. For parenchymal mass lesion, pyrimethamine and sulfadiazine
for 2 weeks; biopsy if not better.
2. Radiation for CNS lymphoma.
3. Amphotericin for fungal meningitis.
4. IV immunoglobulin G (IgG) if neuropathy is demyelinating; oth-
erwise, biopsy to rule out specific infectious or vasculitic etiolo-
gies.
5. Pain treatment with tricyclic antidepressants, gabapentin, lamo-
trigine, carbamazepine.
6. For dementia, treatment protocols include reverse transcriptase
and protease inhibitors.
7. Vacuolar myelopathy has no specific therapy.
2. Herpes Simplex Virus (HSV)
H&P Keys
In the CNS, usually presents with encephalitic symptoms including
clouding of consciousness, aphasia, fever, headache, seizures; exam
denotes aphasia, hemiparesis, nuchal rigidity, confusion, and vari-
able somnolence.
Diagnosis
Brain computed tomography (CT) shows edema and hemorrhage in the
temporal lobe and orbitofrontal cortex; MRI demonstrates areas of high
signal intensity on T2-weighted images (Fig. 10–1); electroencephalo-
260
NEUROLOGY Infectious Diseases of the Central Nervous System (CNS)
B. Meningitis
1. Aseptic
H&P Keys
Preceding upper respiratory infection, exanthem, exposure to rat
excreta; headache, fever, photophobia, nuchal rigidity.
Diagnosis
CT scan to rule out abscess; CSF examination shows pleocytosis, pre-
dominantly lymphocytic, mild protein elevation, normal glucose;
Figure 10–1. MRI of HSV encephalitis with increased T2 signal in both temporal lobes. R, mesial;
L, lateral with T1 hypodensity secondary to hemorrhage.
261
Disease Severity
Coma, focal neurologic signs, signs of herniation, seizures bear poor
prognosis and may suggest abscess formation; sequelae and outcome
directly related to time of institution of therapy.
Treatment Steps
1. Blood culture; CT of head, lumbar puncture (LP).
2. Do not delay antibiotics for LP or LP for CT; cefotaxime in adults
and children > 3 months; ampicillin and cefotaxime in neonates
and infants.
3. Steroids may lessen hearing loss in children.
4. Supportive care.
3. Fungal
H&P Keys
Progressive dementia, headaches, nuchal rigidity, lack of fever,
cranial nerve involvement, often immunosuppression or history of
lymphoma or other malignant disease; exposure to birds; suspect
Cryptococcus in the immunosuppressed, mucormycosis in diabetics.
Diagnosis
CSF with predominantly lymphocytic and monocytic pleocytosis;
positive cryptococcal antigen and positive India ink in cryptococcal
meningitis; positive fungal cultures in weeks.
Disease Severity
Hydrocephalus; arteritis, thrombosis, and infarction of the brain.
Treatment Steps
1. Amphotericin B along with antifungal agents (fluconazole, itra-
conazole).
2. Supportive care.
4. Tuberculous (TB)
H&P Keys
Positive purified protein derivative (tuberculin) (PPD) in 75%;
fever, malaise, headache; development of nuchal rigidity, de-
creased mental status, confusion, dementia, lower cranial nerve in-
volvement.
Diagnosis
Chest x-ray (CXR); PPD; CSF shows lymphocytic pleocytosis, ele-
vated protein (often to several hundred), low glucose (normal in
30%), positive acid-fast bacillus (AFB) stain or immunofluorescence;
cultures take several weeks and often require multiple LPs.
263
C. Abscesses
H&P Keys
History of sinus, ear, periodontal, pulmonary, or head wound infec-
tion, endocarditis; suspect congenital heart disease in children; pre-
sents with focal severe headache, nausea and vomiting, seizures; fo-
cal signs dependent on location.
Diagnosis
MRI or CT with contrast of affected area shows ring-enhancing le-
sion with edema, mass effect, “daughter lesion”; LP shows “aseptic”
pleocytosis but is usually not necessary and may be contraindicated
because of possible herniation.
Disease Severity
Untreated cases produce major disability or death; mortality rate in
treated cases is 30%; 50% suffer neurologic sequelae.
Concept and Application
Focal infection of brain parenchyma, usually without meningitis; en-
capsulated with central necrotic material and pus; solitary 75% of
the time; usually caused by anaerobes or microaerophilic organisms,
most commonly anaerobic streptococci or bacteroides.
Treatment Steps
1. Biopsy using stereotaxic CT guidance.
2. Steroids for management of intracranial pressure.
3. Mechanical hyperventilation and PCO2 of < 30 may be needed if
severe.
4. Anticonvulsants for seizures.
5. IV antibiotics include penicillin and metronidazole if pathogen is
unknown; vancomycin for methicillin-resistant staphylococci and
third-generation cephalosporin for gram-negative bacteria.
D. Spirochetes
1. Lyme Disease
H&P Keys
History of tick bite in about 50% of patients; history of erythema
chronicum migrans in about 60% of patients; influenza-like symp-
264
NEUROLOGY Neuromuscular Disorders
Treatment Steps
1. Treat erythema migrans or Bell’s palsy with oral doxycycline or
amoxicillin for 30 days; for any other neurologic symptoms or
conditions, treat with IV ceftriaxone 2 g daily for 14 days.
2. Corticosteroids are used in failure to respond to antibiotics.
Diagnosis
Wrist x-rays show bony deformities; MRI of wrist demonstrates focal
compression of median nerve; EMG and NCV will show focal me-
dian nerve conduction velocity slowing at the wrist, axonal loss; de-
pending on history, check for diabetes, rheumatoid arthritis, Lyme
disease.
Disease Severity
Severe atrophy of thenar muscles, marked thumb opposition weak-
ness, permanent pain or numbness of first three digits, absent sen-
sory early and, later, motor potentials on nerve conduction of the
median nerve.
B. Guillain–Barré Syndrome
(Acute Inflammatory Demyelinating
Polyneuropathy)
H&P Keys
Progressive ascending weakness a few weeks after an upper respira-
tory or gastrointestinal (GI) illness or surgery; back discomfort in
60%; no sensory level; areflexia; distal, but may be proximal, pro-
gressive weakness mainly in the legs; no fever on presentation; very
symmetrical; maximum evolution of disease in 2 weeks in 50% of pa-
tients, in 4 weeks in 90% of patients.
Diagnosis
CSF with high protein but < 10 white blood cells (WBCs) (50 in HIV
patients); slowing of conduction velocities to conduction block on
EMG and NCV studies; check for Lyme, HIV, urine porphyrins, hep-
atitis; culture stools for Campylobacter jejuni. GM1, Gd1a, and GD1b
myelin glycolipid antibodies in CSF support the diagnosis.
Disease Severity
Poor prognostic indicators include severe tetraparesis, hyperacute
onset, assisted ventilation, low nerve amplitudes on EMG and NCV
(suggesting axonal involvement and likely to result in prolonged se-
quelae), and abnormal phrenic nerve studies; autonomic instability
may increase morbidity.
Concept and Application
Proximal and later distal segmental demyelination with inflamma-
tory cell infiltration in nerve.
Treatment Steps
1. Plasmapheresis in first week for quickly progressing cases (inabil-
ity to stand) or ventilator dependence.
2. Supportive care.
3. Recent evidence favors intravenous immune globulin (IVIG) as
treatment.
4. Corticosteroids are not helpful.
C. Myasthenia Gravis
H&P Keys
Diplopia, ptosis; symptoms worsen at end of day; young females; el-
derly males; demonstrable fatigue on examination, such as worsen-
ing of ptosis on prolonged upward gaze.
Diagnosis
Dramatic improvement with IV edrophonium chloride (Tensilon)
(test double blind); positive antibodies to acetylcholine receptors
266
NEUROLOGY Neuromuscular Disorders
H&P Keys
History of progressive proximal weakness in most cases; myalgias
and cramps are more the exception than the rule; weakness is noted
raising arms overhead and in related activities, or getting up from a
chair; myotonic dystrophy will show myotonia on percussion of small
muscles (e.g., tongue); Duchenne’s dystrophy shows calf muscle en-
largement.
Diagnosis
Creatine kinase (CK) is elevated in most cases of myopathy and mus-
cular dystrophy; EMG and NCV demonstrate small, brief (myo-
pathic) motor unit potentials and normal nerve conduction veloci-
ties; muscle biopsy is diagnostic in most cases; metabolic myopathies
may require biochemical studies; ischemic exercise test is abnormal
in some glycogen metabolic myopathies because of the inability to
utilize energy substrate; electrocardiogram (ECG) is needed for
high incidence of cardiac abnormalities.
Disease Severity
Severe weakness could be associated with respiratory compromise;
high CK might cause myoglobinuria and renal failure in metabolic
myopathies; many myopathies associated with life-threatening car-
diac abnormalities; swallowing and respiratory difficulties could re-
sult in death.
B. Thiamine Deficiency
H&P Keys
Usually undernourished alcoholics, but sometimes patients with gas-
tric carcinoma or hyperemesis gravidarum, who present with ataxia
of gait, gaze palsies or nystagmus, and mental confusion and amne-
sia (Wernicke’s encephalopathy); ocular abnormalities might in-
clude nystagmus that could be either horizontal or vertical, weakness
or paralysis of conjugate gaze, or weakness of the external rectus
muscle, which is always bilateral; the ataxia is one of stance and gait
with no evidence of tremor, and the confusion could present as a
global confusional state, stupor, and coma or as a hallucinatory state
with overactivity; Wernicke’s encephalopathy might progress to an
amnesic syndrome with severe short-term memory impairment and
confabulation (Korsakoff’s psychosis).
268
NEUROLOGY Paroxysmal Disorders
Diagnosis
CSF is almost always normal; blood pyruvate may be elevated; red
blood cell (RBC) transketolase or plasma thiamine activity is
markedly reduced, but the diagnosis remains a clinical one.
Disease Severity
Transition to the Korsakoff state heralds poor outcome, even with
therapy; other symptoms are likely to improve with treatment; con-
current septicemia, pneumonia, and liver disease result in about
15% mortality rate.
Concept and Application
Thiamine deficiency results in necrotic lesions of the mamillary bod-
ies, periaqueductal region, thalamus, hypothalamus, and floor of the
fourth ventricle.
Treatment Steps
1. Administer IV thiamine, 50 mg and IM 50 mg initially; and IM 50
mg daily subsequently until normal diet is resumed.
2. Avoid glucose infusion prior to thiamine administration.
3. Provide supportive care.
4. Evaluate for infections and other conditions.
C. Metabolic Encephalopathy
H&P Keys
Progressive clouding of consciousness in general without any focal
symptomatology; history of medication overdose, infections, anoxia,
hypo- or hyperglycemia, renal insufficiency, liver disease, alcohol in-
toxication; examination demonstrates normal pupillary reactions
with small pupils, normal oculovestibular responses, normal corneal
responses, and no focal deficits in a mentally obtunded patient.
Diagnosis
CT of the head is used to rule out mass lesions in patients with focal
neurologic deficits (hypoglycemia, hypoxia, azotemia, and hepatic
encephalopathies might cause focal neurologic signs); EEG to rule
out nonconvulsive status epilepticus or postictal state; drug screen
and metabolic parameters including sodium, glucose, oxygen, PCO2;
CXR and urinalysis (UA) to rule out infections.
Disease Severity
Prolonged hypoxia or hypoglycemia may result in permanent neuro-
logic injury; ventilatory support might be needed in many instances
of coma; fever should raise suspicion of meningitis or abscess.
Concept and Application
Usually reversible; nonstructural injuries resulting in generalized
cerebral dysfunction.
Treatment Steps
Correction of metabolic derangement.
A. Seizures
H&P Keys
History of abrupt stereotypic transitory loss or alteration of con-
sciousness with or without involuntary movements; short or no warn-
269
Diagnosis
CT or MRI of brain to rule out irritative lesion (stroke, tumor, ab-
scess); routine EEG might be diagnostic in ≤ 20% of patients; pro-
longed EEG monitoring might be necessary in difficult cases; single
photon emission computed tomography (SPECT) and positron-
emission tomography (PET) studies helpful in epileptogenic focus
localization; diagnosis remains a clinical one; cardiac evaluation
might be needed to rule out convulsive syncope in selected cases.
Disease Severity
Seizures, although most often idiopathic, can be the presentation of
otherwise treatable conditions such as brain tumors, abscesses, arte-
riovenous malformations; repeated seizures during the day might re-
sult in severe functional and social disability along with bodily in-
juries; the disease carries a number of social limitations with it; status
epilepticus markedly increases morbidity and mortality.
Treatment Steps
1. Treatment depends on seizure type.
2. When no seizures, patients respond best to valproic acid.
3. Generalized and partial seizures respond to phenytoin, valproate,
zonisamide, lamotrigine, topiramate, tiagabine, carbamazepine,
and levetiracetam.
4. Febrile seizures do not require chronic anticonvulsant therapy.
5. Refractory cases may require surgery or vagal nerve stimulator
placement.
6. Status epilepticus (continuous seizure activity lasting < 20 minutes
or multiple seizures without interictal recovery) are medical emer-
gencies requiring immediate use of IV benzodiazepines followed
by IV phenytoin or valproic acid and, if necessary, barbiturate
coma with mechanical ventilation.
B. Trigeminal Neuralgia
H&P Keys
Brief, sharp, lancinating pain mainly in the third or second division
of the fifth cranial nerve. Precipitated by touch, cold, or chewing.
Diagnosis
Normal neurologic exam; if abnormal, consider other conditions
such as posterior fossa mass lesion; beware of young multiple sclero-
sis (MS) patients with similar symptoms; any neurologic abnormality
should cause a prompt investigation of the posterior fossa with CT
or MRI for cerebellar pontine angle tumors, tumors of the fifth
nerve, and MS.
270
NEUROLOGY Paroxysmal Disorders
Disease Severity
The condition is a painful disease that results in no permanent se-
quelae to the patient if untreated; however, the pain is severe
enough to have caused some patients to commit suicide.
Concept and Application
The specific etiology is unknown, but may involve arterial ectasia
with nerve compression in some cases.
Treatment Steps
1. Medical management is highly satisfactory; oral carbamazepine is
usually started at the beginning (75% response rate); refractori-
ness may require the addition of baclofen, gabapentin, lamotri-
gine, or amitriptyline.
2. Monitoring for dizziness, unsteadiness, bone marrow suppression
with carbamazepine.
3. Acute confusional state and seizures may occur with abrupt dis-
continuation of baclofen.
4. Microvascular decompression of the nerve might result in long-
standing relief.
C. Headaches
H&P Keys
History of intermittent or persistent head pain with or without associ-
ated vegetative symptoms; migraines are usually unilateral, pulsating
headaches and if classic are associated with visual scotomata;
hemisensory disturbances; nausea and vomiting; and photo-, phono-,
and osmophobia; cluster headaches are retro-orbital, occurring in
the adult smoker associated with lacrimation; ipsilateral Horner’s syn-
drome usually lasts 90 minutes, may occur at the same time of day;
tension headaches are chronic, bandlike headaches with no other
symptomatology; examination should be normal.
Diagnosis
CT or MRI of the head to rule out intracranial mass lesion; early
generalized, pulsating headaches might mean nocturnal hypoxemia
and may require oximetry; LP should be done on patients with
nuchal rigidity or fever; sinus x-rays should be done on patients with
percussion tenderness.
Disease Severity
Headaches might be the initial and sole presentation of intracranial
lesions such as brain tumors, abscesses, hydrocephalus, and other
space-occupying lesions (Fig. 10–3); otherwise, these are benign con-
ditions.
Concept and Application
Migraine is a familial disorder characterized with abnormal cerebral
and intra- and extracranial vascular reactivity; chronic tension
headaches are secondary to self-perpetuating muscle tension; cluster
headaches could be paroxysmal parasympathetic discharges through
the superficial petrosal nerve.
Treatment Steps
1. Abortive therapy can be provided by means of nonsteroidal anti-
inflammatory drugs (NSAIDs).
2. 5HT1B and 5HT1D agonists (sumatriptan, zolmitriptan, rizatrip-
tan, frovatriptan, eletriptan, and naratriptan) are highly effective
271
V. CEREBROVASCULAR DISORDERS
Figure 10–4. CT scan demonstrating right frontal and occipital infarcts in a patient with carotid
artery thromboembolism.
Figure 10–5. Diffusion-weighted MRI demonstrating hyperintense lesion in the L thalamus due to
acute infarction.
273
B. Cardioembolic Strokes
H&P Keys
History of rheumatic fever, cardiac dysrhythmias; abrupt onset; hem-
orrhagic strokes or multifocal distribution. Evidence of cortical signs
including aphasia, seizures, more than one arterial distribution in
addition to findings similar to thrombotic strokes.
Diagnosis
CT or MRI of head, ECG, transthoracic and transesophageal
echocardiogram if ECG is normal and suspicion for cardiac source
still high, Holter monitor, blood cultures if endocarditis suspected.
Figure 10–6. Digital subtraction carotid angiogram revealing large atherosclerotic plaque in the
common carotid artery extending to internal and external carotid branches.
274
NEUROLOGY Cerebrovascular Disorders
Disease Severity
Embolic strokes tend to be larger and more commonly hemor-
rhagic, higher incidence of edema with mass effect and seizures in-
crease morbidity and mortality.
Treatment Steps
1. With the exception of myxoma and recent heart attack, long-term
anticoagulation is needed.
2. Anticoagulation with heparin can be started within a few hours if
stroke is not too large or there is no hemorrhage.
3. Switch to warfarin (Coumadin) when therapeutic and follow in-
ternational normalized ratio (INR) recommendations depending
on cause of stroke.
C. Intracerebral Hemorrhage
H&P Keys
Usually, history of uncontrolled hypertension or coagulation deficits,
sudden apoplectic onset, severe headache, nausea, vomiting, and fo-
cal neurologic deficits; exam shows hemiparesis, obtundation, sen-
sory deficits, conjugate eye deviation contralateral to hemiparesis
(toward hemiparesis if brain stem), rapidly developing coma in pon-
tine or cerebellar hemorrhage, elevated blood pressure.
Diagnosis
CT or MRI of head to assess extent of bleeding; LP may be bloody
but nonspecific and may induce herniation.
Disease Severity
Both pontine and cerebellar hemorrhages carry increased risk; cere-
bellar hemorrhage is usually treated surgically if large.
Treatment Steps
1. Supportive therapy, management of intracranial hypertension,
careful control of systemic hypertension.
2. Lobar and cerebellar hemorrhages may be amenable to surgical
resection.
D. Subarachnoid Hemorrhage
H&P Keys
Acute onset of worst headache of patient’s life, nausea, vomiting,
variable loss of consciousness, may have had previous “warning”
headaches; nuchal rigidity, obtundation; hemiparesis in middle cere-
bral territory; third cranial nerve palsy in posterior communicating
artery distribution; leg weakness, confusion in anterior cerebral
artery territory. Two-thirds of ruptured aneurysms occur in anterior
circulation.
275
Disease Severity
Outcome depends on mental status at time of ictus. Lethargic or ob-
tunded patients have poorer outcome. Vasospasm and seizures add
to morbidity. Disease associated with polycystic kidneys. Multiple
aneurysms in many cases.
Treatment Steps
1. Control of hypertension.
2. Reduction of Valsalva with stool softeners, quiet room.
3. Reduction of vasospasm with nimodipine.
4. Reduction of increased intracranial pressure with osmotic agents.
5. Hyperventilation and ventriculostomy if necessary.
6. Treatment of seizures with anticonvulsants.
7. Surgery when stable.
A. Medications
1. Opioids
H&P Keys
High incidence of addiction; inadvertent poisoning or suicidal at-
tempts, results in varying degrees of obtundation followed by de-
creased ventilation, miosis, bradycardia, and hypothermia.
2. Barbiturates
H&P Keys
High incidence of addiction; suicide attempts or accidental poison-
ing result in progressive unarousal, respiratory depression; pupillary
response present and pulmonary edema.
3. Benzodiazepines
H&P Keys
Similar to barbiturates.
4. Antipsychotic Drugs
H&P Keys
Use of phenothiazines and butyrophenones, treatment of schizo-
phrenia; side effects are parkinsonian syndrome, buccolingual invol-
untary movements (tardive dyskinesias), inability to sit still
(akathisia), and a syndrome of severe rigidity, fever, and confusion
276
NEUROLOGY Neoplasms
VII. NEOPLASMS
A. Glioblastoma
H&P Keys
Most common type of primary CNS tumor in adults (Fig. 10–7); his-
tory of hemiparesis or other focal neurologic signs, also seizures,
confusion, obtundation, and late headache. No clear predisposing
factors; most common cause of new-onset seizures in middle age.
Exam correlates with complaints.
Diagnosis
Contrast CT or MRI will show characteristic ring-enhancing lesion;
rarely may be multicentric or across corpus callosum. Other studies
are negative, including search for a metastatic origin. MR spec-
troscopy, PET, functional MRI, and cerebral blood flow techniques
add to the current diagnostic arsenal. Biopsy shows typical
Figure 10–7. Fluid-attenuated inversion recovery MRI of right frontal glial tumor demonstrating
vasogenic edema and midline shift.
277
NEUROLOGY Neoplasms
pseudopallisading, hemorrhage, pleomorphism, and hypercellular-
ity, and necrosis and endothelial hyperplasia.
Disease Severity
Younger patients have better prognosis than older ones. Less than
one-fifth of all patients survive more than a year.
Concept and Application
Likely arises from anaplasia of astrocytes. Secondary characteristics
of the tumor lead to further tissue invasion and mass effect.
Treatment Steps
1. Surgery to debulk tumor.
2. Radiation accompanied by use of corticosteroids (vasogenic
edema) and anticonvulsants is routine because of patient’s symp-
toms.
3. Chemotherapy with timazolamide or bis chloroethyl nitrosourea
(BCNU).
4. New radiothearapy techniques include radioactive implants,
stereotactic radiosurgery, radiation with radiosensitizers, hyper-
thermia.
5. Monoclonal antibodies did not prove useful.
6. Genetic modification is the most promising future approach.
B. Meningioma
H&P Keys
Benign tumor, causes symptoms by compression or irritation. Con-
vexity tumors present with hemiparesis, seizures, and headaches;
parasagittal with bicrural asymmetric weakness and spasticity, with
sphincter disorder. Can happen in spinal canal (mainly females) or
on optic nerve. Exam relates to complaints and presentation.
Diagnosis
CT of head will show a usually rounded dural lesion with mass effect;
MRI may only show lesion clearly with contrast enhancement (Fig.
10–8). It may calcify and show on plain x-rays.
Disease Severity
Location of the tumor and potential for surgical resection deter-
mine outcome. Usually, tumor recurs if not completely resected.
Concept and Application
Arise from arachnoid cells and may attain great size prior to devel-
opment of symptoms. Usually cause exostosis rather than bone ero-
sion. Some have progesterone, somatostatin, epidermal growth fac-
tor, and estrogen receptors and enlarge because of this. Psammoma
bodies can be seen microscopically.
Treatment Steps
1. Surgical resection when accessible; otherwise, radiation if sympto-
matic.
2. Incidental tumors can be observed because of slow growth.
3. Corticosteroids for edema; anticonvulsants for seizures.
C. Metastases
H&P Keys
History of smoking, breast cancer, or other predisposing factors.
Usually presents with focal neurologic signs, behavioral changes,
headaches, or seizures. May be apoplectic if it bleeds.
278
NEUROLOGY Degenerative Diseases
Diagnosis
MRI or CT scan shows single or multiple lesions with surrounding
edema and enhancement. Physical examination including breast, gy-
necologic, and rectal exam; CXR; blood count may lead to diagnosis
of primary. Biopsy of lesion if primary unknown will lead to separa-
tion from other etiologies.
Disease Severity
Choriocarcinoma, melanoma, thyroid carcinoma, and hyper-
nephroma may present with high incidence of bleeding. Solitary le-
sions have better prognosis and can be resected in some cases. Final
outcome is relative to the primary disease itself.
Concept and Application
Most commonly arises from lung, breast, or melanoma.
Treatment Steps
Radiation, steroids, and anticonvulsants. Chemotherapy in appropri-
ate cases.
A. Alzheimer’s Disease
H&P Keys
Onset in late fifties or sixties; presents initially with deficit in reten-
tive memory followed by dysnomia, spatial disorientation, personal-
ity changes, and gait disorder. The mental status examination shows
findings related to these complaints. Seizures, paraparesis, and abu-
lia can be seen in advanced cases.
279
C. Parkinson’s Disease
H&P Keys
Progressive tremor, slowness, festinating gait, stooped posture. Early
on, symptoms may be nonspecific (“arm discomfort”). Exam shows a
resting tremor of 4–6 Hz, difficulty with passive motion (rigidity)
evenly through the full range, decreased expression, paucity of
movements (bradykinesia), and difficulty with posture. When
tremor is added to the rigidity, “cogwheeling” results. Dementia oc-
curs in 30% of cases.
Diagnosis
No routine diagnostic studies exist; have decreased basal ganglia
dopamine activity on PET scan. Exclude medications, progressive
supranuclear palsy, olivopontocerebellar degeneration.
Disease Severity
The disease eventually leads to disability in spite of therapy. Swallow-
ing can be markedly affected.
Concept and Application
Neuronal loss of the substantia nigra and other pigmented nuclei.
Reduced dopamine levels. Similarity to a syndrome caused by the
designer drug methylphenyltetrahydropyridine (MPTP) has raised
question of environmental factor.
Treatment Steps
1. Replace dopamine with L-dopa; added carbidopa (Sinemet) re-
duces peripheral effects. This medication can cause variations in
clinical state not associated with dosing (on–off phenomenon).
2. Dopamine receptor agonists are also helpful (bromocriptine, per-
golide, pramipexole, ropinirole).
3. Anticholinergics improve tremor but can worsen dementia.
4. Rasagaline, a selective monoamine oxidase (MAO)-B inhibitor, is
used to slow down the disease progression.
5. Catechol-O-methyltransferase (COMT) inhibitors prolong L-dopa
availability.
D. Huntington’s Disease
H&P Keys
Progressive mental deterioration; becoming irritable, impulsive, ex-
hibiting poor self-control. Hand and face chorea develops and even-
tually all muscles follow. The exam shows chorea, dementia, oculo-
motor disturbances.
Diagnosis
The CT or MRI shows caudate head atrophy; genetic studies can de-
fine the patient at risk and the subject with overt disease.
Disease Severity
The disease is transmitted as autosomal dominant with complete
penetrance; it is fatal. Childhood cases present with rigidity and
seizures also. Mode of transmission leads to anticipation (subse-
quent generations show earlier and more severe signs).
NEUROLOGY Trauma
Treatment Steps
1. No known treatment. Genetic counseling available for receptive
individuals.
2. Because there is no cure, patients or individuals at risk may com-
mit suicide.
IX. TRAUMA
A. Subdural Hematoma
H&P Keys
History of head trauma with progressive change in mentation, focal
neurologic signs, often loss of consciousness but not always, hemi-
paresis, large unreactive pupil with ophthalmoplegia if acute;
seizures, headache, progressive change in mentation if chronic.
Diagnosis
CT of head (Fig. 10–9), if performed without contrast, may miss an
isodense (chronic) subdural hematoma. MRI of head with contrast
(gadolinium) is the study of choice. Both will show crescentic lesion
with signal compatible with blood.
Disease Severity
Progressive focal neurologic deficit, progressive obtundation, re-
quires quick intervention. Mass effect and shift of intracranial con-
tents in radiologic studies also are usually suggestive of increased
severity.
Figure 10–9. Hyperintense acute subdural hematoma with compression of underlying brain.
282
NEUROLOGY Trauma
B. Epidural Hematoma
H&P Keys
Severe head trauma accompanied by loss of consciousness, transient
recovery of consciousness followed by progressive obtundation, pos-
turing, shallow respirations, seizures, focal neurologic signs, coma.
Diagnosis
CT of head or MRI will show concave blood clot, white on CT, bright
on T1- and T2-weighted images on MRI. Skull x-rays will show frac-
ture through area of middle meningeal artery or, less commonly,
across venous sinus.
Disease Severity
If untreated, the condition is lethal; timing is of the essence.
Concept and Application
Tear of middle meningeal artery or venous sinus results in accumu-
lation of blood at great pressure in a potential space.
Treatment Steps
1. Emergent surgical evacuation.
2. Supportive.
C. Contusion
H&P Keys
History of moderate to severe head trauma (unconscious for > 5
minutes); returns to alertness with confusion and mild mutism.
Exam shows extensor plantar reflexes, mild hemiparesis, elevated
blood pressure and heart rate.
Diagnosis
CT or MRI may show focal parenchymal swelling (most commonly
frontal or temporal tip) or delayed hemorrhage.
Disease Severity
Degree of alertness at the time of evaluation, time unconscious, de-
gree of retrograde amnesia determine severity of injury if otherwise
uncomplicated. Temporal lobe herniation is main cause of morbid-
ity and mortality.
Concept and Application
Sustained head trauma results in neuronal swelling and axonal
shearing, often maximal 24–48 hours after event.
Treatment Steps
Control of intracranial pressure with hyperventilation, ventricu-
lostomy; barbiturate coma may lessen neuronal injury. Delayed phys-
ical and cognitive deficits may occur and will require therapy.
283
NEUROLOGY Demyelination
X. DEMYELINATION
A. Multiple Sclerosis
H&P Keys
History of arm, leg, hand, or foot numbness (50% of patients); vi-
sual loss (25%); diplopia; incoordination; weakness; bladder dys-
function. Exam shows sensory loss in spinal distribution, afferent
pupillary deficit, ataxia, dysarthria, hyperreflexia, internuclear oph-
thalmoplegia.
Diagnosis
MRI will show periventricular white matter demyelination or lesion
in the spinal cord or optic nerve (Fig. 10–10). LP shows lymphocytic
pleocytosis (< 100 cells/mL), increased protein (< 100 mg/mL),
normal glucose, increased IgG intrathecal production, and oligo-
clonal bands. Large myelinated pathways can be assessed with
evoked potentials (visual, auditory, somatosensory). The disease can
be mimicked by syphilis, Sjögren’s syndrome, systemic lupus erythe-
matosus, sarcoidosis, and Lyme disease. The diagnosis remains clini-
cal and depends on finding different lesions on separate occasions.
Disease Severity
Pure spinal forms, chronic progressive, and early presentation are
correlated with worst outcome. Elevation of body temperature
(Uhthoff’s phenomenon), stress, and infection can cause exacerba-
tion.
Concept and Application
The etiology of the disease is multifactorial, with environmental fac-
tors, increased incidence in temperate regions, immunogenetic fac-
tors such as certain human leukocyte antigen (HLA)-Dw or DR, fol-
lowed by an aberrant immunologic response against CNS myelin.
Figure 10–10. MRI of patient with multiple sclerosis demonstrating multiple white matter lesions
with characteristic periventricular localization and perpendicular to ventricle orientation.
284
NEUROLOGY Sleep Disorders
Treatment Steps
1. Acute attacks respond to IV corticosteroids.
2. Chronic prophylaxis can be obtained with interferon-β or glati-
ramer acetate.
3. The tremor responds to isoniazid, propranolol, clonazepam, or
primidone; baclofen orally or intrathecally or tizanidine reduces
spasticity.
4. Fatigue responds to amantadine, pemoline, or modafinil.
5. Gait disability, bladder disorders require appropriate care.
A. Sleep Apnea
H&P Keys
Obesity, short neck, large tongue, large tonsils in children,
myxedema, acromegaly, myotonic dystrophy in obstructive type; po-
liomyelitis, syringobulbia, and brain stem infarct in central type; his-
tory of heavy snoring, daytime sleepiness, fatigue, early morning
headache, impotence.
Diagnosis
Thyroid studies to rule out hypothyroidism; polysomnogram to ex-
clude other etiologies of daytime sleepiness and to assess apneas and
differentiate between central or obstructive type; multiple sleep la-
tencies to assess the degree of nocturnal disturbance.
Disease Severity
Six or more apneas per hour; oxygen desaturation; nocturnal car-
diac arrhythmias. Sleep apnea is associated with cerebrovascular ac-
cidents, heart attacks.
Concept and Application
Upper airway laxity and collapse on inspiration; reduced nocturnal
respiratory drive in central type.
Treatment Steps
1. Weight reduction of as few as 5 pounds may reduce symptoms.
2. Continuous positive airway pressure (CPAP) for obstructive type,
repeat studies and adjust settings.
3. Protriptyline for central apnea.
B. Narcolepsy
H&P Keys
Present with excessive daytime sleepiness; multiple daytime naps;
paralysis with strong emotions in 70% (cataplexy); sleep paralysis;
hypnagogic hallucinations; normal examination.
Diagnosis
Sleep study (polysomnogram) is normal and will exclude other
pathologies; multiple sleep latencies show rapid eye movement
(REM) sleep onset in at least 2 out of 4–5 recordings; same abnor-
mality can be seen with use of drugs, drug withdrawal, or sleep dep-
rivation; therefore, needs good clinical correlation.
Disease Severity
Most patients have narcolepsy and cataplexy by history; there could
be milder cases; few have all symptoms.
285
BIBLIOGRAPHY
Bradley WG, Daroff RB, Fenichel GM, Marsden CD. Neurology in Clinical Practice, 4th
ed. Boston: Butterworth Heinemann, 2004.
Brazis PW, Marsden JC, Biller J, Brazis P. Localization in Clinical Neurology. Philadel-
phia: Lippincott Williams & Wilkins, 2001.
Campbell WW, DeJong RN. DeJong’s The Neurologic Examination, 6th ed. Philadelphia:
Lippincott Williams & Wilkins, 2005.
Goetz CG, Pappert EG. Textbook of Clinical Neurology. Philadelphia: W.B. Saunders,
2003.
Joynt RJ, Griggs RC (eds.). Baker’s Clinical Neurology. Philadelphia: Lippincott Williams
& Wilkins, 2002.
Mayo Clinic Department of Neurology. Mayo Clinic Examinations in Neurology, 7th ed.
St. Louis: Mosby, 1998.
Ropper AH, Brown RJ. Adams & Victor’s Principles of Neurology. New York: McGraw-Hill,
2005.
Rowland LP (ed.). Merritt’s Neurology, 11th ed. Lippincott Williams & Wilkins, 2005.
Samuels MA (ed.). Hospitalist Neurology. Boston: Butterworth–Heinemann, 1999.
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Male and Female
Reproduction 11
I. UTERUS / 289
A. Malignant Neoplasm / 289
B. Leiomyomata Uteri / 289
C. Other Disorders / 290
287
Copyright © 2004, 2001 by The McGraw-Hill Companies, Inc. Click here for terms of use.
288 Male and Female Reproduction
BIBLIOGRAPHY / 304
289
A. Malignant Neoplasm
1. Endometrial Cancer
H&P Keys
Obesity, nulliparity, chronic anovulation, diabetes, hypertension,
postmenopausal bleeding, history of tamoxifen therapy, unopposed
estrogen therapy.
Diagnosis
Endometrial biopsy, transvaginal ultrasonography (thickened en-
dometrial “stripe”), hysteroscopy, fractional dilatation and curettage.
Advanced Disease—Chest x-ray (CXR), intravenous pyelogram (IVP),
computed tomographic (CT) scan, barium enema, cystoscopy.
Disease Severity
International Federation of Gynecology and Obstetrics (FIGO).
FIGO Staging (1988)
Treatment Steps
Early Disease—Total abdominal hysterectomy and bilateral sal-
pingo-oophorectomy (with or without lymph node sampling).
Adjunctive Therapy
1. External beam radiation.
2. Preoperative radiation.
Recurrent Endometrial Carcinoma
1. High-dose progestins, e.g., medroxyprogesterone.
2. Chemotherapy, e.g., adriamycin, platinum.
B. Leiomyomata Uteri
H&P Keys
Most common solid pelvic tumor in women; pain, abnormal uterine
bleeding, pressure, uterine enlargement, urinary frequency, rectal
pressure (Fig. 11–1).
Diagnosis
Complete blood count (CBC), physical exam, pelvic ultrasound.
Disease Severity
Severity of pain, anemia, urinary symptoms, hydronephrosis, uterine
size beyond 12 weeks.
290
MALE AND FEMALE REPRODUCTION Uterus
Figure 11–1. Common types of uterine fibroids. (Reproduced, with permission, from Pernoll ML,
Benson RC [eds.]. Current Obstetric & Gynecologic Diagnosis & Treatment, 9th ed. Appleton & Lange,
2002.)
C. Other Disorders
1. Endometriosis
H&P Keys
Premenstrual dysmenorrhea, premenstrual spotting or staining, dys-
pareunia, infertility, chronic pelvic pain, retroverted uterus,
uterosacral nodularity, nonmobile uterus.
Diagnosis
History and physical examination, bimanual examination, pelvic ul-
trasonography (for presence of endometrioma), laparoscopy (gold
standard of diagnosis; Fig. 11–2), biopsy if possible (histology shows
endometrial glands and stroma). (CA 125 of questionable help and
very nonspecific.)
Disease Severity
American Fertility Society stages I to IV (minimal through severe),
extent of pelvic pain.
291
II. OVARY
A. Malignant Neoplasm
1. Ovarian Cancer
H&P Keys
Fifth most common of all cancers in women, higest mortality rate;
two-thirds present with advanced disease. Affects 1 in 70 women in
the United States.
Diagnosis
Symptoms of ascites, abdominal distention in advanced stages. His-
tory and physical exam, pelvic ultrasonography, CA 125, CXR, surgi-
cal staging.
Disease Severity
management
decisions d Treatment Steps
1. Total abdominal hysterectomy, bilateral salpingo-oophorectomy,
omentectomy, staging.
2. Adjunctive chemotherapy.
BENIGN ADNEXAL MASSES
Overall 5-year survival depends on age, stage, grade, and residual
Ovarian Corpus Luteum
disease after initial surgery.
Cyst
Observation; serial
B. Ovarian Cysts
ultrasonography; cyst H&P Keys
usually resolves within 9 Abdominal pain; anovulation, irregular bleeding, irregular periods,
weeks, with or without oral
reproductive age group; usually unilateral, bimanual exam.
contraceptive suppression.
Rarely, surgical intervention
Diagnosis
is needed.
CBC and differential, pelvic ultrasonography (size, loculations, uni-
Ovarian Hemorrhagic
laterality versus bilaterality, calcifications), β-human chorionic go-
Corpus Luteum Cyst
As above.
nadotropin (β-hCG) to rule out ectopic pregnancy.
Ovarian Follicular Cyst Disease Severity
Observation; usually Amount of pain, cyst size, presence of loculations, fluid in the peri-
associated with
toneal cavity (rare).
anovulatory cycle;
ultrasound for size; Concept and Application
progestin to bring on
Most common is functional cyst related to anovulation. Follicular
menses; occasionally, oral
contraceptives will help cyst continues to develop and enlarge without ovulation, thereby
shrink cyst. Rarely, surgery causing pain, possibly intraperitoneal rupture, and irregular menses.
is needed. Eighty-five percent will resolve by 9 weeks.
Endometrioma
Treatment Steps
Observation; analgesics,
GnRH agonists to 1. Observation, recheck at 3-week intervals.
suppress growth of 2. Serial ultrasonograms.
endometriosis, serial 3. Serial CBC with differential if suspicion of intraperitoneal bleed-
ultrasonography to check ing.
response to GnRH agonist. 4. Surgery if danger of torsion.
Surgery may be needed to
5. Possible laparotomy, ovarian cystectomy, or oophorectomy.
drain cyst, remove cyst
wall, or even perform
oophorectomy.
Pedunculated Fibroid III. CERVIX
Observation, serial
ultrasonography; rarely A. Malignant Neoplasm
need surgery if rapid
H&P Keys
growth in size, or pain from
torsion or pressure.
Human papillomavirus (HPV) infection plays a main role in the
pathogenesis of cervical cancer, cigarette smoking, high-risk sexual
Ovarian Cyst with Torsion
Stat admission to OR, to
behaviors. Postcoital bleeding, abnormal discharge, abnormal Pap
detorse ovary if no smears.
ischemia present;
otherwise, emergency Diagnosis
oophorectomy performed. Pap smear, colposcopy, cervical biopsy, cone biopsy, endocervical
curettage (ECC), CXR, IVP, barium enema, cystoscopy, proctosig-
moidoscopy, magnetic resonance imaging (MRI) (optional).
Disease Severity (FIGO Staging, 1985)
Staging is clinical.
Stage I—Confined to cervix.
Stage II—Upper vagina, not pelvic sidewall.
Stage III—Extending to pelvic sidewall.
Stage IV—Beyond pelvis: distant organs.
293
Disease Severity
HSV—Primary lesions 2–3 weeks, recurrent lesions.
Gonorrhea—Severity of symptoms may progress to overt pelvic in-
flammatory disease (PID) with tubo-ovarian abscess formation;
advanced stages: arthritis.
Syphilis—Primary, secondary, tertiary syphilis; neurosyphilis.
Chlamydia—Cervicitis may progress to overt PID with tubo-ovarian
abscess formation.
Chancroid—Severity of symptoms.
HPV—HPV serotyping: high-risk types versus low-risk types for
progression to dysplasia.
LGV—Severity of symptoms.
Treatment Steps
A. Malignant Neoplasms
1. Vulvar Carcinoma
H&P Keys
Chronic vulvar irritation or itching, labial lesion that does not heal,
history of condyloma; age range 60–80 years.
Diagnosis and Evaluation
Biopsy.
295
Diagnosis
Trichomoniasis—Trichomonas vaginalis.
Bacterial Vaginosis—Gardnerella vaginalis.
Treatment Steps
Trichomoniasis—Metronidazole (PO).
Bacterial Vaginosis—Metronidazole (PO or intravaginal).
V. MENSTRUAL DISORDERS
A. Dysmenorrhea
H&P Keys
Pelvic pain with menses with variable onset of pain compared to day
1 of flow; nausea, diarrhea, headache, ovulatory menstrual cycles.
297
Primary Dysmenorrhea
1. NSAIDs to inhibit prostaglandin synthetase and thereby de-
crease smooth-muscle contractility.
2. Combination oral contraceptives to inhibit ovulation.
3. GnRH agonists in severe cases if suspicion of endemetriosis is
present.
4. If no relief, laparoscopy may be needed to rule out pelvic
pathology (e.g., secondary dysmenorrhea).
C. Disorders of Menstruation
1. Amenorrhea
H&P Keys
Primary amenorrhea is defined as no menses by age 16. Secondary is 3
months or longer of amenorrhea in a normally cycling individual.
Possible sexual ambiguity or virilization; possible absence of sec-
ondary sex characteristics.
Diagnosis
History and physical examination, β-hCG, prolactin, follicle-stimulat-
ing hormone (FSH), luteinizing hormone (LH), progesterone with-
298
MALE AND FEMALE REPRODUCTION Menopause
diagnostic
decisions d drawal test. If virilization, testosterone, dehydroepiandrosterone sul-
fate (DHEAS). Primary: karyotype, FSH, estradiol.
Disease Severity
SECONDARY AMENORRHEA Presence or absence of secondary sex characteristics; no breast de-
velopment by age 14.
Pregnancy
Most common etiology of Concept and Application
secondary amenorrhea in Primary—Müllerian agenesis, testicular feminization (androgen
reproductive age women.
insensitivity), Turner’s syndrome.
Look for sexual activity with
partner—unprotected Secondary—Pregnancy, Asherman’s syndrome (intrauterine syn-
intercourse. Usually 1–2 echiae), polycystic ovarian disease, congenital adrenal hyperplasia,
weeks beyond expected
hyperandrogenism, hypothyroidism, hyperprolactinemia.
date of menses. Check
pregnancy test. Hypothalamic—Associated with weight loss, chronic anxiety, exces-
Menopause sive exercise, eating disorder (anorexia nervosa), marijuana, tran-
Most common etiology of quilizers, head injury, chronic medical illness, central nervous sys-
secondary amenorrhea in tem (CNS) tumor, IV drug use (opium derivatives).
older women. Average age
of menopause is 51.4 Note—In the absence of all the above, diagnosis is dysfunctional
years in the United States. uterine bleeding.
Look for a history of hot
flashes; vaginal dryness; Treatment Steps
abnormal menstrual 1. Primary: Obtain karyotype if < 30 years old. If abnormal kary-
bleeding patterns, followed otype, phenotypic female: estrogen replacement therapy for com-
by amenorrhea. If this pletion of secondary sex characteristics. Müllerian agenesis re-
occurs before age 40, it is
quires surgery.
called premature ovarian
failure. Check FSH levels
2. Secondary: Requires cyclic menstrual function to prevent endome-
and estradiol levels. trial hyperplasia: cyclic combination oral contraceptive therapy or
Hypothalamic
monthly progestin therapy.
Amenorrhea 3. Hypothalamic: Hormone replacement therapy to prevent osteo-
History of exercise; eating porosis and maintain normal physiologic status.
disorder (usually anorexia); 4. Asherman’s syndrome: Amenorrhea not responsive to estrogen–
decreased body fat; high- progesterone cycle; hysteroscopic surgical intervention.
stress lifestyle; heroin or
opiate usage. Measure
patient’s height, weight,
and body mass index
(BMI); check bone density VI. MENOPAUSE
for osteoporosis. Obtain
H&P Keys
good dietary history.
Changes in menstrual cycle regularity, decreased cycle interval, fi-
Polycystic Ovarian
nally cessation of menses; mean age 51.4 years; atrophy of estrogen-
Disease (PCOD)
History of obesity,
dependent tissue (uterus, breasts, vagina), vasomotor symptoms (hot
hirsutism, and infertility. flashes), osteoporosis, urethral changes, increased frequency of cysti-
Usually familial as well; look tis, insomnia.
for height, weight;
↑ BMI; hirsutism on face, Diagnosis
chest, abdomen, male FSH; estradiol. Menopause prior to age 40 means premature ovarian
escutcheon; bilateral failure; if menopause prior to age 30, check karyotype.
ovarian cystic enlargement;
obtain LH, FSH, and check Disease Severity
if positive withdrawal bleed 1. Skin collagen content decreases.
to progestins. 2. Worsening serum lipid profile.
3. Vaginal dryness with atrophy.
4. Breast atrophy.
5. Osteopenia leading to osteoporosis and possibly stress frac-
tures.
6. Atherosclerotic coronary vessel disease.
7. Senility, Alzheimer’s disease.
299
VII. BREAST
A. Malignant Neoplasm
H&P Keys
Family history (5–10% of breast cancers show genetic component),
long history of estrogen exposure, nulliparity, high fat intake; dis-
crete lump, retracted nipple, puckering of breast skin (peau d’or-
ange), axillary lymphadenopathy, nipple bleeding or discharge
(Table 11–1).
Diagnosis
Mammography, physical examination, biopsy.
11-1
RISK FACTORS FOR BREAST CANCER
Reproduced, with permission, from Gant NF, Cunningham FG. Basic Gynecology and Obstetrics. Norwalk, CT: Appleton & Lange,
1993.
300
MALE AND FEMALE REPRODUCTION Breast
Disease Severity
Stated by T (tumor size), N (regional lymph nodes), and M (distant
metastases). Evaluations done for hormone receptors reflect tumor
responsiveness to chemotherapy.
Treatment Steps
Surgery
1. Mammography.
2. Biopsy.
3. Lumpectomy.
4. Simple mastectomy (if necessary).
5. Radical mastectomy (if necessary).
Hormonal Therapy—If positive hormone receptors, progestins.
B. Fibroadenoma
Benign breast neoplasm.
H&P Keys
Younger women, ages 20–35; single breast mass, smooth, well-
circumscribed, firm, mobile, and rubbery nodule.
Diagnosis
Mammography, sonography.
Disease Severity
Severity of symptoms.
Treatment Steps
1. Fine-needle aspiration to make diagnosis.
2. Subsequent observation.
3. Possible lumpectomy needed.
C. Intraductal Papilloma
Benign breast lesion.
H&P Keys
Unilateral bloody or serous nipple discharge, no palpable mass.
Diagnosis
History, exam, mammogram, and duct evaluation.
Disease Severity
Amount of nipple discharge.
Treatment Steps
Local excision of lesion and duct.
301
management decisions d
INFERTILITY
Ovulatory Dysfunction
One-third of all infertility etiologies. Determine cause of oligo/anovulation. If abnormal thyroid
function, replace thyroid hormone, then await resumption of menses. If elevated prolactin levels,
determine etiology—use bromocriptine (dopamine agonist) to decrease prolactin, then await
resumption of menses. If elevated androgen levels of adrenal origin, give glucocorticoid to inhibit
adrenals, then add ovulation induction agent (e.g., clomiphene, human menopausal
gonadotropins). If elevated androgen levels of ovarian origin (e.g., PCOD), give ovulation induction
agents. If hypothalamic amenorrhea, remove etiologic agent if possible (e.g., dietary correction,
ceasing drug usage, decreasing severe exercise, etc.).
Tubal Obstruction
One-third of all infertility etiologies. Associated with poor prognosis if long-standing. Also risk of
ectopic pregnancies. Surgery is first-line treatment; attempt to open tube(s) via
laparoscopy/hysteroscopy or laparotomy. Overall success rate is 10%. ART if surgery not
possible. IVF provides up to 20–25% success rate/cycle. Involves ovulation induction with potent
injectable gonadotropins, oocyte retrieval, and embryo transfer.
Male Factor
Third main cause of overall infertility. Semen analysis shows most abnormalities, although sperm
antibody testing and postcoital testing very important. If low count, or antisperm antibody
positive, then intrauterine insemination is suggested (with/without ovulatory induction drugs). If
unsuccessful, ART with intracytoplasmic sperm injection. Finally, donor semen is used in the most
severe cases.
303
management
decisions d Diagnosis
Pap smear, lipid profile, STD screening, mammogram, colonoscopy,
bone density at appropriate ages.
FAMILY PLANNING Note—General gynecologic exam should be done annually along
with counseling about tobacco, alcohol, caffeine, exercise, and
Teenage Girl diet.
The most important two
issues here are to prevent B. General Counseling for Contraception
pregnancy and to prevent Types—Natural family planning; spermicides and barrier contra-
transmission of STDs,
ceptives (spermicide, condoms, diaphragms, sponges, cervical
especially in light of multiple
partners. Condoms plus
caps); intrauterine devices (IUDs) (Progesta-Sert, Paraguard);
hormonal contraception steroid contraceptives: contraceptive patch, vaginal ring, combi-
would provide this nation oral contraceptive, progestin-only contraceptives; in-
coverage. Hormonal jectable and implantable contraceptives (medroxyprogesterone
contraception consists of acetate); postcoital contraception (emergency contraception),
either oral contraceptives oral abortifacients (RU-486, methotrexate and misoprostol).
or long-acting injectable
progestins (e.g., Depo- Effectiveness (Given as Failure Rate)—Oral contraceptives, < 1–2%;
Provera), contraceptive IUD, 2–4%; diaphragm with spermicide, 10–20%; condom,
patch, or vaginal ring. 5–15%.
Woman in Long-Term Surveillance of Prescribed Contraceptives—Oral contraceptives: regular
Monogamous
breast, thyroid, liver, and pelvic examinations, initial blood pres-
Relationship
Completed childbearing: sure check; IUD: string check 6 weeks after insertion.
Laparoscopic tubal
sterilization or vasectomy is C. Sterilization
most highly recommended Most frequent method of controlling fertility in the United States.
if permanent contraception
Male Sterilization—Vasectomy failure rate 1%.
desired. Otherwise, long-
acting progestins, or IUD. Female Sterilization—Postpartum failure rate 1 in 250; interval (be-
Not completed tween pregnancies) failure rate 1 in 500.
childbearing: Long-acting
progestins, or oral Counseling must include permanent nature of procedure, opera-
contraceptives, or IUD. tive risk, failure rate. Despite careful counseling, approximately 1%
Woman over 35, Smoker, of patients undergoing sterilization subsequently request reversal.
in Monogamous Most common reason: new sexual partner.
Relationship
Condoms or long-acting
injectable progestins, or
BIBLIOGRAPHY
IUD.
Perimenopausal Woman Beckman CRB, Ling FW, et al. Obstetrics and Gynecology, 4th ed. Baltimore: Lippincott
Ultra-low-dose oral Williams & Wilkins, 2002.
contraception assuming no DeCherney AH, Pernoll ML. Current Obstetric and Gynecologic Diagnosis and Treatment.
Norwalk, CT: Appleton & Lange, 2002.
contraindications to Gant NF, Cunningham FG. Basic Gynecology and Obstetrics. Norwalk, CT: Appleton &
estrogen; long-acting Lange, 1993.
progestins; tubal
sterilization if permanent
contraception desired.
Obstetrics 12
I. UNCOMPLICATED PREGNANCY / 307
A. Health and Health Maintenance / 307
B. Prenatal Diagnosis / 307
C. Postpartum Care of the Mother / 308
D. Lactation / 308
E. Normal Labor and Delivery / 309
305
Copyright © 2004, 2001 by The McGraw-Hill Companies, Inc. Click here for terms of use.
306 Obstetrics
BIBLIOGRAPHY / 326
307
B. Prenatal Diagnosis
H&P Keys
Exposure to medications or teratogens (i.e., alcohol, radiation, tetra-
cycline, antiepileptics, folic acid antagonists, warfarin, mercury,
syphilis, rubella, toxoplasmosis), pedigree, accurate pregnancy dat-
ing, exam looking for expression of genotype.
Diagnosis
Specific for condition evaluated: biochemical screening tests:
MSAFP, unconjugated estriol, and β-hCG and inhibin A. Abnormal
patterns are associated with neural tube defects and aneuploidy (tri-
somies 18 and 21). Ultrasound screening: major structural anom-
alies, thickened nuchal fold, growth lag, and abnormal amniotic
fluid volume.
Disease Severity
Specific for condition evaluated.
308
OBSTETRICS Uncomplicated Pregnancy
d
Concept and Application
diagnostic Hundreds of biochemical, chromosomal, and genetic disorders can
decisions be diagnosed by condition-specific tests including ultrasonography,
identified structural change, karotyping, DNA analysis technologies,
fetal tissue enzyme activity, and metabolic product accumulation in
PRENATAL DIAGNOSIS
fetal cells obtained by chorionic villus sampling (CVS), amniocente-
Chorionic Villus Sampling
sis, percutaneous umbilical blood sampling, or fetal biopsy. Major
Provides direct DNA congenital anomalies occur in 3% of all pregnancies.
analysis of fetus by
Treatment Steps
sampling chorionic tissue.
Evaluation can be done 1. Specific for the condition evaluated.
between 10 and 14 weeks. 2. Nondirective counseling is used.
Amniocentesis
Provides direct DNA C. Postpartum Care of the Mother
analysis of fetus by H&P Keys
sampling the somatic cells Childbirth in the preceding 6 weeks. Lochia changes from red to
of the fetus. Evaluation is
brown to serous over first 2 weeks. Episiotomy heals rapidly. Uterus
done after 15–16 weeks.
is in pelvis at 2 weeks and normal size at 6 weeks. If mother is Rh
Biochemical Screen
negative, baby needs to be tested; if baby is Rh positive, Rh Ig must
Provides indirect
biochemical analysis of be given.
AFP, estriol, and
Diagnosis
β-hCG as markers for fetal
abnormalities, specifically Pap smear at postpartum visit if no recent Pap smear.
open neural tube defects
Disease Severity
and chromosomal
abnormalities. Evaluation is Infection: endomyometritis (mixed aerobic and anaerobic flora),
done between 15 and 18 mastitis (Staphylococcus aureus), breast abscess. Depression: > 50% will
weeks. have a week of “the blues,” 10% will be depressed, and 0.05% suici-
Ultrasound dal.
Provides a visual image of
the fetus at any gestational Concept and Application
age. Fifty percent of nonlactating women ovulate between 28 and 90 days
postpartum. Cardiac output normalizes in several hours. Glomerular
filtration rate (GFR) is down to normal in a few weeks.
Treatment Steps
Initial Care
1. Evaluation, support, and problem-specific therapy.
2. Rh Ig if indicated.
Emergency Care—Problem specific.
D. Lactation
H&P Keys
Absence of pain, erythema, localized induration, abscess, nipple fis-
sures or cracks. Prefeeding engorgement and discomfort is common
in the first few weeks. Limit medications to those necessary and not
contraindicated.
Diagnosis
None.
Disease Severity
Same as history and physical examination.
Concept and Application
Human breast milk is the best nutrition and immunologic stimula-
tion for the baby. Prolactin is essential. Suckling stimulates the neu-
rohypophysis to release oxytocin, which contracts the breast’s my-
309
Figure 12–1. Stages of labor. (Reproduced, with permission, from Cohen W, Friedman EA, eds. Man-
agement of Labor, 2nd ed. University Park Press, 1988.)
310
OBSTETRICS Complicated Pregnancy
Treatment Steps
A. Adolescent Pregnancy
H&P Keys
Nineteen years old and younger, sexually transmitted disease (STD)
surveillance, nutritional deficiency, substance abuse, sexual abuse.
Diagnosis
Same for pregnancy; assist with social support network.
Disease Severity
Disease specific.
Management
Routine prenatal care with a special emphasis in support services
and screening and intervention for problems.
B. Ectopic Pregnancy
H&P Keys
Early pregnancy symptoms and signs; amenorrhea; abnormal vaginal
bleeding; and abdominal pain, colicky and lateralizing to the af-
fected side. Shoulder pain, rectal pain, syncope, and peritoneal signs
are associated with intraperitoneal bleeding. Passage of the decidual
cast is confused with spontaneous abortion (SAB).
Diagnosis
Quantitative β-hCG rising < 66% every 48 hours, serum proges-
terone < 5 ng/mL, and inability to identify an intrauterine preg-
nancy by transabdominal ultrasonography when the β-hCG is
> 5,000 mIU/mL or by transvaginal ultrasonography if the β-hCG is
> 2,000. Culdocentesis to identify hemoperitoneum if ultrasonogra-
phy is not available. Blood type and Rh.
Disease Severity
Transvaginal sonography and serial measurement of the β-hCG diag-
nose many unruptured ectopics.
Treatment Steps
Initial Care
1. Threatened abortion is usually managed by observation and
prevention of the introduction of infection. There is no evi-
dence to support that bed rest will prevent early SAB.
2. Incomplete abortion is treated by uterine evacuation.
3. Rh Ig for the Rh-negative woman.
Emergency Care—Same as initial care.
312
OBSTETRICS Complicated Pregnancy
Continued Care
1. Emotional support for the patient and family.
2. Prepregnancy evaluation if recurrent (three or more).
D. Induced Abortion
H&P Keys
Termination of an otherwise viable pregnancy for medical or per-
sonal reasons. A legal termination is consistent with statutory author-
ity. An illegal or criminal one is not.
Diagnosis
Same as initial pregnancy evaluation.
Disease Severity
Advanced gestational age.
Concept and Application
Compliance with the patient’s wishes. Procedures are safest when
performed in the first trimester. They can be done either surgically
or medically using a combination of methotrexate followed by
prostaglandins.
Treatment Steps
E. Septic Abortion
H&P Keys
Same as SAB, except for symptoms of infection, fever, foul-smelling
discharge, and pain. Possible history of illegal abortion.
Diagnosis
Same as SAB except for associated infection, septic shock, renal fail-
ure.
Disease Severity
Same as SAB except for associated infection, septic shock, renal fail-
ure. Can be lethal.
Concept and Application
Same as SAB except for increased risk of perforation and associated
organ injury at dilatation and evacuation.
Treatment Steps
Same as SAB, plus inspection of injury and administration of broad-
spectrum antibiotics prior to or during procedure pending culture
results.
F. Placenta Previa
H&P Keys
Frequent incidental ultrasonographic finding in early pregnancy. Pa-
tients present with painless vaginal bleeding in the third trimester.
313
Initial Care
1. Asymptomatic patient education, and coital restriction in the
third trimester.
2. Serial ultrasonograms for fetal growth and placental location.
Emergency Care—A patient who has bleeding is hospitalized for he-
modynamic stabilization and fetal assessment.
Continued Care
1. Delivery is usually by cesarean section after 36 weeks with doc-
umented fetal maturity or uncorrectable fetal compromise.
2. Patients with marginal placenta previa may attempt a vaginal
delivery.
G. Abruptio Placentae
H&P Keys
Painful contractions, tender uterus, fundus remains firm between
contractions, and vaginal bleeding. Fetal monitoring may be nonre-
assuring.
Diagnosis
Clinical diagnosis, CBC, platelet count, fibrin split products, fibrino-
gen and prothrombin time (PT) and partial thromboplastin time
(PTT) for disseminated intravascular coagulation (DIC). Ultra-
sonography is of limited value.
Disease Severity
Shock out of proportion to the observed blood loss, especially if con-
cealed hemorrhage. Fetal demise possible if > 50% abruption.
Concept and Application
Bleeding into the decidua basalis. Couvelaire uterus is caused by
blood extravasating into the myometrium (ecchymosis). Abruptio
may be caused by hypertension, cocaine, smoking, uterine decom-
pression, and trauma.
Treatment Steps
diagnostic
decisions d 3. Immature fetus may be expectantly managed in mild abrup-
tions.
H. Preeclampsia
THIRD TRIMESTER H&P Keys
BLEEDING
Hypertension (140/90 mm Hg) and proteinuria (> 300 mg/24
hours) and edema after 20 weeks. Signs and symptoms of HELLP
Placenta Previa
Look for painless bleeding
syndrome (Hemolysis, Elevated Liver functions, Low Platelets).
with the placenta located
Diagnosis
low in the uterus in the area
of the cervix. Careful blood pressure (BP) measurement, 24-hour urine for pro-
tein and creatinine clearance, CBC with platelets, and uric acid. As-
Placental Abruption
Look for bleeding partate aminotransferase (AST, formerly SGOT), bilirubin, and lac-
associated with pain and tic dehydrogenase (LDH) may be elevated. Fetal growth and
contractions. Look carefully well-being assessment.
for fetal compromise.
Disease Severity
Bloody Show
Painless bleeding often Mild preeclampsia is without symptoms and signs of severe
mixed with mucus. preeclampsia. Severe preeclampsia if BP > 160/110 mm Hg, > 5 g of
Bleeding is usually minimal proteinuria in 24 hours, visual disturbances, headache, pulmonary
and is often accompanied edema, cyanosis, epigastric pain, right upper quadrant pain, liver
by early labor. dysfunction, oliguria, thrombocytopenia, intrauterine growth retar-
dation (IUGR), or oligohydramnios.
Concept and Application
Diffuse multiorgan vasospastic disease starting months before diag-
nosis, characterized by decreased sensitivity to angiotensin II and in-
creased thromboxane-to-prostacyclin ratio. Delivery is the only spe-
cific therapy.
Treatment Steps
I. Eclampsia
H&P Keys
Preeclampsia with tonic–clonic seizures of no other etiology.
Diagnosis
Same as preeclampsia.
Disease Severity
Same as preeclampsia.
Treatment Steps
J. Premature Labor
H&P Keys
Uterine contractions with cervical change prior to 37 weeks. Symp-
toms: cramps, backache, pressure, and uterine contractions.
Diagnosis
Monitor for contractions, rule out rupture of membranes, check for
cervical change, culture vagina for group B streptococcus, evaluate
for urinary tract infection or other processes that might be causing
the contractions, confirm dating if indicated.
Disease Severity
No tocolysis if rupture of membranes, advanced cervical dilatation
(5+ cm), fetal distress, fetal anomalies, mature fetus, in utero infec-
tion, and conditions made worse by tocolysis.
Treatment Steps
Emergency Care
1. Monitoring, hydration, and evaluation of the patient; adminis-
tration of tocolytic (SQ terbutaline, 0.25 mg, 3–6 doses every
20–30 minutes or incremental increases from IV 0.050
mg/min or IV MgSO4 6-g load and 2–3 g/hr) and monitoring
for complications (pulmonary edema, etc.).
2. Penicillin for group B streptococcus prophylaxis.
3. Betamethasone IM, 12 mg every 12 hours for two doses, to
hasten pulmonary maturity and decrease hemorrhagic disor-
ders and necrotizing enterocolitis.
Continued Care—Outpatient on oral β-sympathomimetics.
Initial Care
Lower Tract—Antibiotic therapy by local sensitivities (usually
7–10 days of ampicillin or nitrofurantoin).
Pyelonephritis—IV, then oral therapy. Monitoring for preterm la-
bor.
Continued Care—Reculturing monthly. If recurrent infection, pro-
phylactic antibiotics.
L. Incompetent Cervix
H&P Keys
Painless preterm (second trimester) effacement and dilatation of
the cervix. Patient complains of several days of vaginal or pelvic pres-
sure, watery-to-pink vaginal discharge, or backache prior to rupture
of the membranes. There is no history consistent with uterine con-
tractions. The process usually recurs in multiple pregnancies if there
is no intervention. Although there is no specific known cause, risk
factors include trauma to the cervix including surgery and diethyl-
stilbestrol (DES) exposure.
317
Disease Severity
Associated with deep lacerations or conizations and cervical amputa-
tions.
Treatment Steps
Diagnosis
Elevated MSAFP, amniotic fluid (AF) AFP, elevated AF acetyl-
cholinesterase. Directed scan (Fig. 12–3).
Disease Severity
Anencephaly (failure of development of the forebrain) occurs in
50% of NTDs. Spina bifida may occur at any level.
Treatment Steps
N. Trisomy
H&P Keys
Advanced maternal age, previous history, balanced translocation,
low MSAFP. Trisomy 21 is most common, accounting for 1 in 800 live
births.
318
OBSTETRICS Complicated Pregnancy
Diagnosis
Fetal sampling via CVS, amniocentesis, or percutaneous umbilical
blood sample (PUBS) is definitive.
Disease Severity
Babies with a mosaicism may not fully express the typical phenotype.
Concept and Application
Aneuploidy results from nondisjunction in the first meiotic division
or from an unbalanced translocation. Trisomy 21 is most common,
followed by trisomy 18 and trisomy 13.
319
O. Rh Incompatibility or Isoimmunization
H&P Keys
Inadequate or no Rh Ig after Rh-positive RBC exposure in Rh-
negative woman; Kell-negative recipient of Kell-positive transfusion.
Diagnosis
Serial maternal antibody titering; amniocentesis or PUBS once
greater than the critical titer. Middle cerebral artery (MCA)
Dopplers can be used as a noninvasive measure for anemia in isoim-
munization.
Disease Severity
Delta OD450 (a measure of bilirubin in amniotic fluid) in zone III is
associated with imminent fetal death from erythroblastosis fetalis.
PUBS is used for both diagnosis (hemoglobin and hematocrit and
antigen determination) and treatment (transfusion).
Concept and Application
Maternal hemolytic immunoglobulin G (IgG) antibody formation to
fetal red cell antigens (Rh, Kell, Duffy, Kidd, etc.), extramedullary
hematopoiesis in the fetal liver, decreased oncotic protein produc-
tion and edema, anasarca (erythroblastosis fetalis). Rh-negative
woman has a 15% risk of sensitization in the first exposed Rh-posi-
tive, ABO-compatible pregnancy if not treated with RhoGAM.
Treatment Steps
Initial Care
1. Serial measurement of the indirect Coombs’ and antibody ti-
tering.
2. MCA Dopplers.
3. Amniocentesis or PUBS (diagnostic and therapeutic) is per-
formed once critical titer is reached or MCA Doppler indi-
cates severe anemia.
Emergency Care—PUBS or delivery.
Continued Care—Serial studies and therapy are determined by pre-
vious results.
P. Multiple Gestation
H&P Keys
High index of suspicion when size greater than dates, multiple
heartbeats, positive family history, ovulation induction; 1 of 80 black
women, 1 of 100 white women.
Diagnosis
Ultrasonogram, elevated MS-AFP. See Figure 12–4.
Disease Severity
Serial ultrasonographic evaluation for growth and polyhydramnios.
Concept and Application
Seventy percent of twins are dizygotic, wherein two eggs are fertil-
ized by two sperm. This type of twinning is affected by race, heredity,
age, and parity as well as infertility treatments.
320
OBSTETRICS Complicated Pregnancy
Treatment Steps
Q. Maternal Mortality
H&P Keys
Maternal mortality is defined as the death of a woman from any
pregnancy-related problem during pregnancy or within 42 days of
termination of pregnancy.
Diagnosis
Self-evident.
Disease Severity
Self-evident.
R. Depression
H&P Keys
Mean age 40 years; disturbance of mood, intense anguish, and loss
of a sense of control; predisposing factors include victim of abuse,
childhood loss of a parent, genetic predisposition, deprivation, and
lifestyle stress.
Diagnosis
High index of suspicion: a high score on the Beck Depression Inven-
tory.
Disease Severity
Suicide threats and attempts.
Concept and Application
Most common psychiatric disorder in women.
Treatment Steps
Initial Care
1. Mild to moderate depression is treated with psychotherapy.
2. Severe, chronic, recurrent depression is treated with antide-
pressants and psychotherapy.
Emergency—Hospitalization and psychotherapy for suicidal ideation.
T. Hyperemesis Gravidarum
H&P Keys
Intractable nausea and vomiting associated with significant weight
loss, dehydration, electrolyte imbalance, or ketonemia. This occurs
predominantly prior to 16 weeks of gestation.
322
OBSTETRICS Complicated Pregnancy
d
Diagnosis
management Rule out other causes: gastroesophageal reflux, pancreatitis, hepato-
decisions biliary disease, and other gastrointestinal disorders.
Disease Severity
EVALUATION OF RUPTURE Protracted negative protein balance and ketonemia adversely affect
OF MEMBRANES
fetal growth.
Pooling Concept and Application
Speculum exam reveals Etiology unknown.
large amount of fluid in the
vagina. False-positive Treatment Steps
results can come from Frequent small, bland meals with liquids at separate intervals,
recent intercourse.
antiemetics.
Nitrazine
Amniotic fluid turns Emergency Care
nitrazine paper blue. False- 1. Intravenous hydration.
positive results can result 2. Antiemetics.
from urine, semen, cervical 3. Psychosocial support.
mucus, blood
4. Hyperalimentation is used rarely.
contamination, antiseptic
solution, or vaginitis. False-
U. Abnormalities of Labor, Dystocia
negative results can be
from minimal leakage or H&P Keys
exam remote from the time Patient in labor with abnormal progress based on graphic analysis
of rupture of membranes. (see Table 12–1).
Ferning
The deposition of salt Diagnosis
crystals on a glass slide Graphic analysis of labor, assessment of pelvis and fetal attitude, po-
when the amniotic fluid sition, and presentation.
dries. False-positive can
result from cervical mucus. Disease Severity
False-negative can result Prolonged latent phase: no progress from latent to active phase
from contamination with
(nulligravidas > 20 hours, multiparas > 40 hours). Protracted active
blood or urine or antiseptic
solution or very early
phase dilatation: nulligravidas 1.2 cm/hr, multiparas 1.5 cm/hr. Ar-
gestational age. rest of dilatation: no change in 2 hours. Arrest of descent: no de-
scent in 1 hour.
12-1
ABNORMAL LABOR PATTERNS, DIAGNOSTIC CRITERIA, AND METHODS OF TREATMENT
Diagnostic Criterion
Prolongation disorder
Prolonged latent phase > 20 hr > 14 hr Therapeutic rest Oxytocin or cesarean deliveries for
urgent problems
Expectant and supportive Cesarean delivery for CPD
Protraction disorders
Protracted active phase dilatation < 1.2 cm/hr < 1.5 cm/hr
Protracted descent < 1.0 cm/hr < 2 cm/hr
Arrest disorders
Prolonged deceleration rate > 3 hr > 1 hr Without CPD: oxytocin Rest if exhausted
Secondary arrest of dilatation > 2 hr > 2 hr With CPD: cesarean delivery Cesarean delivery
Arrest of descent > 1 hr > 1 hr
Failure of descent No descent in deceleration
phase or second stage of labor
V. Postpartum Hemorrhage
H&P Keys
Uterine Atony—Predisposing factors: prolonged labor, precipitous
labor, infection, uterine overdistention, multiparity, fibroids, oxy-
tocin augmentation, and magnesium sulfate.
Genital Tract Laceration—Predisposing factors: instrumented vaginal
delivery, precipitous delivery, macrosomic infant, previous geni-
tal tract laceration, and in utero manipulation.
Retained Placental Fragments—From incomplete removal of normal
placenta, retained accessory lobe, or partial placenta accreta.
Diagnosis
Vital signs, CBC, blood product availability, and search for the
source of the bleeding.
Disease Severity
Normal blood loss: singleton, vaginal, 500 mL; twin, vaginal, 1,000
mL; cesarean section, 1,000 mL. Normal parturient can lose 900 mL
without any symptoms; 1,500 mL is associated with tachycardia, nar-
rowed pulse pressure, positive blanch test; 2,000 mL is associated
with hypotension; 2,400 mL is associated with profound hypotension
and vasoconstriction.
Concept and Application
Uterine blood flow is 500–600 mL/min. Interference with the nor-
mal mechanisms of hemostasis may result in significant blood loss.
Treatment Steps
Emergency Care
1. Uterine massage, oxytocin, methylergonovine maleate
(Methergine) or 15-methylprostaglandin f2α.
2. Transfusion if hemodynamically unstable.
3. Determination of etiology of the hemorrhage; definitive treat-
ment.
4. Hysterectomy is last resort.
W. Postpartum Sepsis
H&P Keys
Predisposing factors: cesarean section, prolonged rupture of mem-
branes, chorioamnionitis, prolonged labor, multiple pelvic exams,
internal monitors, obesity, and anemia. Febrile morbidity: tempera-
ture of > 100.4°F on two occasions at least 6 hours apart 24 hours
postpartum.
324
OBSTETRICS Complicated Pregnancy
Diagnosis
Endomyometritis presents with uterine tenderness and fever; cul-
tures are not reliable. Wound infection presents with fever, pain, ten-
derness, erythema, and swelling; wound cultures are helpful.
Pyelonephritis, pneumonia, and atelectasis should be ruled out.
Disease Severity
Evaluate for septic pelvic thrombophlebitis, which presents as persis-
tent fever and tachycardia after presumed effective antibiotic treat-
ment; responds to the addition of heparin.
Concept and Application
Uncontrolled sepsis results in physiologic instability, organ failure,
and death.
Treatment Steps
Y. Diabetes
1. Gestational
H&P Keys
Universal screening or screen if risk factors are present (prior his-
tory, obesity, macrosomia, hydramnios, family history, excessive
weight gain).
Diagnosis
If 50-g glucola screen > 140 mg/dL, then proceed to 3-hour oral glu-
cose tolerance test (GTT); abnormal if any two values exceed fasting
blood sugar (FBS) > 105, > 190 at 1 hour, > 165 at 2 hours, or > 145
at 3 hours.
325
Z. Asthma
H&P Keys
Acute dyspnea, wheezing, cough. One-third become better, one-
third stay the same, one-third experience a worsening of the condi-
tion in pregnancy. Patients usually tolerate labor well.
12-2
PRISCILLA WHITE CLASSIFICATION
on rounds
PROBLEMS IN OBSTETRICS
Preeclampsia
• Hypertension and proteinuria or edema after 20 weeks.
• Diagnosis by blood pressure measurement, 24-hour urine for protein.
• Treatment includes bed rest (if preterm) and delivery (if term). Magnesium sulfate to prevent
seizures.
Eclampsia
• Preeclampsia with tonic–clonic seizures.
• Diagnosis as in preeclampsia.
• Treatment includes stabilization of the mother and then delivery of the fetus.
Abruptio Placenta
• Vaginal bleeding, tender uterus, shock.
• Clinical diagnosis, ultrasound no help.
• Treatment includes hemodynamic stabilization and delivery (with mature fetus).
Placenta Previa
• Painless third-trimester vaginal bleeding, often at night.
• Diagnosis by ultrasound.
• Treatment for asymptomatic cases:
Education
Hematinics
Serial ultrasounds
Coital restriction in third trimester
• Urgent cases: hemodynamic stabilization.
Diagnosis
Peak flow monitoring.
Disease Severity
Accessory muscle usage, respiratory rate, pulse oximetry, arterial
blood gases (ABGs).
Concept and Application
Bronchospasm in response to allergens, antigens, or irritants; infec-
tion.
Treatment Steps
Acute
1. β-Agonist inhalers, parenteral glucocorticoids.
2. Evaluation of fetal well-being.
Continued Care
1. β-Agonist, inhaled glucocorticoids, cromolyn.
2. Peak flow monitoring.
BIBLIOGRAPHY
ACOG. Compendium of Selected Publications. Washington, DC: American College of Ob-
stetricians and Gynecologists, 2005.
Cunningham FG. Williams Obstetrics, 22nd ed. Stamford, CT: Appleton & Lange, 2005.
327
329
Copyright © 2004, 2001 by The McGraw-Hill Companies, Inc. Click here for terms of use.
330 Ophthalmology
BIBLIOGRAPHY / 354
331
OPHTHALMOLOGY Conjunctivitis
U
I. CONJUNCTIVITIS
Hyperemia of conjunctival blood vessels. Types: allergic, bacterial, vi-
ral; common, often not serious. cram facts
H&P Keys
Red eye, usually without blurred vision, pain, or colored halos. Exu- RED EYE
dation is more severe in bacterial, moderate in viral, and least in al-
lergic (watery). Itching is pronounced in allergic. Ciliary flush is ab- Uveitis
sent. Conjunctival injection is prominent in bacterial, moderate in Eye pain, blurred vision,
viral, and least in allergic. Corneal disturbance may be present in vi- injected conjunctiva,
ral, absent in bacterial and allergic. Pupil, anterior chamber depth, photophobia, diagnosis on
and intraocular pressure are normal. Preauricular lymph node may slit lamp showing “cells and
be present in viral but absent in bacterial and allergic (Tables 13–1, flare” (protein and white
blood cells [WBCs] in
13–2).
aqueous humor).
Diagnosis
Correct diagnosis for cause of red eye determines effectiveness of Keratitis
Severe pain, photophobia,
treatment and reduces complications. Aside from conjunctivitis,
diagnosis with fluorescein
other causes of red eye must be ruled out. Differentiate from acute stain showing multiple
glaucoma, acute iridocyclitis, and keratitis corneal lesions (Tables punctate lesions.
13–3, 13–4, 13–5).
Most cases are managed without laboratory studies. Smear of ex- Glaucoma
udates. Conjunctival scrapings for culture and sensitivity studies. Eye pain, nausea, vomiting,
1. Allergic conjunctivitis: eosinophils. headache, hazy cornea
2. Bacterial conjunctivitis: polymorphonuclear cells and bacteria. diagnosis, pupil not
reacting, increased ocular
3. Viral conjunctivitis: lymphocytes.
pressure.
Disease Severity
Viral Conjunctivitis
Assess vision, pupils, amount of pain, and sensitivity to light. Rule
Profuse water discharge.
out red eye caused by acute glaucoma, corneal lesions, or iridocycli-
tis. Allergic Conjunctivitis
Allergic Conjunctivitis—Itching, watery discharge, chemosis, history Itchy, bilateral.
of allergies, edematous lids, and no preauricular nodes.
Bacterial Conjunctivitis
Bacterial Conjunctivitis—Severe purulent discharge; may have sub- Purulent discharge.
conjunctival hemorrhage. Red eye subconjunctival hemorrhage.
Gram stain for causative bacteria.
Viral Conjunctivitis—History of recent upper respiratory tract infec-
tion or contact with someone 7–10 days prior with a red eye. Usu-
ally gets worse the first few days after onset and may last for 2–3
weeks. Watery or mucous discharge, red and edematous eyelids.
Eyelid sticking, worse in the morning. May have subconjunctival
13-1
SYMPTOMS OF CONJUNCTIVITIS
Exudation +++ ++ +
Itching 0 0 ++
Blurred vision 0 0 0
Colored halos 0 0 0
Pain 0 0 0
Photophobia 0 0 0
13-2
SIGNS OF CONJUNCTIVITIS
d
Pupil N N N
Anterior chamber depth N N N
management Intraocular pressure N N N
decisions N, normal; +, present; 0, absent.
Viral Conjunctivitis
1. Usually subsides after its natural course. It is very contagious
about 5 days after onset. Patients with red and weeping eyes
should avoid spreading to other people.
2. Isolation from group gatherings.
3. Artificial tears and cold compresses as often as needed for
comfort.
4. Frequent hand washing.
A. Blepharitis
Chronic bilateral inflammation of the lid margins.
H&P Keys
Itching, irritation, burning of lid margins, red rims, scales of lashes,
ulcerated areas along lid margins.
Diagnosis
Lid margin ulceration or no ulceration, scales oily or dry.
Disease Severity
Check scalp, brows, ears for seborrhea. Smear and stain of scraping
from lid margins. Stain cornea. Evaluate for ulceration.
13-5
ASSOCIATED SYSTEMIC DISEASES IN CONJUNCTIVITIS
Pharyngoconjunctival fever
(adenovirus type 3, type 7) 0 + 0
Seasonal rhinitis of hay fever 0 0 +
Erythema multiforme
(Stevens–Johnson syndrome) 0 0 +
+, present; 0, absent.
334
OPHTHALMOLOGY Disorders of the Eyelids
13-6
MANAGEMENT OF CONJUNCTIVITIS
+, of some benefit; ++, moderate benefit; +++, treatment of choice; ±, may or may not help; 0, no benefit; NSAIDs, nonsteroidal anti-
inflammatory drugs.
Treatment Steps
B. Stye
External hordeolum (glands of Zeis or Moll, hair follicles).
H&P Keys
Recent onset; localized red, swollen, tender area of the lid, fre-
quently preceded by diffuse edema of the lid.
Diagnosis
Red, swollen gland with pore opening can be seen at the lid margin.
Tender area can be identified with a cotton tip.
Disease Severity
Pain is related to the amount of swelling. Pus tends to point to the
skin surface of the lid margin.
Treatment Steps
1. Warm compresses over affected lids 10 minutes three times a day
after application of antibiotic ointment.
2. Incision and drainage of pus when needed.
335
D. Entropion
Turning inward of the lid margin, usually affects the lower lid.
H&P Keys
Tearing, irritation of the cornea. Eyelashes turn inward, touching
cornea.
Diagnosis
Check the lid margin, position of the eyelashes, and the integrity of
the cornea. Early detection of corneal ulcer.
Disease Severity
Entropion can cause trichiasis, the turning inward of the lashes so
that they rub on the cornea. Irritation of the cornea can predispose
to corneal ulcer.
Concept and Application
Senile type caused by degeneration of fascial attachments in the
lower lid. Cicatricial type caused by scarring of the palpebral conjunc-
tiva and the tarsus. Common in trachoma, Stevens–Johnson syn-
drome, chemical burns, and trauma.
Treatment Steps
1. Temporary taping to evert lid.
2. Corrective surgery.
E. Ectropion
Turning outward of lower lid.
H&P Keys
Tearing, irritation. Sagging and eversion of lid margin.
336
OPHTHALMOLOGY Disorders of the Eyelids
Diagnosis
Test closure of lids and integrity of cornea.
Disease Severity
Exposure keratitis. Fluorescein stain for corneal integrity.
Concept and Application
Bilateral. Older persons. Relaxation of orbicularis oculi. Present in
aging and seventh cranial nerve palsy.
Treatment Steps
1. Protection of cornea.
2. Lubrication with artificial tears or ointment.
3. Surgical correction of deformed lid.
F. Epicanthus
Wide nasal folds.
H&P Keys
Vertical folds of skin over medial canthi; in Asians and most children
of all races.
Diagnosis
Corneal light reflex test is normal. So-called esotropia, or turning in
of the eye, appears present on side gaze.
Disease Severity
Prominent epicanthal folds in children becomes less obvious as
child grows older.
Concept and Application
The large skin fold covers the nasal sclera and causes pseudo-
esotropia. Frequent concern as strabismus. A common cause for re-
ferral.
Treatment Steps
Explanation and understanding. No treatment needed.
G. Blepharoptosis
Droopy eyelids.
H&P Keys
May be noted at birth; congenital or acquired. Drooping of upper
lids when both eyes are open. Unilateral or bilateral. Constant or in-
termittent.
Diagnosis
Determine amount of movement of upper lid and severity in block-
ing of vision. Assess cosmesis and skin position.
Disease Severity
Degree of upper lid movement and occlusion of pupillary axis for vi-
sion. Concern with visual development in children. May cause am-
blyopia.
Concept and Application
Treatment Steps
1. Conservative: no cosmetic or visual acuity disturbance.
2. Myasthenia gravis: neostigmine.
3. Special spectacle frames.
4. Surgery for improvement of vision or cosmesis: frontalis sling for
no action of levator, to lift upper lid; levator strengthening for
partial weakness of levator.
A. Undersecretion
Dry-eye syndrome, Sjögren’s syndrome.
1. Dry-Eye Syndrome (Keratoconjunctivitis Sicca)
H&P Keys
Dry, irritative conjunctiva. Dry, sore mouth. Foreign-body sensation,
scratchy and sandy feeling of the eyes. Itchy, burning, photosensitiv-
ity, excessive mucus, redness, pain, dry lids. Grossly, normal-looking
eyes.
Diagnosis
Dry undersecretion is confirmed by Schirmer’s test with strip of blot-
ting paper (4 mm × 30 mm). The strip is inserted onto the lower lid
margin so that the strip protrudes forward from the eye. The rate of
lacrimation is measured. By the end of 5 minutes the strip should be
moistened for at least 15 mm in a normal response. In Sjögren’s syn-
drome, the moistening rarely extends beyond 5 mm along the strip,
suggesting inadequate tear production.
Disease Severity
Undersecretion of tears. Evaluation studies for corneal epithelium
and conjunctival defects with vital stain such as rose bengal (1%).
The affected areas will be colored bright red. Collagen-vascular
diseases, conjunctival scarring, drugs, and vitamin A deficiency
should be investigated, if not yet diagnosed.
Treatment Steps
1. Search for cause.
2. Artificial tears, methylcellulose are used frequently.
338
OPHTHALMOLOGY Disorders of the Optic Nerve
B. Acute Dacryocystitis
H&P Keys
Infection of lacrimal sac. Common acute or chronic in infants or in
patients over 40. Tearing and discharge. Swelling lump, redness,
pain, tenderness over tear sac area. May have fever.
Diagnosis
Persistent tearing. Purulent material can be expressed from tear sac.
Nasolacrimal duct is blocked. Staining of conjunctival smear to iden-
tify infectious organisms.
Disease Severity
Tearing in mild cases. Purulent discharge in moderate to severe
blockage of nasolacrimal ducts.
Treatment Steps
In children, forceful massage of the tear sac is tried initially. Irriga-
tion and probings of nasolacrimal duct are usually effective in
adults.
Acute
1. Warm compresses.
2. Appropriate antibiotics.
3. Incision and drainage if necessary.
Chronic—Surgical removal of obstruction of nasolacrimal duct
(dacrocystorhinostomy).
H&P Keys
No symptoms, blurred disc margin, elevated disc substance, obliter-
ated cup, no edema, no hemorrhage.
Diagnosis
Dilated ophthalmoscopy; document with optic disc photography,
pseudopapilledema; visual field, intravenous fluorescein angiogra-
phy (Tables 13–7, 13–8).
Disease Severity
Benign, nonprogressive; rule out true papilledema.
Treatment Steps
Detection and follow-up through serial examinations to monitor
possible progression.
339
Etiology Acute swelling of optic disc Acute blurred disc margin Blurred disc
• Increased intracranial pressure • Ischemia • Congenital, developmental anomalies
• Brain tumor • Inflammation • Hyperopia
• Hypertension • Multiple sclerosis
• Pseudotumor cerebri
Acute loss of vision + +++ (Severe) 0
Headaches +++ 0/+ 0
Retrobulbar pain on
eye movement 0 +/+++ 0
2. Papilledema
Swelling of optic disc caused by increased intracranial pressure (Fig.
13–1).
H&P Keys
Symptoms of brain tumor, hyperemia of optic disc, tortuosity of
veins and capillaries, blurring and elevation of disc margin, hemor-
rhages on and surrounding nerve head.
Diagnosis
Studies for brain lesions, computed tomographic (CT) scan, mag-
netic resonance imaging (MRI), magnetic resonance angiography
(MRA), intravenous fluorescein angiography.
Disease Severity
Brain tumor, pseudotumor cerebri, severe systemic hypertension.
Concept and Application
Swelling of optic disc secondary to increased intracranial pressure,
brain tumor 50%.
Treatment Steps
Detection and referral for evaluation by neurologists or neurosur-
geons.
3. Papillitis
Anterior optic neuritis. Inflammatory edema of the optic nerve head
visible by ophthalmoscope.
13-8
SIGNS OF BLURRED OPTIC NERVE HEAD
H&P Keys
Sudden loss of central vision, usually in one eye. Pain behind the
eyeball on movement of the affected eye. Reduced vision. Swinging
light test for relative afferent pupillary defect.
Diagnosis
Ophthalmoscopy, visual field test for central scotoma, CT of orbits
and chiasm, MRI and MRA of the brain.
Disease Severity
Search for multiple sclerosis, Lyme disease, and neurosyphilis. Rule
out compressive lesions of optic nerve and chiasm.
Concept and Application
Inflammation of optic nerve. Idiopathic. Associated with multiple
sclerosis, Lyme disease, and neurosyphilis. Prognosis for return vi-
341
Treatment Steps
1. IV corticosteroid versus no treatment for acute phase.
2. Oral prednisone treatment is contraindicated in the treatment of
idiopathic optic neuritis.
Diagnosis
Vision, pupillary reaction to light, swinging light test, ophthal-
moscopy; compare both discs for asymmetry of color, cupping, fine-
vessels pattern.
Disease Severity
Intraocular pressure for glaucoma, orbital mass compression of
nerve. Intravenous fluorescein angiography of disc.
Treatment Steps
Detection and referral for investigation and treatment.
H&P Keys
Loss of central vision in one eye. Reduction of visual acuity (Fig.
13–2).
Diagnosis
Visual acuity testing, pupillary reaction to light, swinging light test
for relative afferent pupillary defect, ophthalmoscopy, visual field
testing, red-color appreciation, Amsler grid.
Disease Severity
Ophthalmoscopy for macular and optic nerve diseases. Rule out reti-
nal detachments, vascular occlusions, such as central retinal artery
occlusion, branch retinal artery occlusion, central retinal vein occlu-
sion. Intravenous fluorescein angiography.
Figure 13–2. Visual pathways with associated field defects. (Reproduced, with permission, from Schwartz SH.
Visual Perception, 2nd ed. New York: McGraw-Hill, 1999).
B. Bitemporal Defects
At the chiasm.
H&P Keys
Visual field defect affects both eyes, bitemporal hemianopsia, side vi-
sion defect of both eyes (see Fig. 13–2). Confrontation testing with
red object.
Diagnosis
Vision, visual fields testing, CT scan, MRI/MRA.
Disease Severity
CT scan. Check out enlarged sella turcica for pituitary tumor.
Concept and Application
Bitemporal visual field defects means chiasmal lesions; suspect pitu-
itary tumor.
Treatment Steps
Detection and referral.
C. Homonymous Defects
Posterior to chiasm.
H&P Keys
Loss of visual fields of both eyes on the same side (see Fig. 13–2). Vi-
sual field defects on the right or left side. Confrontation testing with
red object.
Diagnosis
Visual field testing, intracranial studies, CT scan, MRI.
343
OPHTHALMOLOGY Amblyopia
Disease Severity
Check out neurologic symptoms and signs for brain tumor and cere-
bral vascular lesions, stroke.
management decisions d
Amblyopia Glasses
Occlusion therapy
Surgery for strabismus
Diabetic retinopathy Retinal laser treatment for clinically significant macular edema and/or
proliferative retinopathy
Vitrectomy
344
OPHTHALMOLOGY Strabismus
13-9
PROGRESSION OF DIABETIC RETINOPATHY
Nonproliferative retinopathy
Microaneurysms
Hemorrhages (dot and blot, flame)
Hard exudates
Retinal edema
Preproliferative retinopathy
Retinal nerve fiber layer infarcts (cotton-wool patches)
Venous dilation, loops, and beading (irregularities of the vein caliber)
Telangiectasias (intraretinal microvascular abnormalities)
Proliferative diabetic retinopathy
Neovascularization of the retina, optic disc, or vitreous
Preretinal and vitreous hemorrhages
Fibrous proliferation
Tractional retinal detachment
VII. STRABISMUS
Misalignment of two eyes so that both eyes cannot be directed to-
ward the object of fixation (Fig. 13–3).
H&P Keys
Family history, head trauma, systemic disease, neurologic disorder.
Figure 13–3. (A) Right esotropia. (B) Right exotropia. (Reproduced, with permission, from Vaughan DG [ed.]. General Ophthalmology, 15th ed. Stamford, CT: Apple-
ton & Lange, 1999).
345
Comitant Noncomitant
(Nonparalytic) (Paralytic)
Strabismus Strabismus
Diagnosis
General inspection, corneal light reflex, cover test, dilated ophthal-
moscopy.
Disease Severity
Check for intraocular lesions, cataract, retinoblastoma, other retinal
abnormalities. Examine for neurologic disorder.
Concept and Application
Early detection (Tables 13–10, 13–11). Delayed diagnosis affects vi-
sion, eye, and systemic problems.
Treatment Steps
1. Detection early to allow diagnosis and treatment.
2. Search for serious organic conditions that cause strabismus.
13-11
TYPES OF IMBALANCE OF THE TWO EYES
Horizontal
Inward turning Esotropia Esophoria
Outward turning Exotropia Exophoria
Vertical
Upward turning Hypertropia Hyperphoria
Downward turning Hypotropia Hypophoria
346
OPHTHALMOLOGY Diabetes with Ophthalmologic Manifestations
Diagnosis
Direct ophthalmoscopy detects most ophthalmic clinical manifesta-
tions of diabetes, including retinopathy, optic neuropathy, blockage
of the red reflex by cataract (Fig. 13–4). Indirect ophthalmoscopy:
wider, stereoscopic view of retina. Biomicroscopy with a slit lamp:
useful in evaluating for diabetic changes in anterior segment includ-
ing cataracts and iris neovascularization. Pupillary dilation for easier,
more thorough examination of retina and lens. Ultrasonography for
internal structure (e.g., tractional retinal detachment), when blood,
debris, or membranes impede direct visualization of retina.
Disease Severity
Biomicroscopy with a contact lens system provides stereoscopic, de-
tailed view of structures of retina; especially valuable in diagnosis of
macular edema. Fluorescein angiography to assess extent of disease,
such as leakage from abnormal vessels in suspected neovasculariza-
tion, and in guiding laser photocoagulation treatment of clinically
evident macular edema by revealing characteristic lesions and patho-
logic processes of retinopathy, e.g., microaneurysms, capillary leak-
age, and nonperfusion areas. Images of retina.
Concept and Application
Cellular edema, pericyte destruction, endothelial damage and dys-
function from increased intracellular sorbitol and hypoxia lead to
capillary hyperpermeability. Abnormal retinal vascular permeability
leads to macular edema, which threatens vision and may be an indi-
cation for laser therapy. Increased hemoglobin A1c oxygen affinity
impairs oxygen release, resulting in retinal hypoxia. Basement mem-
brane thickening and increased platelet and red blood cell aggrega-
bility predispose to regions of microvascular occlusions, retinal
hypoxia, and release of vasogenic factors with subsequent neovascu-
larization of the retina, optic nerve, or iris. Widespread panretinal
laser photocoagulation reduces release of vasogenic stimuli and there-
fore neovascularization.
Treatment Steps
1. Tight medical control with long-term near-normalization of
blood sugar level slows the progression of diabetic retinopathy.
Aggressive control of blood pressure to 130/80 or better.
A. Corneal Ulcers
H&P Keys
Pain, photophobia, blepharospasm, lacrimation, discharge, and de-
creased vision often present. The lesion begins as a dull, grayish, cir-
cumscribed, superficial infiltration of the cornea that subsequently
ulcerates. Conjunctival and limbal injection is usual and blood ves-
sels may grow in from the limbus in long-standing cases (pannus).
Pus may appear in the anterior chamber (hypopyon). Corneal per-
foration with iris prolapse may occur.
Diagnosis
The ulcer stains green with fluorescein. Bacterial and fungal cul-
tures from corneal scrapings should be obtained prior to starting an-
tibiotic treatment.
Disease Severity
Slit-lamp biomicroscopy may be used to determine the extent of
corneal thinning and infiltration and the presence of hypopyon.
Concept and Application
Bacterial or fungal infection following trauma, corneal foreign body,
contact lens wear, or previous corneal disease. Assume bacterial until
proven otherwise.
Treatment Steps
1. Cycloplegia (scopalamine), broad-spectrum or fortified topical
antibiotics, subconjunctival antibiotics.
13-12
DISEASES OF THE GLOBE
A. Cornea
1. Ulcers
2. Herpes simplex keratitis
3. Ophthalmic herpes zoster
4. Dry eye (keratoconjunctivitis sicca)
5. Interstitial keratitis
B. Sclera
1. Scleritis
C. Uvea
1. Uveitis
a. Anterior
b. Posterior
D. Retina
1. Retinal detachment
2. Vascular occlusions
a. Arteriolar
b. Venous
3. Hypertensive retinopathy
4. Retinitis pigmentosa
5. Age-related macular degeneration
E. Penetrating injuries
348
OPHTHALMOLOGY Disorders of the Cornea
D. Interstitial Keratitis
H&P Keys
Acute: pain, tearing, photophobia, red eye. Corneal stromal blood
vessels, corneal edema, anterior uveitis. Old disease: deep corneal
scarring and haze, corneal stromal thinning, and ghost vessels. Asso-
ciated with maternal venereal disease, saddle nose, frontal bossing,
Hutchinson’s teeth, chorioretinitis, optic atrophy (congenital
syphilis); hearing deficit, tinnitus, vertigo, polyarteritis nodosa (Co-
gan’s syndrome); hypopigmented or anesthetic skin lesions, loss of
temporal eyebrow or eyelashes, thickened corneal nerves, iris nod-
ules (leprosy); tuberculosis.
Diagnosis
Slit-lamp and dilated fundus examination. Rapid plasma reagin
(RPR), fluorescent treponemal antibody absorption (FTA-ABS), pu-
rified protein derivative (PPD) (tuberculin) tests with anergy panel,
chest x-ray, sedimentation rate.
Disease Severity
Slit-lamp examination for corneal edema, scarring or thinning, or
uveitis.
Concept and Application
Chronic nonulcerative infiltration of the deep layers of the cornea
with uveal inflammation.
Treatment Steps
1. Acute corneal involvement: topical cycloplegia, topical steroids,
treatment of underlying disease.
2. Corneal transplant surgery for central corneal scarring with im-
paired vision.
A. Glaucoma
H&P Keys
Disease Severity
The severity of disease is based on the clinical appearance of the op-
tic nerve and on the visual field. Response to treatment is evaluated
with regard to relationship of lowering of intraocular pressure to sta-
bility of changes in the optic nerve appearance and visual field (Fig.
13–5).
management decisions d
GLAUCOMA
Open-Angle Glaucoma
1. β-Adrenergic antagonists (e.g., timolol), topical carbonic an-
hydrase inhibitors (e.g., dorzolamide, brinzolamide), and
adrenergic agonists (e.g., epinephrine) decrease aqueous pro-
duction.
α-Adrenergic agonists (e.g., brimonidine, clonidine, apra-
clonidine), prostaglandin analogs (e.g., latanoprost, travaprost),
and miotics (e.g., pilocarpine) reduce outflow resistance.
2. Laser trabecular burns promote aqueous outflow.
3. Surgical filtration procedures promote aqueous outflow.
Angle-Closure Glaucoma
1. Acutely treat with topical β-blockers, topical pilocarpine, oral
carbonic anhydrase inhibitors, and osmotic agents such as
oral glycerin or intravenous mannitol.
2. A laser or surgical peripheral iridectomy must be performed
for definitive treatment of the anatomic problem.
Figure 13–5. (A) Normal optic disc. (Photo by Diane Beeston.) (B) Glaucomatous optic disc. (Reproduced, with permission, from Vaughan DG [ed.]. General Ophthal-
mology, 15th ed. Stamford, CT: Appleton & Lange, 1999).
352
OPHTHALMOLOGY Disorders of the Lens
B. Cataract
H&P Keys
A cataract is a lens opacity. Complaints of painless blurred vision,
glare, increasing nearsightedness, or monocular double vision. His-
tory of previous eye conditions, injury, surgery, or concurrent dis-
eases may suggest cause of cataract. Assessment of visual acuity with
optical correction by means of Snellen letter chart or a number
chart, or picture charts or ability to fixate on and follow or reach for
a moving object for young children or adults with severe mental dis-
ability. Preferential viewing techniques may estimate visual acuity in
infants. The lens is best examined for cataracts with a dilated pupil.
The simplest way is with a handheld direct ophthalmoscope showing
dark lenticular opacities blocking the normal red reflex of the
retina. The slit-lamp biomicroscope is routinely used to detect the
location and density of any opacity within the lens.
Diagnosis
Potential visual acuity that might be expected with removal of the
cataract may be estimated by the use of a potential acuity meter
(PAM) or a laser interferometer. Both instruments are based on the
ability of a patient to resolve letters or lines projected onto the
retina.
When significant lens opacification prevents direct retinal exami-
nation, B-scan ultrasonography may be used to detect severe abnor-
malities of the retina, such as retinal detachment or tumors.
Disease Severity
The clinical degree of cataract formation, assuming that no other
eye disease is present, is judged primarily by visual acuity. With direct
ophthalmoscopy of the posterior pole through the cataract, the ocu-
lar fundus becomes increasingly more difficult to visualize as the
lens opacity becomes denser. Slit-lamp biomicroscopy allows judg-
ment of lens clarity and location of the cataract. Glare testing and
contrast sensitivity testing are new methods for quantitatively esti-
mating the functional impact a cataract has on vision.
13-13
CLASSIFICATION OF CATARACTS
A. Age related
1. Nuclear sclerotic
2. Posterior subcapsular
3. Cortical
4. Mature
5. Morgagnian and hypermature
B. Congenital
C. Juvenile
1. Inborn errors of metabolism
2. Chromosomal abnormalities
D. Secondary
1. Traumatic or physical damage
2. Associated with intraocular disease
a. Chronic or recurrent uveitis
b. Retinitis pigmentosa
c. Retinal detachment or tumors
3. Associated with systemic disease (e.g., hypoparathyroidism, Down syndrome,
diabetes mellitus)
4. Toxic
a. Steriods
b. Miotic agents
c. Intraocular metals: copper or iron
353
BIBLIOGRAPHY
Foundation of the American Academy of Ophthalmology. Ophthalmology Monographs.
Italy: 1999.
Kanski JS. Clinical Ophthalmology: A Systematic Approach, 5th ed. Boston:
Butterworth–Heinemann Medical, 2003.
Spalton DJ, Hitchings RA, Hunter PA. Atlas of Clinical Ophthalmology, 2nd ed. London:
Wolfe Publishing, 1994.
Vaughan D. General Ophthalmology, 16th ed. Stamford, CT: Appleton & Lange, 2003.
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Pediatrics 14
I. NEUROLOGY / 357
A. Febrile Seizures / 357
B. Infantile Botulism / 357
C. Neural Tube Defects (NTDs) / 358
D. Malformations / 359
355
Copyright © 2004, 2001 by The McGraw-Hill Companies, Inc. Click here for terms of use.
356 Pediatrics
F. Pharyngitis / 370
G. Bacterial Meningitis / 370
H. Congenital Infections (STORCH) / 371
I. Hemolytic–Uremic Syndrome / 373
BIBLIOGRAPHY / 381
357
PEDIATRICS Neurology
I. NEUROLOGY
A. Febrile Seizures
H&P Keys
Occurs in children 6 months to 6 years of age. Signs and symptoms
include generalized, tonic, atonic, tonic–clonic, or focal seizures and
fever. Risk factors include positive family history, developmental de-
lay, day care, prematurity, perinatal maternal smoking.
Diagnosis
Diagnosis of exclusion based on history and physical exam. Evaluate
for source of infection or metabolic imbalance. May include lumbar
puncture (LP), blood and urine cultures, electrolytes, glucose, cal-
cium, and complete blood count (CBC) based on clinical presenta-
tion.
Disease Severity
B. Infantile Botulism
H&P Keys
Caused by the toxin of Clostridium botulinum. C. botulinum spores can
be found in soil, dust, lakes, and contaminated food products, in-
cluding honey. Signs and symptoms include weakness, poor feeding,
weak cry, constipation, respiratory failure, symmetric descending
flaccid paralysis, loss of a gag reflex, and ptosis.
Diagnosis
Stool culture for C. botulinum and toxin, electrophysiologic studies.
Disease Severity
Neurologic changes, pulmonary function tests, arterial blood gases
(ABGs).
Treatment Steps
1. Supportive care.
2. Antitoxin (neutralizes circulating toxin before it binds to nerve
endings). Must be given expediently.
3. No antibiotics. Aminoglycosides contraindicated.
358
PEDIATRICS Neurology
Diagnosis
Prenatal ultrasonography, maternal α-fetoprotein (AFP), clinical
exam, magnetic resonance imaging (MRI), and computed tomo-
graphic (CT) scanning.
Disease Severity
Asymptomatic or disturbances of bowel, bladder, motor function.
Degrees of severity include: spina bifida occulta (incomplete closure
of the posterior lumbosacral spinal cord) (see Fig. 14–1), meningo-
cele (herniation of the meninges through the spinal canal defect
without neural tissue), encephalocele (herniation of the meninges
and brain substance through a skull defect), myelomeningocele (se-
vere spinal dysraphism with herniation of the meninges and spinal
cord), anencephaly (congenital absence of the cerebral hemisphere
and cranial vault). More than 75% of myelomeningoceles are associ-
ated with hydrocephalus (usually related to an Arnold–Chiari mal-
formation). Majority have normal intelligence.
Treatment Steps
Multidisciplinary approach.
PEDIATRICS Neurology
D. Malformations
1. Hydrocephalus
H&P Keys
A congenital or acquired condition resulting from impaired circula-
tion, absorption, or overproduction of cerebrospinal fluid (CSF).
Can cause increased intracranial pressure. Signs and symptoms in-
clude headaches, vomiting, nausea, irritability, lethargy, third and
sixth cranial nerve palsies, papilledema, bulging anterior fontanelle,
rapid increase in head circumference, setting-sun sign, long-tract
signs, vital sign changes.
Diagnosis
Clinical exam, CT scan. Avoid LPs (risk of herniation).
Disease Severity
Evidence of herniation: Cushing’s triad (bradycardia, hypertension,
and irregular breathing), vomiting, change of mental status.
Treatment Steps
Placement of a ventriculoperitoneal (VP) shunt if needed.
2. Congenital Glaucoma
H&P Keys
Incidence of about 1 in 100,000 births; 30% are unilateral. Signs and
symptoms include photophobia, tearing, blepharospasm, and cloud-
ing or enlargement of the cornea.
Diagnosis
Intraocular pressure measurements. Clinical exam.
Disease Severity
Blindness if not treated promptly.
Treatment Steps
Surgery.
360
PEDIATRICS Rheumatology
3. Congenital Cataracts
H&P Keys
Unilateral or bilateral opacification of the lens. Signs and symptoms
include decreased visual attentiveness, light sensitivity, decreased vi-
sion, and opacification of the lens.
Diagnosis
Ophthalmologic exam, ocular ultrasonography. Rule out associated
systemic conditions.
Disease Severity
Varies by the degree of opacification. Blindness, amblyopia.
Concept and Application
Often idiopathic but may have an autosomal dominant inheritance
pattern. May occur as part of a systemic disease including intrauter-
ine infections (rubella, cytomegalovirus [CMV]), metabolic diseases
(galactosemia), syndromes (Marfan, Alport’s), or be associated with
chromosomal disorders (trisomies).
Treatment Steps
1. Surgical removal of lens.
2. Evaluate for associated underlying problems.
II. RHEUMATOLOGY
A. Henoch-Schönlein Purpura
H&P Keys
Small-vessel vasculitis occurring in children ages 1–7 years. Male-to-
female ratio 2:1. Signs and symptoms include abdominal pain, rash,
vomiting, hematemesis, and joint pain. Purpura, most commonly
found on the buttocks and lower extremities, is the first sign in
> 50% of cases. Patients may also have fever, gastrointestinal (GI)
bleeding, intussusception (3%), and hematuria.
Diagnosis
No pathognomonic laboratory tests. Normal platelet count and co-
agulation studies. Mild increase in white blood cell count (WBC)
and erythrocyte sedimentation rate (ESR). Elevation of serum im-
munoglobulins A (IgA) and M (IgM) in 50% of cases. Skin lesions
show leukocytoclastic vasculitis.
Disease Severity
Morbidity and mortality related to the extent of GI or kidney in-
volvement. May lead to GI hemorrhage, intussusception, or end-
stage renal disease.
Treatment Steps
1. Supportive care.
2. Corticosteroids (if GI hemorrhage).
3. Anti-inflammatory agents if needed for arthritis.
4. Urinalysis (UA) to evaluate for hematuria.
361
A. Hypospadias
H&P Keys
Urethral meatus located proximal to its normal position at the tip of
the glans (any point along the course of the anterior urethra from
the perineum to the tip of the glans). Abnormal urinary stream.
Diagnosis
Clinical presentation.
Disease Severity
Dependent on the location.
Concept and Application
Failure of the urethral folds to fuse completely over the urethral
groove.
Treatment Steps
Surgical correction. Avoid circumcision (foreskin used in repair).
C. Vesicoureteral Reflux
H&P Keys
Often asymptomatic. May develop features of urinary tract infection
(fever, vomiting, flank pain, dysuria, frequency, urgency, irritabil-
ity).
Diagnosis
Voiding cystourethrogram.
Disease Severity
Renal scarring, pyelonephritis.
Concept and Application
Abnormal backflow of urine from the bladder into the ureters or
kidneys. Increased incidence of reflux in siblings.
Treatment Steps
Management depends on grade of reflux and the amount of renal
scarring.
1. Medical management includes treatment of the urinary tract in-
fection and prophylactic antibiotics.
2. Surgical correction of the anatomic defect if severe reflux or scar-
ring.
PEDIATRICS Neonatology
cram facts
Involvement Inherited
Multicystic kidney disease Unilateral No
Polycystic kidney disease Bilateral Yes
Treatment Steps
1. Evaluation of renal function.
2. Monitoring.
3. Kidney not routinely removed.
F. Undescended Testis
H&P Keys
Nonpalpable testis.
Diagnosis
Clinical exam, ultrasonography (location of testis).
Disease Severity
More prone to testicular torsion, malignant degeneration, and im-
paired fertility.
Concept and Application
Result of mechanical hindrance, abnormal epididymal develop-
ment, or endocrine dysfunction during fetal development.
Treatment Steps
1. Surgical orchiopexy before 2 years of age.
2. Consider hormonal therapy.
IV. NEONATOLOGY
Diagnosis
CXR shows a fine reticular granularity of the lung fields and air
bronchograms (Fig. 14–2). Must differentiate from other causes of
respiratory distress in newborns (sepsis, heart disease, CNS disor-
ders, lung anomalies).
Disease Severity
ABGs, pulse oximetry, hemoglobin, metabolic disturbances, cardio-
vascular compromise. Long-term complications include bronchopul-
monary dysplasia (BPD), cor pulmonale, retinopathy of prematurity,
poor growth, and persistent patent ductus arteriosus.
Treatment Steps
1. Prevention of prematurity.
2. Maternal steroid administration 48–72 hours prior to delivery to
stimulate fetal surfactant production.
3. Neonatal surfactant via endotracheal tube at delivery for prema-
ture infants (surfactant serves to reduce surface tension of the
alveoli).
PEDIATRICS Neonatology
4. Supportive care (correction of hypoxia, acidosis, and hypercap-
nia).
C. Meconium Aspiration
H&P Keys
Presence of meconium in the amniotic fluid and the newborn’s tra-
chea. Signs and symptoms include retractions, increased respiratory
rate, cyanosis, and hypoxia.
Diagnosis
Clinical presentation, CXR.
Disease Severity
Severe respiratory distress with increased respiratory rate, retrac-
tions, and hypoxia requiring ventilatory support; pneumothorax;
pulmonary hypertension.
Concept and Application
Aspiration of meconium-contaminated amniotic fluid leading to air-
way obstruction and poor gas exchange.
Treatment Steps
1. Suctioning the infant on the perineum.
2. Direct visualization and suctioning of the trachea in distressed in-
fants.
3. ABCs.
4. Chest physiotherapy.
5. Supplemental oxygen.
6. Mechanical ventilation if needed.
D. Rh Incompatibility
H&P Keys
Infant of a gravida 2 Rh-negative mother. Signs and symptoms in-
clude pallor, respiratory distress, cardiomegaly, edema.
Diagnosis
Rh type, ABO group, Coombs’ test (positive), hemoglobin, blood smear
(hemolysis), reticulocyte count (increased), and bilirubin (increased).
Disease Severity
Hydrops fetalis; severe anemia leading to heart failure, ascites, pleural
and pericardial effusions; thrombocytopenia; organomegaly; elevated
bilirubin leading to kernicterus (staining of the basal ganglia).
cram facts U
EMESIS IN CHILDREN
E. Neonatal Hyperbilirubinemia
H&P Keys
Signs and symptoms depend on the etiology. Ascertain a maternal
history (blood type, race, illnesses during pregnancy, drug usage,
family history of anemia), and an infant history (birth weight, onset
of jaundice, stooling pattern, trauma, feeding pattern [breast-feed-
ing], emesis).
Diagnosis
Total and direct bilirubin (normal total levels rise to a mean of 6.5 ±
2.5 mg/dL in a full-term formula-fed infant). Hemoglobin, smear,
reticulocyte count, maternal and infant blood type, direct Coombs’
(evidence of hemolysis), albumin level; urine Clinitest; culture if
sepsis is suspected.
Disease Severity
Elevated bilirubin in the first 24 hours of life is pathologic. Ker-
nicterus (bilirubin staining and necrosis of neurons in the basal gan-
glion) especially with hemolytic disease.
Concept and Application
d
Physiologic jaundice is found in 60% of newborns with maximum
management values reached in the second to fourth day (6–8 mg/dL) as a result
decisions of inefficient bilirubin conjugation and excretion. Jaundice due to
breast-feeding reaches a maximum level of 7.3 ± 3.9 mg/dL. The eti-
Febrile Seizures ology is unknown. Elevated levels of bilirubin also occur with in-
ABCs (airway, breathing, creased turnover of RBCs from hemolysis (ABO or Rh incompatibil-
circulation); lorazepam ity), significant bruising, cephalhematomas, structural or metabolic
(Ativan) or diazepam abnormalities of RBCs, or hereditary defects of bilirubin conjuga-
(Valium) for prolonged tion (Crigler–Najjar syndrome, Gilbert disease).
seizures; prophylactic
anticonvulsants are Treatment Steps
controversial. 1. Evaluation and treatment for underlying pathologic etiologies.
Kawasaki Disease 2. Encouragement of fluid intake.
Intravenous γ globulin; 3. Phototherapy with pathologically elevated bilirubin levels.
aspirin; cardiac evaluation
4. Exchange transfusion in severe cases.
(ECG, echocardiogram).
Neonatal F. Developmental Dysplasia of the Hip
Hyperbilirubinemia
Evaluation and treatment H&P Keys
for underlying etiology; A higher incidence with breech deliveries, first-born children, fe-
encouragement of fluid males, and oligohydramnios. Left hip more commonly involved.
intake; phototherapy with Twenty percent have a positive family history. Signs and symptoms
pathologically elevated include Barlow sign (posterosuperior movement of the femur over
bilirubin levels; exchange
the limbus with adduction and posteriorly directed pressure), Or-
transfusion in severe cases.
367
V. INFECTIOUS DISEASES
A. Bronchiolitis
H&P Keys
Common (60% of infants). Winter-to-spring months. Peak age
< 24 months. Signs and symptoms include cough, rhinorrhea, with
or without low-grade fever, scattered wheezing with or without rales,
labored breathing.
Diagnosis
Clinical features (age, season, physical). Evaluation of nasopha-
ryngeal secretions (viral culture, enzyme-linked immunosorbent
assay [ELISA], or immunofluorescence). CXR has nonspecific
changes (hyperinflation, atelectasis, with or without interstitial in-
filtrates).
Disease Severity
General appearance, mental status changes, labored breathing
(tachypnea, nasal flaring, retractions, accessory muscle use),
cyanosis, pulse oximetry, ABG if severe distress. Higher morbidity
and mortality if infant has underlying pulmonary or cardiac disease,
immunodeficiency disease, underlying chronic disease, history of
prematurity, or is < 6 weeks old.
Concept and Application
Viral infection (primary cause is respiratory syncytial virus) of lower
respiratory tract. Transmission by direct contact. Respiratory mu-
cosal injury, inflammation or edema, mucus production.
Treatment Steps
1. Prevention by hand washing and monoclonal antibody injections
(for children at risk).
2. Supportive care including oxygen if hypoxic.
3. Aerosolized β2-adrenergic agent trial.
4. Use of ribavirin and steroids controversial.
368
C
PEDIATRICS Infectious Diseases
on rounds
PEDIATRIC INFECTIONS
Croup
• Ages 6–36 months, also termed laryngotracheitis.
• Barklike cough, hoarseness, inspiratory stridor, CXR positive steeple sign (subglottic swelling).
• Worse at night.
• Supportive treatment, mist, O2.
Epiglottitis
• Ages 2–7.
• Fever, drooling, dysphagia, stridor, may have abnormal lateral neck x-ray, possible toxic
appearance, anxious.
• Winter most often.
• Support airway, visualization in OR followed by intubation.
Bronchiolitis
• Peak age < 2 years.
• Cough, wheezing, labored breathing, nonspecific CXR.
• Winter to spring occurrence.
• Supportive treatment.
B. Otitis Media
H&P Keys
Peak age < 24 months. Winter season. Signs and symptoms include
pain, fever, hearing loss, cough, and congestion as well as hyper-
emia, dullness, decreased mobility, retraction or bulging (loss of
landmarks) of tympanic membrane. Asymptomatic in 50% of cases.
Diagnosis
Pneumatic otoscopy, tympanometry, audiometry.
Disease Severity
Overall appearance, degree of discomfort or hearing loss, response
to therapy. Higher morbidity if young age (< 6 months), environ-
mental setting (passive smoking, day care), genetic factors (familial,
trisomy 21, Native American), immunodeficiency, congenital anom-
alies (cleft palate, choanal atresia).
Treatment Steps
1. First-line antibiotics include aminopenicillins.
2. Consider coverage for pneumococcal resistent organisms in high-
risk groups (e.g., day care, age < 2 years, recent antibiotic use).
3. Consider myringotomy tubes if persistent effusion with hearing
loss, failure of prophylaxis, or high-risk groups (e.g., trisomy 21,
cleft palate, known sensorineural hearing loss).
4. Prevention with pneumococcal vaccine.
369
D. Laryngotracheitis (Croup)
H&P Keys
Ages 6–36 months. Nocturnal. Severity of disease peaks at 3–5 days.
Signs and symptoms include barklike cough, hoarseness, upper res-
piratory symptoms, inspiratory stridor.
Diagnosis
Clinical presentation, steeple sign (subglottic swelling) on CXR.
Disease Severity
Overall appearance, mental status changes, respiratory distress, hy-
poxia and hypercapnia.
Concept and Application
Mucosal edema and swelling of subglottis and trachea leading to hy-
poxia and atelectasis. Primarily caused by parainfluenza virus.
Treatment Steps
1. Supportive (especially airway).
2. Minimal disturbance.
3. Mist.
4. Oxygen if hypoxemia.
5. Racemic epinephrine or dexamethasone if severe respiratory dis-
tress.
E. Varicella (Chickenpox)
H&P Keys
Incubation period of 14–15 days. Winter or spring months. Conta-
gious period is 4–5 days prior to exanthem and until rash scabs.
370
PEDIATRICS Infectious Diseases
Diagnosis
Characteristic rash. Fluorescein monoclonal antibody.
Disease Severity
Worse course if < 1 year of age, teenager or adult, or visceral involve-
ment (lungs, CNS, liver, joints, heart, kidneys).
Treatment Steps
1. Prevention is key (varicella vaccine).
2. Antipruritic drug.
3. Antimicrobial therapy if secondary infection (usually group A
streptococcus).
4. Acyclovir if child is immunocompromised, older, or severely in-
volved.
F. Pharyngitis
H&P Keys
Signs and symptoms include fever, sore throat, hoarseness, cough,
abdominal pain, tonsillar erythema or enlargement or exudate, pe-
techiae of palate, anterior cervical adenopathy, “sandpaper” rash.
Concomitant nasal symptoms suggest viral etiology.
Diagnosis
Throat culture, rapid Streptococcus antigen test.
Disease Severity
Clinical discomfort. Suppurative complications (cervical adenitis, otitis
media, septicemia). Nonsuppurative complications (rheumatic fever,
nephritis).
Treatment Steps
1. Self-limiting if viral etiology.
2. Penicillin (erythromycin if penicillin allergy) for 10 days if group
A β-hemolytic streptococci.
G. Bacterial Meningitis
H&P Keys
Signs and symptoms include fever, lethargy, irritability, neck stiffness
or pain, headache, altered consciousness, nuchal rigidity, Kernig’s
sign, Brudzinski’s sign.
Diagnosis
Bacterial culture of CSF is gold standard. CSF cytology and biochem-
ical parameters. Bacterial antigen test if child currently on antibi-
otics.
371
PEDIATRICS Gastroenterology
Diagnosis
Organism isolation (Tzanck test, immunofluorescent studies).
Concept and Application
HSV-2 (70%). Transmission more likely during primary maternal in-
fection.
Treatment Steps
1. Antiviral (acyclovir) therapy.
2. Supportive care.
I. Hemolytic–Uremic Syndrome
H&P Keys
Children usually between 2 months and 8 years of age. Triad of ure-
mia, thrombocytopenia, and microangiopathic hemolytic anemia.
Signs and symptoms include preceding illness (diarrhea or upper
respiratory infection [URI]), irritability, bloody diarrhea, poor uri-
nary output, pallor, petechiae, edema, hypertension.
Diagnosis
CBC with platelets and smear (red blood cell [RBC] morphology),
Coombs’ test (negative), electrolytes with blood urea nitrogen
(BUN) and creatinine (Cr).
Disease Severity
Seizures, stroke, coma, cardiac failure, metabolic imbalance (meta-
bolic acidosis).
Concept and Application
No one causative factor (viral, bacterial, drugs). Peripheral destruc-
tion of platelets, RBC hemolysis secondary to mechanical destruc-
tion by fibrin strands in small renal vessels.
Treatment Steps
1. Supportive care.
2. Correction of fluid and electrolyte imbalance.
3. Correction of hypertension.
4. Dialysis and RBC transfusion if needed.
VI. GASTROENTEROLOGY
C. Pyloric Stenosis
H&P Keys
Average age 3–4 weeks of life. First-born males. Signs and symptoms
include progressive nonbilious projectile emesis, peristaltic abdomi-
nal waves in epigastrium, palpable right upper quadrant mass
(“olive”).
Diagnosis
Clinical. Sonogram or upper GI (UGI) series (“tram-track” sign) if
necessary. Hypochloremic hypokalemic metabolic alkalosis.
Disease Severity
Cachexia, profound dehydration, severe metabolic imbalance.
Concept and Application
Mechanical gastric outlet obstruction results from congenitally hy-
pertrophied pyloric muscle.
Treatment Steps
1. Correction of fluid and electrolyte abnormality.
2. Surgical repair (pyloromyotomy).
Treatment Steps
Surgery (15–20% mortality rate).
375
PEDIATRICS Gastroenterology
E. Intussusception
H&P Keys
Most common cause of intestinal obstruction ages 3–12 months.
Signs and symptoms include intermittent colicky pain, bilious eme-
sis, mental status changes, palpable “sausage-shaped” abdominal
mass, “currant jelly” stools (Fig. 14–3).
Diagnosis
Clinical. Barium enema.
Disease Severity
Severe metabolic acidosis, evidence of intestinal ischemia or infarc-
tion, severe dehydration.
Concept and Application
Invagination or telescoping of proximal bowel into more distal
bowel. Lymphatic and venous compromise. Primarily ileocolic re-
gion. Leadpoint lesion (e.g., polyp, lymphoma) in older children.
Treatment Steps
1. Barium enema reduction.
2. Antibiotics if peritoneal signs.
3. Occasionally, surgical reduction.
Diagnosis
Clinical. Blood or reducing substances in stool, thrombocytopenia,
prolonged prothrombin time (PT) and partial thromboplastin time
(PTT). Pneumatosis intestinalis, bowel wall edema, biliary air, or
free air in peritoneum on abdominal x-ray.
Disease Severity
Evidence of sepsis, hemorrhage, disseminated intravascular coagula-
tion (DIC), shock, severe metabolic acidosis, mental status changes.
Concept and Application
Unknown. Presentation consistent with bowel ischemia or infarc-
tion.
Treatment Steps
1. Bowel rest.
2. Fluid resuscitation.
3. Parenteral nutrition.
4. Broad-spectrum antibiotics.
5. Surgery if failure of medical management or if complications.
G. Colonic Aganglionosis
(Hirschsprung’s Disease)
H&P Keys
Absence of meconium stools in first week of life. Signs and symp-
toms include emesis, abdominal distention, minimal stool in rectal
vault.
PEDIATRICS Genetics
Diagnosis
Clinical. Rectal biopsy. Barium study in older children (Fig. 14–4).
Disease Severity
Presentation can simulate sepsis or NEC.
Concept and Application
Absence of colonic ganglia resulting in persistent colonic contrac-
tion.
Treatment Steps
Surgery.
VII. GENETICS
B. Trisomy 18
H&P Keys
Characteristic features include IUGR, micrognathia, clenched hands
with overlapping fingers, congenital heart disease (ventricular septal
defect [VSD], patent ductus arteriosus [PDA]), mental retardation.
Diagnosis
Clinical. Chromosome analysis.
d
Treatment Steps
diagnostic Same as for trisomy 21.
decisions
C. Trisomy 13
VIII. PULMONOLOGY
PEDIATRICS Pulmonology
Disease Severity
Respiratory distress (tachypnea, retractions, poor air movement).
Concept and Application
Delayed maturation of newborn larynx, resulting in increased flexi-
bility.
Treatment Steps
Positioning (self-limited).
D. Pulmonary Sequestration
H&P Keys
Fever, hemoptysis, and respiratory symptoms if intralobar. Respira-
tory symptoms, heart failure, or no symptoms if extralobar. Dullness
to percussion, heart murmur.
Diagnosis
CXR (mass). Further investigation includes bronchoscopy, sono-
gram, and aortogram.
Concept and Application
Segments of nonfunctioning embryonic lung tissue that are nour-
ished by anomalous systemic circulation. Intralobar type occurs
within normal visceral pleura. Extralobar type has separate visceral
pleural covering.
Treatment Steps
Surgical resection.
380
PEDIATRICS Disorders of the Musculoskeletal System
A. Scoliosis
Description
A lateral curvature of the spine involving the thoracic and/or lum-
bar vertebrae.
Diagnosis
Clinical evaluation of patient’s back in an erect and a bent-at-the-
hips position. X-rays can quantitate any curvature > 10 degrees.
Treatment
Depends on severity of curvature. Follow curves < 15–20 degrees
closely at interval health maintenance visits. If curvature is > 20 de-
grees, the patient should be referred to an orthopedic surgeon for
additional therapy, which may include bracing or surgical interven-
tion.
B. Kyphosis
Description
An excessive roundback of the thoracic spine.
Diagnosis
Physical examination. X-rays may be used to differentiate a postural
and structural defect.
Treatment
Improvement of posture, back exercises, braces.
Diagnosis
History significant for pain in hip or knee region. X-ray of the hip
shows femoral head displacement.
Treatment
Surgical intervention required.
D. Osgood–Schlatter Disease
Description
Stress changes at the tibial tuberosity. Most commonly seen in ado-
lescent males during growth spurt.
Diagnosis
Clinical. Point tenderness at tibial tuberosity with occasional associ-
ated swelling.
Treatment
Supportive.
381
383
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384 Psychiatry
BIBLIOGRAPHY / 416
385
PSYCHIATRY Psychoses
I. PSYCHOSES
A. Schizophrenia
H&P Keys
signs).
Disease Severity
Worse prognosis with poor premorbid social history, early onset, in-
sidious onset, lack of insight, nonparanoid subtype, absence of
mood symptoms, neurologic abnormalities, male gender, negative
symptoms, brain imaging abnormalities. Increased risk for medical
illness, incarceration, violence, substance use, victimization, poverty,
suicide (10–15%).
cram facts U
Concept and Application SYMPTOMS SUGGESTING
MEDICAL OR SUBSTANCE-
Associated with altered dopaminergic activity in the mesolimbic and
INDUCED PSYCHOSIS
mesocortical tracts. Negative symptoms associated with enlargement
of cerebral ventricles and cortical atrophy. Functional neuroimaging • Abrupt onset
demonstrates hypofrontality. Both (polygenic) genetic and environ- • Older age
mental contributions. • Physical or laboratory
abnormalities
Treatment Steps • Disorientation, clouded
sensorium, memory
Acute
impairment
1. Hospitalization for stabilization, protection of self and others • Visual, olfactory, tactile
(may require involuntary commitment). hallucinations without
2. Benzodiazepines and antipsychotics for acute agitation. auditory hallucinations
3. Rule out general medical, substance-induced etiologies.
4. Antipsychotic medication: “atypicals” (olanzapine, risperi-
done, quetiapine) may treat negative symptoms better than
older antipsychotics. Clozapine indicated for refractory symp-
toms (weekly CBC to rule out agranulocylosis).
386
PSYCHIATRY Psychoses
Continued Care
management
decisions d 1. Continuation of antipsychotics (oral or long-acting intramus-
cular).
2. Assess for development of side effects, e.g., tardive dyskinesia
(5% incidence per year with older antipsychotics); anticholin-
ACUTE PSYCHOSIS ergic effects, sexual dysfunction, weight gain, sedation, ex-
trapyramidal symptoms: lipid and glucose abnormalities with
Maintain Safety, Manage atypicals.
Agitation/Arousal
3. Addition of lithium (Li++), anticonvulsants may benefit some.
Calming environment and
staff behavior. “Show of
4. Psychosocial treatments, including patient and family educa-
force” if necessary. tion, social skills training, residential and day treatment, voca-
Benzodiazepine, e.g., tional rehabilitation, supportive psychotherapy.
lorazepam, 2 mg PO or IM,
and/or neuroleptic, e.g., B. Affective Psychoses: Mania
haloperidol, 5 mg PO or and Psychotic Depression
IM, given q 30–60 min until
patient is calm. Seclusion, H&P Keys
restraint only if necessary History—Personal and family history of affective disorder; clinical
after previous interventions.
features of mood disorders have been present preceding psy-
Identify Serious Medical chosis; illness course generally episodic; alcohol and drug abuse
Problems
common.
History, vital signs, physical
exam. Review all Physical and Mental Status Exam—Mania: hyperactivity, rapid and
medications (prescribed, pressured speech, flight of ideas, elated or irritable affect with la-
over-the-counter, bility, decreased need for sleep, distractibility, impulsivity, poor
borrowed). Drug and
judgment. Forty percent have psychotic symptoms, e.g.,
alcohol screen, CBC,
chemistries, LFTs, TFTs,
grandiose or paranoid delusions. Depression: psychomotor retar-
brain imaging for first or dation or agitation, slowed and impoverished speech, signs of
atypical presentations, weight loss, poor self-care, guilty ruminations, somatic delusions,
abnormal neuro exam. derogatory hallucinations, suicidal preoccupation. Either mania
Assessment and or depression may present with catatonia.
Tentative Diagnosis
Further treatment dictated Diagnosis
by diagnostic category. No pathognomonic signs or studies; rule out general medical etiolo-
gies (thyroid and adrenal dysfunction, medications [e.g., sympath-
omimetics, L-dopa, steroids, antidepressants], Parkinson’s disease,
head injury, multiple sclerosis [MS], dementing illnesses, cere-
brovascular accidents [CVAs], pancreatic and other malignancies,
epilepsy, lupus, CNS tumors, viral illnesses); psychiatric disorders in-
cluding personality disorders, substance intoxication or withdrawal
(e.g., stimulants, anabolic steroids, alcohol).
Disease Severity
PSYCHIATRY Psychoses
differential diagnosis
e
PSYCHOTIC MANIA OR DEPRESSION VERSUS SCHIZOPHRENIA
Treatment Steps
Acute
1. Hospitalization for protection of self/others, treatment (may
need involuntary commitment).
2. Rule out general medical, substance-induced etiologies.
3. For mania: may require restraint, IV/IM benzodiazepines;
may use loading doses of lithium or valproate; use IM antipsy-
chotic if necessary for control of agitation. Electroconvulsive
therapy (ECT) for toxic mania, rapid control, pregnancy.
Lamotrigine, carbamazepine, atypical antipsychotics also used
for mania.
4. For psychotic depression: ECT most effective and rapid; anti-
depressant (AD) with antipsychotic also effective; poor re-
sponse to AD alone.
Continued Care—See section II.
C. Delusional Disorder
H&P Keys
Fixed, nonbizarre, systematized delusion; common types are ero-
tomanic (one is loved by a famous other), grandiose, jealous, perse-
cutory (most common), somatic. Age of onset and psychosocial
functioning variable. No hallucinations.
Diagnosis
Rule out general medical disorders (early dementias, CNS tumor,
endocrine and metabolic disorders, stimulant abuse, basal ganglia
trauma).
Disease Severity
Course is variable; worsens with stress.
Concept and Application
Increased in immigrants, deafness, severe stress, visuospatial deficits.
Treatment Steps
Acute
1. Hospitalize for suicidal or homicidal risk; extreme impair-
ment, danger associated with delusions.
2. General medical workup.
388
PSYCHIATRY Psychoses
D. Other Psychoses
1. Schizoaffective Disorder
H&P Keys
Concurrent symptoms of schizophrenia and depression or mania,
with at least 2 weeks of psychotic symptoms alone.
Disease Severity
Poorer prognosis with depressive subtype, family history of schizo-
phrenia, insidious onset, poor premorbid history, predominance of
psychotic symptoms.
Concept and Application
Mood syndrome defines subtype (manic versus depressive); depres-
sive subtype thought to be related to schizophrenia; “bipolar” sub-
type to bipolar disorder.
Treatment Steps
1. Hospitalization as in other psychoses.
2. Rule out general medical etiology.
Bipolar Type—Li++ and/or other mood stabilizer, e.g., valproic acid
(VPA), carbamazepine (CBZ) with antipsychotic if necessary;
ECT if necessary. Li++, VPA, CBZ maintenance; atypical neurolep-
tics also used.
Depressive Type—Antipsychotic with or without AD; ECT, Li++,
mood stabilizers also used.
2. Schizophreniform Disorder
H&P Keys
History, signs, symptoms, and differential as in schizophrenia but du-
ration < 6 months.
Disease Severity
Better prognosis with acute onset, confusion and disorientation, full
affect, good premorbid functioning.
Treatment Steps
1. Hospitalize as needed.
2. Antipsychotics for at least 6 months.
3. Brief Psychotic Disorder
H&P Keys
Acute-onset psychosis with emotional turmoil and confusion often
following obvious stressor, duration < 1 month, full return to pre-
morbid functioning. Young adult. Predisposed by personality disor-
der, posttraumatic stress disorder (PTSD). Rule out schizophreni-
form and mood disorders, general medical etiology, factitious
disorder, malingering, substance induced.
Disease Severity
Prognostic factors as in schizophreniform disorder. Suicide risk.
389
PSYCHIATRY Psychoses
Treatment Steps
1. Hospitalization as needed.
2. Antipsychotic or antianxiety agent.
3. Psychotherapy when stabilized.
Diagnosis
Neurologic evaluation. No pathognomonic tests. Screening for hear-
ing and vision, EEG, computed tomography (CT) or magnetic reso-
nance imaging (MRI), heavy metals, serum ceruloplasmin, phenylke-
tonuria (PKU), karyotype. Developmental assessment.
Disease Severity
Better prognosis with higher intelligence quotient (IQ), language,
and social skills.
Treatment Steps
1. Structured classroom training, behavioral modification, parent
training, family support.
3. Antipsychotics may decrease hyperactivity, stereotypies, irritability.
Selective serotonin reuptake inhibitors (SSRIs) may help with so-
cial interaction, compulsive behaviors.
390
PSYCHIATRY Mood Disorders
diagnostic
decisions d pattern of mood instability; may be comorbid); dementia (less likely
history of affective disorder; deficits stable; patient may try to hide
deficits, rather than complain about them; insidious onset); bipolar
depression (history of mania); bereavement (self-limiting; lacks mor-
MAJOR DEPRESSION bid preoccupation with worthlessness, suicidal ideation, psychomo-
tor retardation, marked functional impairment). Diagnosis difficult
Sustained, distinct in medically ill due to overlapping symptoms.
depressed mood for 2
weeks plus at least four of Disease Severity
the following Same as for affective psychoses; untreated episodes last > 6 months;
(SIG E CAPS): 60% recover, 30% partially recover, 5–10% develop chronic course.
Sleep (increased or
Worse prognosis with late-life onset, concurrent personality, anxiety,
decreased)
Interest (loss of interest or substance abuse, chronic medical problems, dysthymic disorder, psy-
pleasure) chosocial stress. Aggressive treatment reduces suicide risk (15%)
Guilt (worthlessness) and improves comorbid medical disorders. Each recurrence in-
Energy (decreased) creases risk of further recurrence.
Concentration (decreased)
Appetite (increased or Concept and Application
decreased) Dysregulation of neurotransmitters and neuroendocrine system, es-
Psychomotor change pecially in HPA axis; decreased serotonergic activity associated with
(retardation or agitation)
suicide. Late-onset may have subcortical cerebrovascular disease.
Suicidality (active or
passive)
ADs increase available monoamines at nerve terminals, alter recep-
tor sensitivity and density. Genetic component. Twenty-five percent
identify precipitant, often loss. Higher incidence and earlier age of
391
B. Dysthymia
Chronic depression of at least 2 years’ duration with at least two of
the following: appetite disturbance, sleep disturbance, fatigue, low
self-esteem, difficulty concentrating, hopelessness; no history of ma-
jor depression or manic episode within first 2 years. May be sec-
ondary to other psychiatric or general medical illness, chronic stress.
Insidious onset childhood through early adulthood; women > men;
family history of depression. Rule out major depression, personality
disorder (may coexist), chronic illnesses, such as uncontrolled dia-
betes, hypothyroidism, chronic fatigue syndrome. Ten percent per
year develop “double depression” (major depression superimposed
on dysthymia), worsens prognosis of both. Treat with ADs, psy-
chotherapy, possibly exercise.
C. Bipolar Disorders
H&P Keys
See section I.B. above. One or more manic episodes (distinct period
of elated or irritable mood with at least three of the following:
grandiosity, decreased need for sleep, talkativeness, racing thoughts,
distractibility, hyperactivity or increased goal-directed activity, rapid
and pressured speech, increased energy, impulsivity [e.g., excessive
spending, gambling, promiscuity], causing marked impairment or
392
PSYCHIATRY Mood Disorders
Treatment Steps
D. Cyclothymia
Chronic fluctuating mood disturbance of at least 2 years’ duration
with symptoms of hypomania and depression insufficient to be diag-
nosed as major depression or bipolar disorder. Onset adolescence,
early adulthood. Family history of affective disorder. Rule out per-
sonality disorder, substance abuse. May respond to lithium or anti-
convulsants, psychotherapy.
A. Panic Disorder
H&P Keys
Acute
1. Emergency management of panic attack via reassurance, ben-
zodiazepine.
2. Rule out myocardial infarction (MI), pulmonary embolism
(PE), substance withdrawal, CNS insult, hypoglycemia, elec-
trolyte abnormalities.
3. Prompt psychiatric referral; avoidance of interminable med-
ical workups.
Continued Care
1. Antidepressants first line; start with low doses and increase
slowly; high-potency benzodiazepines also effective but may be
difficult to discontinue. Buspirone ineffective. Taper should
be attempted in 6–12 months; 70% eventually relapse.
2. Cognitive–behavioral therapy (CBT) effective for agoraphobia
and relapse prevention.
3. Support, education.
4. Eliminate caffeine.
5. Diagnose and treat comorbid substance and psychiatric disor-
ders.
H&P Keys
More than 6 months of unrealistic, persistent anxiety and worry un-
related to another psychiatric disorder. Associated with ≥ 3 of the fol-
lowing: restlessness, fatigability, difficulty concentrating, irritability,
muscle tension, sleep disturbance. Other somatic complaints com-
mon. Often present in primary care.
Diagnosis
Differential is same as for panic disorder.
Disease Severity
Ninety percent psychiatric comorbidity; 25% develop panic disorder.
Depression common.
Treatment Steps
1. CBT effective for milder cases.
2. Medications for more severe, chronic disorder; many require pro-
longed or intermittent treatment. ADs effective, especially with
comorbid depression. Buspirone as effective as benzodiazepines
without causing sedation, psychomotor impairment, risk of toler-
ance, abuse potential, rebound; delayed onset of action,
headache, nausea, dizziness. Benzodiazepines give immediate re-
lief; abuse rare without substance abuse history.
Diagnosis
Toxicology, head injury workup as indicated. Chronic PTSD may
present with a variety of somatic and psychiatric symptoms; obtain
396
PSYCHIATRY Anxiety Disorders
Acute
1. Rapid evaluation and treatment.
2. Hospitalize for suicide, violence risk.
3. Rape victims require careful documentation of findings, eval-
uation for sexually transmitted diseases (STDs) and preg-
nancy, plan for immediate safety, legal advice.
3. CBT, exposure therapy, patient education, group therapy all of
value.
4. Cautious use of mild sedation, e.g., with benzodiazepine, as
indicated initially.
Continued Care
1. Individual, CBT and supportive therapy, peer group therapy,
family education for chronic symptoms.
2. SSRIs are first-line treatment.
3. Treatment of comorbid disorders.
Treatment Steps
1. Psychotherapy.
2. Mild sedation as indicated.
F. Phobias
1. Social Phobia
H&P Keys
Fear of scrutiny of others; may be specific (e.g., eating in public,
public speaking) or more generalized. Situations avoided or en-
dured with anxiety. Onset in adolescence, chronic course, may de-
velop depression, substance abuse.
Treatment Steps
1. CBT.
2. Breathing retraining.
3. ADs (SSRIs, monoamine oxidase inhibitors [MAOIs]) for gener-
alized symptoms; high-potency benzodiazepines also used.
4. β-Blockers used for performance anxiety.
397
Disease Severity
Greater vulnerability with history of childhood parental loss, serious
medical illness, but 20% of adults and 40% of adolescents have men-
tal disorder at 5-year follow-up. Increased risk of suicide, medical
noncompliance.
Treatment Steps
1. Evaluate risk of suicide, medical noncompliance, substance
abuse.
2. Psychotherapy to help patient clarify concerns, resources, op-
tions, develop plan of action.
3. Stress reduction may include social support, exercise, relaxation,
cognitive reframing techniques, attention to health habits, peer
group support.
4. Antianxiety/antidepressant medication as indicated for short-
term symptom relief.
Odd, Eccentric
1. Paranoid: suspicious, hypervigilant; ascribes malicious intent,
hidden meanings to others; hypersensitivity to criticism;
nonpsychotic.
2. Schizoid: isolated, indifferent to social relationships, re-
stricted affective expression.
3. Schizotypal: minor thought disorder (e.g., ideas of reference,
magical thinking), peculiar behavior, constricted affect, social
anxiety.
Dramatic, Emotional
1. Antisocial (ASP): pattern of exploitative, socially irresponsible,
destructive, impulsive behavior with no remorse; comorbid
substance abuse; conduct disorder in childhood.
2. Borderline: problems with intense, unstable relationships; af-
fect regulation; impulse control; identity. Suicide gestures
common.
3. Histrionic: self-absorbed; seductive, with shallow, labile affect;
excess need for praise, reassurance.
4. Narcissistic: grandiose, exploitative, entitled, rageful if humili-
ated, lacks empathy.
Anxious, Fearful, Inhibited
1. Avoidant: fearful of rejection, timid, inhibited but desirous of
relationships (as versus schizoid); comorbid anxiety disorders.
2. Dependent: submissive, passive, clingy; lets others make im-
portant decisions; easily hurt; preoccupied with abandon-
ment; predisposed by chronic illness.
3. Obsessive–compulsive: preoccupied with rules; rigid, perfec-
tionistic, ambivalent, stingy, controlling, restricted affect; per-
sistent, task-oriented.
Diagnosis
Onset late teens to early twenties. Acute personality changes in
adulthood due to Axis I or general medical disorders (e.g., sub-
stance intoxication, abuse; CNS disease, e.g., MS; mood disorders;
psychosis; psychic trauma); diagnose coexistent Axis I mood, anxiety,
psychotic, substance use, eating, somatization disorders. Personality
disorders must have long-term, persistent pattern; may need to defer
diagnosis in presence of Axis I pathology.
Disease Severity
Hospitalize for protection of self and others (suicide risk significant
in borderlines, antisocials, schizotypals, particularly in presence of
substance use); psychotic decompensation (borderline, schizotypal,
schizoid, paranoid); severe Axis I pathology.
Concept and Application
Personality disorders result from interaction of constitution, tem-
perament, developmental experiences, environment; genetic contri-
bution in antisocial, schizotypal, paranoid; childhood sexual or phys-
ical abuse in many borderlines, avoidants, antisocials; nonspecific
neurologic abnormalities; decreased CNS serotonin functioning as-
sociated with impulsivity, aggression; schizotypals genetically and bi-
ologically related to schizophrenia.
Treatment Steps
1. Evaluate suicide and violence risk; hospitalize if necessary.
2. Diagnose and treat comorbid Axis I disorders.
399
A. Somatization Disorder
H&P Keys
Recurrent, multiple, unfeigned somatic complaints not fully ex-
plained by physical disorder, for which medical attention has been
sought. Young women, often with menstrual difficulties. Frequent,
vague, dramatic complaints involve chronic pain; GI symptoms, e.g.,
irritable bowel, vomiting; cardiopulmonary symptoms, e.g., unex-
plained dyspnea, dizziness; pseudoneurologic symptoms, e.g., paraly-
sis, blindness; reproductive tract, sexual problems. High medical uti-
lizers, multiple doctors; extensive, costly evaluations; may have
polysurgeries, e.g., early hysterectomy. Childhood sexual abuse pre-
disposes; at risk for concurrent spousal abuse. Common comorbid
disorders: alcohol, analgesic, sedative abuse; depression (80–90%);
anxiety (25–45%); personality disorders.
Diagnosis
Rule out disorders that present with vague, multiple, confusing com-
plaints, e.g., MS, porphyria, lupus, hyperparathyroidism; physical
symptoms of panic disorder occur only during attacks; conversion
disorder involves only pseudoneurologic symptoms; in factitious dis-
order, person consciously controls production of symptoms.
Disease Severity
Chronic course; suicide associated with substance abuse.
Concept and Application
Both genetic and environmental factors contribute; familial associa-
tion with substance abuse and antisocial personality in males. Psy-
chologically interpreted as expression of psychological pain, elicits
care, secondary gain.
Treatment Steps
1. Regularly scheduled visits with primary care physician; team ap-
proach with mental health specialist.
2. Tests, consultations only with evidence of illness.
3. Treat depression, anxiety with ADs.
4. Avoid opiates, benzodiazepines.
B. Pain Disorder
H&P Keys
Persistent preoccupation with pain without adequate physical find-
ings or pathophysiologic mechanism to account for intensity or dis-
abling psychosocial sequelae. Onset usually age 30–50, women >
men; high utilizers of medical care, surgery; complaints may be
vague, diffuse; frequent analgesic, substance abuse; comorbid de-
pression, anxiety, insomnia; inactivity, invalidism, isolation. Resistant
to psychological interpretation.
400
PSYCHIATRY Somatoform Disorders
Diagnosis
Dramatic presentation of pain (due to cultural or personality traits)
lacks related impairment; lacks plethora of symptoms of somatiza-
tion disorder; psychogenic disorders with known pathophysiologic
mechanisms, e.g., tension headache, diagnosed as psychological fac-
tors affecting physical condition; in malingering, symptoms inten-
tionally produced in pursuit of obvious goal. Rule out reflex sympa-
thetic dystrophy (RSD).
Disease Severity
Prescription drug addiction may require detoxification. Evaluate
psychosocial impairment. Suicide associated with severe depression.
Concept and Application
Subjective pain experience has cognitive, affective, behavioral com-
ponents, which may be modified through cognitive–behavioral inter-
ventions; serotonergic, adrenergic, endorphin systems involved in
pain may be modulated with ADs, analgesics.
diagnostic
decisions d Treatment Steps
1. As with somatization disorder.
2. CBT and supportive therapies (behavioral evaluation, relaxation,
contingency management).
SOMATOFORM AND 3. Nonsteroidal anti-inflammatory agents, TCAs for pain.
RELATED DISORDERS
4. Family therapy, education.
5. Vocational rehab.
Somatoform Disorders
Symptoms are not
C. Conversion Disorder
intentionally produced.
Symptoms not fully H&P Keys
explained by medical Involuntary psychogenic loss or alteration of functioning suggesting
condition.
a physical disorder, not limited to pain or sexual disturbance. Tem-
Symptoms linked to
psychological factors:
poral relationship between psychologically painful stressor and onset
a. Somatization disorder: of symptoms. Classic cases involve pseudoneurologic symptoms, e.g.,
multiple somanic blindness, paralysis, mutism, pseudoseizures, difficulty swallowing
complaints because of lump in throat (globus hystericus). Antecedent physical
b. Conversion disorder: disorder (e.g., seizure disorder), exposure to persons with physical
neurologic symptoms disorders, preexisting gross brain pathology predispose. Women,
c. Pain disorder
rural, lower socioeconomic strata. Comorbid somatization, depres-
d. Hypochondriasis: fear
of specific disease
sive, panic, substance, personality, dissociative disorders.
e. Body dysmorphic
Diagnosis
disorder:
preoccupation with Diagnosis of exclusion, requiring careful neurologic exam and test-
defect in appearance ing, psychiatric consultation. May have inconsistency of symptoms
Factitious Disorder with known neuroanatomy and physiology, e.g., glove distribution of
Intentional production of anesthesia. Thirty percent subsequently develop neurologic or other
symptoms for unconscious illness that explains symptom, e.g., MS, lupus, especially with onset
psychological reasons (the after age 35.
sick role).
Malingering Disease Severity
Intentional production of Improvement or complete resolution associated with abrupt onset,
symptoms for recognizable clear precipitant, absence of general medical or psychiatric illness;
gain. recurrence predicts chronicity.
Psychological Factors
Affecting a Medical Treatment Steps
Condition 1. Rule out neurologic, medical disorders (e.g., lupus, MS, HIV en-
Known medical condition cephalopathy).
develops/is exacerbated by 2. Reassurance, suggestion, attention to any stressful precipitant.
psychological factors. 3. Diagnose and treat underlying psychopathology.
4. Hypnosis or amobarbital interview may be helpful.
401
F. Related Disorders
1. Psychological Factors Affecting Physical Condition
(Psychosomatic Disorders)
A psychological factor is believed to have contributed significantly to
the development or exacerbation of physical symptom or illness, evi-
denced by temporal relationship with psychologically meaningful
stressor; commonly, headaches, peptic ulcer disease, asthma, skin
diseases, vomiting, obesity, low-back pain, ulcerative colitis, arrhyth-
mias. Physical condition on Axis III. Stress management, psychother-
apy may increase adjustment, reduce medical costs.
2. Factitious Disorders
Intentional (but often compulsive) production of or feigning of
physical or psychological symptoms, presumably for psychological
reasons unknown to the patient, e.g., unsatisfied dependency needs
met in achieving “sick role.” Includes reporting or intentionally pro-
ducing false symptoms, e.g., injection of contaminated substance,
surreptitious use of medications, thermometer manipulation, self-
induced bruises. Early adult onset; chronic, severe impairment; co-
morbid severe personality disorders, substance abuse; may have
medical occupation, history of illness. Psychiatric consultation, con-
frontation.
3. Malingering
Intentional production of physical or psychological symptoms for
obvious recognizable external incentive, e.g., to avoid military ser-
vice, financial reward, evading prison, obtaining drugs. Discrepancy
with objective findings, vague, uncooperative. Comorbid antisocial
personality, substance abuse.
402
PSYCHIATRY Conduct Disorder
A. Anorexia Nervosa
H&P Keys
Refusal to maintain minimal normal body weight (< 85%), intense
fear of gaining weight, normal appetite, distorted body image, 3
months’ amenorrhea. Associated features: obligate exercise, peculiar
food behaviors, bulimic symptoms, emaciation, hypothermia, hy-
potension, lanugo hair, bradycardia, edema, hair loss, hyposexuality,
compulsive behaviors. Onset adolescence, 95% women, mid- to up-
per socioeconomic strata, premorbid history of perfectionism, in-
flexibility, need for control, anxiety, possibly sexual abuse. Precipi-
tated by stress, dieting. Comorbid depression, personality, OCD.
Diagnosis
Rule out weight loss due to depression, AIDS, cancer, GI disorders,
substance abuse. In depression, appetite is diminished whereas
anorexics have normal appetite; in schizophrenia, bizarre eating pat-
terns related to psychosis; in bulimia, weight does not fall below
85%; underweight persons without anorexia recognize their low
weight. May have leukopenia, anemia, electrolyte abnormalities, de-
hydration, decreased TFTs, prepubertal hormone levels, sinus brady-
cardia.
Disease Severity
Thirty percent have chronic course, 5–18% mortality. Poorer out-
come associated with longer duration of illness, older age at onset,
prior psychiatric hospitalizations, poor premorbid adjustment, co-
404
PSYCHIATRY Eating Disorders
Acute
1. Hospitalize for starvation, dehydration, electrolyte imbalance,
hypotension, hypothermia, suicide risk.
2. Monitor and treat metabolic imbalances.
3. Strict enforcement of treatment contract for weight goal with
daily weights, nutritional supplements, intake–output, super-
diagnostic
decisions d
SIGNS AND SYMPTOMS OF SUBSTANCE INTOXICATION AND WITHDRAWAL
B. Bulimia Nervosa
H&P Keys
Recurrent binge eating (2 episodes/wk × 3 months) with lack of
control over eating; self-induced vomiting (70–95%), use of laxatives
or diuretics, strict dieting, vigorous exercise to prevent weight gain;
overconcern with body weight, which is usually normal. Onset late
adolescence, early adulthood, often during strict dieting; 95%
women; mid- to upper socioeconomic strata. Complications: dental
erosion and caries, parotid enlargement, calloused fingers, elec-
trolyte abnormalities (50%), dehydration, weakness, lethargy, GI
problems, cardiac arrhythmias; rarely, esophageal tears, gastric rup-
ture, pancreatitis, sudden death. Comorbid anorexia, depression,
personality disorders, stealing, substance abuse common.
Diagnosis
Rule out seizure disorder, CNS tumor, Klüver–Bucy and Kleine–
Levin syndromes; obtain ECG, electrolytes, amylase, LFTs.
Disease Severity
Chronic intermittent disorder with range of impairment.
Concept and Application
Genetic component; decreased CNS serotonin, NE activity; family
history of mood disorders, obesity, substance abuse.
Treatment Steps
1. Hospitalization for severe electrolyte abnormalities, suicide risk.
2. SSRIs reduce binging. Avoid bupropion.
3. CBT; individual, group, and family therapies.
4. Nutritional counseling, monitor weight.
X. SUBSTANCE-RELATED DISORDERS
A. Overview
Definitions
Abuse—Recurrent maladaptive pattern of use during 12-month
period despite physical hazard or legal, social, or occupational
problems.
Dependence—Psychological (craving) or physical (withdrawal syn-
drome, tolerance); loss of control over use; preoccupation with
obtaining and using substance; continued use despite adverse so-
cial, occupational, or health consequences. Frequent denial,
minimization.
Intoxication—Maladaptive behavior associated with recent inges-
tion.
406
PSYCHIATRY Substance-Related Disorders
Treatment Steps
Acute
1. Intoxication: observation and medical treatment for overdose,
multiple substance ingestion; protection from injury.
2. Withdrawal: symptomatic treatment; may need to detoxify (es-
pecially sedatives, alcohol, opioids) under medical supervi-
sion.
3. Diagnosis and treatment of concurrent nonpsychiatric med-
ical problems.
Continued Care
1. Confront denial.
2. Initiate and maintain abstinence (or substitution pharma-
cotherapy, e.g., methadone), through inpatient, residential,
partial and outpatient rehabilitation programs.
3. Individual, CBT, and group therapies; family therapy; self-help
groups, e.g., Alcoholics Anonymous (AA).
407
B. Alcohol Dependence
H&P Keys
Early: injuries, accidents, gastritis, diarrhea, headaches, insomnia,
absenteeism, blackouts, irritability. Later: nutritional deficiencies,
hepatitis, cirrhosis, GI bleeding, pancreatitis, heart disease, hyper-
tension, palmar erythema, acne rosacea, gynecomastia, testicular at-
rophy, peripheral neuropathy, GI cancers, cardiomyopathy, fetal al-
cohol syndrome (craniofacial abnormalities, mental retardation,
behavior problems, congenital malformations), depression, anxiety,
insomnia, neuropsychiatric disorders (below). Onset teens to 30s,
men > women, family history. Associated abuse of other substances.
Rule out underlying bipolar, anxiety, attention deficit, antisocial dis-
orders. Twenty to 40% of homeless have diagnosis.
Diagnosis
Clinical diagnosis; screen with CAGE (attempts to Cut down, Annoy-
ance with criticism of drinking, Guilt, morning Eye-opener; two posi-
tives suggest problem drinking, and three positives give 95% certain
diagnosis). Blood level of > 150 mg/dL without intoxication evi-
dence of tolerance. May have increased γ-glutamyl transferase
(GGT), mean corpuscular volume (MCV), asparatate transaminase
(AST), alanine transaminase (ALT), uric acid, alkaline phosphatase,
vertebral or rib fractures, abnormal liver, hepatic disease stigmata,
hypertension, peripheral neuropathy on exam.
Disease Severity
One-fourth to one-third have early onset; male, antisocial, family his-
tory; poor prognosis; responds best to structured milieu treatments.
Two-thirds to three-fourths have later, gradual onset; equal sex distri-
bution; better prognosis. Women have later onset but more virulent
course, family and personal history of mood disorder; complications
include motor vehicle accidents (MVAs), job loss, assaults, suicide,
falls (rule out subdural hematoma), fires, poisonings, drownings.
Concept and Application
Multiple causes: genetic factors, cultural patterns, learning. High
heritability in males; nondrinking sons of alcoholics have altered
evoked potentials, possibly greater tolerance.
Treatment Steps
Acute
1. Treat withdrawal, detoxification as below.
2. Diagnose and treat acute comorbid medical and psychiatric
problems.
Continued Care
1. Confront denial.
2. Inpatient or outpatient rehabilitation programs include group,
individual, family, 12-step AA, educational components.
3. Disulfiram, naltrexone, acamprosate, SSRIs used to promote
abstinence in some.
4. Anxiety, depression lasting more than 2–4 weeks past detoxifi-
cation may require specific treatment.
408
PSYCHIATRY Substance-Related Disorders
D. Drug Dependence
1. Stimulants (Amphetamines, Cocaine,
“Diet Pills,” Others)
Highly addictive; ingested, injected, snorted, purified to free-base
form and smoked (crack). Intoxication produces euphoria, alertness,
increased energy, anxiety or panic, talkativeness, psychomotor agita-
tion, impaired judgment, sexual arousal, anorexia, insomnia, pupil-
lary dilatation, hypertension, tachycardia; may progress to hyper-
pyrexia; nausea and vomiting; visual or tactile hallucinations
(“cocaine bugs”); paranoia; seizures, CVA, myocardial infarction
(MI), sudden cardiac death. Treat symptomatically, e.g., severe agita-
tion with benzodiazepine, tachyarrhythmia with antiarrhythmic;
acidify urine. Assess for suicidality. Delirium lasting 1–6 hours with ol-
factory or tactile hallucinations, may lead to seizures, death. Chronic
use and dependence associated with tolerance to euphoric effects; se-
vere social, financial, health losses including STD risk through IV
use, prostitution, poor judgment; weight loss; depression, irritability,
sexual dysfunction, memory impairment, paranoia, persecutory
delusions (delusional disorder). Withdrawal (crash) may be self-treated
with sedatives, e.g., alcohol, marijuana; associated with insomnia or
hypersomnia, hunger, fatigue, dysphoria, agitation, anxiety, suicidal
ideations, craving. Not physically dangerous; assess suicide risk, treat
supportively, refer for drug treatment. Treat depression persisting
> 2–4 weeks after withdrawal.
3. Sedative–Hypnotic Dependence
(Benzodiazepines, Barbiturates,
Methaqualone, Others)
Young, polydrug abusers (frequently combined with alcohol, opi-
oids, stimulants) or middle-aged women with iatrogenic depen-
dence. Dependence produces tolerance to euphoriant and sedative ef-
fects, fatigue, psychomotor impairment, amnesia, depression,
headaches, GI disturbances; intoxication causes slurred speech,
drowsiness, incoordination, ataxia, impaired attention and memory,
disinhibition. Flumetrazepam (“roofies”) associated with date rape.
Barbiturates have low therapeutic index, frequently used in suicide;
overdose causes respiratory depression, coma; gastric lavage, charcoal,
monitor closely, maintain airway and blood pressure. Benzodi-
azepines have high therapeutic index but may be lethal in combina-
tion with other CNS sedatives. Flumazenil (benzo antagonist) does
not reverse respiratory inhibition. Mild withdrawal syndrome of anxi-
ety, insomnia, headache, anorexia, dizziness common. Severe with-
drawal syndrome is a medical emergency: nausea, vomiting, malaise,
autonomic hyperactivity, anxiety, photophobia, tremor, hyper-
reflexia, hyperthermia, insomnia, delirium, seizures, death; short-
acting drugs cause most severe syndrome. Pentobarbital challenge
test determines substitute phenobarbital or long-acting benzodi-
azepine dose for gradual withdrawal. Pentobarbital 200 mg PO, then
100 mg q2h (max 500 mg) until intoxication observed; substitute
phenobarbital, 30 mg/each 100 mg pentobarbital; taper ≈ 10%/day,
adjust for signs of intoxication, withdrawal.
4. Cannabinoids (THC, Marijuana, Hashish,
Bhang, Ganja)
Intoxication may produce euphoria or dysphoria, heightened sensa-
tion, time distortion, increased humorousness, impaired judgment,
dry mouth, increased appetite, pupillary dilatation, conjunctival in-
jection, suspiciousness, anxiety, tachycardia, depersonalization, inco-
ordination; rarely hallucinations, mild persecutory delusions. Very
high doses may cause prolonged psychosis, mild delirium, panic.
Chronic use: apathetic, amotivational syndrome, memory impair-
ment, depression, anxiety, respiratory and reproductive problems.
No characteristic withdrawal syndrome. Urine toxicology positive up
to 4 weeks after cessation of heavy use.
5. Hallucinogens (LSD, Mescaline, DMT, Psilocybin,
MDMA, Others)
Eaten, sucked from paper, smoked; intoxication produces halluci-
nosis, sympathomimetic effects (pupillary dilatation, tachycardia, di-
aphoresis), perceptual changes, emotional intensity and lability. May
cause depression, paranoia, panic reactions with belief that percep-
tions are real (“bad trips”); treat with reassurance, safe environment;
benzodiazepines, antipsychotics for severe symptoms. Prolonged psy-
chosis may develop in vulnerable patients; posthallucinogen percep-
tion disorder (“flashbacks”) distressing persistent reexperiencing of
hallucinations with intact reality testing. Low-dose benzodiazepine
acutely, antipsychotic if persistent.
6. Phencyclidine (PCP, Angel Dust)
Hallucinogen, smoked with marijuana, eaten, injected, snorted; eu-
phoriant, commonly causes unpredictable, paranoid, agitated, as-
saultive behavior; accompanied by dysarthria, diaphoresis, vertical
and horizontal nystagmus, hypertension, tachycardia, analgesia, in-
411
cram facts U ders, hepatic or other systemic disease, surgical procedures; medica-
tions, commonly antihypertensives, anticholinergics, antihistamines,
ADs, antipsychotics, steroids. Spontaneous erections, morning erec-
tions, erections with masturbation rule out organic etiology of impo-
tence. Complete gynecologic or urologic exam, measurement of
SAFE SEX GUIDELINES
nocturnal penile tumescence, pudendal nerve latency, penile blood
pressure, serum glucose, LFTs, TFTs, prolactin, luteinizing hormone
Safe Sex Practices
• Masturbation
(LH), FSH as indicated.
• Dry kissing
Disease Severity
• Hugging, massage
• Use of unshared Primary and chronic disorders more difficult to treat. May have his-
vibrators, sex toys tory of sexual victimization.
Low-Risk Sex Practices
Concept and Application
• Wet kissing without
mouth sores
Illnesses, substance abuse, or medications that interfere with normal
• Mutual masturbation endocrine, neural, and vascular systems may produce sexual dys-
• Intercourse (vaginal or function. Psychological etiologies include ignorance and misinfor-
anal) with condom mation; unconscious guilt, anger and anxiety; conditioned re-
• Oral sex with use of sponses; performance anxiety or fear of rejection; lack of
barrier communication between partners; physical and psychological
• Skin contact with semen,
stresses.
urine with no skin sores
or breaks Treatment Steps
Unsafe Sex Practices 1. Rule out medical or substance-related etiology.
• Intercourse (vaginal or 2. Cognitive therapy, specific behavioral therapies, marital therapy,
anal) without condom
education (e.g., clitoral stimulation for orgasm in women). Be-
• Oral sex without barrier
• Blood contact
havioral therapies (e.g., sensate focus for erectile dysfunction,
• Sharing sex instruments squeeze technique for premature ejaculation, directed masturba-
• Semen, urine, feces in tion for anorgasmia) desensitize patient and decrease perfor-
mouth, vagina mance anxiety.
3. Somatic treatments include alprostadil injections, sildenafil and
others for impotence (contraindicated with use of nitrates, heart
disease), hormone replacement therapy (HRT), SSRIs for prema-
ture ejaculation.
XII. DELIRIUM
H&P Keys
Syndrome of global cognitive impairment with reduced attention;
disorganized thought with rambling or incoherent speech; reduced
and fluctuating level of consciousness (clear, drowsy, stupor, coma);
413
PSYCHIATRY Delirium
diagnostic decisions d
DELIRIUM VERSUS DEMENTIA
Delirium Dementia
Rapid onset Insidious onset
Fluctuating, clouded consciousness Clear sensorium until late in course
Often reversible Most irreversible and progressive
Perceptual disturbances, sleep–wake cycle These symptoms uncommon until late in
abnormalities, incoherent speech common course
Diagnosis
Rule out unintentional trauma, bleeding diathesis, dermatologic
conditions, vitamin deficiencies, osteogenesis imperfecta, self-
inflicted injuries; thorough physical exam; skeletal series, serologies,
bleeding screening, CBC, creatine kinase (CK) as indicated.
Disease Severity
Deaths usually occur only after numerous episodes; psychiatric se-
quelae (PTSD, depression, substance abuse, personality disorders,
dissociative identity disorder, sexual dysfunction, somatic com-
plaints, repetition of abuse, suicidal or self-destructive behavior)
worse with early-age onset, chronicity, severe abuse, use of force,
multiple perpetrators, abuse by parental figure, lack of support.
Treatment Steps
Acute
1. Interview child alone; expert may be necessary to elicit abuse
history; interview parents separately.
2. Document findings, including pictures.
3. All states mandate reporting of suspected abuse (physical,
emotional, sexual, severe neglect).
4. Ensure safety of child.
Continued Care
1. Treatment of child for physical, emotional sequelae; individ-
ual, group psychotherapy.
415
PSYCHIATRY Bereavement
2. Treatment for abusers ranges from support (emotional sup-
port, social services, education, Parent’s Anonymous groups,
hotlines) through mandated therapy, removal of parent
abuser or child, legal prosecution.
C. Elder Abuse
Abuser generally relative/caretaker; victim may fear disclosure due
to dependency; family system with frustration, financial or health
stress, substance abuse, history of violence; previous injuries, physi-
cal deterioration: bruising, head injury, burns, decubiti, contrac-
tures, dehydration, lacerations, diarrhea, impaction, malnutrition,
urine burns, signs of neglect, sexual assault, PTSD symptoms. Inter-
view privately; social service for assessment of living situation;
mandatory reporting in most states.
XIV. BEREAVEMENT
A. Uncomplicated Bereavement
Normal reaction to death of loved one or other significant loss;
acute grief characterized by intense emotional distress, somatic
symptoms, dissociation, preoccupation with deceased, anger, loss of
416
PSYCHIATRY Bereavement
diagnostic decisions d
DEPRESSION VERSUS BEREAVEMENT
Depression Bereavement
Mood pervasive, unremitting Mood fluctuates
Pervasive low self-esteem, worthlessness Self-reproach regarding deceased
May be suicidal Usually not suicidal
May have sustained psychotic symptoms May transiently hear voice or see image of
deceased
Does not improve without treatment; Symptoms improve with time; severe symptoms
average episode 6–9 months usually gone by 2–6 months
Social withdrawal Often welcomes social support
BIBLIOGRAPHY
American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders.
4th ed. Text Revision. Washington, DC: American Psychiatric Association, 2000.
Fauman MA. Study Guide to DSM-IV-TR. Washington, DC: American Psychiatric Press,
2002.
Kaplan HI, Sadock BJ. Synopsis of Psychiatry, 9th ed. Baltimore: Williams & Wilkins,
2002.
Manley MRS. Psychiatric Clerkship Guide. St. Louis: Mosby, 2003.
Taylor MA. The Fundamentals of Clinical Neuropsychiatry. New York: Oxford University
Press, 1999.
Pulmonary Medicine 16
I. INFECTIOUS DISORDERS / 419
A. Croup / 419
B. Acute Epiglottitis / 419
C. Acute Bronchitis / 419
D. Acute Bronchiolitis / 420
E. Pertussis / 420
F. Bacterial Bronchopneumonia / 421
G. Atypical Pneumonias / 422
H. Pulmonary Tuberculosis / 424
I. Fungal Pneumonias / 424
Copyright © 2004, 2001 by The McGraw-Hill Companies, Inc. Click here for terms of use.
418 Pulmonary Medicine
BIBLIOGRAPHY / 445
419
A. Croup
H&P Keys
Children under 6 years old following upper respiratory illness. Bark-
ing cough, inspiratory stridor, dyspnea, hoarseness, usually worse at
night.
Diagnosis
X-ray or magnetic resonance imaging (MRI) of upper airway (glottic
and subglottic swelling). Diagnosis usually clinical.
Disease Severity
Respiratory rate, pulse oximetry, accessory muscle use, stridor.
B. Acute Epiglottitis
H&P Keys
Children < 7 years of age most common. High fever, stridor, dys-
pnea, hoarseness, dry cough, drooling, dysphagia, systemic toxicity,
cherry-red epiglottis.
Diagnosis
Lateral neck x-ray (enlarged epiglottis), blood culture, throat cul-
ture (but see below).
Disease Severity
X-ray findings, pulse oximetry, clinical distress, accessory muscle use.
Concept and Application
Edema of epiglottis obstructing upper airway. Etiologic agent usually
Haemophilus influenzae type b.
Treatment Steps
1. Antibiotics active against H. influenzae (cefuroxime, ampicillin
plus clavulanic acid).
2. Endotracheal intubation or tracheostomy in severe cases.
Note: Airway examination and throat culture may provoke laryn-
gospasm and cardiopulmonary arrest!
C. Acute Bronchitis
H&P Keys
Severe, prolonged productive cough, fever, dyspnea.
Diagnosis
History and physical, sputum culture, chest x-ray (CXR) to rule out
bronchopneumonia.
420
PULMONARY MEDICINE Infectious Disorders
Disease Severity
Respiratory rate, temperature.
Concept and Application
Infection and inflammation of large airways. Usually viral (influenza,
adenovirus). May be bacterial (Mycoplasma pneumoniae, Bordetella per-
tussis, H. influenzae, Streptococcus pneumoniae).
Treatment Steps
1. Hydration, cough suppressants.
2. If bacterial: broad-spectrum oral antibiotic (e.g., macrolide, tetra-
cycline derivative, broad-spectrum quinolone).
D. Acute Bronchiolitis
H&P Keys
Children < 2 years old following upper respiratory infection; tachy-
pnea, inspiratory and expiratory wheezing, intercostal and supra-
sternal retractions, nasal flaring, hyperresonant chest, inspiratory rales.
Diagnosis
CXR: hyperinflated lungs, peribronchial thickening; may have con-
current bronchopneumonia. Normal white blood cell (WBC) count.
Inspiratory “click.”
Disease Severity
Respiratory rate, intercostal retractions, pulse oximetry.
Concept and Application
Acute inflammation of small airways causing hyperinflation, obstruc-
tion, and atelectasis. Majority associated with RSV.
Treatment Steps
Oxygen, hydration, aerosol ribavirin for RSV.
E. Pertussis
H&P Keys
Usually occurs in infants < 2 years old.
Catarrhal Stage—Lasts 1–2 weeks. Presents similarly to viral illness:
low-grade fever, injected conjunctiva.
Paroxysmal Stage—Lasts 2–4 weeks. Severe, paroxysmal, short
coughs with inspiratory “whoop.” Thick, tenacious secretions,
usually afebrile.
Diagnosis
Nasopharyngeal culture (requires special medium), elevated white
blood cell (WBC) count (mostly lymphocytes); increased polymor-
phonuclear neutrophils (PMNs) suggest bacterial superinfection.
Disease Severity
WBC count. Presence of bacterial superinfection.
Concept and Application
Infection of tracheobronchial tree with Bordetella pertussis. In severe
cases, mucopurulent exudate obstructs small airways.
Treatment Steps
1. Macrolide antibiotic in catarrhal stage (does not help in paroxys-
mal stage).
2. Supportive care.
3. Antibiotics for treatment of superinfection if present.
421
Diagnosis
CXR (lobar infiltrate), sputum Gram stain, sputum culture, blood
culture, WBC.
Disease Severity
Pulse oximetry, arterial blood gases (ABG), tachypnea, CXR. Multi-
lobed involvement, low WBC count, positive blood culture, and
older age associated with worse prognosis.
Treatment Steps
1. Begin antibiotic immediately (penicillin G, erythromycin, broad-
spectrum quinolone). Use vancomycin or quinolone if penicillin
resistance is present or suspected.
2. Chest tube drainage if empyema present. Pneumococcal vaccine
for high-risk individuals after acute episode resolves.
2. Staphylococcal Pneumonia
H&P Keys
Fever, dyspnea, cough with purulent sputum.
Diagnosis
CXR (multifocal infiltrates, abscess, pneumatocele, effusions), other
tests as above for pneumonia.
Disease Severity
Pulse oximetry, ABG, tachypnea, CXR. Metastatic infection (central
nervous system [CNS], bone, endocarditis, sepsis).
Treatment Steps
1. Begin antibiotic immediately (β-lactamase–resistant penicillin; if re-
sistant: vancomycin or linezolid).
2. Oxygen if hypoxic.
3. Haemophilus influenzae Pneumonia
H&P Keys
Young children, chronic lung disease, alcoholics. Fever, cough, dys-
pnea, purulent sputum. Subacute presentation over several weeks
possible.
422
PULMONARY MEDICINE Infectious Disorders
Diagnosis
CXR (multilobar patchy infiltrates), as above for pneumonias.
Disease Severity
Pulse oximetry, ABG, respiratory rate, CXR. Empyema is rare.
Concept and Application
Pulmonary infection caused by H. influenzae. More common in
COPD and smokers.
Treatment Steps
Begin antibiotic immediately (broad-spectrum quinolone if > 18
years of age, cefuroxime, ampicillin/clavulanic acid, others).
4. Gram-Negative Bacillary Pneumonias
H&P Keys
Usually nosocomial, fever, chills, dyspnea, cough productive of puru-
lent and sometimes bloody sputum.
Diagnosis
CXR (lobar or multilobar, cavitary infiltrates), as above for pneumo-
nia.
Disease Severity
Pulse oximetry, ABG, respiratory rate, CXR (multilobed involvement
and cavitation). High mortality.
Concept and Application
Aspiration of gram-negative bacilli from colonized oropharynx. Kleb-
siella pneumoniae, Acinetobacter and Pseudomonas species, Enterobacte-
riacae genera; common in immunocompromised and hospitalized
patients. Klebsiella common in alcoholics.
Treatment Steps
1. Third-generation cephalosporin, semisynthetic penicillin (ti-
carcillin, piperacillin), or carbopenem plus either an aminoglyco-
side or a broad-spectrum quinolone.
2. Check antibiotic sensitivities and adjust for resistance.
G. Atypical Pneumonias
1. Legionnaire’s Disease
H&P Keys
Lethargy, headache, fever, rigors, anorexia, myalgias, nonproductive
cough, GI symptoms. Rales and rhonchi, abdominal tenderness, rel-
ative bradycardia.
Diagnosis
CXR (lobar, nodular, or patchy subsegmental), low sodium and
phosphate, elevated WBC. Sputum culture (requires special media),
serologic titers, urinary antigen.
Disease Severity
Symptoms, respiratory rate, pulse oximetry, ABG, CXR.
Disease Severity
Respiratory rate, clinical appearance.
Concept and Application
Viral pneumonia caused by influenza A.
Treatment Steps
1. Zanamivir, oseltamivir, or rimantadine if administered within 48
hours of onset; otherwise, symptomatic treatment.
2. Prophylactic influenza vaccine for high- and moderate-risk groups.
3. Consider postexposure prophylaxis of high-risk patients with
zanamivir, oseltamivir, rimantadine, or amantadine.
H. Pulmonary Tuberculosis
H&P Keys
Fever, malaise, weight loss, dyspnea, night sweats; productive cough
with hemoptysis, rales in area of involvement; amphoric breath
sounds may indicate cavity.
Diagnosis
CXR (upper-lobe cavitary disease if reactivation, lower-lobe infil-
trates in primary infection, upper-lobe scarring may indicate latent
infection). Sputum culture, acid-fast smear; bronchoscopy if unable
to get diagnosis on sputum studies. Purified protein derivative
(PPD) (tuberculin) skin test.
Disease Severity
CXR. Extrapulmonary involvement (lymphatic, pleural, peritoneal,
genitourinary, miliary, bone and joint, meningeal) may be more
problematic.
Concept and Application
Inhalation of Mycobacterium tuberculosis leads to primary lower-lobe
infection. Localized inflammatory response usually halts infection.
Reactivation disease occurs in upper lobes of lung or other areas of
high oxygen content.
Treatment Steps
I. Fungal Pneumonias
1. Histoplasmosis
H&P Keys
Mostly asymptomatic, can have abrupt onset of flulike illness, with
fever, chills, substernal chest pain, nonproductive cough with myal-
gias, arthralgias, and headache.
Diagnosis
CXR: acute disease often normal, hilar adenopathy with lower-lobe
alveolar infiltrates, leading to chronic calcification). Progressive
425
Sources: American Thoracic Society (ATS)/Centers for Disease Control and Prevention (CDC). Diagnostic standards and
classifications of tuberculosis in adults and children. Am J Respir Crit Care Med 161:1376–1395, 2000; and ATS/CDC. Targeted
tuberculin testing and treatment of latent tuberculosis infection. Am J Respir Crit Care Med 161 (4 Pt 2):S221–47, 2000.
3. Coccidioidomycosis
H&P Keys
Cough, fever, pleuritic chest pain, headache (may be indicative of
meningitis); erythematous rash, “valley fever”: erythema nodosum,
erythema multiforme, arthralgias.
Diagnosis
CXR (patchy pneumonitis, hilar adenopathy, “coin lesions,” cavitary
lesions), KOH preparation of sputum, lung biopsy, complement fixa-
tion, skin test.
Disease Severity
Disseminated disease (meningitis, skin lesions, bone, etc.). Dissemi-
nation more common in AIDS, steroids, malignant disease, African-
Americans, Native Americans, Mexicans.
Concept and Application
Infection with Coccidioides immitis. Spherules are pathogenic form.
Mostly mild, self-limited, but can be life threatening and dissemi-
nated. Southwestern United States and California valley regions,
northern Mexico.
Treatment Steps
Nonmeningeal Disease
1. Fluconazole or amphotericin B.
2. Alternative: ketoconazole, itraconazole.
Disseminated Disease or Meningitis
1. Fluconazole, miconazole, or amphotericin B.
2. Intrathecal amphotericin if fluconazole fails.
4. Cryptococcosis
H&P Keys
Disease Severity
Disseminated disease (CNS), presence of meningitis, CXR.
427
16-2
CHARACTERISTIC CHANGES IN LUNG VOLUMES AND FLOW RATES IN PATIENTS
WITH RESTRICTIVE, OBSTRUCTIVE, AND COMBINED VENTILATORY DISORDERS
B. Asthma
H&P Keys
Acute onset of dyspnea, wheezing, cough that remit spontaneously
or with treatment.
Diagnosis
PFT (obstructive), response to bronchodilators, response to bron-
choconstricting provocational agents.
Disease Severity
PFT, use of accessory muscles, ABG, paradoxical pulse, respiratory
rate, pulse oximetry, symptoms.
Concept and Application
Bronchospasm, inflammation, hyperreactivity to inhaled antigens
and irritants, mucus plugging. Obstruction may improve to normal
with treatment.
Treatment Steps
Acute
1. Bronchodilators:
• Inhaled β-agonist, inhaled ipratropium bromide.
• If fails, consider subcutaneous epinephrine, and/or intra-
venous aminophylline.
2. Anti-inflammatory agents: systemic corticosteroids; use if se-
vere.
Chronic
1. Bronchodilators: β-Agonists (inhaled, subcutaneous, oral) as
“rescue,” theophylline.
2. Anti-inflammatory agents:
• Inhaled corticosteroids, cromolyn sodium, nedocromil
sodium, or oral leukotriene blocker (montelukast, zafir-
lukast). Omalizumab (anti–immunoglobulin E [IgE] anti-
body) if sensitivity to perennial aeroallergens and high IgE
level.
• Choose one as “controller”; may add a second for poor con-
trol. Combining inhaled steroid with long-acting inhaled
β-agonist improves control.
3. Avoidance of causative agents.
429
1. Chronic Bronchitis
H&P Keys
Defined as presence of chronic productive cough for 3 months in 2
successive years without other discernible cause. Dyspnea, recurrent
productive cough, “blue bloater”; cyanosis with edema, wheezes.
Diagnosis
PFTs (obstructive), history, CXR (usually clear or hyperinflated).
Sputum cultures for acute exacerbations.
Disease Severity
PFTs, pulse oximetry, ABG.
Treatment Steps
1. Smoking cessation.
2. Ipratropium bromide.
3. Add inhaled β-agonist bronchodilator if needed.
4. Influenza and pneumococcal vaccination.
5. Antibiotics for acute bacterial exacerbation.
6. Oxygen if saturaton ≤ 88% or PO2 ≤ 55 (or ≤ 60 with cor pul-
monale).
7. Corticosteroids if severe.
2. Emphysema
H&P Keys
Dyspnea, wheezing, cough. “Pink puffer”: thin, not cyanotic, tachy-
pneic. Diminished breath sounds, hyperinflated chest, hyperresonant
to percussion.
Diagnosis
Obstructive PFTs (TLC may be increased), CXR, history, and physi-
cal. Serum protein electrophoresis or α1-protease inhibitor level if de-
ficiency suspected.
Disease Severity
PFTs, ABG, exercise tolerance.
Treatment Steps
Same as for chronic bronchitis, above. α1-protease inhibitor if de-
ficiency proven.
3. Cystic Fibrosis (CF)
H&P Keys
Persistent cough, recurrent pneumonia and bronchitis, recurrent
abdominal pain, meconium ileus, failure to thrive, steatorrhea, in-
fertility, diabetes, family history. Usually diagnosed in childhood.
Diagnosis
Sweat chloride test (> 60 mEq/L before age 20; < 80 in adults) diag-
nostic. Genetic testing available for more common genotypes of CF.
Mucoid Pseudomonas lung infection, unexplained azoospermia, and
obstruction on PFTs suggests diagnosis. CXR (hyperinflation, en-
larged pulmonary arteries, bronchiectasis, cystic areas).
Disease Severity
PFTs, pulse oximetry, ABG, CXR.
Concept and Application
Autosomal recessive disorder; CF gene locus located on long arm of
chromosome 7; genetic defect in chloride permeability in exocrine
glands due to cystic fibrosis transmembrane regulator protein
(CFTR); affects all exocrine secretions; frequent Pseudomonas and
Staphylococcus infections.
Treatment Steps
1. Chest physiotherapy.
2. Inhaled β-agonist and mucolytic agents (DNase, acetylcysteine).
3. Antibiotics prn; aerosol tobramycin for chronic Pseudomonas.
4. Influenza vaccine.
5. Pancreatic enzymes.
6. Lung transplant in selected cases.
4. Bronchiectasis
H&P Keys
Chronic cough, copious purulent sputum, recurrent fever, weakness,
weight loss, hemoptysis, clubbing, cyanosis, edema.
Diagnosis
History, CXR, high-resolution computed tomographic (CT) scan,
bronchography (rarely used), sputum culture.
Disease Severity
PFTs (obstructive), stigmata of cor pulmonale.
Concept and Application
Abnormal dilatation of bronchi from inflammation and destruction
of bronchial wall. Often associated with infection, bronchial obstruc-
tion, immotile-cilia syndrome, CF, allergic bronchopulmonary as-
pergillosis, immunoglobulin deficiency.
Treatment Steps
1. Chest physiotherapy.
2. Antibiotics for infection.
3. Steroids for allergic bronchopulmonary aspergillosis if present.
4. Surgery or bronchial artery embolization for severe hemoptysis.
431
A. Idiopathic
1. Sarcoidosis
H&P Keys
May be asymptomatic. Dyspnea, cough, wheezing, hemoptysis, skin
lesions (erythema nodosum, nodules, plaques), eye pain, arthralgias,
cardiac arrhythmias, cranial nerve palsies. More common in African-
Americans, Scandinavians.
Diagnosis
CXR (bilateral hilar adenopathy, interstitial lung disease), tissue
biopsy (noncaseating granuloma). Elevated serum calcium or an-
giotensin-converting enzyme suggestive. Exposure history to rule out
occupational granulamatous disease or intravenous injection of for-
eign matter (i.e., talc).
Disease Severity
PFTs (restriction, low diffusion), CXR, gallium scan (reflects disease
activity); presence of CNS and cardiac involvement.
Treatment Steps
1. May be self-limited.
2. Corticosteroids for significant pulmonary, CNS, or cardiac dis-
ease.
3. Hydroxychloroquine or topical steroids for skin involvement.
4. Alternative agent: methotrexate.
2. Idiopathic Pulmonary Fibrosis
H&P Keys
Insidious onset of dyspnea (may progress over many years), nonpro-
ductive cough, clubbing, fine crackles.
Diagnosis
CXR (diffuse interstitial infiltrates), lung biopsy, PFTs (restriction,
low diffusion).
Disease Severity
PFTs, high-resolution chest CT scan, ABG.
Treatment Steps
1. Corticosteroids.
2. Treat cor pulmonale with diuretics, oxygen.
3. Lung transplant.
b. Desquamative Interstitial Pneumonitis (DIP)—Predomi-
nant alveolar component, more responsive to drugs, mostly in smok-
ers.
432
PULMONARY MEDICINE Restrictive Pulmonary Diseases
Diagnosis
CXR (diffuse interstitial infiltrates), lung biopsy, PFTs (restriction,
low diffusion).
Disease Severity
PFTs, high-resolution chest CT scan, ABG.
Treatment Steps
1. Stop smoking.
2. Corticosteroids.
3. Treat cor pulmonale with diuretics, oxygen.
B. Pneumoconiosis
1. Silicosis
H&P Keys
Sandblasters, miners, stoneworkers. Asymptomatic, or progressive
dyspnea, cough.
Diagnosis
Exposure history, CXR (upper lobe nodules, “eggshell calcification”
of hilar nodes), PFTs (restriction).
Disease Severity
CXR and PFTs. Progressive massive fibrosis: coalescence of small
nodules into larger conglomerate lesions.
Concept and Application
Inhalation of quartz particles causes reactive fibrosis. Increased risk
for tuberculosis.
Treatment Steps
1. Avoidance of further exposure.
2. No medical treatment known to be effective.
3. Lung transplantation in severe cases.
2. Asbestosis
H&P Keys
History of asbestos exposure (mining, shipbuilding, insulation work-
ers, construction). Asymptomatic, or progressive dyspnea, persistent
cough, basilar inspiratory crackles, clubbing.
Diagnosis
Clinical diagnosis: interstitial disease with exposure history. CXR
(lower-lobe linear infiltrates, pleural plaques and thickening); high-
resolution chest CT scan, PFTs (restriction), ferruginous bodies (he-
mosiderin-coated asbestos fibers) in sputum, alveolar lavage fluid, or
lung tissue suggestive but not diagnostic.
Disease Severity
CXR, PFTs.
Concept and Application
Inhalation of asbestos fibers releases damaging enzymes and inflam-
matory mediators. Increased risk of lung cancer, especially in smok-
ers.
Treatment Steps
1. Avoidance of further exposure.
2. Stop smoking.
433
H&P Keys
Coal miners, carbon manufacturers.
Simple Coal Workers’ Pneumoconiosis—Asymptomatic radiographic
finding.
Complicated Coal Workers’ Pneumoconiosis (Progressive Massive Fibrosis—
PMF)—Dyspnea, signs of cor pulmonale.
Chronic Obstructive Lung Disease—Dyspnea, wheezing, productive
cough.
Diagnosis
Occupational exposure, CXR (small round opacities, usually upper
lobes), PFTs. X-ray findings are not necessary for the diagnosis.
Disease Severity
PFTs (usually normal in simple disease; restrictive in PMF, obstruc-
tive in COPD), CXR. Simple has small round opacities; PMF has en-
larging, irregular nodular infiltrates, which may cavitate.
Treatment Steps
1. Avoidance.
2. Surveillance for COPD, PMF, tuberculosis.
3. Stop smoking.
4. Treatment of COPD if present.
4. Hypersensitivity Pneumonitis
H&P Keys
Diagnosis
Exposure history key to diagnosis. Common antigens include ther-
mophilic Actinomyces (farmer’s lung: moldy hay; bagassosis: moldy
sugar cane); avian secretory proteins (pigeon breeder’s disease).
Serum precipitins can help identify agent but are not diagnostic of
disease. High-resolution CT pattern suggestive.
434
PULMONARY MEDICINE Pleural Diseases
Disease Severity
CXR (interstitial and alveolar infiltrates); PFTs (restriction, de-
creased diffusion).
Concept and Application
Immune complex–mediated and cell-mediated hypersensitivity re-
sponses to inhaled antigen.
Treatment Steps
1. Removal and avoidance of offending antigen.
2. Corticosteroids for severe attacks or if symptoms persist.
A. Pleural Effusion
H&P Keys
Asymptomatic or signs and symptoms of underlying cause. Dys-
pnea, pleuritic chest pain. Decreased breath sounds, dullness to per-
cussion.
Diagnosis
CXR with lateral decubitus views. Pleural fluid chemistries (LDH,
protein, glucose) (Table 16–3), cell count, cultures.
Disease Severity
CXR.
Concept and Application
16-3
CHARACTERISTIC CHEMISTRIES OF PLEURAL EFFUSIONS
Exudate Transudate
B. Pleurisy (Pleuritis)
H&P Keys
Pleuritic chest pain (sharp pain on inspiration), usually rapid onset,
dyspnea, low-grade fever.
Diagnosis
CXR, history and physical.
Disease Severity
Usually self-limited.
Treatment Steps
1. Nonsteroidal anti-inflammatory agents.
2. If fails—corticosteroids if indicated.
C. Pneumothorax
H&P Keys
Chest pain, dyspnea, enlarged hemithorax, hyperresonance to per-
cussion, absent or reduced breath sounds and fremitus on involved
side, tracheal shift away from involved side.
Diagnosis
CXR (air in pleural space).
Disease Severity
CXR, pulse oximetry and ABG, pulse rate, respiratory rate, blood
pressure.
Treatment Steps
1. Observation if stable.
2. Chest tube if large, symptomatic, or if tension pneumothorax is
present or suspected.
436
PULMONARY MEDICINE Diseases of Pulmonary Circulation
Diagnosis
CXR (diffuse granular or ground-glass infiltrate), pulse oximetry,
ABG.
Disease Severity
Clinical appearance of respiratory distress, ABG.
Treatment Steps
1. Prevention of premature labor and use of prenatal steroid ther-
apy.
2. Oxygen.
3. Continuous distending airway pressure (CDAP or CDP).
4. Mechanical ventilation.
5. Surfactant replacement therapy.
D. Pulmonary Embolism
H&P Keys
Acute onset of dyspnea and pleuritic chest pain. Cough, hemoptysis,
tachypnea, tachycardia; look for pleural rub, edema, or tenderness
in lower extremity (Fig. 16–1).
Diagnosis
ABG (acute respiratory alkalosis, usually decreased PO2), CXR (clear,
or wedge-shaped infiltrate, effusion), ECG (sinus tach, or S1, Q3, T3
inversion pattern), ventilation perfusion lung scan (perfusion defect
with normal ventilation), noninvasive (e.g., duplex ultrasound) or
venogram studies for deep vein thrombosis (DVT) in lower extremi-
ties, pulmonary angiogram, contrast helical “spiral” CT, quantitative
D-dimer levels (if < 500 ng/mL, PE unlikely).
Disease Severity
ABG, vital signs.
Treatment Steps
1. Anticoagulation (heparin, then warfarin for 3–6 months).
2. Thrombolytic agents for massive embolism with shock.
3. Embolectomy in unresponsive cases.
4. Vena caval interruption if anticoagulation is contraindicated
(vena caval clip, Greenfield filter, others).
5. Prophylaxis of high-risk patients with subcutaneous low-dose
heparin, warfarin, or compression boots.
438
PULMONARY MEDICINE Diseases of Pulmonary Circulation
Clinical Suspicion
Low Intermediate or
High
Treat
Follow Treat
Figure 16–1. Management of Acute Pulmonary Thromboembolism. (Adapted, with permission, from Schulman ES. Management of acute pulmonary thromboem-
bolism. In: Sherman MS, Schulman ES, eds. The Pocket Doctor 2001. Educational Communications, Mt. Kisko, NY, 2001 and www.pocketdoctor.com.)
E. Pulmonary Vasculitis
H&P Keys
Dyspnea; symptoms of underlying disease process.
Diagnosis
CXR, ABG, urinalysis, creatinine, serologic studies for systemic vas-
culitic processes (ANA, ANCA, hepatitis B and C, rheumatoid factor,
cryoglobulins, complement). Tissue biopsy usually required.
Disease Severity
ABG, exercise tolerance, serologic studies of underlying disease.
Concept and Application
Inflammation of pulmonary blood vessels.
Leukocytoclastic Vasculitis—Neutrophilic inflammation caused by
drugs, neoplasms, infection.
439
Treatment Steps
Leukocytoclastic Vasculitis
1. Usually self-limited.
2. Steroids if hypoxemia present.
Wegener’s Granulomatosis—Cyclophosphamide with or without corti-
costeroids.
Allergic Granulomatosis, Collagen Vascular Diseases
1. Corticosteroids; azathioprine or cyclophosphamide in severe
cases.
2. Progressive systemic sclerosis usually does not respond.
F. Goodpasture’s Syndrome
H&P Keys
Hemoptysis, hematuria, fever, dyspnea.
Diagnosis
Antiglomerular basement membrane antibodies, renal biopsy (lin-
ear immunofluorescence pattern).
Disease Severity
Amount of hemoptysis, CXR, blood urea nitrogen (BUN), creati-
nine, ABG.
Treatment Steps
Corticosteroids plus cyclophosphamide or azothioprine; plasma-
pheresis.
G. Cor Pulmonale
Defined as right ventricular failure secondary to pulmonary disease.
H&P Keys
Breathlessness, hepatic discomfort, symptoms of underlying disease.
Right ventricular heave, loud split S2, peripheral edema, ascites.
Diagnosis
CXR (large pulmonary artery, right ventricular hypertrophy
[RVH]); ECG (RVH, pulmonale), echocardiogram (RVH, elevated
pulmonary artery pressures), PFTs (reflect underlying disease).
Disease Severity
ABG, cardiac catheterization (direct measurement of pulmonary
artery pressures), symptoms, and exercise tolerance.
Treatment Steps
1. Oxygen, diuretics.
2. Treatment of underlying disease.
3. Endothelin receptor antagonist (Bosentan) in selected cases (i.e.,
primary pulmonary hypertension or scleroderma pulmonary vas-
culopathy).
A. Bronchogenic Carcinoma
H&P Keys
Smoking, asbestosis produce highest risk. Asymptomatic or cough,
hemoptysis, dyspnea, chest pain, fever, weight loss, hoarseness, focal
wheezing or decreased breath sounds, adenopathy.
Diagnosis
CXR (coin lesion or mass, adenopathy), CT scan, bronchoscopic or
percutaneous needle biopsy or cytologic aspiration.
Disease Severity
Severity depends on stage, cell type, and patient’s general condition.
Concept and Application
Non–Small Cell
1. Staging, surgery if resectable.
2. Radiation for palliation.
Small Cell
1. Chemotherapy.
2. Rarely resectable (usually metastatic when discovered).
All
1. May metastasize to other lung, mediastinum, brain, adrenal
glands, other organs.
2. Watch for superior vena cava syndrome, hypercalcemia.
B. Carcinoid Tumors
H&P Keys
Asymptomatic or wheezing, cough, hemoptysis, obstruction. Carci-
noid syndrome (episodic flushing, bronchospasm, and diarrhea) is
rare.
Diagnosis
CXR (clear, mass, or obstructive pneumonia). Lung biopsy, urine
5-hydroxyindoleacetic acid.
441
A. Cough
H&P Keys
Smoking history, sputum characteristics (color, viscosity), postnasal
drip, throat clearing, reflux symptoms, wheezing, occupational his-
tory, medication.
Diagnosis
History and physical, PFTs, bronchoprovocation challenge tests,
CXR, sputum culture, trial of medication. Common causes of cough:
Postnasal Drip—History of rhinitis, frequent throat clearing.
Asthma—Wheezing may not be present. Diagnose by history, bron-
choprovocation challenge.
COPD
Gastroesophageal Reflux—Worse at night and recumbent.
Others—Recurrent aspiration, lung carcinoma, congestive heart
failure (CHF), medications (β-blockers, ACE inhibitors),
bronchiectasis, others.
442
PULMONARY MEDICINE Ill-Defined Symptom Complex
Disease Severity
Cough intensity and number.
Concept and Application
Nonspecific symptom caused by (1) direct stimulation of cough re-
ceptors (foreign body, tumor); (2) increased sensitivity of cough re-
ceptors (asthma); (3) inadequate glottic closure (aspiration, reflux);
or (4) altered mucus quantity or quality (chronic bronchitis,
bronchiectasis).
Treatment Steps
1. Treatment of underlying disorder if identified.
2. Therapeutic trials of bronchodilators, antacids, antihistamines,
proton pump inhibitors, or decongestants may be diagnostic.
3. Guaifenesin, dextromethorphan, or codeine may alleviate symp-
toms.
B. Dyspnea
H&P Keys
Shortness of breath, chest tightness, air hunger, signs and symptoms
of underlying disease.
Diagnosis
History and physical examination, PFTs, exercise testing, ECG, CXR.
Differential diagnosis of dyspnea includes pulmonary diseases, CHF,
neuromuscular disease, anemia, hyperventilation disorders (e.g.,
metabolic acidosis, psychogenic).
Disease Severity
Exercise tolerance, symptom scores. Usually dyspnea correlates with
degree of pulmonary dysfunction.
Concept and Application
Sensation of increased respiratory effort.
Treatment Steps
1. Specific treatment of underlying disease.
2. General supportive measures include oxygen, nutritional and psy-
chological support.
3. Pulmonary rehabilitation.
C. Chest Pain
H&P Keys
Quality of pain, location, and relationship to breathing keys to diag-
nosis. Signs and symptoms of underlying disease.
Diagnosis
D. Hemoptysis
H&P Keys
Cough productive of bloody or blood-tinged sputum.
Diagnosis
Differential diagnosis (common causes).
• Tracheobronchial disorders: Acute or chronic bronchitis, bron-
chogenic carcinoma, bronchiectasis, cystic fibrosis, trauma.
• Cardiovascular disorders: Pulmonary infarction, mitral stenosis,
CHF, atrioventricular malformation, aneurysm, others.
• Hematologic disorders: Anticoagulation, thrombocytopenia, hemo-
philia, disseminated intravascular coagulation.
• Parenchymal lung disorders: Bacterial pneumonia, tuberculosis,
paragonimiasis, contusion.
• Vasculitic disorders: Systemic lupus erythematosus, Goodpasture’s
syndrome.
Disease Severity
Massive hemoptysis: > 600 mL blood/24 hr.
Treatment Steps
1. Diagnostic workup (CXR, complete blood count [CBC], platelet
count, prothrombin time [PT], partial thromboplastin time
[PTT], sputum culture, cytology, acid-fast bacilli [AFB] smear, cy-
tology).
2. Quantitate hemoptysis.
3. Bronchoscopy to localize source.
4. Treatment of coagulation abnormalities and CHF if present.
5. Cough suppressant (codeine).
6. Antibiotic if infection present.
7. Surgical excision of bleeding segment or angiographic emboliza-
tion if persists.
Diagnosis
PFT, flow volume loop, bronchoprovocation challenge, CXR, soft-
tissue neck films, bronchoscopy.
Stridor—Inspiratory “wheeze” heard best over trachea and upper
airway (e.g., epiglottitis, croup, tracheal stenosis, angioedema, la-
ryngeal (“psychogenic”) asthma.
444
PULMONARY MEDICINE Ill-Defined Symptom Complex
Disease Severity
Clinical symptoms, PFT.
Concept and Application
Sound caused by vibrations of airway narrowed by bronchospasm, in-
flammation, mass, or edema.
Treatment Steps
Treatment of underlying disease, bronchodilators, oxygen if hy-
poxic.
Disease Severity
Lung biopsy.
Concept and Application
Round mass may be benign or malignant tumor, tuberculoma, gran-
uloma, artifact, cyst, resolving pneumonia, others.
Treatment Steps
1. Locate old films: if lesion unchanged more than 2 years, probably
benign. If unavailable or new lesion, CT scan and biopsy mass.
Unless a firm benign diagnosis can be made, surgical excision
usually required. Laminated or solid calcification suggests benign
granuloma. A negative positron emission tomography (PET) scan
also suggests a benign lesion.
2. Consider periodic CXRs over 2 years if nonsmoker, age < 45, with
nonspiculated lesion < 2.2 cm.
Diagnosis
Physical examination (obesity, narrowed pharyngeal opening);
polysomnography.
Disease Severity
Polysomnography, degree of nocturnal oxygen desaturation.
Concept and Application
BIBLIOGRAPHY
American Thoracic Society/Centers for Disease Control and Prevention. Diagnostic
standards and classifications of tuberculosis in adults and children. Am J Respir Crit
Care Med 161:1376–1395, 2000.
Fishman AP, ed. Pulmonary Diseases and Disorders, 3rd ed. New York: McGraw-Hill,
2002.
Light RW. Pleural Diseases, 4th ed. Philadelphia: Williams & Wilkin, 2001.
Murray JF, Nadel JA, eds. Textbook of Respiratory Medicine, 3rd ed. Philadelphia: W.B.
Saunders, 2000.
NHLBI/WHO workshop report: Global Strategy for Asthma Management and Pre-
vention, 2002 update. NIH Pub No. 02-3659.
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Diseases of the Renal
and Urologic Systems 17
I. INFECTIOUS DISEASES AND INFLAMMATORY CONDITIONS / 449
A. Urinary Tract Infection (UTI) / 449
B. Painful Bladder and Urethral Syndromes / 449
C. Prostatitis / 450
D. Epididymitis / 450
E. Urethritis / 450
F. Orchitis / 451
G. Gonorrhea / 451
H. Syphilis / 452
I. Chlamydia / 452
J. Herpes / 452
K. Human Immunodeficiency Virus (HIV/AIDS) / 453
447
Copyright © 2004, 2001 by The McGraw-Hill Companies, Inc. Click here for terms of use.
448 Diseases of the Renal and Urologic Systems
BIBLIOGRAPHY / 475
449
DISEASES OF THE RENAL AND UROLOGIC SYSTEMS Infectious Diseases and Inflammatory Conditions
I. INFECTIOUS DISEASES
AND INFLAMMATORY CONDITIONS
C. Prostatitis
H&P Keys
Slow or sudden onset, perineal discomfort, voiding dysfunction,
prostate tenderness on digital rectal exam, UTI.
Diagnosis
Urine culture and sensitivity, extraprostatic secretion culture, UA
(pyuria).
Disease Severity
Fever or rigors, urinary retention, constant or intermittent discom-
fort, degree of voiding dysfunction.
Concept and Application
Distinguish true bacterial prostatitis (acute and chronic) from non-
bacterial (inflammatory states, chlamydia) and prostatodynia. Acute
bacterial prostatitis responds dramatically to antibiotics. Fifty per-
cent empiric response to antibiotics in other conditions. Prostatody-
nia associated with pelvic floor or sphincter spasm.
Treatment Steps
1. Acute bacterial infection requires culture-appropriate antibiotics.
2. Chronic bacterial or nonbacterial infection requires cipro-
floxacin or levofloxacin or tetracyclines for 4–6-week course.
3. Warm soaks and nonsteroidal anti-inflammatory drugs (NSAIDs).
4. Chronic pain evaluation for prostatodynia.
D. Epididymitis
H&P Keys
Pain, tenderness, discomfort distinct from testis parenchyma. Nor-
mal testis, palpable epididymal discomfort. Slow or sudden onset.
No trauma, sexual activity, voiding symptoms, genitourinary (GU)
instrumentation.
Diagnosis
Pyuria; culture and sensitivity are usually negative. Scrotal ultra-
sound or Doppler good flow state, inflammation of epididymis.
Disease Severity
Fever or rigors, testis and epididymis are indistinguishable, scrotal
induration, incapacitation.
E. Urethritis
H&P Keys
Purulent or mucoid discharge. Dysuria and frequency during urina-
tion. History of sexual activity.
451
DISEASES OF THE RENAL AND UROLOGIC SYSTEMS Infectious Diseases and Inflammatory Conditions
Diagnosis
Gonococci (GC) culture in Thayer–Martin media, gram-negative
diplococci, or specific chlamydia culture.
Disease Severity
Ranges from painless discharge to severe voiding symptoms, epididy-
mal and testicular involvement, systemic illness.
Concept and Application
Generally gonococcal and nongonococcal (Chlamydia trachomatis,
Ureaplasma urealyticum, Trichomonas) complications of urethral stric-
ture or Reiter’s syndrome.
Treatment Steps
1. Gonococcal infection: ceftriaxone, 250 mg intramuscularly (IM);
levofloxacin, 500 mg, one dose.
2. Nongonococcal infection: tetracycline, 500 mg qid; doxycycline,
100 mg bid for 7–10 days; or levofloxacin, 250–500 mg qd for
7–10 days.
3. Metronidazole for Trichomonas; acyclovir for herpes.
F. Orchitis
H&P Keys
Rapid or slow onset, fever and malaise, absence of trauma. Scrotal
contents can be normal, inflamed, or distorted (torsion). Sexual or
voiding dysfunction. Child or adolescent versus adult.
Diagnosis
Pyuria, scrotal ultrasound, urine culture.
Disease Severity
Scrotal induration, fistula, abscess on ultrasound, rule out
torsion–hyperemic blood flow and systemic infection.
Concept and Application
Rule out torsion in young men; bacterial infection in older men and
sexually transmitted disease (STD) in younger men. Mumps orchitis
in children and adolescents. Chronic orchalgia can be present with-
out infection.
Treatment Steps
1. Four to six weeks of ciprofloxacin, levofloxacin, doxycycline.
2. NSAIDs, warm soaks, and elevation.
G. Gonorrhea
H&P Keys
Sexual exposure; thick, creamy urethral discharge; urethritis. May
involve epididymis.
Diagnosis
Gram-negative diplococci. GC culture in Thayer–Martin media.
Disease Severity
Severity of pain and discharge; associated epididymitis or orchitis.
Concept and Application
β-Lactamase plasmid–penicillin resistance. Pili contribute to viru-
lence. Different infection rate per exposure (male 20%, female
90%). Coexisting chlamydial infection.
452
DISEASES OF THE RENAL AND UROLOGIC SYSTEMS Infectious Diseases and Inflammatory Conditions
Treatment Steps
Ceftriaxone, 125 mg IM; ciprofloxacin, 500 mg PO, single dose, plus
azithromycin, 1 g PO single dose, and doxycycline, 100 mg bid for 7
days.
H. Syphilis
H&P Keys
Sexual activity. Painless genital ulcer, lack of vesicles. Negative travel
history.
Diagnosis
Rapid plasma reagin (RPR) and fluorescent treponemal antibody-
absorption test (FTA-ABS) for syphilis, patient’s history.
Disease Severity
Primary, secondary, or tertiary disease; systemic symptoms, neu-
rosyphilis; cardiovascular changes.
Treatment Steps
1. Benzathine, 2.4 million units IM, one dose.
2. Erythromycin base, 2 g/day for 2 weeks.
3. Late latent: benzathine, 2.4 million units IM weekly for 3 weeks.
I. Chlamydia
H&P Keys
Sexual activity, younger age group, clear or mucoid discharge.
Diagnosis
No routine culture; tissue culture 8–10 days, fluorescent antibody
stains, history.
Disease Severity
Pain, spread to testis or prostate.
Treatment Steps
Doxycycline; tetracycline; quinolones; azithromycin, 1 g PO, single
dose.
J. Herpes
H&P Keys
Genital ulcer, painful vesicles, multiple lesions.
Diagnosis
Tzanck test with Wright or Giemsa stain and cell culture.
Disease Severity
Pain, coalesced vesicles, persistence or recurrence of infection.
453
DISEASES OF THE RENAL AND UROLOGIC SYSTEMS Benign Conditions of the Genitourinary Tract
Concept and Application
Double-strand DNA virus. Subclinical infections, systemic complica-
tions—aseptic meningitis, fever, urinary retention in women, and, in
rare cases, hepatitis or pneumonia.
Treatment Steps
1. Oral acyclovir, 200 mg 5 times/day for 10 days. Topical applica-
tion for pain relief.
2. Suppressive treatment, acyclovir, 400 mg PO bid.
Diagnosis
HIV antibody test. Western blotting.
Disease Severity
HIV positivity versus systemic disease.
Treatment Steps
1. Per current therapy.
2. Evaluation of GU symptoms as per uninfected patients. Minimize
invasive procedures in immunocompromised patients.
A. Cryptorchidism
H&P Keys
Immature birth, absence of testis on scrotal exam. If hypospadias is
present, consider sex ambiguity.
Diagnosis
Physical exam. In adults, consider computed tomographic (CT)
scan.
Disease Severity
Retractile testis; inguinal canal versus intra-abdominal, unilateral, or
bilateral.
Treatment Steps
1. Surgical correction at 6–12 months of age.
2. Hormonal manipulation.
454
DISEASES OF THE RENAL AND UROLOGIC SYSTEMS Benign Conditions of the Genitourinary Tract
B. Testicular Torsion
H&P Keys
Common scrotal swelling in children. Acute severe onset. Possible
nausea and vomiting, abdominal pain.
Diagnosis
Negative UA, distorted or rotated gonad, Doppler ultrasound, nu-
clear flow scan.
Disease Severity
Degree of pain and scrotal swelling. Duration (less than or more
than 5 hours). Absence of contralateral testis.
Treatment Steps
Surgical correction in 5 hours. Bilateral orchiopexy with permanent
suture.
C. Intersex
H&P Keys
Salt loss, salt retention. Phallic enlargement. Precocious pubic hair,
early masculinization, early epiphyseal closure (congenital adrenal
hyperplasia [CAH]). Sparse axillary and pubic hair (testicular femi-
nization). Penile scrotal hypospadias and bilateral cryptorchidism.
Groin mass (gonad).
Diagnosis
Buccal smear, karyotype. Metabolic studies for CAH. Genitography,
ultrasound gonadal histology.
Disease Severity
Degree of underdevelopment or ambiguity of genitalia. Neonatal
versus pubertal diagnosis. Reproductive and gender role dysfunc-
tion.
Treatment Steps
1. Variable. Treatment depends on specific syndrome and time of
recognition.
2. Possible gender reassignment, usually male to female.
3. Support in assigned role (testicular feminization).
455
DISEASES OF THE RENAL AND UROLOGIC SYSTEMS Benign Conditions of the Genitourinary Tract
D. Infertility
H&P Keys
Failure to conceive after 1 year of unprotected intercourse. Asym-
metric or undescended testicles. Testicular mass. Varicocele. Sec-
ondary sexual characteristics. Drug and chemical exposure. Stress.
Diagnosis
Semen analysis (> 20 million/cc, 1.5–5 cc volume). UA, semen fruc-
tose (obstruction/dysfunction of seminal vesicles). Luteinizing hor-
mone (LH), follicle-stimulating hormone (FSH), and testosterone
level. Sperm function tests.
Disease Severity
Mild disorders of semen parameters (motility or morphology) to
azoospermia (total absence of sperm).
Treatment Steps
1. Varicocele repair.
2. Repair of anatomic blockage.
3. Clomiphene citrate administration (idiopathic infertility).
4. Assisted reproductive techniques.
5. Adoption.
Diagnosis
Physical exam, UA, scrotal ultrasound. Check tumor marker in
young men if diagnosis is questionable.
Disease Severity
Hydrocele—Cosmetic deformity.
Varicocele—Deformity, pain, testicular atrophy, infertility prob-
lems.
Treatment Steps
Hydrocele
1. Drainage with sclerotherapy or surgical correction (5–15% re-
currence).
2. Observation.
Varicocele
1. Venous ligation or embolization.
2. Observation.
G. Peyronie’s Disease
H&P Keys
Painless, firm, nonindurated area on penile shaft.
Diagnosis
Physical exam. Color-flow Doppler ultrasound.
Disease Severity
Firm area, degree of pain with erection, deformity of erection, impo-
tence.
Concept and Application
Idiopathic fibrosis of tunic of corpora cavernosa. Asymmetric thick-
ening leads to erectile thickening and pain. Natural history is un-
clear. Ten percent are associated with Dupuytren’s contracture.
Treatment Steps
1. Observation.
2. Oral antioxidants.
3. Surgical excision and graft repair.
H. Erectile Impotence
H&P Keys
Neurological disease, diabetes, peripheral vascular disease, medica-
tions, radical pelvic surgery, Peyronie’s plaque, psychological factors.
457
DISEASES OF THE RENAL AND UROLOGIC SYSTEMS Benign Conditions of the Genitourinary Tract
Diagnosis
History and physical exam, color-flow Doppler ultrasound, noctur-
nal penile tumescence studies, hormone profile (testosterone), LH,
FSH, prolactin.
Disease Severity
Occasional or permanent inability to attain erection sufficient for
vaginal penetration.
Concept and Application
Vascular, muscular, or neurologic etiology is the site of primary dys-
function; psychogenic issues much less common.
Treatment Steps
1. Sildenafil, 25 to 100 mg PO.
2. Intracavernosal injection (prostaglandin E1 or papaverine–
regitine).
3. Vacuum suction device.
4. Penile prosthesis.
I. Hypospadias
H&P Keys
Ectopic position of urethral meatus on ventral shaft of penis. Lack of
ventral foreskin. Check for undescended testicles.
Diagnosis
Physical examination.
Disease Severity
Glandular position to more proximal location on shaft (penile or
scrotal). Associated anomalies.
Concept and Application
Occurs at the rate of 1 in 300 live male births. Failure of mesothelial
folds to close in the midline. Epispadias (dorsal urethral opening) is
extremely rare. Associated with midline closure defects.
Treatment Steps
Surgical correction.
J. Vesicoureteral Reflux
H&P Keys
Associated family history. Occurs in 50% of infant UTIs and 30% of
childhood UTIs; recurrent UTIs in infancy and childhood.
Diagnosis
Ultrasound, IV urography, renal nuclear scan.
Disease Severity
Grades 1–5, international classification.
Concept and Application
Ectopic ureteral bud leading to lateral placement of ureter in bladder.
Decreased flap-valve mechanism. May improve with maturity. High-
pressure voiding states also can overwhelm normal anatomy. Goal is to
preserve upper tracts. Avoid renal scarring and hypertension.
Treatment Steps
1. Prophylactic antibiotics: TMP-SMZ, nitrofurantoin. Initial med-
ical management of grades 1–3; grade 4, medical or surgical;
grade 5, surgery. Cohen reimplant.
458
DISEASES OF THE RENAL AND UROLOGIC SYSTEMS Benign Conditions of the Genitourinary Tract
K. Urolithiasis
H&P Keys
Prior history of stone, geography, metabolic disorders, flank or groin
tenderness.
Diagnosis
Urinalysis, IV urogram, ultrasound.
Disease Severity
Stone burden on imaging studies; degree of urinary obstruction or
renal impairment; pain, nausea, vomiting.
Concept and Application
Most stones are “idiopathic” calcium oxalate. Struvite stones are as-
sociated with infections (Proteus) and uric acid; cysteine is less com-
mon.
Treatment Steps
1. Small calculi: hydration, pain control, spontaneous passage.
2. Upper tract: extracorporal shockwave lithotripsy.
3. Lower tract: ureteroscopic extraction or lithotripsy.
management
decisions d L. Neurogenic Bladder
H&P Keys
Neurologic exam, palpable bladder, rectal exam. Rule out stricture
GENITOURINARY TRACT disease.
Urolithiasis Diagnosis
Size and location of stone. Urodynamics: pressure-flow, cystometrogram, electromyography,
Observe with spontaneous uroflowmetry, cystoscopy.
passage, extracorporeal
shock wave lithotripsy Disease Severity
(ESWL), ureteroscopy for Voiding dysfunction versus complete retention, total incontinence,
lower ureter. chronic UTIs, deterioration of renal function.
Chronic/recurrent stones—
medical management. Concept and Application
Prostate Cancer Several classification schemes: (1) failure to empty versus failure to
Patient age, disease stage store is most functional scheme, (a) emptying failure resulting from
and performance status:
decompensated detrusor mechanical obstruction or overactive
Local disease: younger
patients
sphincters (smooth and striated), (b) failure to store because of
(< 70) surgery > radiation, overactive detrusor or incompetent sphincters; and (2) motor versus
older patients radiation sensory. Classification of uninhibited versus autonomous is used
> surgery. Androgen least often.
ablation in advanced
disease. Treatment Steps
Vesicoureteral Reflux 1. Failure to empty (detrusor): clean intermittent catheterization;
Initial antibiotic therapy bethanechol is ineffective.
(grades 1–3), surgery 2. Failure to empty (mechanical): cystoscopy to rule out stricture or
(ureteral reimplant) higher enlarged prostate.
grade or failed medical 3. Failure to empty (sphincter dyssynergia): α-blockage, bladder
therapy.
neck incision, sphincterotomy. Failure to store (detrusor): anti-
Enuresis cholinergics, surgical bladder augmentation. Failure to store
Reassurance with
(sphincter incompetence): bladder sling surgery, collagen injec-
spontaneous resolution,
behavioral modification,
tions, artificial sphincter placement.
intranasal DDAVP. Note: Diabetes can induce a sensory neurogenic bladder. Initial
good motor function can be maintained with timed voiding.
459
DISEASES OF THE RENAL AND UROLOGIC SYSTEMS Benign Conditions of the Genitourinary Tract
Chronic overdistention creates dilated myopathy with motor de-
compensation as well. Treatment involves intermittent catheteri-
zation.
N. Enuresis
H&P Keys
Lack of neurologic history, structural disorder that precludes normal
toilet training, polyuria.
Diagnosis
UA and culture and sensitivity. Radiography if infection is pres-
ent, wetting is diurnal and present beyond age 12 years.
Disease Severity
Frequency and persistence into adolescence.
Concept and Application
Persistence of immature reflex pattern of bladder emptying.
Treatment Steps
1. Reassure patient about spontaneous resolution. Restrict fluids in
evening.
2. 1-deamino-8-D-arginine vasopressin, 10–40 µg intranasally.
P. Urologic Trauma
H&P Keys
Cardiovascular stability, hematuria, pelvic stability, prostate location
on rectal exam. Penile and scrotal ecchymosis, blood at urethral
meatus, blunt or penetrating trauma.
Diagnosis
Urinalysis, CT scan, IV urogram, retrograde urethrogram, cys-
togram. Ultrasound for oliguria.
Disease Severity
Mild contusions treated with observation. Renal fracture involving
collecting system, ureteral disruption, intra- versus extraperitoneal
bladder extravasation. Penile or testicular fracture.
Treatment Steps
Renal (stable):
1. Observation and bed rest, serial imaging; renal (unstable). Diver-
sion of urine with stent; ureteral (major). Catheter drainage;
bladder (extraperitoneal). Drainage and later repair in most
cases. Debridement and repair.
2. Surgery or angiography. Ureteral (minor). Early or immediate re-
pair (major, delayed diagnosis). Open repair. Urethra (suprapu-
bic): Penile–testicular.
3. Urine diversion with later repair. Bladder (intraperitoneal).
Diagnosis
Uroflowmetry, postvoid residual urine, UA, pressure-flow uro-
dynamics if indicated, cystoscopy if indicated, serum prostate-
specific antigen (PSA).
Disease Severity
American Urological Association symptom score of 0–35. UTIs, re-
nal deterioration, urinary retention.
C 461
DISEASES OF THE RENAL AND UROLOGIC SYSTEMS Neoplasias of the Genitourinary Tract
on rounds
UROLOGIC DISORDERS
Prostate Cancer
• Diagnosis by digital rectal exam and PSA followed by needle biopsy, bone scan.
• Staging: Jewett–Whitmore A–D.
A = Local, nonpalpable
B = Palpable, confined
C = Extracapsular
D = Lymph node or distant mets
• Treatment if local includes radical prostatectomy or radiation.
• Treatment if extracapsular is radiation.
• Treatment for advanced disease includes androgen ablation (orchiectomy, LH-releasing
hormone agonists).
Wilms’ Tumor
• Pediatric tumor (peak age 3), flank mass, hematuria.
Bladder Cancer
• Risk factors include exposure to tobacco, cyclophosphamide, and aniline dyes.
Hydrocele
• Painless scrotal mass, transillumination, fluid in tunical layer of testes, a cosmetic problem.
Varicocele
• Spermatic venous varicies, “bag of worms” feel, pain, infertility.
Testicular Torsion
• Scrotal swelling in children, acute severe onset, nausea, abdominal pain.
Treatment Steps
1. Expectant management.
2. Decrease tone with α-blocker (several) hs.
3. Medical bulk reduction with finasteride.
4. Surgical bulk reduction with transurethral resection of prostate
(TURP).
B. Prostate Cancer
H&P Keys
Digital rectal exam and serum PSA. Ultrasound is not part of routine
exam. Hematuria and obstruction symptoms are less specific.
Diagnosis
Needle biopsy of prostate. Staging: Bone scan. Endorectal coil mag-
netic resonance imaging (eMRI) investigational. CT scan and MRI
of body are not useful.
Disease Severity
Tumor grade and stage (Jewett–Whitmore, A-D, TNM [tumor, node,
metastasis] system). (A/T1) local, PSA, or incidental biopsy, nonpal-
pable disease; (B/T2) palpable organ, confined disease; (C/T3) ex-
tracapsular disease; (D/T4, N+, M+) lymph node or distant metastasis.
462
DISEASES OF THE RENAL AND UROLOGIC SYSTEMS Neoplasias of the Genitourinary Tract
Treatment Steps
Prostate cancer is a progressive disease. Slow rate of progression
suggests that active observation is an option in patients with local
disease and expected life span of < 10 years. Local disease: radical
prostatectomy or radiation therapy. Extracapsular disease: radia-
tion therapy. Advanced disease: androgen ablation (orchiectomy,
LH-releasing hormone agonists with or without antiandrogen). Bra-
chytherapy and cryosurgery for local disease is investigational. Pal-
liative therapy for hormone-refractory disease.
C. Bladder Carcinoma
H&P Keys
Gross or microscopic hematuria. Irritative voiding symptoms. Occa-
sionally, pelvic pain. Exposure to tobacco, cyclophosphamide,
analine dyes.
Diagnosis
IV urogram, cystoscopy, barbitage cytology. Transurethral resection
biopsy of bladder lesion.
Disease Severity
Key determinant is presence or absence of muscle invasion by the tu-
mor. Degree of hematuria does not correlate with tumor stage. TNM
staging system. Patients with gross nodal and distant disease do
poorly.
Concept and Application
Cancer-related deaths per year: 10,000. Two-thirds of tumors are su-
perficial and treated by resection; two-thirds of these will recur, and
10–20% progress to muscle-invasive disease. Associated carcinoma in
situ is a bad prognostic feature regarding recurrence and progres-
sion. Tumor grade is strong indicator of recurrence.
Treatment Steps
Superficial
1. Resect initial tumor and observe, on standard schedule (see
below), multiple tumors or recurrence. Treat with intravesical
bacillus Calmette–Guérin (BCG).
2. Mitomycin decreases papillary recurrence, decreases recur-
rence and progression in carcinoma in situ.
Cysto Schedule—Every 3 months for 1 year, every 6 months for 1
year, yearly thereafter. Recurrence resets schedule.
Muscle Invasive
1. Radical cystectomy and urinary tract reconstruction (ileal con-
duit or continent neobladder).
2. Radiation therapy, and chemotherapy used in combination.
3. Transurethral resection.
463
DISEASES OF THE RENAL AND UROLOGIC SYSTEMS Neoplasias of the Genitourinary Tract
Advanced Disease
1. Methotrexate, vinblastine, Adriamycin, and cisplatin (MVAC),
with 40–50% response and 15% sustained complete remis-
sion.
2. Paclitaxel or combinations.
Diagnosis
Mass on IV urogram, ultrasound, CT, or MRI. Angiography rarely
performed now. CT criteria: mass with slight increase in Hounsfield
units after contrast. Paraneoplastic effect of hypercalcemia and ele-
vated liver function tests (LFTs) (Stauffer’s syndrome) does not indi-
cate metastases. Anemia is more common than erythrocytosis.
Disease Severity
Robeson or TNM tumor stage. Weight loss, fatigue, and large-mass
organ-confined disease easily treated with surgery. Metastatic disease
responds poorly to therapy.
Treatment Steps
1. Radical nephrectomy is treatment of choice if no evidence of
metastatic disease.
2. Interleukin-2 or 5-fluorouracil (FUDR), or research protocol.
3. Progesterones are used for palliation and have few side effects.
Some positive results with biological response modifiers.
E. Wilms’ Tumor
H&P Keys
Pediatric tumor. Noticed as flank mass on exam. Hematuria.
Diagnosis
History and physical exam, CT scan or ultrasound, IV urogram.
Disease Severity
Clinical staging, performance status.
Treatment Steps
1. Radical nephrectomy for localized or regional disease and
chemotherapy (actinomycin and vincristine).
2. Radiation therapy for advanced disease.
464
DISEASES OF THE RENAL AND UROLOGIC SYSTEMS Renal Disorders
F. Testicular Carcinoma
H&P Keys
Painless testicular mass, testis rupture after mild trauma. Advanced
disease includes gynecomastia, shortness of breath, adenopathy, ab-
dominal mass.
Diagnosis
Scrotal ultrasound, tumor markers (β-human chorionic gonadotropin
[β-hCG] and α-fetoprotein [AFP]). Diagnosis by radical orchiectomy
(inguinal incision).
Disease Severity
Marker level, retroperitoneal CT scan, organ confined versus subdi-
aphragmatic lymph nodes versus pulmonary/visceral disease.
Treatment Steps
Radical orchiectomy. Staging. Seminoma (local): 2,500 cGy of radia-
tion; seminoma (node positive): chemotherapy. Nonseminoma (lo-
cal): retroperitoneal lymph node dissection (RPLND); nonsemi-
noma (advanced, six positive nodes or > 2.5 cm): chemotherapy
followed by salvage RPLND. Bleomycin, etoposide, and cisplatin
(BEP).
Seminoma
1. Radical orchiectomy and radiation.
2. Chemotherapy.
Nonseminoma
1. Radical orchiectomy.
2. RPLND for localized.
3. Chemotherapy and RPLND; advanced: BEP, usually 3 cycles.
A. Pyelonephritis
diagnostic
decisions d
H&P Keys
RENAL DISORDERS
History of UTIs, voiding dysfunction, flank pain, fever, malaise. Dif-
ferentiate acute from chronic pyelonephritis.
Polycystic Kidney
Disease
Diagnosis Positive family history in
Urine culture, urinalysis (pyuria, white blood cell casts), renal ultra- 75%, clinical (bilateral flank
sound to rule out obstruction by stone or other cause. masses, elevated BP,
uremia).
Disease Severity Alport’s Syndrome
Discomfort to frank sepsis. Acute infection versus chronic deteriora- Hematuria, proteinuria,
tion (renal scarring, insufficiency, proteinuria, hypertension). hearing loss, ocular
disorders.
Treatment Steps
Assess clinical and laboratory parameters.
466
DISEASES OF THE RENAL AND UROLOGIC SYSTEMS Renal Disorders
Prerenal
1. Restore adequate circulating plasma volume.
2. Correct nonrenal pathology.
Renal
1. Convert to nonoliguric state, remove toxins, dialyze as
needed.
2. Urologic evaluation (ultrasound, cystoscopy, retrograde stent
placement) as needed.
Diagnosis
Shrunken kidneys on imaging; uremia; creatinine clearance; edema;
hyperkalemia; normochromic, normocytic anemia.
Disease Severity
Creatinine clearance, edema, electrolyte derangement, neurologic
complications of uremia.
D. Tubulointerstitial Disease
H&P Keys
Toxin exposure (analgesics, heavy metal), immune disorders, neo-
plasia, vascular disease, family history of renal disorders.
Diagnosis
Electrolyte irregularities, eosinophilia, impaired creatine clearance,
renal biopsy.
Disease Severity
Acute or chronic, tubular defects versus marked GFR deterioration.
Concept and Application
Pathology is morphologically in tubules and interstitium, not
glomerulus. Acute disease: inflammation, tubule necrosis, edema.
Chronic forms: fibrosis is common. Condition has many causes. Look
for dysfunction in tubular transport. Proteinuria usually not severe.
Eventual GFR dysfunction.
Treatment Steps
1. Remove offending agent.
2. Compensate tubular defect.
3. Treat primary disease.
467
Treatment Steps
F. Nephrotic Syndrome
H&P Keys
Edema.
Diagnosis
Urinalysis, proteinuria, 24-hour collection, hypoalbuminemia, renal
biopsy.
Disease Severity
Rapid versus chronic course. Degree of proteinuria/renal insuffi-
ciency.
Concept and Application
Albuminuria, hypoalbuminemia, hyperlipidemia, and edema. Mini-
mal change disease, mesangial proliferative immunoglobulin A
(IgA) glomerulonephritis (Berger’s disease), focal and segmental
glomerulosclerosis, membranous Berger’s disease.
Treatment Steps
Treatment with combination of steroids, cytotoxic drugs, cy-
closporine.
G. Glomerulonephritis
H&P Keys
Infectious disease, primary renal disease, multisystem disease.
Abrupt azotemia, oliguria, edema, hypertension.
Diagnosis
Serum electrolytes, proteinuria, urinalysis for hematuria, red cell
casts.
468
DISEASES OF THE RENAL AND UROLOGIC SYSTEMS Renal Disorders
Disease Severity
Degree of edema, renal insufficiency, acute versus rapidly progress-
ing disease.
Treatment Steps
H. Diabetic Nephropathy
H&P Keys
History of diabetes, diabetic stigmata, edema, hypertension.
Diagnosis
BUN and creatinine, creatinine clearance, oliguria.
Disease Severity
Degree and duration of diabetes, level of renal impairment.
I. Renal Osteodystrophy
H&P Keys
Renal insufficiency, growth retardation, rickets. Bone pain or proxi-
mal muscle weakness in adults.
Diagnosis
Calcium and phosphorus levels. Parathyroid activity. Plain film find-
ings.
Disease Severity
Renal dwarfism versus growth retardation or maturation, degree of
orthopedic disability in adults. Extent of calcium imbalance, patho-
logic calcification.
Concept and Application
Impaired vitamin D metabolism, parathyroid hormone overproduc-
tion. Dialysis accelerates bone pathology secondary to aluminum de-
position. Osteitis fibrosa cystica, renal rickets, osteosclerosis.
469
J. Papillary Necrosis
H&P Keys
Hematuria and flank pain, patient may be asymptomatic, disease of-
ten associated with severe infection and other conditions.
Diagnosis
Urinalysis, culture, filling defect on urogram; ring shadow may be
present.
Disease Severity
Asymptomatic or flank pain infection, obstructive uropathy with
papillary sloughing.
Concept and Application
Infection or microangiopathy of renal pyramids. Associated with dia-
betes, alcoholism, sickle cell anemia.
Treatment Steps
1. Asymptomatic finding: treat primary disease.
2. Mechanical removal of obstruction.
K. Renovascular Hypertension
H&P Keys
Hypertension, rapid onset, poorly controlled, epigastric bruit.
Diagnosis
Renal vein renin sampling (ratio > 1.5); angiography (classic, digital,
MRI); occasionally, IV urogram (not a screening test).
Disease Severity
Pharmacologic control, severity of stenosis on imaging.
Concept and Application
Usually secondary to atherosclerotic vascular disease; several forms
of fibromuscular hyperplasia.
Treatment Steps
1. Medical.
2. Angioplasty.
3. Surgical repair.
L. Preeclampsia
H&P Keys
Pregnancy related, edema, proteinuria, and hypertension after 24th
week of pregnancy.
Diagnosis
Blood pressure 140/90, proteinuria, 30 mm Hg systolic or 15 mm
Hg diastolic relative to earlier pregnancy readings.
Disease Severity
Progression to eclampsia; need for intervention beyond bed rest.
470
DISEASES OF THE RENAL AND UROLOGIC SYSTEMS Electrolyte and Acid–Base Disorders
M. Eclampsia
H&P Keys
Preeclampsia and seizures.
Diagnosis
Preeclampsia and seizure evaluation.
Disease Severity
Proteinuria, level of hypertension and degree of seizure activity.
Concept and Application
Etiology unknown.
Treatment Steps
Control blood pressure and seizures.
O. Nephrosclerosis
H&P Keys
Mild, moderate, or malignant hypertension; neurologic signs; pa-
pilledema.
Diagnosis
UA, proteinuria, renal imaging (size).
Disease Severity
Mild sclerosis with mild to moderate physiologic changes (slight
azotemia, exaggerated natriuresis with fluid challenge versus malig-
nant hypertension, neurologic symptoms.
471
DISEASES OF THE RENAL AND UROLOGIC SYSTEMS Electrolyte and Acid–Base Disorders
Concept and Application
Mild to moderate secondary changes of essential hypertension, vas-
cular atherosclerotic changes (afferent arterioles). Severe disease
with fibrinoid necrosis and hyperplastic arteriolitis.
Treatment Steps
Control hypertension (acute and chronic).
P. Lupus Nephritis
H&P Keys
Associated history of systemic lupus erythematosus (SLE) and physi-
cal exam, edema.
Diagnosis
Urinalysis, azotemia, low C3 and C4 concentrations. Positive double-
stranded DNA antibody, proteinuria, nephrotic syndrome, renal
biopsy.
Disease Severity
Asymptomatic, clinical SLE, mild to severe renal status.
A. Hyponatremia
H&P Keys
Nausea, confusion, lethargy, coma, seizures, decreased deep tendon
reflexes.
Diagnosis
Serum sodium < 130 mg/dL.
Disease Severity
Abnormal laboratory value to severe clinical derangement.
Concept and Application
Free-water retention, exogenous free water, TURP or water intoxica-
tion, syndrome of inappropriate secretion of antidiuretic hormone
(SIADH), renal and cardiac decompensation.
Treatment Steps
Restrict free water, replace salt.
B. Hypernatremia
H&P Keys
Dehydration, hyperpnea, oliguria, thirst, hypotension.
Diagnosis
Serum sodium > 145 mg/dL.
472
DISEASES OF THE RENAL AND UROLOGIC SYSTEMS Electrolyte and Acid–Base Disorders
Disease Severity
Laboratory finding to severe clinical derangement.
Concept and Application
Impaired thirst mechanism, excessive water loss, solute and free-
water loss (diabetic ketoacidosis).
Treatment Steps
Slow replacement of free water to avoid cerebral edema.
C. Hypokalemia
H&P Keys
Dysrhythmia, rhabdomyolysis, muscle weakness or cramps.
Diagnosis
Serum potassium < 3.5 mg/dL. Changes in electrocardiogram
(ECG): wide decrease in T wave, U wave, atrioventricular block.
Disease Severity
Laboratory finding to severe clinical derangement.
Concept and Application
Inappropriate gastrointestinal (GI) or GU loss, cellular sequestra-
tion, decreased intake. Because of body stores, small decrease in
serum value can indicate significant total-body depletion.
Treatment Steps
1. Oral replacement for chronic loss (diuretic use).
2. IV replacement is not advised except in monitored situation
(keep below 20 mEq/hr).
D. Hyperkalemia
H&P Keys
Renal insufficiency, diarrhea, weakness.
Diagnosis
Laboratory findings, ECG: widened QRS waves and peaked T waves.
Disease Severity
Laboratory value or severe clinical derangement.
Concept and Application
Reduced renal excretion of potassium; adrenal insufficiency, exces-
sive intake; hyperchloremic acidosis.
Treatment Steps
1. Limit exogenous potassium.
2. Correct underlying acidosis.
3. Exchange resin, insulin/D50 glucose.
4. Dialysis.
E. Volume Depletion
H&P Keys
Decreased skin turgor, orthostasis, thirst, coma, sunken eyes.
Diagnosis
Serum electrolytes, increased sodium, BUN, osmolality increased.
Disease Severity
Laboratory derangement to severe clinical compromise.
473
DISEASES OF THE RENAL AND UROLOGIC SYSTEMS Electrolyte and Acid–Base Disorders
Concept and Application
Third spacing, insufficient replacement of free water, excessive loss
of free water.
Treatment Steps
1. Slow replacement of free water.
2. Replace sodium as needed.
F. Volume Excess
H&P Keys
Renal insufficiency, CHF, pathologic free-water consumption (water
intoxication), syndrome of inappropriate antidiuretic hormone se-
cretion (SIADH), nausea, seizures, weakness, coma.
Diagnosis
Serum electrolytes, clinical scenario.
Disease Severity
Mild electrolyte disturbance to severe clinical derangement.
Concept and Application
Excessive exogenous free water or poor elimination of free water.
Treatment Steps
Restrict fluids.
G. Metabolic Alkalosis
H&P Keys
GI loss, renal loss, H+ translocation (hypokalemia), NaHCO3 admin-
istration.
Diagnosis
Elevation of arterial pH, increase in plasma HCO3, compensatory hy-
poventilation (increased PCO2).
Disease Severity
Compensated disturbance or severe metabolic derangement.
Concept and Application
Generally a loss of H+, retention of bicarbonate, or contraction alka-
losis.
management
decisions d
Treatment Steps ELECTROLYTE AND
ACID–BASE DISORDERS
1. Correct primary cause.
2. Compensate electrolyte abnormality.
Metabolic Acidosis
Correct primary pathology
H. Respiratory Alkalosis (anion gap or nonanion gap
H&P Keys causes), assess renal
Hypoxemia, CHF, pulmonary disease, severe anemia, gram- function and GI function,
evaluate for lactic acidosis
negative sepsis, hepatic failure.
or ketoacidosis.
Diagnosis Hypercalcemia
Elevated arterial pH, hypocapnea, plasma HCO3 decreased. Discern appropriate
etiology—neoplasia,
Disease Severity hyperparathyroidism,
Mild compensated disorder or severe metabolic derangement. sarcoidosis; evaluate ECG;
saline hydration and
Concept and Application furosemide diuresis;
Hyperventilation caused by hypoxemia or central stimulation of res- calcitonin or
piration. Primary respiratory disease and mechanical ventilation also bisphosphonates.
a cause.
474
DISEASES OF THE RENAL AND UROLOGIC SYSTEMS Electrolyte and Acid–Base Disorders
Treatment Steps
1. Correct underlying medical defect.
2. Rebreathing (increase PCO2).
I. Metabolic Acidosis
H&P Keys
Low arterial pH, reduced plasma HCO3 concentration, compen-
satory hyperventilation.
Diagnosis
Electrolytes, blood gas, associated metabolic disorders.
Disease Severity
Mild electrolyte disturbance to severe metabolic derangement.
Concept and Application
Generally characterized as anion gap (ingestions, ketoacidosis, lactic
acidosis, renal failure, rhabdomyolysis), and hyperchloremic (nor-
mal anion gap) renal dysfunction, GI loss of HCO3, renal loss of
HCO3, ingestion.
Treatment Steps
1. Correct primary etiology.
2. Replace HCO3 with accompanying additional cation load (Na+).
J. Respiratory Acidosis
H&P Keys
Medications, acute cardiac arrest, obesity, upper-airway obstruction,
chest wall pathology, adult respiratory distress syndrome, and
chronic obstructive pulmonary disease.
Diagnosis
Blood gas and electrolytes. Elevated serum HCO3, reduced arterial
pH. Elevated PCO2.
Disease Severity
Mild disorder to severe decompensation.
Concept and Application
Inability to excrete respiratory CO2, multiple mechanical and struc-
tural disorders.
Treatment Steps
Correct primary ventilatory defect.
K. Hypomagnesemia
H&P Keys
Alcoholism, malnutrition, diuretics, diabetic ketoacidosis, leth-
argy, delirium, irritability of central nervous system.
Diagnosis
Low serum magnesium (< 1.1 mEq/dL); ECG, prolonged QT waves;
hypokalemia; hypocalcemia.
Disease Severity
Electrolyte abnormality to severe neurologic decompensation.
Concept and Application
Metabolism similar to calcium; generally difficult to deplete body
stores.
475
DISEASES OF THE RENAL AND UROLOGIC SYSTEMS Electrolyte and Acid–Base Disorders
Treatment Steps
IV or IM exogenous replacement.
L. Hypercalcemia
H&P Keys
Carcinoma, hyperparathyroidism or hyperthyrosis, sarcoidosis, milk
alkali syndrome.
Diagnosis
Serum free calcium > 2.9 mEq. ECG: short QT and long PR waves.
Disease Severity
Abnormal electrolytes to tetany and cardiac arrest.
Concept and Application
Inappropriate calcium storage mobilization, hormonal etiology, neo-
plasia. Excretion linked to sodium and state of hydration.
Treatment Steps
1. Saline infusion, furosemide.
2. Diuresis.
3. Calcitonin, mithramycin.
4. Sodium etidronate.
M. Hypocalcemia
H&P Keys
Renal failure, hypoparathyroidism, vitamin D deficiency, malabsorp-
tion, Chvostek’s sign, Trousseau’s sign, perioral paresthesias, muscle
cramps.
Diagnosis
Serum calcium (free Ca < 2.2 mEq).
Disease Severity
Electrolyte finding to tetany, neurologic, cardiovascular complica-
tions.
Concept and Application
Secondary to parathyroid surgery, poor absorption, inability to ac-
cess bone stores.
Treatment Steps
1. Correct underlying defect.
2. Administer exogenous calcium and vitamin D.
BIBLIOGRAPHY
Gillenwater J, Grayhack J, Howards S, Duckett JW. Adult and Pediatric Urology, 4th ed.
St. Louis: Mosby, 2002.
Walsh PC, Retik AB, Vaughn ED, Wein AJ (eds.). Campbell’s Urology, 8th ed. Philadel-
phia: W.B. Saunders, 2002.
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Surgical Principles 18
I. DISORDERS OF THE SKIN AND SUBCUTANEOUS TISSUE / 480
A. Cellulitis / 480
B. Lipoma / 480
C. Hemangioma / 480
D. Neurofibroma / 481
E. Basal Cell Carcinoma / 481
F. Squamous Cell Carcinoma / 481
G. Melanoma / 482
H. Sarcoma / 483
I. Decubitus Ulcer / 483
J. Venous Ulceration / 483
K. Arterial Ulcer / 484
477
Copyright © 2004, 2001 by The McGraw-Hill Companies, Inc. Click here for terms of use.
478 Surgical Principles
V. TRAUMA / 491
A. Multiple Injuries / 491
B. Cranial Injury / 491
C. Abdominal Injury / 492
BIBLIOGRAPHY / 525
480
SURGICAL PRINCIPLES Disorders of the Skin and Subcutaneous Tissue
A. Cellulitis
H&P Keys
Erythematous and edematous skin; red or tender streaks; indistinct
advancing edge; history of venous or lymphatic insufficiency.
Diagnosis
Physical examination, culture blister aspirate.
Disease Severity
Malaise, high fever, lymphangitis, bullae, necrosis, sepsis.
Concept and Application
Injury to skin, bacterial invasion of skin and subcutaneous tissue
(Streptococcus, Staphylococcus, anaerobes); lymphatic spread.
Treatment Steps
1. Warm packs, rest, limb elevation.
2. Antibiotics.
3. Remove the infective source (e.g., intravenous [IV] line, infected
bullae).
B. Lipoma
H&P Keys
Soft subcutaneous lobulated mass.
Diagnosis
Physical examination; excisional biopsy.
Disease Severity
Hard mass, rapid growth.
Concept and Application
Benign tumor of mature fat cells (malignant counterpart: liposar-
coma).
Treatment Steps
Surgical excision (cosmesis, local symptoms, persistent growth).
C. Hemangioma
H&P Keys
Red or bluish, raised lesion; present soon after birth; regress sponta-
neously.
Diagnosis
Physical examination.
Disease Severity
Degree of disfigurement, ulceration, high-output cardiac failure (ar-
teriovenous shunting), intravascular coagulation.
D. Neurofibroma
H&P Keys
Mass, pain, sensory deficit, muscular weakness. Multiple in von Reck-
linghausen’s disease (neurofibromatosis, café au lait spots).
Diagnosis
Physical examination, nerve conduction studies, magnetic reso-
nance imaging (MRI).
Disease Severity
Peripheral nerve dysfunction, malignant transformation, involve-
ment of craniospinal axis.
Concept and Application
Proliferation of perineurial/endoneurial fibroblasts and Schwann
cells.
Treatment Steps
Observation or surgical excision (for malignant transformation,
functional impairment, cosmesis).
Diagnosis
Physical examination; biopsy.
Disease Severity
Burn-scar carcinoma (Marjolin’s ulcer); fixed to surrounding struc-
tures, immune status, regional lymphadenopathy.
Treatment Steps
1. Surgical excision, regional lymph node excision (if clinically pal-
pable.
2. Radiotherapy.
G. Melanoma
H&P Keys
Increase in size or change in color of mole or any pigmented nevus,
bleeding, itching, pain, family history. Risk factors: sun exposure,
dysplastic nevus syndrome, xeroderma pigmentosum, > 50 moles,
history of nonmelanoma skin cancer.
Disease Severity
MELANOMA: POOR
PROGNOSTIC FACTORS Lymphatic and hematogenous metastatic spread; most common
sites: liver and lung.
• Head, neck, or trunk
lesion Concept and Application
• Thick tumor (Breslow Malignant tumor of melanocytes. Majority arise de novo; up to 50%
classification) in existing nevi; 90% occur in skin (other sites: eye, anus, viscera).
• Multiple, congenital, or
dysplastic nevi Treatment Steps
• Ulceration
1. Wide surgical excision, elective lymph node dissection for inter-
• Lymphadenopathy
• Vertical growth
mediate-thickness lesions (sentinel node biopsy in selected
• Subungual lesions cases).
2. Chemotherapy, immunotherapy.
3. Regional hyperthermic limb perfusion for recurrent or unre-
sectable extremity lesions.
cram facts U
BRESLOW THICKNESS PROGNOSIS AND MARGIN OF EXCISION
I. Decubitus Ulcer
H&P Keys
Blanching erythema, shallow or extensive dermal defects, fever, cel-
lulitis. Immobilized patient.
Diagnosis
Physical examination, x-ray of ulcer to assess osteomyelitis and sub-
cutaneous extension (air).
Disease Severity
Depth and size of defect, underlying osteomyelitis.
Concept and Application
Microcirculatory ischemia from prolonged pressure due to immobi-
lization. Malnutrition and incontinence contribute. Muscle necrosis
always more extensive than skin (more sensitive to ischemia).
Treatment Steps
1. Prevention: offloading and mobilization (e.g., turn patient fre-
quently, use air or foam mattress), improve nutrition.
2. Definitive: drainage of infected spaces, debridement, musculocu-
taneous flaps or skin grafts.
J. Venous Ulceration
H&P Keys
Ulceration proximal to medial malleolus; history of deep vein
thrombosis (DVT); saphenofemoral or perforator incompetence, in-
competence; brawny, hyperpigmented skin in distal, medial leg; lipo-
dermatosclersosis; dependent edema; cellulitis (stasis dermatitis).
Diagnosis
Clinical examination, Doppler ultrasound, plethysmography to as-
sess for venous reflux.
Disease Severity
Cellulitis, subfascial perforator incompetence, obesity, cardiac fail-
ure.
484
SURGICAL PRINCIPLES Breast Disorders
Treatment Steps
K. Arterial Ulcer
H&P Keys
Smoking, claudication, pain at rest, increased low-density lipopro-
tein (LDL), diabetes. Punched-out appearance of ulcer, absent
pulses, painful ulcer. Ulcer location at toes or pressure point.
Diagnosis
Ankle–brachial index (ABI < 0.5), digital pressures (< 35 mm Hg),
arteriography (for intervention plans only).
Disease Severity
Pain at rest, gangrene, diabetes, previous bypass.
Treatment Steps
Angiography, debridement, and closure or skin graft after revascu-
larization (bypass or angioplasty).
Diagnosis
Regular self-examination; bilateral mammograms further define le-
sion features and assess contralateral breast. Fine-needle aspiration
cytology; ultrasound; core or excisional biopsy.
Disease Severity
A mass that is discrete, hard, fixed, irregular, or associated with lym-
phadenopathy or skin changes is suggestive of breast cancer. Fever
and fluctuation indicate abscess.
cram facts U
BREAST CANCER STAGES
A. Thyroid Neoplasm
H&P Keys
Neck mass, dysphagia, dysphonia, respiratory difficulty, hoarseness,
vocal cord paralysis, female patient, childhood neck irradiation, lym-
phadenopathy, goiter. Medullary cancer in multiple endocrine neo-
plasia type II (MEN II) syndrome.
Diagnosis
Serum thyroid-stimulating hormone (TSH) level, fine-needle aspira-
tion cytology, high-resolution ultrasound, radioisotope scan, calci-
tonin if suspect medullary thyroid cancer. Four types of primary thy-
roid cancer: papillary (85%), follicular (10%), medullary (4%), and
anaplastic (1%).
Disease Severity
Symptoms of local invasion, metastatic disease, older age, size of tu-
mor, histology, lymphadenopathy, cellular differentiation. Anaplastic
carcinoma—poor prognosis. Papillary and follicular cancer—good
prognosis.
B. Hyperparathyroidism
Diagnosis
ETIOLOGY OF Hypercalcemia with elevated or normal level of parathyroid hor-
HYPERPARATHYROIDISM mone (PTH), hypophosphatemia, ultrasonography, sestamibi scan.
d
C. Cushing’s Disease/Cushing’s Syndrome
H&P Keys diagnostic
Cushing’s syndrome is caused by an excess of adrenocortical hor- decisions
mones from any source. Cushing’s disease refers to pituitary hyper-
secretion of adrenocortical hormone (ACTH). Signs: “mooning” of CUSHING’S SYNDROME
face, central obesity, wide purple striae, spontaneous ecchymosis,
hirsutism, buffalo hump, impotence or amenorrhea, hypertension, Adrenal Hyperplasia
High plasma ACTH,
diabetes.
secondary to pituitary
hypersecretion, pituitary
Diagnosis
tumor (image with MRI), or
Twenty-four-hour urine cortisol and low-dose dexamethasone sup- ectopic tumor.
pression test confirm Cushing’s syndrome; plasma ACTH levels;
Adrenal Adenoma
high-dose dexamethasone suppression test distinguishes between pi- Low plasma ACTH.
tuitary (suppresses) and ectopic sources (does not suppress) of Abdominal CT scan or MRI
ACTH; isotope-scanning, CT scan of abdomen, angiography, venous to localize.
sampling, MRI, NP.59 (iodocholesterol scanning). Adrenal Carcinoma
Low ACTH. Palpable
Disease Severity
abdominal mass. Markedly
Hypertension, stroke, diabetes mellitus, muscle wasting, osteoporosis elevated urinary 17-
(pathologic fractures). ketosteroids and plasma
dehydroepiandrosterone
Concept and Application
(DHEA).
1. Excess cortisol production: pituitary adenoma producing ACTH,
adrenal adenoma or carcinoma, ACTH-secreting tumors.
2. Ectopic ACTH secretion results from bronchial carcinoid, thymic
U
carcinoid, or pulmonary neoplasm.
3. High ACTH indicates pituitary or ectopic ACTH tumor.
4. Low ACTH indicates hypersecretion from adrenal source. cram facts
Treatment Steps
Medical ETIOLOGY OF CUSHING’S
1. Mitotane for metastatic adrenal cancer (blocks β2 hydroxyla- SYNDROME
tion, reversible adenolytic).
2. Lifelong mineralo- and glucocorticoid replacement after bilat- 1. Autonomous adrenal
eral adrenalectomy. tumor (20%)
3. Temporary steroid replacement after unilateral adrenalec- 2. Pituitary adenoma
tomy (because of contralateral atrophy). (60%)
3. Ectopic ACTH
Surgical production (10%)
1. Pituitary ablation (transsphenoidal), adrenalectomy (laparo- 4. Iatrogenic Cushing’s
scopic if < 6 cm). 5. Adrenocortical cancer
2. Postoperative corticosteroid therapy. (rare)
D. Pheochromocytoma
H&P Keys
Classic triad—hypertension, palpitation, diaphoresis.
Diagnosis
Twenty-four-hour urine catecholamines, metanephrine, vanillylman-
delic acid (VMA), serum catecholamine, CT scan of abdomen,
metaiodobenzylguanidine (MIBG) isotope scanning (for extra-
abdominal or malignant pheochromocytoma).
488
SURGICAL PRINCIPLES Disorders of the Kidney and Urinary Tract
Disease Severity
A. Testicular Tumors
H&P Keys
Asymptomatic painless swelling (85%), history of cryptorchidism
(15%), associated hydrocele (5%).
Diagnosis
Physical examination, ultrasound, orchiectomy.
Disease Severity
Staging: CT scan (chest, abdomen), tumor markers (β-human chori-
onic gonadotropin [β-hCG], and α-fetoprotein [α-FP]).
Concept and Application
Ninety-five percent are germ cell tumors (seminomas most com-
mon).
Treatment Steps
1. Orchiectomy by inguinal approach.
2. For seminoma: radiation to retroperitoneum.
3. For nonseminoma germ-cell tumor: retroperitoneal lympha-
denectomy, adjuvant chemotherapy.
Diagnosis
Urinary cytology, cystoscopy with biopsy, CT scan and MRI to detect
local invasion and distant metastases.
Treatment Steps
Carcinoma In Situ
1. Intravesical therapy (bacillus Calmette–Guérin [BCG], doxo-
rubicin, mitomycin C).
2. Intravesical fulguration.
Superficial
1. Intravesical chemotherapy, local immunotherapy (BCG).
2. Transurethral endoscopic resection.
3. Recurrence common, may require cystectomy.
Invasive—Radical cystectomy, chemotherapy.
C. Prostate Cancer
H&P Keys
Often asymptomatic; average age 73 years. Dysuria, urinary fre-
quency or retention, hematuria. Risk factors: age, race (African-
Americans twofold increase), family history.
Diagnosis
Digital rectal examination, prostate-specific antigen (PSA). Confir-
mation by needle biopsy, transrectal ultrasound.
Treatment Steps
Diagnosis
CT scan, MRI, renal ultrasound, cystoscopy (for hematuria).
490
SURGICAL PRINCIPLES Disorders of the Kidney and Urinary Tract
E. Renal Calculi
H&P Keys
Excruciating pain (upper back to testicle or vulva) secondary to dila-
tion of urinary tract, hematuria, nausea, vomiting, urinary frequency
or urgency.
Diagnosis
Urinalysis (UA): hematuria, crystals; ultrasound: hydronephrosis or
acoustic shadow, noncontrast CT scan.
Disease Severity
Recurrent calculi, hyperparathyroidism, decreased renal function,
hydronephrosis.
Treatment Steps
Medical (50% Pass Spontaneously)
1. Analgesics.
2. Diuretics, cholestyramine (oxalate-binding resin).
3. Fluids, low-purine diet, alkali, and allopurinol for uric acid
stones.
Surgical (Indicated for Infection, Complete Obstruction, Intractable Pain, Pro-
gressive Renal Damage)
1. Shock wave lithotripsy.
2. Transurethral extraction if < 4 mm.
3. Nephrostomy.
Diagnosis
Digital rectal examination, UA, PSA.
Disease Severity
Indication for biopsy of BPH prostate: PSA > 4.
V. TRAUMA
diagnostic
decisions d
A. Multiple Injuries GLASGOW COMA SCALE
U
Presence of immediately life-threatening conditions, old age, preex-
isting diseases, intoxication (alcohol, drugs), multiple systems af-
fected, hypothermia, severe neurologic deficit. cram facts
Concept and Application
Death and sequelae may result from initial injury (e.g., aortic tran-
CLUES FOR THORACIC
section, severe brain damage) or complications (e.g., brain edema, AORTIC INJURY
sepsis, renal failure).
• Severe deceleration
Treatment Steps
mechanism
1. ABCs (airway, breathing, circulation), oxygen, fluid replacement, • Hypotension
warming; tetanus toxoid, antibiotics. • Left hemothorax; apical
2. Treatment of life-threatening conditions and management ac- pleural hematoma
cording to specific injuries. • Fractures: ribs, sternum,
clavicle, scapula
B. Cranial Injury • Widened mediastinum
• Obliteration of aortic
H&P Keys knob contour
Loss of consciousness, amnesia, headache, lethargy, seizures, weak- • Deviated nasogastric
ness, scalp laceration or hematoma, hemotympanum, rhinorrhea, tube
otorrhea, periorbital or mastoid ecchymosis.
492
SURGICAL PRINCIPLES Trauma
management decisions d
LIFE-THREATENING CONDITIONS REQUIRING IMMEDIATE TREATMENT
Airway Obstruction
Signs: respiratory distress; cyanosis. Treatment: avoid tongue drop (most common cause),
remove blood, foreign body. If face, oropharynx, or larynx severely damaged, emergency surgical
airway is required (cricothyroidotomy or tracheostomy).
Tension Pneumothorax
Signs: respiratory distress, cyanosis, hypotension, tracheal deviation to the contralateral side,
unilateral absence of breath sounds, neck vein distention. Insert large-bore needle into anterior
chest (2nd intercostal), followed by tube thoracostomy.
Open Pneumothorax
“Sucking chest wound,” occurs when a chest wall defect is greater in diameter than two-thirds of
the trachea. Requires closure of the defect and tube thoracostomy.
Flail Chest
Unstable chest wall from sternum or rib fractures. Intubation and ventilation. Treat associated
hemo- or pneumothorax.
Exsanguinating Hemorrhage
Stop obvious bleeding by applying direct pressure. Establish large-bore IV lines. Massive
hemothorax—shock, loss of breath sounds, shifting of mediastinum—require chest tube drainage
and urgent thoracotomy.
Cardiac Tamponade
Penetrating parasternal or upper abdominal trauma. Cyanosis, hypotension, distended neck
veins, and muffled heart sounds. Needle pericardiocentesis for temporary relief; sternotomy or
thoracotomy for definitive treatment.
Diagnosis
Figure 18–1. Computed tomography (CT) scan showing ruptured spleen following abdominal
trauma in patient with human immunodeficiency virus (HIV) infection and splenomegaly.
Note intra- and perisplenic hematoma (arrows).
494
SURGICAL PRINCIPLES Postoperative Infections
A. Wound Infections
H&P Keys
Fever, wound pain, malaise, anorexia, tachycardia, local redness, ten-
derness, crepitance, swelling, discharge (bloody, serous, or puru-
lent), postoperative days 3–7 most commonly.
Diagnosis
Physical examination, wound exploration, culture.
Disease Severity
Abscess, fascial dehiscence, evisceration, poor nutrition.
Treatment Steps
1. Evacuate pus, debridement.
2. Antibiotics.
3. Local wound care.
4. Optimize nutrition/hemodynamics.
management decisions d
POSTOPERATIVE INFECTIONS
Wound
Erythema, edema, pain/tenderness, drainage, fever. Open and pack the wound. Antibiotics if
extensive cellulitis present or patient immunocompromised. Check for foreign body or fistula.
Respiratory Tract
Productive cough, yellow or green sputum; fever, tachycardia, tachypnea; leukocytosis; CXR
infiltrate. Treat with intensive respiratory physiotherapy and IV antibiotics.
IV Lines
Superficial phlebitis (erythema, and tenderness over IV line insertion area): Remove line; treat with
warm compresses and analgesic anti-inflammatory drugs. Central venous catheter infection
(positive blood cultures): remove line and give antibiotics if infection persists or patient
immunocompromised.
Intra-abdominal
Peritonitis requires exploratory laparotomy or laparoscopy. Abscess may be drained
percutaneously. Both require IV antibiotics that cover enteric gram-negatives and anaerobes.
Genitourinary
History of catheterization or instrumentation of urinary tract. Remove catheters if possible.
Maintain diuresis. Oral or IV antibiotics depending on severity of infection.
Gastrointestinal
Fever, leukocytosis, and diarrhea. Stool culture and Clostridium difficile toxin assay. Treat with
metronidazole or vancomycin.
Prosthetic device
Fever, leukocytosis, and bacteremia. CT scan, MRI, radionuclide scan, blood cultures. Give IV
antibiotics. Definitive treatment is removal of prosthesis.
495
Definitions
• Transient ischemic attack (TIA): brief paresis or numbness of
an arm or leg contralateral to the affected carotid territory (<
24 hours in duration).
• Amaurosis fugax (AF): transient episode of monocular or par-
tial blindness.
• Stroke: permanent neurologic deficit.
Symptoms—TIAs, amaurosis fugax, or resolving or permanent neu-
rologic deficit. Vertebrobasilar artery symptoms consist of ver-
tigo, drop attacks, or dizziness due to cerebellar or brain stem is-
chemia.
496
SURGICAL PRINCIPLES Diseases of the Circulatory System
Figure 18–2. Carotid angiogram showing a high-grade stenosis of the origin of the internal (ar-
row) as well as the external carotid artery. This study is currently reserved for recurrent carotid artery
stenosis (atherosclerotic or radiation therapy related), high carotid bifurcation, stenotic “string sign”
lesion of the internal carotid artery, or if the patient is being considered for carotid angioplasty and
stenting.
497
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carotid.
Definitions
• Claudication: deep ache or cramping (most commonly in the
calf) secondary to muscle ischemia during exercise.
• Rest pain: burning pain usually in the forefoot; indicates severe
disease.
498
SURGICAL PRINCIPLES Diseases of the Circulatory System
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Risk Factors—Smoking, hypertension, diabetes, hyperlipidemia.
Diagnosis
cram facts
Physical Examination—Diminished or absent pulses distal to the ar-
terial stenosis, pallor on elevation, rubor with dependency.
ANKLE–BRACHIAL INDEX Noninvasive Vascular Tests—ABI, segmental pressures with pulse vol-
ume recordings or Doppler waveforms, arterial duplex.
1.0–1.2 Normal
< 0.9 Claudication Imaging Studies—Arteriography reserved for preoperative patients
< 0.4 Rest pain to define the site of arterial obstruction and delineate arterial
< 0.5 Tissue loss anatomy. MRA provides arterial anatomy without contrast, but
may overestimate stenosis.
Disease Severity
Heart disease, previous bypasses, small-vessel disease (limits revascu-
larization attempts).
Concept and Application
Atherosclerotic narrowing (stenosis) causes decreased blood flow
during exercise (mild disease, claudication) or at rest (severe dis-
ease, rest pain).
Treatment Steps
Medical
1. Reduction of risk factors (smoking cessation is most impor-
tant), exercise program.
2. Antiplatelet and vasodilatory agents (cilostazol).
Surgical
1. Indication: incapacitating claudication or limb salvage.
2. Aortoiliac or aortofemoral reconstruction: 80% patency after
5 years. Iliac angioplasty (stenting) also has good results. Ex-
tra-anatomic bypass (femorofemoral, iliofemoral, or axillob-
ifemoral) for high-risk patients.
3. Femoropopliteal and infrapopliteal reconstruction ideally
with ispsilateral greater saphenous vein. Reconstructions to
the tibial, peroneal, or pedal arteries are generally performed
for limb salvage.
D. Aneurysms of the Thoracic Aorta
H&P Keys
Majority are asymptomatic. Chest pain and pressure, hoarseness, su-
perior vena cava syndrome, and cough and dyspnea from tracheo-
bronchial obstruction. Hemoptysis may indicate erosion into the tra-
chea and mainstem bronchus.
Diagnosis
CT scan and MRI; echocardiography and anteriography; trans-
esophageal echo is a sensitive diagnostic method.
Disease Severity
Ascending Aortic—Mortality of surgical repair < 10%.
Aortic Arch—Mortality rate 10–15%; neurologic complications 10%.
Descending Aortic—Mortality rate 10%; paraplegia, 5–20%.
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E. Aortic Dissection
H&P Keys
Severe, sudden-onset pain radiating to the back. May exhibit neuro-
logic deficit, dyspnea, pulmonary edema, nausea, and vomiting.
More common in men; typical age 45–70. Hypertensive history in
80–90%. Shock, pulmonary edema, and a murmur of aortic insuffi-
ciency may be noted.
Differential diagnosis: myocardial infarction, cerebrovascular ac-
cident, pulmonary embolism, aortic thrombosis, and acute abdomi-
nal disorders.
Diagnosis
CXR may show a dilated aorta, widened mediastinum, pulmonary
edema, or mass effect with or without pleural effusion (rupture). CT
(Fig. 18–3A), MRI, and transesophageal echocardiogram. Aor-
togram usually shows splitting of contrast column (Fig. 18–3B).
Disease Severity
Dissections classified as ascending (usually involve the entire aorta
or descending aorta distal to the left subclavian artery).
Concept and Application
Degeneration of medial layer can be caused by hypertension, athero-
sclerosis, coarctation, endocrine factors, Marfan’s syndrome,
trauma. Hemodynamic forces, shear stress, and weakened arterial
wall lead to development of an intimal tear. A hematoma forms
within the torn aorta and dissects distally and proximally within the
media.
Treatment Steps
Figure 18–3. Dissecting thoracoabdominal aneurysm in a 70-year-old man with history of hypertension. (A) Contrasted computed tomography (CT) scan of
descending thoracic aorta showing dilatation (arrowheads), double lumen, and mural thrombus. (B) Aortogram demonstrating true and false lumina, separated
by a septum. The dissection column (arrowheads) extends to the suprarenal portion of the aorta. (Courtesy of Dr. Oswaldo Yano, Mount Sinai School of Medicine,
New York, NY.)
Diagnosis
Ultrasound to assess size of aneurysm (most rapid diagnosis). CT
scan to assess size of aneurysm as well as extent (Fig. 18–4). Aortog-
raphy used selectively to define suprarenal involvement, suspected
renovascular disease, visceral arterial involvement or stenosis, possi-
ble distal occlusive disease.
Disease Severity
Risk of rupture increases with increasing diameter. Majority of
aneurysms enlarge slowly over time (2–4 mm/yr). Risk of rupture
5–7%/yr for aneurysms 5–7 cm; 20%/yr for aneurysms > 7 cm.
Treatment Steps
1. Recommend repair of all aneurysms ≥ 5 cm in diameter in rea-
sonable-risk patients.
2. Aortic replacement with prosthetic grafts.
3. Endovascular repair for high-risk patients.
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cram facts
I. Arterial Embolism/Thrombosis
H&P Keys
Acute occlusion: six Ps—pulselessness, pallor, paresthesia, pain,
paralysis, and poikilothermia. Irreversible limb loss may occur within
6–8 hours.
Diagnosis
ABI; angiography; echocardiography because cardiac system is most
common source of emboli.
Disease Severity
Embolism usually secondary to cardiac source (atrial fibrillation, my-
ocardial infarction, congestive heart failure).
Concept and Application
Embolic occlusion is more common than thrombotic. Thrombosis
generally occurs in area of atherosclerotic disease. Compromise of
oxygenation leads to anaerobic metabolism, acidosis, membrane
destabilization, and cellular edema and death.
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2. Embolectomy.
3. Thrombolytic agents (tissue plasminogen activator [tPA]) are
used when thrombus has progressed into small vessels. cram facts
4. Fasciotomy for reperfusion after prolonged ischemia
5. Maintenance of urine output recommended to prevent renal in-
jury from reperfusion of ischemic limb. DVT RISK FACTORS
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knee thrombi: 90–100%).
Concept and Application
cram facts
Thrombosis is related to three factors—endothelial abnormalities,
blood stasis, and hypercoagulability (Virchow’s triad).
PULMONARY EMBOLISM
Treatment Steps
1. Prevent with exercise, elastic or pneumatic stockings, and early Signs and Symptoms
ambulation after surgery. Important role for DVT prophylaxis • Dyspnea
with anticoagulant medications in patients at high risk (orthope- • Tachypnea
dic procedures, etc.). • Pleuritic chest pain
2. Bed rest with leg elevation to reduce the edema after DVT. • Hemoptysis
3. Drug therapy to prevent propagation of the thrombus and pul- • Bulging neck veins
monary embolus includes IV unfractionated heparin or low-mole- Specific Diagnostic
cular-weight heparin. Warfarin therapy is then initiated, and con- Tests
• Ventilation–perfusion lung
tinued for 3–6 months.
scan
4. Vena cava filter placement is indicated when anticoagulation is • Spiral CT scan
contraindicated or patient has pulmonary embolism or recurrent • Pulmonary arteriography
DVT on adequate anticoagulation.
5. Thrombolytic therapy is used in selected cases of iliofemoral DVT
to decrease the late sequelae of venous thrombosis (valvular in-
competence, chronic venous insufficiency).
K. Varicose Veins
H&P Keys
Affect 10–20% of the population. Patients may be asymptomatic or
complain of aching, swelling, heaviness, cramps, itching; occasion-
ally hemorrhage. Physical findings: dry, scaling skin; edema and
brawny induration; dilated, tortuous, subcutaneous veins of the
thigh and leg.
Diagnosis
Valvular competence of the deep, superficial, and perforating veins
must be determined and can be performed most accurately with a
duplex ultrasound examination.
Concept and Application
Caused by incompetent valves and abnormalities in the structure of
the vein wall.
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SURGICAL PRINCIPLES Diseases of the Gastrointestinal (GI) Tract
Treatment Steps
1. Nonoperative management consists of improving venous return
and reducing venous pressure by elevation and graduated elastic
stockings.
2. Surgical therapy is indicated for severe symptoms, large varicosi-
ties, superficial phlebitis, hemorrhage, ulceration, and cosmesis.
It consists of saphenous vein ablation (stripping, radiofrequency,
or laser) and removing branch varicosities.
3. Sclerotherapy is used for small and “spider” varicosities.
L. Superficial Thrombophlebitis
H&P Keys
History of IV catheters or drug abuse. Cancer may cause recurrent
or migratory superficial thrombophlebitis (Trousseau’s sign). Signs
include erythema, pain, induration, heat, tenderness along the
course of a superficial vein.
Diagnosis
History and physical examination.
Disease Severity
Disease usually has a short and uncomplicated time course.
Concept and Application
Introduction of bacteria with either IV catheters or IV drug abuse.
Local infection and inflammatory response result in thrombosis.
Treatment Steps
1. Conservative measures: nonsteroidal anti-inflammatory agents, lo-
cal heat, and elastic compression bandages.
2. If inflammation of saphenous vein involves the saphenofemoral
junction, pulmonary embolism may result, and ligation of the
greater saphenous vein or anticoagulation is indicated.
3. In cases of suppuration, surgical excision of involved vein is indi-
cated.
A. Zenker’s Diverticulum
H&P Keys
Dysphagia, regurgitation of undigested food, coughing, mass on the
left side of the neck.
Diagnosis
Barium swallow showing cricopharyngeal bar and hypopharyngeal
diverticula.
Disease Severity
Pulmonary complications, poor nutrition, weight loss, size of pouch.
Most common esophageal diverticulum.
Concept and Application
Abnormal cricopharyngeus muscle (more scar, smaller opening di-
mensions) exerts increased hypopharyngeal bolus pressure during
swallowing.
505
C. Achalasia
H&P Keys
Dysphagia, regurgitation of undigested food, weight loss, dyspnea,
cough.
Diagnosis
Barium esophagogram showing dilated esophagus with tapered dis-
tal end (“bird’s beak”), esophagoscopy, esophagomanometry.
Disease Severity
Aspiration, pneumonia, old age, recurrent dysphagia. Increased risk
for squamous cell carcinoma.
Concept and Application
Dilatation, absent esophageal peristalsis, incomplete relaxation of
lower esophageal sphincter, hypertensive lower esophage-al sphinc-
ter, decreased ganglion cells in Auerbach’s plexus.
Treatment Steps
Medical––Calcium channel blockers, esophageal balloon dilata-
tion.
Surgical––Distal esophagomyotomy (Hellar myotomy) and antire-
flux procedure (laparoscopic or open).
D. Esophageal Varices
H&P Keys
Hematemesis, melena, signs of liver failure (ascites, encephalopathy,
clonus, jaundice, hepatomegaly, palmar erythema, cachexia). His-
tory of alcohol abuse.
Diagnosis
Esophagogastroduodenoscopy.
Disease Severity
Recurrent bleeding. Child’s classification.
Concept and Application
Significant elevation of portal pressures. Shunting of blood through
left gastric vein (coronary vein) into submucosal plexus. Majority of
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506
SURGICAL PRINCIPLES Diseases of the Gastrointestinal (GI) Tract
cram facts
A B C
Bilirubin <2 2–3 >3
Albumin > 3.5 3–3.5 <3
Ascites None Mild Severe
Mortality 5% 25% 50%
F. Gastric Volvulus
H&P Keys
Abdominal pain; retching with inability to vomit; inability to pass na-
sogastric tube.
Diagnosis
Upper GI series, endoscopy.
Disease Severity
Perforation or gangrene of stomach, shock.
Concept and Application
Presence of diaphragmatic defect (paraesophageal hernia in adults).
Rotation around the longitudinal axis (organoaxial) or vertical axis
(mesenteroaxial).
507
H&P Keys
Periumbilical pain shifting to right lower quadrant, anorexia, tender
right lower quadrant, tender on rectal examination.
Diagnosis
History and physical, leukocytosis, ultrasound (especially in chil-
dren). Fecalith (uncommon), CT scan with oral or rectal contrast.
Disease Severity
Perforation and abscess. Marked leukocytosis, high fever, peritonitis,
elderly, pregnancy, delayed diagnosis.
Concept and Application
Obstruction of appendix lumen and bacterial invasion.
Treatment Steps
1. Fluid resuscitation, antibiotics.
2. Appendectomy (open or laparoscopic).
H. Ulcerative Colitis
H&P Keys
Diarrhea, rectal bleeding, weight loss, abdominal cramps/tender-
ness.
Diagnosis
Colonoscopy, barium enema; rectum always involved; continuous,
uninterrupted inflammation.
Disease Severity
Dehydration, malnutrition, cecal perforation, massive lower GI bleed-
ing, total colonic involvement. Increased risk of colon carcinoma.
Concept and Application
Etiology unclear; immunologic injury to colon mucosa, defect of
suppressor T cells in intestinal wall.
Treatment Steps
Acute
1. Bed rest, sulfasalazine, steroids, nothing by mouth.
2. Total parenteral nutrition.
3. Subtotal colectomy and ileostomy (if fails medical manage-
ment, persistent GI bleeding, or toxic megacolon).
Chronic––Sulfasalazine, steroids, immunosuppressive agents, proc-
tocolectomy with ileoanal pull-through and J-pouch.
I. Crohn’s Disease
H&P Keys
Abdominal pain, diarrhea, weight loss, perianal fistula and abscess.
Diagnosis
Small-bowel series, endoscopy, cobblestone mucosa, skip lesions, fis-
tula.
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SURGICAL PRINCIPLES Diseases of the Gastrointestinal (GI) Tract
cram facts
Disease Severity
Severe anal disease, extraintestinal manifestations, malnutrition, ab-
dominal mass, intra-abdominal abscesses, amount of small bowel
and colon involved.
Concept and Application
Possible immunologic mechanism that leads to inflammatory reac-
tion and transmural damage.
Treatment Steps
Medical
1. Bowel rest.
2. Low-residue, high-protein diet.
3. Sulfasalazine, metronidazole, aminosalicylates orally or rec-
tally.
4. 6-Mercaptopurine and cyclosporin in refractory cases.
Surgical
1. Small-bowel resection, segmental colectomy, stricturoplasty.
2. Surgery is indicated only for complications (obstruction, per-
foration, GI bleeding, failure of medical therapy, malignancy).
3. Unlike ulcerative colitis, surgery is not definitive treatment.
J. Acute Mesenteric Ischemia
H&P Keys
Abdominal pain (out of proportion to physical findings), rectal
bleeding, atrial fibrillation.
Diagnosis
Leukocytosis, elevated lactic acid, angiography (definitive).
Disease Severity
Peritonitis (bowel infarction).
Concept and Application
Four mechanisms—embolic, thrombosis, vasoconstriction, venous
thrombosis (see Cram Facts).
Treatment Steps
1. IV fluids, antibiotics, thromboembolectomy, bowel resection, an-
ticoagulation, intra-arterial papaverine.
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509
Mechanism Treatment
Embolic occlusion (Fig. 18–5) Embolectomy; control dysrhythmia
Thrombosis of preexisting stenosis Thrombectomy; endarterectomy or bypass
Severe splanchnic vasoconstriction due Intra-arterial papaverine; improve hemodynamics
to low flow state
Mesenteric vein thrombosis Anticoagulation
K. Small-Bowel Obstruction
H&P Keys
Colicky abdominal pain, vomiting, failure to pass flatus or feces; ab-
dominal distention, dehydration, previous surgery.
Diagnosis
Plain abdominal x-rays (distended small bowel with air–fluid levels)
(Fig. 18–6), barium small-bowel follow-through, CT scan.
Disease Severity
Poor urine output, leukocytosis, unremitting pain, high fever, peri-
tonitis.
Concept and Application
Narrowing or occlusion of bowel lumen, proximal bowel distention
with gas and fluid. Distention leads to increased intestinal wall pres-
sure, ischemia, and gangrene. Adhesions, hernia, and tumor are the
most common causes.
Treatment Steps
1. Fluid and electrolyte resuscitation, nasogastric decompression.
2. Laparotomy, with correction of cause with or without bowel resec-
tion.
Figure 18–5. Acute mesenteric ischemia in a 74-year-old man with history of atrial fibrillation and
heart failure, who developed acute abdominal pain and shock. Exploratory laparotomy disclosed
extensive areas of intestinal necrosis and embolic occlusion of the superior mesenteric artery.
510
SURGICAL PRINCIPLES Diseases of the Gastrointestinal (GI) Tract
Figure 18–6. Upright abdominal x-ray showing small-bowel obstruction secondary to Crohn’s
disease. Note distended loops of small-bowel and multiple air–fluid levels (arrows).
L. Large-Bowel Obstruction
H&P Keys
Crampy abdominal pain, nausea, obstipation; abdominal distention;
abdominal tenderness.
Diagnosis
Plain x-ray of abdomen, Gastrografin enema, colonoscopy. Evaluate
for volvulus.
Disease Severity
Low urine output, leukocytosis, obstructing tumor, peritonitis.
Concept and Application
Five percent of large-bowel obstruction is caused by volvulus, a twist-
ing of bowel on mesenteric axis.
Treatment Steps
Medical
1. Fluid and electrolyte resuscitation, nasogastric decompres-
sion, monitor urine output.
2. Endoscopic decompression (volvulus, partially obstructing le-
sion).
Surgical
1. Decompression (colostomy or cecostomy).
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M. Diverticulitis
H&P Keys
Abdominal pain, left lower quadrant tenderness, constipation, low- cram facts
grade fever.
Diagnosis UNCOMPLICATED
History and physical, CT scan of abdomen and pelvis, water-soluble DIVERTICULITIS
contrast enema (rarely).
Medical Management
Disease Severity • Bowel rest
Perforation, abscess formation, fecal peritonitis, septic shock. • IV fluid resuscitation
• Antibiotics (ciprofloxacin
Concept and Application and metronidazole)
Raised intracolonic pressure leading to pulsion diverticulum; en- • High-fiber diet after
trapped fecalith causes obstruction of the diverticulum with result- resolution of symptoms
ing inflammation and potential perforation. Indications for Surgery
• Second episode of
Treatment Steps uncomplicated
diverticulitis
Medical (Indicated for Uncomplicated Initial Episode)
• Mass or abscess
1. Systemic antibiotics. • Fistula
2. Fluid resuscitation. • Perforation
3. Bowel rest. • Obstruction
Medical (Indicated for Complicated or Recurrent Diverticulitis) • Bleeding
1. Drainage of abscess (may be percutaneous).
2. Colectomy (Hartman’s procedure).
3. Colectomy with primary anastomosis (can be done laparo-
scopically).
Figure 18–7. Benign tumor of sigmoid colon (submucosal lipoma) in a 35-year-old man. Note regular and smooth appearance of the lesion and normal distensi-
bility of the colonic wall.
Disease Severity
Family history of polyposis or colon cancer, size of polyp, degree of
dysplasia, multiple adenomas.
Concept and Application
Neoplasia of intestinal epithelium (tubular, villous), abnormal mix-
ture of normal tissue (hamartomas), other benign growths (e.g.,
lipoma).
Treatment Steps
Polypectomy, bowel resection, panproctocolectomy and J-pouch (for
familial polyposis).
Treatment Steps
1. Surgical resection usually indicated—segment of colon along
with regional lymph nodes.
2. Resection of liver metastases can contribute to cure.
3. Radiotherapy useful for rectal tumors.
4. Chemotherapy indicated for Dukes’ C stage or distant metastasis
(5-fluorouracil/leukovorin).
Q. Rectal Tumor
H&P Keys
Rectal bleeding, mucous discharge, change of bowel habits, tenes-
mus, rectal mass.
Diagnosis
Colonoscopy, rigid proctoscopy, endorectal ultrasound (to deter-
mine depth of invasion), MRI with endorectal coil, CT scan.
Disease Severity
Anemia, abnormal liver function tests (LFTs), sphincter destruction,
lymph node involvement, fixed tumor.
Treatment Steps
1. Local excision for superficial lesions.
2. Low anterior resection or abdominoperineal resection.
3. Radiation, chemotherapy (often preoperatively for tumor down-
staging).
R. Hemorrhoids
H&P Keys
Rectal bleeding, mucous discharge, prolapse spontaneously or with
defecation.
Diagnosis
Rectal examination, proctosigmoidoscopy.
Disease Severity
Degree of hemorrhoidal prolapse, thrombosis.
Treatment Steps
1. Evaluate for coexistent rectal or sigmoid pathology (e.g., cancer),
sigmoidoscopy.
2. High-fiber diet, warm sitz baths.
3. Rubber band ligation.
4. Hemorrhoidectomy.
514
SURGICAL PRINCIPLES Diseases of the Gastrointestinal (GI) Tract
S. Perirectal Abscess
H&P Keys
Deep buttock or rectal pain, fever, perianal mass.
Diagnosis
Rectal–perianal examination, CT scan sometimes indicated.
Disease Severity
Coexisting Crohn’s disease, extension into adjacent anatomic space,
complex fistula.
Concept and Application
Infection in anal gland starts as intersphincteric abscess; can spread
to become supralevator, perianal, or ischioanal abscess.
Treatment Steps
1. Examination under anesthesia.
2. Incision and drainage.
3. Warm sitz baths and local wound care.
T. Anorectal Fistula
H&P Keys
Chronic purulent discharge from perianal opening, history of
perianal abscess.
Diagnosis
Rectal examination, anoproctoscopy, rectal ultrasound, fistulogra-
phy.
Disease Severity
Coexisting Crohn’s, complex or high fistulas, TB, incontinence,
HIV.
U. Pilonidal Cyst
H&P Keys
Purulent drainage from sacrococcygeal sinus; pain, tender mass, in-
duration.
Diagnosis
Physical examination.
Disease Severity
Multiple tracts, multiple recurrences.
Concept and Application
Macerated skin, suction effect of buttock when walking, loose hair
embedded in skin.
Treatment Steps
1. Incision and drainage of abscess; wide local excision of sinus
tract, down to sacral fascia.
2. Healing by primary closure (if not infected), secondary intention,
local flaps.
515
A. Duodenal Atresia
H&P Keys
Congenital, diagnosed shortly after birth; bile-stained vomiting, post-
feeding vomiting, distended upper abdomen, antepartum polyhy-
dramnios, stigmata of Down’s syndrome (30%).
Diagnosis
Plain x-ray (“double-bubble” sign), upper GI series and follow-
through, barium enema, evaluate cardiac system with echocardiogra-
phy.
Disease Severity
Prematurity, associated anomalies (e.g., congenital heart disease),
low birth weight, trisomy 21.
Concept and Application
Hypoplasia or atresia of duodenum at level of ampulla. Evaluate for
annular pancreas.
Treatment Steps
1. Elevate head of bed, nasogastric decompression.
2. Correct fluid and electrolyte imbalance.
3. Duodenoduodenostomy, decompression gastrostomy; correct as-
sociated malrotation.
B. Biliary Atresia
H&P Keys
Jaundice, dark urine, pale-colored stools, hepatomegaly, spleno-
megaly, usually in 2- to 4-week-old infants.
Diagnosis
Technetium-iminodiacetic acid (Tc-IDA) hepatobiliary scan, con-
jugated hyperbilirubinemia, abdominal ultrasound; needle biopsy
of liver, exploratory laparotomy. Must differentiate from α-anti-
trypsin deficiency.
Disease Severity
Jaundice, fever, cirrhosis, sepsis, esophageal varices, liver failure, de-
layed diagnosis, age (< 12 weeks).
Concept and Application
Absence of patent bile ducts, periportal fibrosis, cirrhosis.
Treatment Steps
1. IV fluids, vitamin K.
2. Roux-en-Y hepaticojejunostomy, liver transplantation.
C. Malrotation
H&P Keys
Biliary vomiting, hematemesis, heme-positive nasogastric aspirate,
failure to thrive, mild abdominal distention.
Diagnosis
Plain x-ray of abdomen, upper GI series, barium enema.
Disease Severity
Peritonitis, tachycardia, hypotension.
516
SURGICAL PRINCIPLES Congenital Abnormalities
D. Hirschsprung’s Disease
H&P Keys
Failure to pass meconium, chronic constipation, bile-stained vomit-
ing, reluctance to feed, diarrhea, irritability, abdominal distention,
palpable stool in lower abdomen, male infant.
Diagnosis
Plain abdominal x-ray, barium enema, rectal biopsy, and rectal
manometry.
Disease Severity
Enterocolitis, malnutrition, length of bowel involved.
Concept and Application
Interrupted development of myenteric nervous system (lack of gan-
glion cells in distal intestine) causes a functional obstruction; rec-
tum always involved.
Treatment Steps
1. Rectal tube and colonic washing.
2. Colostomy, endorectal pull-through.
E. Imperforate Anus
H&P Keys
Anal dimple but no orifice; ectopic anal opening or fistula; meco-
nium in vagina, urethra, or urine.
Diagnosis
Physical examination, test urine for meconium, ultrasound of kid-
neys and heart, sacrum x-ray.
Disease Severity
Associated anomalies (70%)—VACTERL (vertebral, anorectal, car-
diac, tracheal, esophageal, renal, limb), acidosis, neurologic deficit,
agenesis of sacral vertebrae, incontinence.
Concept and Application
Abnormal growth and fusion of embryonic anal hillocks, faulty divi-
sion of the cloaca by urorectal septum.
Treatment Steps
1. Posterior sagittal anoplasty, sigmoid colostomy, division of fistula.
2. Repeated anal dilation.
F. Diaphragmatic Hernia
H&P Keys
Gasping respiration, cyanosis, absent breath sound on left, bowel
sounds on affected hemithorax, scaphoid abdomen.
517
A. Acute Cholecystitis
H&P Keys
Right upper quadrant pain, tenderness, guarding, subscapular radia-
tion, nausea and vomiting, anorexia, fever, history of fatty food intol-
erance.
Diagnosis
Ultrasound, plain x-ray of abdomen (radiopaque stones), hepatobil-
iary iminodiacetic acid (HIDA) scan, LFTs.
Disease Severity
Unremitting fever, leukocytosis, elevated amylase, palpable gallblad-
der suggesting empyema or pericholecystic abscess, chills, common
duct stones, diabetes.
Concept and Application
Obstruction of cystic duct with stone, secondary bacterial invasion.
Treatment Steps
1. IV fluids, antibiotics, and cholecystectomy.
2. Cholecystostomy if patient is too ill for cholecystectomy (Fig.
18–8).
B. Choledochal Cyst
H&P Keys
Abdominal pain, episodic jaundice, mass in right upper quadrant,
fever.
Diagnosis
Ultrasound and CT scan of abdomen, endoscopic retrograde
cholangiopancreatography (ERCP), percutaneous transhepatic
cholangiography (PTC).
Disease Severity
Fever, recurrent pancreatitis.
Concept and Application
Persistence of embryonic hepaticopancreatic duct, regurgitation of
pancreatic juice in bile duct, cystic changes in bile duct, fibrosis, in-
flammation. If untreated, risk of carcinoma is increased.
518
SURGICAL PRINCIPLES Diseases of the Gallbladder and Liver
Figure 18–8. A cholecystostomy tube cholangiogram showing a somewhat decompressed gallbladder filled
with gallstones. Arrows point to the portion of the gallbladder containing stones. This patient had calculus cholecystitis
in the setting of severe hemodynamic instability and was too sick for an open cholecystectomy.
Treatment Steps
1. Excision of choledochal cyst.
2. Cholecystectomy and biliary reconstruction with a Roux-en-Y
limb.
C. Choledocholithiasis
H&P Keys
Biliary colic, pruritus, chills, fever, jaundice, dark urine, pale stools,
right upper quadrant tenderness.
Diagnosis
LFTs, ultrasound, ERCP, PTC, amylase, lipase.
Disease Severity
Cholangitis: fever, right upper quadrant pain, jaundice (Charcot’s
triad).
Concept and Application
Secondary stones originate in gallbladder (more common); primary
stones arise in intrahepatic duct or common bile duct.
Treatment Steps
1. IV fluids, IV antibiotics.
2. ERCP and sphincterotomy, cholecystectomy and common bile
duct exploration with possible transduodenal sphincteroplasty, or
choledochoduodenostomy.
E. Hepatic Adenoma
H&P Keys
Right upper quadrant pain, palpable liver mass, use of oral contra-
ceptives, female gender.
Diagnosis
Ultrasound, CT scan of the abdomen, technetium colloid sulfur
scan, MRI.
Disease Severity
Abdominal distention, guarding, large adenoma, spontaneous rup-
ture, hemoperitoneum.
Concept and Application
Encapsulated homogeneous mass of hepatocyte, no bile ducts or
central vein present.
Treatment Steps
1. < 6 cm: observation, discontinue oral contraceptive.
2. > 6 cm: surgical resection.
Disease Severity
Extension, compression of adjacent structures, symptoms.
Treatment Steps
Conservative, observation with imaging follow-up.
Diagnosis
Elevated α-fetoprotein (AFP), alkaline phosphatase, direct bilirubin.
Ultrasound, CT scan, or MRI. Percutaneous or laparoscopic needle
biopsy.
Disease Severity
Hepatocellular carcinoma (HCC) can undergo spontaneous rup-
ture. Invasion of diaphragm and adjacent organs. Metastases to
lymph nodes, lung, bone, adrenals, and brain.
Concept and Application
HCC is the most common fatal cancer worldwide; risk factors in-
clude hepatitis, cirrhosis, aflatoxin exposure. Cholangiocarcinoma
involves the biliary tree and is often unresectable. Hepatoblastoma is
a distinctive tumor of infants and children.
Treatment Steps
1. Liver resection, radiotherapy, chemotherapy.
2. Liver transplantation may be indicated in select patients.
H. Liver Metastasis
H&P Keys
History of cancer, especially GI (portal drainage). Anorexia, fatigue,
weight loss, abdominal and shoulder pain. Jaundice, hepatomegaly,
ascites.
Diagnosis
High alkaline phosphatase. Ultrasound, CT scan (Fig. 18–9), needle
biopsy.
Figure 18–9. Abdominal CT scan of a cachectic patient showing bilobar liver metastatic lesions (arrows). This
patient will be managed nonsurgically.
521
A. Acute Pancreatitis
H&P Keys
Epigastric and back pain, nausea and vomiting, retching, hypoten-
sion, fever, left pleural effusion, abdominal tenderness, abdominal
mass, jaundice, abdominal distention, Cullen’s sign (periumbilical
ecchymosis). Alcohol abuse.
Diagnosis
Amylase, lipase, ultrasound, CT scan.
Disease Severity
Predicted mortality increases with number of Ranson’s criteria.
Concept and Application
Enzymatic digestion of gland, duct obstruction (gallstones and pro-
tein), chemical injury to the gland. Most common causes: alcohol in-
gestion, gallstones.
cram facts U
RANSON’S CRITERIA (PREDICTING THE SEVERITY OF ACUTE PANCREATITIS)
On Admission:
W WBC > 16,000/mm3
A Age > 55 yr
G Glucose > 200 mg/dL
A AST > 250 IU/dL
L LDH > 350 IU/L
At 48 hrs:
B Base deficit > 4 mEq/L
E Estimated fluid gain >6L
C Calcium < 8 mg/dL
H Hct fall > 10%
U Urea rise > 5 mg/dL
P PaO2 < 60 mm Hg
Mortality rate in acute pancreatitis closely related to the number of positive Ranson signs (1% if
up to 2 signs, 15% if 3–4, 40% if 5–6, and 100% if 7–8 signs present).
NOTE: Amylase is not part of the Ranson’s criteria.
522
SURGICAL PRINCIPLES Diseases of the Pancreas
Treatment Steps
1. Fluid replacement, GI rest, calcium and magnesium replacement.
2. ERCP; analgesia; cholecystectomy; biliary drainage; debridement
of necrotic, infected pancreatic tissue.
B. Pancreatic Carcinoma
H&P Keys
Vague abdominal pain, back pain, weight loss, pruritus, painless
jaundice, hepatomegaly, migratory thrombophlebitis.
Diagnosis
Ultrasound, CT scan (Fig. 18–10), ERCP, endoscopy, magnetic reso-
nance cholangiopancreatography (MRCP), biopsy of pancreatic
mass.
Concept and Application
Malignant change in ductal epithelium, increased risk with severe
smoking.
Treatment Steps
Curative––Pancreaticoduodenectomy, total or distal pancreatec-
tomy, external-beam radiation, multidrug chemotherapy.
Palliative––Bilioenteric bypass, biliary stent, celiac plexus nerve
block.
C. Gastrinoma
H&P Keys
Severe peptic ulcer symptoms, diarrhea, previous ulcer operation.
Diagnosis
Basal acid output/maximal acid output ratio, serum gastrin level, se-
cretin provocative test, intraoperative endoscopic ultrasound, gastri-
Figure 18–10. Abdominal CT scan showing an oval-shaped, distal pancreatic mass (arrows). This
patient will be managed with a distal pancreatectomy and possible adjuvant therapy depending on the
stage of the tumor.
523
XII. HERNIA
A. Inguinal Hernia
H&P Keys
Aching in groin, bulge or lump in groin. Symptoms may be precipi-
tated by exercise or straining.
Diagnosis
Physical examination.
Disease Severity
Hernia complications: incarceration (sac contents not reducible);
strangulation (loss of blood supply to sac contents leading to infarc-
tion/necrosis).
Concept and Application
Persistent peritoneal diverticulum, increased intra-abdominal pres-
sure (more severe in chronic obstructive pulmonary disease
[COPD], chronic constipation, prostatism), weakness of transversalis
fascia, patent process vaginalis.
Treatment Steps
Standard or laparoscopic herniorrhaphy.
B. Femoral Hernia
H&P Keys
Groin discomfort; mass below inguinal ligament, medial to femoral
vessels. Rule out lymphadenopathy.
Diagnosis
Physical examination, ultrasound.
Disease Severity
Intestinal obstruction, irreducible hernia, compression and/or
thrombosis of femoral vein.
Concept and Application
Protrusion of intra-abdominal contents through femoral canal.
Treatment Steps
1. Excision of sac, closure of femoral canal.
2. For intestinal obstruction, exploratory laparotomy, and possible
bowel resection.
524
SURGICAL PRINCIPLES Hernia
C. Umbilical Hernia
H&P Keys
Bulge in umbilicus; fascial defect felt.
Diagnosis
Physical examination.
Disease Severity
Associated diseases such as cirrhosis and intra-abdominal tumor;
pregnancy.
Concept and Application
Gradual yielding of the umbilical scar tissue.
Treatment Steps
1. < 6 years: observation.
2. > 6 years: repair fascial defect.
D. Incisional Hernia
H&P Keys
Pain; swelling adjacent to scar.
Diagnosis
History and physical examination.
Disease Severity
Large multiple defects, bowel obstruction, steroids, malnutrition,
history of COPD.
Treatment Steps
Tension-free repair, usually with mesh.
Disease Severity
Fever, low urine output, tachycardia, hypotension, abdominal guard-
ing, advanced age.
527
Copyright © 2004, 2001 by The McGraw-Hill Companies, Inc. Click here for terms of use.
528 Otolaryngology and Respiratory System Diseases
BIBLIOGRAPHY / 549
529
Disease Severity
Expanding soft-tissue involvement with intracranial spread and de-
creased function of cranial nerve.
Concept and Application
Severe infection of periauricular soft tissue and bone in immuno-
compromised host, with rapidly expanding and infiltrating infec-
tion with potential for cranial nerve destruction, central nervous
system (CNS) involvement. Most common organism is Pseudomonas
aeruginosa.
Treatment Steps
1. Intravenous antibiotics, with judicious debridement as necessary.
2. Despite aggressive treatment, still significant percentage of mor-
tality.
Treatment Steps
1. Systemic antibiotics, usually amoxicillin (30–40 mg/kg/day
for uncomplicated infections and for children under age 12;
other antibiotics used are amoxicillin–clavulanate, erythromy-
cin, sulfa for children allergic to penicillin, and trimethoprim–
sulfamethoxazole and cephalosporins as necessary.
531
Treatment Steps
1. Initial management should be observation for approximately 3
months, during which 90% resolve.
2. Generally, antibiotics are offered initially; some suggest that anti-
histamine decongestants are not uniquely helpful. In adults with
serous otitis media, if unilateral, one must pay careful attention
to the nasopharynx to rule out nasopharyngeal lesions—again,
particularly in immunocompromised hosts.
3. If the serous otitis media does not resolve and hearing loss per-
sists after a period of careful observation and treatment, myringo-
tomy with aspiration of the middle ear content and subsequent
placement of ventilation tubes is the treatment of choice. In
adults, attempts at autoinflation with Valsalva’s maneuver is often
effective in resolving serous otitis media; in children with recur-
rent nonresolving serous otitis media, adenoidectomy with or
without tonsillectomy is often recommended.
Diagnosis
Direct visualization via otoscopy.
Disease Severity
Quality of hearing loss and degree of cerumen impaction. Rule out
foreign body within the external canal, particularly in children and
retarded patients.
533
G. Vertigo
H&P Keys
Vertigo is a complex complaint; it must be determined whether the
vertigo is otologic, central, or medical in origin. Determining whether
the disease is peripheral is made easier by the symptom of definite
sensation of movement, most often rotatory. When the vertigo is
paroxysmal and severe, it is more likely to be peripheral; attacks may
last minutes to hours (seldom longer) and may be associated with veg-
etative signs such as sweating, nausea, and vomiting. Patient never
loses consciousness. Conversely, central vertigo is more often mild and
described as a sensation of light-headedness or unsteadiness. It is
vague, without specific onset or termination, and may be constant; at-
tacks may last weeks or months, often without an obvious nystagmus.
Associated symptoms of vertigo may be nystagmus (with peripheral
pathology, the nystagmus can often be seen); with irritative lesions,
nystagmus is often to the side of involved ear; nystagmus with chang-
ing of direction is more often central than peripheral. Causes of ver-
tigo of otologic origin are acute otitis media, serous otitis media, head
trauma with involvement of labyrinthine apparatus, and trauma to
middle ear by penetrating wound, with dislocation of ossicles and pro-
duction of vertigo and hearing loss, Cogan’s syndrome, vestibular neu-
ronitis, temporal bone fractures, acute barotrauma with perilymph fis-
tulas, and endolymphatic hydrops (Ménière’s disease). Ménière’s
disease is a disease process involving abnormal absorption or produc-
tion of endolymph, which produces a quadrad or triad of symptoms of
tinnitus, vertigo, fluctuant hearing loss, and sensations of fullness or
blockage in the ear; the disease process may begin suddenly with tin-
nitus or any of the other symptoms; vertigo is severe and unrelenting
for minutes to hours; nausea and vomiting are often present.
Diagnosis
History of fluctuant hearing loss, tinnitus, vertigo, neurosensory
hearing loss on audiometric evaluation, evidence of canal paresis
with vestibular studies involving the affected ear, negative examina-
tions with intracranial MRI with gadolinium for cranial nerve VIII
and neurovascular bundles. Electronystagmography (ENG) docu-
menting canal paresis or hypoactivity of affected ear.
Disease Severity
Severity of vertigo, length of episodes, frequency of attacks, degree
of hearing loss.
Concept and Application
Temporal bone studies indicate presence of hydrops of the endolym-
phatic space with destruction of neuroepithelium thought to be sec-
ondary to abnormality of stria vascularis, endolymphatic sac mecha-
nism, or both.
534
OTOLARYNGOLOGY AND RESPIRATORY SYSTEM DISEASES Diseases of the Ear
Treatment Steps
1. For acute cases, benzodiazepam-like drugs are effective if nausea
and vomiting are not a problem.
2. For long-term management of Ménière’s disease, diuretics and
low-sodium diet are often effective.
3. In patients with continuing sensations of disequilibrium who fail to
respond to medical therapy, endolymphatic sac decompression;
cranial nerve VIII section; or, in patients who have nonfunctioning
ears from auditory standpoint and unilateral disease for > 5 years,
labyrinthectomy is procedure of choice; diazepam and antihista-
mine group such as meclizine hydrochloride, diphenhydramine
hydrochloride (Benadryl), or dimenhydrinate (Dramamine).
H. Otalgia
H&P Keys
Otalgia may represent pain of otologic origin or of distant disease
referred to the ears, such as dental infection, pharyngitis, or tonsilli-
tis. Symptoms include ear pain (sharp, constant, dull, or burning).
Determination of duration of pain and exacerbating and remitting
factors are essential. Physical examination includes inspection of the
external ear, otoscopy with examination of external canal and tym-
panic membrane with middle ear; examination of the temporo-
mandibular joints with direct pressure both externally and on the
pterygoid muscles within the oral cavity; and complete examination
of the upper aerodigestive tract, nasopharynx, oropharynx, tongue,
larynx, and hypopharynx.
Diagnosis
If cause is not obvious, diagnostic studies such as CT scan and MRI
of upper aerodigestive tract and neck are useful. Studies also include
direct laryngoscopy, nasopharyngoscopy, audiologic testing, and
tympanometry, as well as palpation of tonsillar fossae, tongue base,
and neck. Direct laryngoscopy and cervical esophagoscopy also may
be indicated.
Disease Severity
Presence of tumors or lesions in the upper aerodigestive tract refer-
ring pain to the ear are of potentially great concern and may be life
threatening.
Concept and Application
Direct stimulation of nerves supplying sensation to the ear via in-
flammatory process or direct pressure, transmission via the same
nerves through the temporomandibular joint and mechanism of re-
ferred pain via myositis and muscle spasm from associated joint mus-
culature. Referred pain from tongue base, larynx, or pyriform sinus
occurs via the vagus or glossopharyngeal nerve.
Treatment Steps
1. Management will vary, depending on underlying disease process.
2. It may be as simple as cerumen removal or as complex as cancer
extirpation and adjunctive treatments.
I. Hearing Loss
H&P Keys
Obvious loss of hearing acuity is noted either by patient or by friends
and family; may be associated with other otologic signs such as tinni-
535
Figure 19–1. Pedigree of a typical family with autosomal dominant hearing loss. Note the multiple
affected family members; transmission can be either maternal or paternal. , Affected male, female;
, unaffected male, female.
536
OTOLARYNGOLOGY AND RESPIRATORY SYSTEM DISEASES Diseases of the Ear
Treatment Steps
1. For sensorineural hearing losses of acquired type and of mild to
moderate or even severe degree, amplification by means of hear-
ing aid is available.
2. For profound losses not amenable to amplification, cochlear im-
plant surgery is available.
3. For conductive hearing losses secondary to middle-ear disease, as-
piration of fluid and myringotomy (as noted), ossicular recon-
struction by means of stapes surgery or ossiculoplasty, and tym-
panoplasty for correction of tympanic membrane perforations.
Diagnosis
Audiometric testing, including air and bone conduction, ENG and
calorics, testing of auditory brain responses, CT scan and MRI of the
temporal bone, blood sugar, fluorescent treponemal antibody ab-
sorption (FTA-ABS) testing and sedimentation rate, and direct ex-
amination.
Disease Severity
Degree of hearing loss as determined by audiogram; presence or ab-
sence of vertigo or tinnitus and patient’s disability.
diagnostic decisions d
SUDDEN HEARING LOSS
Indications Testing
All patients Audiologic/tympanometric testing
Acoustic neuroma/skull base lesions MRI or CT with contrast
Autoimmune inner-ear disease Lymphocyte transformation testing
Western blot immunoassay
Syphilis FTA-ABS
MHA-TP
Bacterial infections Appropriate culture and Lyme disease titer
Viral infections Appropriate titers
HIV testing
Miscellaneous ENG, ABR, ECoG, perilymphic fistula test, CBC,
blood chemistries, metabolic studies
ABR, auditory brain stem evoked responses; CBC, complete blood count; CT, computed tomography; ECoG,
electrocochleography; ENG, electronystagmography; FTA-ABS, fluorescent treponemal antibody absorption; HIV, human
immunodeficiency virus; MHA-TP, microhemagglutination assay for Treponema pallidum; MRI, magnetic resonance imaging.
537
K. Barotrauma
H&P Keys
History of recent scuba diving or air flight with inability to equalize
pressure between external environment and middle ear; pain, tinni-
tus, hearing loss, and vertigo. Physical examination may reveal he-
motympanum, eardrum perforation, nystagmus, nausea, vomiting,
and hearing loss.
Diagnosis
Direct inspection of ear via otoscopy and pneumatic otoscopy; audi-
management
decisions d
ologic and tympanometric testing; vestibular testing, including ENG.
TREATMENT MODALITIES
Disease Severity UTILIZED FOR SUDDEN
SENSORINEURAL HEARING
Degree of vertigo and patient’s functioning, including hearing loss
LOSS
and duration of symptoms.
Anti-inflammatory and
Concept and Application
Immunologic Agents
Acute changes in barometric pressure and failure of the eustachian Steroids
tube to function properly. A large pressure gradient across the mid- Prostaglandin
dle ear may result in trauma and the destruction of middle ear, in- Antivirals
ner ear, or both. Eustachian tube dysfunction may be a result of in- Acyclovir
fectious, anatomic, or neoplastic abnormalities. The difference in Calcium Antagonists
barometric pressure may result in rupture of round or oval window Nifedipine
seals, causing acute inner-ear abnormalities that lead to hearing loss Diuretics
or vertigo. Disruption of the tympanic membrane or vessels within Hydrochlorothiazide
middle ear may result in tympanic membrane perforation or hemo- Furosemide
tympanum. Vasodilators
Carbogen
Treatment Steps
Papaverine
1. For uncomplicated barotrauma, appropriate nasal decongestants, Nicotinic acid
systemic decongestants, and watchful waiting for resolution of he- Pentoxifylline
motympanum or tympanic membrane perforation. Volume Expanders
2. For inner-ear dysfunction, bed rest for 24 hours may result in res- Hydroxyethyl starch
olution if no significant hearing loss or vertigo is present; should Dextran
these symptoms persist, middle-ear exploration with patching of Other Therapies
oval and round windows is treatment of choice. Vitamins
3. Prevention of barotrauma can be helped with appropriate use Acupuncture
of nasal decongestants and systemic decongestants before Procaine
scuba diving or flying. Patients’ tolerance to these medications
538
OTOLARYNGOLOGY AND RESPIRATORY SYSTEM DISEASES Diseases of the Mouth and Throat
L. Tinnitus
H&P Keys
History of noise in the ear, which is generated endogenously, not
from the environment. Complaints are about continuous humming,
hissing, or whistling. Pulsatile tinnitus that is synchronous with
heartbeat may accompany hearing loss or vertigo.
Diagnosis
Tinnitus that is bilateral, symmetrical, and of reasonably long stand-
ing is most often benign and requires audiometry. Unilateral tinni-
tus or pulsatile tinnitus requires workup with MRI, magnetic reso-
nance angiography (MRA), auscultation of the chest and neck to
determine presence of transmitted or carotid bruits, auscultation
within the ear to determine presence or absence of lesions, and CT
scan to rule out vascular lesions of the ear.
Disease Severity
Tinnitus may be extremely loud and produce inability to concen-
trate, sleep, or function. Tinnitus matching audiogram, CT scan,
MRI, auscultation of neck, ultrasound, noninvasive studies of great
vessels of neck, and transcranial Dopplers. Examination of the ear
for vascular lesions involving middle ear.
Treatment Steps
As per etiology.
Diagnosis
Clinical examination with Giemsa stain evaluation of vesicular fluid
revealing syncytial giant cells with intranuclear inclusions.
Disease Severity
Degree of symptoms listed above.
Concept and Application
Initial herpesvirus type I. Infection usually occurs in children ages
2–5 years.
539
OTOLARYNGOLOGY AND RESPIRATORY SYSTEM DISEASES Diseases of the Mouth and Throat
Treatment Steps
1. Symptomatic therapy includes saltwater gargles and irrigations,
soft diet, antipyretics, and topical anesthetics as needed.
2. Intravenous (IV) hydration for severe debilitation.
Treatment Steps
1. Depends on type of lesion noted.
2. Benign processes respond most often to conservative manage-
ment or surgical extirpation.
3. Malignancies may require extirpation and irradiation,
chemotherapy, or both.
4. Lesions of the ear secondary to masses in the nasopharynx may
require myringotomy and tube placement to correct serous otitis
media.
E. Hoarseness
H&P Keys
Presence of infectious disorder, local use/abuse, history of smoking
and ethanol use, history of arthritis, history of trauma and intuba-
tion, possible endocrinopathy, benign and malignant neoplasms,
functional disorders, reflux symptomatology. Physical examination
includes indirect mirror examination and direct laryngoscopy as
well as complete physical examination of the upper aerodigestive
tract. Symptoms include possible referred otalgia, possible throat/la-
ryngeal pain, dysphagia, dyspnea, cough, etc.
Diagnosis
Thorough examination of the larynx using indirect and direct meth-
ods, complete examination of the upper aerodigestive tract; adjunc-
541
OTOLARYNGOLOGY AND RESPIRATORY SYSTEM DISEASES Diseases of the Mouth and Throat
tive radiologic studies include CT scan, MRI scan, barium swallow,
thyroid function tests, biopsy as appropriate.
Disease Severity
Due to vast etiologic sources, a large variety of disease severity occurs.
Concept and Application
Disruption of normal mucosal wave of the vocal cords with creation
of turbulent air flow resulting in hoarseness, edema, vocal masses
and irregularities, as well as limited function or hyperfunctioning of
the vocal cords.
Treatment Steps
Directed toward etiology.
Diagnosis
Complete examination of the upper aerodigestive tract, including
indirect and direct laryngoscopy, bimanual palpation, CT scan of
neck and larynx, MRI.
Disease Severity
Depends on TNM staging and extent of disease process.
Concept and Application
Squamous cell carcinoma is most frequent malignant neoplasm of
the larynx (95%). Tumor initially remains confined to the larynx,
then spreads to cervical lymph nodes and ultimately metastasizes to
distant areas.
Treatment Steps
Management includes surgery, radiation therapy, and chemother-
apy, depending on extent and stage of disease.
A. Acute Sinusitis
H&P Keys
History of recent upper respiratory tract infection associated with
purulent rhinorrhea, headache, pain, and pressure over the affected
sinus (cheek—maxillary, forehead—frontal, periorbital—ethmoid,
and occipital—sphenoid). Other signs and symptoms include puru-
lent postnasal drip, pressure and headache, nasal obstruction, re-
ferred otalgia, and orbital pain.
Diagnosis
Nasal endoscopy, both standard and endoscopic; culturing of puru-
lent discharge; sinus x-rays or CT scan of sinuses; sinus tap to deter-
mine presence of pus for culture and treatment.
Disease Severity
Fever, chills, sinus pressure and pain; possible periorbital cellulitis,
edema, proptosis, blindness, headache; intracranial complication
such as meningitis, brain abscess, or cavernous sinus thrombosis.
Concept and Application
Obstruction of ostea of sinuses in middle meatus (osteomeatal com-
plex) leading to negative pressure, transudate followed by exudate,
and acute infection. Presence of anatomic abnormalities such as sep-
tal deviation, concha bullosa, turbinate hypertrophy, nasal polyposis,
and allergic rhinitis. Bacteriology is similar to that of acute otitis me-
dia, including H. influenzae, S. aureus, group A β-streptococcus,
pneumococcus, and more unusual organisms in immunocompro-
mised hosts.
Treatment Steps
1. Antibiotics such as amoxicillin, ampicillin, or amoxicillin with
clavulanic acid to cover suspected organisms; both systemic and
topical decongestants to nasal mucosa.
2. Surgical drainage of affected sinuses as indicated by severity of
disease and degree of patient’s illness.
3. Steroids, antihistamines, or both for patients with a significant al-
lergic component to their sinusitis.
543
D. Chronic Rhinitis
H&P Keys
Long-term nasal obstruction, postnasal discharge, sneezing, rhinor-
rhea, and possible purulence. Patients may complain of seasonal
symptoms or have symptoms referable to emotional or temperature
change. Physical examination reveals erythema of mucous mem-
brane, often with crusting and bleeding and occasional purulence.
Diagnosis
Gram stain of nasal smears for eosinophils or polymorphonuclear
cells; sinus x-rays, CT scan, or both to rule out occult sinusitis. Al-
lergy studies to rule out allergic disease as primary causative factor.
Disease Severity
Persistence of nasal obstruction, postnasal discharge, pressure and
pain, inability to sleep because of nasal obstruction, fatigue, loss of
concentration, and loss of time from work.
Treatment Steps
Determination of etiology and direction of therapy to allergic, infec-
tious, or vasomotor disease process; also included in therapy would
be antihistamine decongestants, steroid nasal sprays, cromolyn
sodium as a nasal spray, systemic steroids, and intranasal medica-
ments such as lubricating drops when indicated.
E. Allergic Rhinitis
H&P Keys
Nasal obstruction and congestion, nasal pruritus, rhinorrhea, sneez-
ing, and symptoms related to seasons. History of presence or ab-
sence of animals, specific plants, flowers, molds, and conditions
(e.g., feather pillows) that would support the growth of molds or al-
lergies. Physical examination may reveal swollen, pale blue nasal mu-
cosa and turbinates with nasal obstruction and generally clear rhin-
545
Disease Severity
Degree of function during allergic periods (i.e., potential loss of
school or work time).
Treatment Steps
1. If possible, avoiding specific allergen is most useful for mild to
moderate symptoms.
2. Treatment with antihistamines, nasal steroids, systemic steroids,
sympathomimetic medications as well as sodium cromolyn nasal
spray are indicated.
3. Also, immunotherapy with allergy shots for desensitization as well
as diet control are useful adjuncts.
F. Epistaxis (Nosebleed)
H&P Keys
Episode can be intermittent or acute; bleeding may be from anterior
nares or may produce postnasal bleeding. Patient may give history of
digital trauma to nose or history of nasal obstruction, particularly in
boys < 15 years old. Bleeding may respond to anterior nares pressure
or may require intranasal packing, posterior nasal packing, or both.
Diagnosis
Direct examination of nose after careful intranasal vasoconstriction
and local anesthesia permits examination of anterior nares, particu-
larly in Kiesselbach’s (Little’s) area (Fig. 19–2). Postnasal space can
be examined by fiberscope under local anesthesia; sinus x-rays or CT
scans should be done to rule out intrasinus occult malignancies.
Disease Severity
Minor intermittent bleeding stops spontaneously with gentle pres-
sure. Severe postnasal bleeding is life threatening and requires post-
nasal packing, hospitalization, and intensive care observation; neces-
sity for blood transfusion and surgical intervention with ligation of
sphenopalatine or maxillary artery.
D. Distributive Shock
Characteristic of sepsis, also anaphylaxis and neurogenic, toxic, or
endocrinologic shock.
H&P Keys
Fever, chills, rigor, respiratory alkalosis, bee sting or toxic ingestion,
hypotension, peripheral vasodilation with warm extremities.
Disease Severity
Level of blood pressure, evidence of poor organ perfusion.
Diagnosis
Blood cultures, white blood count (WBC), ABG, pH, lactate, toxin
screen, hemodynamic monitoring.
Concept and Application
Diffuse arterial and venous dilatation in response to endotoxins, ex-
otoxins, and cytokines. Other mediators include kinins, histamine,
and prostaglandins. Cardiac output is increased and peripheral oxy-
gen demand also markedly increased. Inadequate tissue oxygen de-
livery and failure of microcirculation, with inappropriate vasodilata-
tion and vasoconstriction. Later, sepsis leads to depression in
myocardial function.
Treatment Steps
1. Antibiotic coverage, including two bactericidal agents for likely
organisms.
2. Initial rapid volume infusion with crystalloid and colloid solutions
and blood to maintain hemoglobin- and oxygen-carrying capac-
ity.
3. Vasoactive drugs including dopamine to maintain perfusion pres-
sure of 60 mm Hg.
4. Initial characterization of the site of infection and appropriate
treatment, e.g., drainage of an abscess.
12
CARDIOVASCULAR MEDICINE Myocardial Diseases
A. Dilated Cardiomyopathy
H&P Keys
Symptoms suggest low cardiac output and congestion of systemic
and pulmonary vasculature: dyspnea, fatigue, orthopnea, PND,
edema. Exam often reveals hypotension; tachycardia; cool extremi-
ties; pulmonary rales; displaced cardiac impulse, often of diminished
intensity; and S3, mitral, or tricuspid murmur. Signs of RV failure
(edema, ascites, elevated JVD) often present.
Diagnosis
CXR often shows cardiomegaly, often with signs of congestion, possi-
bly with pleural effusions. ECG often shows signs of ventricular or
atrial enlargement, atrial fibrillation; premature ventricular com-
plexes (PVCs) are often seen. Echo and RNA often show ventricular
or four-chamber enlargement with diffuse hypocontractility and re-
duced cardiac output.
Disease Severity
Symptoms may correlate poorly with degree of ventricular impair-
ment. Echo and RNA document degree of ventricular dilatation and
dysfunction. Echo documents associated valvular abnormalities. Car-
diac catheterization documents degree of hemodynamic abnormal-
ity and presence of CAD. In absence of CAD, catheterization rarely
alters therapy.
Concept and Application
Various etiologies, most common being idiopathic. Other etiologies
include ischemic (postinfarction), viral (e.g., coxsackie), peripar-
tum, toxic (e.g., alcohol, doxorubicin). Myocyte injury occurs, caus-
ing reduction in contractility, leading to ventricular dilation, with
compensation by activation of sympathetic nervous system and
renin–angiotensin–aldosterone axis.
Treatment Steps
1. Removal of inciting cause (e.g., discontinue alcohol) if one can
be found.
2. Remainder of treatment plan as detailed in section II.
C. Restrictive Cardiomyopathy
H&P Keys
Patient may have an underlying disease (e.g., hemachromatosis, sar-
coidosis, amyloidosis, or cancer). Symptoms: fatigue, weakness, dysp-
nea. Physical findings as described for diastolic dysfunction. ECG of-
ten shows low voltage.
Disease Severity
Echo shows LV thickening and systolic impairment (if any). Doppler
and cardiac catheterization can show augmented early diastolic fill-
ing. Catheterization demonstrates lowered cardiac output and ab-
normal RV filling. RV biopsy can identify a specific cause.
Concept and Application
Restrictive cardiomyopathy is the least common of the cardiomy-
opathies. Systemic disorders (e.g., hemachromatosis, sarcoidosis,
amyloidosis, metastatic cancer), patients with thoracic radiation or
with other disorders (e.g., endomyocardial fibrosis) caused by my-
ocardial infiltration, fibrosis, etc. have elevated filling pressures,
which lead to signs and symptoms of right and left heart congestion.
Reduced cavity size causes decreased cardiac output.
Treatment Steps
1. Identify and treat the underlying illness if possible (e.g., steroids
in sarcoidosis or chelation therapy in hemachromatosis).
2. Symptomatic treatment includes diuretics and salt restriction.
Digitalis is often not helpful and may be harmful in these disor-
ders.
3. The prognosis depends on the severity of the underlying condi-
tion.
14
CARDIOVASCULAR MEDICINE Cardiac Arrhythmias
V. CARDIAC ARRHYTHMIAS
A. Supraventricular Arrhythmias
Rhythm disturbances involving sinus node, atria, and atrioventricu-
lar (AV) node.
1. Bradyarrhythmias
H&P Keys
U
Fatigue, dizziness, weakness, syncope, low rhythm and pulse.
Diagnosis
cram facts
ECG, cardiac rhythm monitoring, electrophysiologic studies.
Disease Severity
TREATMENT DECISIONS
Depressed mental status, fatigue, limitation of activity, shortness of
breath.
Cardiac Arrhythmias
Bradyarrhythmias Concept and Application
Withdrawal of medications Disruption of normal structures and conduction pathways resulting
that produce bradycardia; from collagen deposition, hypertension, ischemia, atrial stretch, id-
temporary intravenous or
iopathic causes, autonomic nervous system.
external pacemaker for
acute management;
Treatment Steps
permanent pacemaker for
definitive treatment. 1. Acute symptomatic: Temporary intravenous pacemaker or exter-
nal pacing device.
Atrial Fibrillation
β-Blockers, calcium 2. Chronic symptomatic: Permanent pacemaker, withdrawal of of-
channel blockers for rate fending drugs.
control; anticoagulation
with Coumadin if b. AV Node
underlying structural heart
First-Degree AV Block—PR interval > 0.20 seconds. May or may not
disease; DC cardioversion
or antiarrhythmic drugs
be associated with bradyarrhythmia.
such as amiodarone or Second-Degree AV Block—Intermittent failure of atrial activity to
sotalol to restore sinus reach ventricles:
rhythm. Catheter-based
radiofrequency ablation • Mobitz Type I (Wenckebach). Usually at level of AV node, pro-
techniques are also useful gressive PR prolongation prior to blocked QRS. Does not in-
in restoring and maintaining clude blocked premature atrial contraction.
sinus rhythm.
• Mobitz Type II Block. Usually distal to AV node (bundle of
Ventricular Tachycardia His). High risk for complete AV block. Often associated with
Immediate DC
wide QRS.
cardioversion if patient is
hemodynamically unstable; Complete (Third-Degree) AV Block—May be at level of AV node, bun-
drugs including lidocaine, dle of His, or bundle branches. Atrial and ventricular activity are
procainamide, amiodarone, independent. QRS usually wide. Often associated with symptoms.
or pronestyl. Implantable
defibrillator to treat high- H&P Keys
risk patients.
Fatigue, shortness of breath, CHF, dizziness, syncope. Slow or irregu-
Ventricular Fibrillation lar pulse.
Immediate DC
cardioversion. Diagnosis
ECG, cardiac rhythm monitoring, electrophysiologic studies.
15
Disease Severity
Frequency of symptoms, including fatigue, lethargy, dizziness, chest
pain, shortness of breath, syncope. Stroke is associated with atrial
fibrillation.
B. Ventricular Arrhythmias
Disorders of rhythm isolated to ventricles.
1. Bradyarrhythmias
Conduction disturbances within the His–Purkinje system and bundle
branches. Often symptomatic. Usually caused by second- and third-
degree heart block.
H&P Keys
Light-headedness, fatigue, syncope, intermittent cannon A waves in
jugular venous pulse exam (third-degree heart block), slow pulse
(intermittent or chronic).
Diagnosis
ECG, auscultation (dissociation of atrial [S4] and ventricular [S1 and
S2] activity), electrophysiologic studies.
Disease Severity
Frequency of symptoms including dizziness, light-headedness, fa-
tigue, shortness of breath, chest pain, syncope. Trifascicular block
may be asymptomatic but predictive of complete heart block.
Disease Severity
Cardiac arrest has a high recurrence rate if untreated. Prognosis re-
lated to LV function, CAD. 2-D echo, cardiac catheterization to as-
sess severity of LV dysfunction.
Treatment Steps
A. Acute Pericarditis
Most common pericardial disorder, consisting of acute inflammation
of the pericardium from a wide variety of causes.
H&P Keys
Retrosternal and left precordial sharp chest pain, often radiating to
the back and left trapezius, with strong pleuritic component. Dysp-
nea is common. Symptoms often are worse in supine position, allevi-
ated by sitting forward. Friction rub (three-component) best heard in
left precordium with patient sitting forward during held exhalation.
Diagnosis
ECG can show evolutionary changes consisting of widespread ST ele-
vations with upward concavity and depressed PR interval, reduction
of T wave amplitude or T wave inversion; with large associated peri-
cardial effusions, reduction and respiratory variation in R wave am-
plitude (electrical alternans). Tests for etiology, e.g., antinuclear an-
tibody (ANA), purified protein derivative (PPD).
Disease Severity
The underlying disease is an important determination of outcome.
Echo used to assess for presence and degree of associated pericar-
dial effusion.
18
CARDIOVASCULAR MEDICINE Pericardial Diseases
B. Pericardial Effusion
Fluid accumulation in the pericardial space. The amount of fluid
and the rapidity of accumulation of the effusion determines the ob-
served signs and symptoms.
H&P Keys
Symptoms of pericarditis, or patient may be asymptomatic. Previ-
ously present friction rub may diminish in intensity. In large effu-
sion, heart sounds may be muffled.
Diagnosis
CXR shows cardiomegaly with “water bottle” shape. ECG, especially
in large effusions, shows reduced QRS amplitude; electrical alter-
nans may be seen. Echo for direct visualization of size and presence
of effusion.
Disease Severity
Determined by rapidity of accumulation and size of effusion. Echo
documents size of effusion and extent of the hemodynamic impair-
ment; hemodynamic effects can be also documented by cardiac
catheterization.
Concept and Application
Any cause of pericarditis can lead to the formation of a pericardial
effusion. Pericardial effusion can also occur with a variety of systemic
disorders such as hypothyroidism.
Treatment Steps
1. Pericardiocentesis for diagnosis and treatment.
2. Treatment of underlying cause.
3. Removal of the pericardium may be required to prevent recur-
rent tamponade.
C. Cardiac Tamponade
An accumulation of pericardial fluid under high pressure, which
limits the ability of the heart to fill.
H&P Keys
May have antecedent history of pericarditis or chest trauma. Symp-
toms: dyspnea, fatigue. Physical findings: JVD, hypotension, shock,
distant heart sounds, tachycardia, pulsus paradoxus.
19
D. Constrictive Pericarditis
Obliteration of pericardial space or scarring of pericardial tissue,
causing cardiac enclosure, resulting in compromised cardiac filling.
H&P Keys
Symptoms: fatigue, hypotension, weakness. Physical signs: ascites,
edema, JVD, often with Kussmaul’s sign (elevation of venous pres-
sure during inspiration), pericardial knock in diastole, reduced am-
plitude of apical impulse.
Diagnosis
ECG may show a low-voltage QRS complex with diffuse T wave
changes. CXR may show pericardial calcification, present in approxi-
mately 50% of patients. Echo and Doppler demonstrate normal cav-
ity size with enhanced early diastolic filling. Pericardial thickening
can be suggested by echo; however, computed tomography (CT)
and magnetic resonance imaging (MRI) are more accurate in diag-
nosis.
Disease Severity
Hemodynamic features seen during catheterization include eleva-
tion and equalization of all diastolic pressures; LV and RV pressure
tracings are equal in diastole and have a dip and plateau configura-
tion (square root sign); RA pressure tracing shows an M configura-
tion with prominence of the Y descent.
Treatment Steps
1. Pericardial resection is the definitive therapy.
2. Diuretics can afford symptomatic improvement. Risks of the oper-
ation and prognosis postoperatively depend on the degree of in-
volvement of the epicardium in the scarring, calcific process.
20
CARDIOVASCULAR MEDICINE Valvular Diseases
A. Acute
1. Rheumatic Fever
Delayed sequela to pharyngeal infections with group A streptococci.
Pathology involves the heart, joints, central nervous system (CNS),
skin, and subcutaneous tissues.
2. Endocarditis
Native valve endocarditis usually with prior valve pathology; endocardi-
tis in prior drug users; and prosthetic valve endocarditis are the most
common forms. May be acute or subacute. Acute endocarditis is often
caused by Staphylococcus aureus and is rapidly destructive, and often fatal
(if untreated) in < 6 weeks. Subacute endocarditis has a longer, smol-
dering course and is frequently caused by viridans streptococci. With-
out effective treatment, death usually occurs in 2–6 months.
3. Ischemic
Acute valvular decompensation resulting from ischemic heart dis-
ease, e.g., acute ischemia or myocardial infarction: may lead to car-
diac decompensation and death. Usually acute MR caused by poste-
rior papillary muscle ischemia or infarct.
H&P Keys
B. Chronic
Degeneration of cardiac valvular structures over a prolonged time.
Progressive dilation of the LV or RV. Chronic degenerative disorders
including mitral valve prolapse (myxomatous degeneration) and cal-
cific valvular disease in the elderly. Mitral and aortic valves are most
commonly involved in endocarditis and degenerative valvular disor-
ders including MR, aortic regurgitation (AR), aortic stenosis (AS).
Acquired mitral stenosis (MS) occurs almost exclusively from
rheumatic fever. Pulmonary and tricuspid valves less often involved.
Bicuspid aortic valve is a common congenital abnormality predomi-
nantly in men, and is associated with AS later in life.
H&P Keys
CHF, shortness of breath, fatigue, pedal edema, cardiac murmurs
consistent with severe stenotic or regurgitant lesions, elevated JVP,
pulse alterations, e.g., delayed upstroke with aortic stenosis, bound-
ing pulse with aortic insufficiency.
Diagnosis
CXR, echo, cardiac catheterization.
Disease Severity
Heart rate, respiratory rate, auscultatory findings, peripheral edema,
pulmonary rales, blood pressure, oxygen saturation, level of con-
sciousness.
Concept and Application
H&P Keys
Harsh systolic murmur at left sternal border radiating to right pre-
cordium, palpable thrill over precordium. Large left-to-right shunt
associated with CHF. Pulmonary hypertension causes reversal of flow
(right-to-left shunt), resulting in the Eisenmenger syndrome charac-
terized by cyanosis, pedal edema, syncope, and CHF.
Diagnosis
Physical exam, CXR, oxygen saturation of blood in the ventricles,
2-D echo, cardiac catheterization, and angiography.
Disease Severity
Mentation, heart rate, cyanosis, growth retardation. In children,
30–50% spontaneous closure. May be asymptomatic if shunt is small.
C. Tetralogy of Fallot
Most common congenital heart lesion over the age of 1 year charac-
terized by the following four signs:
1. VSD
2. RV outflow narrowing
3. Overriding aorta
4. RV hypertrophy
H&P Keys
Growth retardation, cardiac murmurs, cardiac arrhythmias, poly-
cythemia and cyanosis, exercise limitation with squatting maneuver
to restore normal breathing, clubbing, thrill at left sternal border.
Diagnosis
Physical exam, CXR, ECG (RV hypertrophy), 2-D echo, cardiac
catheterization.
Disease Severity
Activity level, growth, ABG, mentation, heart rate, cyanosis and
pedal edema, heart size, CHF, syncope.
24
CARDIOVASCULAR MEDICINE Cardiac Life Support
Treatment Steps
1. Total surgical correction.
2. Bacterial endocarditis prophylaxis.
H&P Keys
More common in males. Differential development of upper and
lower body, differential pulses in upper and lower extremities, a
cause of secondary hypertension.
Diagnosis
Brachial and femoral artery pulses, blood pressure in upper versus
lower extremities, CXR (rib notching), 2-D echo in children, aortog-
raphy, CT scan.
Disease Severity
Depends on extent of luminal narrowing. Severe hypertension may
result, producing headache, CHF, CNS hemorrhage. Lower extrem-
ity claudication, aortic rupture possible. May be associated with bi-
cuspid aortic valve (AS or AR).
Treatment Steps
1. Surgical correction optimally at age 4–8.
2. Bacterial endocarditis prophylaxis.
H&P Keys
Dizziness, dyspnea, palpitations, chest pain may precede cardiac ar-
rest and loss of consciousness that is not spontaneously terminated.
Examination: cyanotic, pulseless, unconscious, or unarousable pa-
tient with absent or agonal respirations. Children often are cyanotic.
Diagnosis
Most cardiopulmonary arrests occur outside the hospital and are fa-
tal. Only 25% of cardiopulmonary arrest victims survive to hospital
admission. Cardiopulmonary arrest victims are identified as unre-
sponsive and pulseless patients. ECG, electrolytes, myocardial en-
zymes, and cyanosis.
25
XI. HYPERTENSION
Hypertension is defined as a diastolic pressure > 90 and a systolic
pressure > 140. Severe hypertension includes a diastolic blood pres-
sure > 115. Hypertension is an important public health problem and
a major cause of CHF. Uncontrolled hypertension is associated with
a marked reduction in life expectancy.
A. Essential Hypertension
The etiology is unknown in 95% of patients with essential hyperten-
sion. There is a strong family occurrence.
H&P Keys
The vast majority of patients are asymptomatic. Symptoms of occipi-
tal headache are seen with severe hypertension. Other associated
symptoms reflect end-organ damage, e.g., hematuria, blurring of vi-
sion, CHF. Exam is tailored to detect evidence of end-organ damage,
e.g., retinal hemorrhages, S4, S3.
Diagnosis
All patients with hypertension should have urinalysis for protein,
blood, potassium, creatinine, BUN, and an ECG for evidence of LV
hypertrophy. Other testing is performed to exclude a suspected sec-
ondary cause.
26
CARDIOVASCULAR MEDICINE Hypertension
Disease Severity
Evidence of end-organ damage including CNS, renal, and cardiac
dysfunction.
CNS—Lacunar infarcts and stroke.
Renal—Elevated creatinine, BUN.
Cardiac—LV hypertrophy, cardiomegaly. CHF, MI, aortic enlarge-
ment, and aortic dissection. Natural history depends on the level
of blood pressure and the extent and severity of organ involve-
ment.
Concept and Application
The cause of essential hypertension remains unknown. Possible
mechanisms include complex multifactorial genetic factors as well as
abnormal responsiveness to sodium and calcium. Associated with di-
abetes and obesity.
Treatment Steps
Both isolated systolic and diastolic hypertension should be treated.
1. In general, weight reduction in the obese patient and some re-
striction in salt are often very valuable in control. Step therapy
has been evaluated over a 25-year period, with evidence of im-
proved life expectancy.
2. Diuretics and β-blockers improve outcomes and are recom-
mended as the initial drug choices.
3. Other agents including ACE inhibitors and calcium antagonists
are useful as secondary agents.
4. As severity increases, antiadrenergic drugs such as clonidine and
vasodilators (e.g., minoxidil) may be valuable.
B. Secondary Hypertension
A host of disease entities produce hypertension, including en-
docrine abnormalities, such as adrenal cortical hyperfunction
(Cushing’s disease), primary hyperaldosteronism, and pheochromo-
cytoma; neurogenic, renovascular, and parenchymal diseases; and
entities associated with increasing stroke volume, e.g., hyperthy-
roidism and aortic insufficiency.
H&P Keys
Wide-ranging disease process may be detected such as cushingoid
features, episodic marked elevation in blood pressure in pheochro-
mocytoma, polyuria, polydipsia, and muscle weakness secondary to
hypokalemia.
Diagnosis
BUN, creatinine level, thyroid-stimulating hormone (TSH) level, re-
nal artery Doppler flow studies or angiography, urine and plasma
catecholamine levels, plasma renin activity, serum and urine cate-
cholamine levels.
Disease Severity
Depends on specific etiology and its natural history as well as re-
sponse of the blood pressure to therapy.
Concept and Application
Elevations of catecholamine levels, activation of the renin–
angiotensin system with vasoconstriction, excessive sodium retention
in primary and secondary aldosteronism, and excessive glucocorti-
coid production.
27
C. Malignant Hypertension
Sudden and severe elevation of blood pressure with evidence of
acute end-organ damage. Includes hypertensive encephalopathy,
rapidly deteriorating renal function, and acute CHF.
H&P Keys
Visual disturbances, severe headache, confusion, coma, seizures,
edema, dyspnea, blood pressure > 200/115, papilledema, hemor-
rhage, spasm on ophthalmoscopic exam, S3 gallop, oliguria.
Diagnosis
BUN, creatinine levels, peripheral smear for microangiopathic he-
molytic anemia, chest roentgenographic evidence of CHF.
Disease Severity
Depends on the extent and severity of end-organ damage and may
include stroke, refractory pulmonary edema, and progressive olig-
uric renal failure.
Concept and Application
The trigger for malignant hypertension is unknown. There is wide-
spread fibrinoid necrosis of the arterial walls. Cerebral autoregula-
tion is impaired and cerebral blood flow is increased, which con-
tributes to the encephalopathy.
Treatment Steps
Acute
1. Nitroprusside infusion in doses of 0.5–8 mg/kg/min can pro-
duce rapid, graded control of blood pressure and is recom-
mended initial therapy.
2. Also, continuous labetalol infusion in doses in the range of 2
mg/min have been effective.
Long-Term—Other agents for long-term control are needed.
1. Diuresis is useful to contract volume and decrease blood pres-
sure and also to assist with CHF and encephalopathy.
2. β-Blockers, ACE inhibitors, and calcium antagonists are all ef-
fective. Renal function may deteriorate in the early phase of
treatment, but persistent blood pressure control is needed to
resolve the arterial lesions.
A. Hyperlipoproteinemia
A wide array of disorders resulting from abnormalities in metabo-
lism of the lipoproteins that transport exogenously and endoge-
nously produced cholesterol and triglycerides. The major lipopro-
teins are chylomicrons and very-low-density lipoprotein (VLDL),
28
CARDIOVASCULAR MEDICINE Lipoproteins and Atherosclerosis
Treatment Steps
General
1. Dietary restriction of fats and weight reduction.
2. Control of secondary factors, e.g., cessation of alcohol, opti-
mum control of diabetes.
3. Elimination of other risk factors for CAD.
Drug Therapy—Dependent on lipid abnormality, severity, other
coronary risk factors, and associated disease. In patients with
known atherosclerosis or significant, multiple risk factors, lipid
therapy should be considered when LDL is > 100 mg/dL. HMG
CoA reductase inhibitors are the most effective agents and have
been demonstrated to substantially reduce atherosclerotic event
rates and are widely used for both primary and secondary pre-
vention. Available agents include:
1-1
LIPOPROTEIN ABNORMALITY TYPES
I Chylomicrons TG
IIA LDL Cholesterol
IIB LDL and VLDL Cholesterol and TG
III Chylomicrons and IDL TG and cholesterol
IV VLDL TG
V VLDL and chylomicrons TG and cholesterol
29
B. Atherosclerosis
Results as a response to injury of vascular endothelium with throm-
bus formation. Initially, isolated macrophages or foam cells infiltrate
endothelium. Later, lipid-rich lesions with smooth muscle and fi-
brous collagen cap form mature atheromatous plaque. Acute coro-
nary syndromes (unstable angina and MI) occur when there is acute
plaque rupture and thrombus formation.
H&P Keys
Anginal chest pain, stroke syndromes with neurologic deficits, in-
cluding motor or sensory abnormalities, intermittent claudication,
reduction in peripheral pulses and pressure.
Diagnosis
ECG, stress testing, and myocardial perfusion imaging, noninvasive
vascular assessment, including carotids, arterial Doppler, cardiac
catheterization, and angiography (Fig. 1–3).
Disease Severity
Extent of motor and sensory deficit after stroke, impairment in car-
diac function after MI, exercise limitation, claudication.
Concept and Application
The development of atherosclerosis is multifactorial and related to
injury of the endothelium as well as the factors that enhance throm-
bosis. Endothelial cells produce a variety of substances that cause
vasoconstriction and smooth-muscle proliferation. Acute plaque dis-
ruption occurs as a result of alterations of stress at the plaque sur-
face. Exposure of damaged vessel wall leads to the adherence of
platelets, development of thrombus, and, ultimately, occlusion of the
vessel.
30
CARDIOVASCULAR MEDICINE Peripheral Arterial Vascular Disease
Figure 1–3. Normal coronary angiogram of left coronary (A) and right coronary (C). Figure B and D arrows point to
atherosclerotic narrowing in abnormal left and right coronary arteries.
Treatment Steps
1. Prevention of atherosclerosis and plaque rupture with thrombosis
is complex and requires multiple interventions. Major risk factors
include cigarette smoking, hypertension, diabetes, and elevated
cholesterol. Management is directed at eliminating these risk fac-
tors. Thrombosis is enhanced by catecholamines (stress), ciga-
rette smoking, and familial predisposition.
2. Aspirin therapy is effective in both primary and secondary pre-
vention.
Medical
1. Control of atherosclerotic risk factors.
2. Smoking cessation most important.
3. Exercise may stimulate collateral blood flow. Vasodilators are
generally ineffective.
4. Pentoxifylline improves exercise ability in approximately one-
third of patients.
Surgical
1. Revascularization with bypass procedure or angioplasty for
limiting symptoms or for limb salvage.
2. Sympathectomy for pain relief.
3. Amputation for severely damaged ischemic limbs.
C. Vasculitis Syndromes
Encompasses a wide variety of conditions, characterized by inflam-
mation of the blood vessel wall. The syndromes include:
1. Necrotizing vasculitis: polyarteritis nodosa.
2. Hypersensitivity angiitis.
3. Giant cell arteritis: Takayasu’s, temporal arteritis.
4. Other: thromboangiitis obliterans; mucocutaneous lymph node
syndrome.
H&P Keys
Wide-ranging because arterial thrombosis can affect any organ sys-
tem. Systemic signs and symptoms include fever, weight loss, arthri-
tis, organ dysfunction from occlusion of cerebral vessel limb or digi-
tal arteries, absent or decreased pulses, differential blood pressures,
vascular bruits. Also, elevated sedimentation rate, leukocytosis.
Diagnosis
Arterial biopsy, e.g., in temporal arteritis to confirm vessel inflamma-
tion. Arterial Doppler studies, PVR, and aortography.
Disease Severity
Dependent on extent and severity of organ dysfunction or compro-
mising blood flow to the limbs. Patients may develop stroke, gan-
grenous bowel, MI, ischemia to limbs and digits.
Treatment Steps
1. Many of these entities respond partially to treatment with sys-
temic glucocorticoids, e.g., temporal arteritis and Takayasu’s dis-
ease.
2. Occasionally, responses are seen from other cytotoxic agents.
3. Cessation of smoking in thromboangiitis.
4. Sympathectomy is of value to relieve pain.
A. Venous Thrombosis
Occurs in a wide variety of settings. Predisposing factors include
trauma or surgery, especially orthopedic, prolonged bed rest for any
reason, pregnancy, and a variety of neoplasms. The disease com-
monly affects the lower extremities, although upper extremities can
be involved.
H&P Keys
Recognition of the appropriate setting and predisposing factors.
Clinical signs (calf swelling, tenderness, and warmth) are nonspe-
cific and frequently missed.
33
Disease Severity
Diagnosis
Impedance plethysmography, Doppler ultrasonography, ventila-
tion–perfusion lung scan, spiral CT, and pulmonary angiography.
Treatment Steps
Acute
1. Heparin in continuous infusion for 7–10 days, adjusted so par-
tial thromboplastin time (PTT) is approximately two times
control value.
Chronic
1. Anticoagulation for 6–12 months with warfarin, longer (indef-
inite) if DVT recurs.
2. Elevation of legs when possible.
3. Full waist-length, graduated compression stockings.
4. Meticulous skin care.
Prophylaxis
1. With 5,000 U heparin every 8–12 hours in high-risk clinical situ-
ations needs to be initiated prior to surgical procedure.
2. Below-the-knee thrombosis can be followed if no proximal ve-
nous disease is detected; anticoagulation may not be required.
B. Pulmonary Embolism
A common event in hospitalized patients, frequently unrecognized,
with high morbidity and mortality.
H&P Keys
Sudden dyspnea, pleuritic chest pain, hemoptysis, syncope, unex-
plained tachycardia, supraventricular dysrhythmias, pulmonary hy-
pertension with a right ventricular lift; wide, persistent splitting; and
loud pulmonic component of the second heart sound.
Diagnosis
can occur with shock and peripheral hypoperfusion and is more se-
vere with preexisting heart or lung disease. Impairment of gas ex-
change and arterial hypoxemia.
Concept and Application
The majority of pulmonary embolisms occur in the setting of DVT.
Embolization of clot from below the knee is unusual. The best ap-
proach to the disease is prophylaxis, prevention, early detection, and
treatment of DVT.
Treatment Steps
1. Heparin, with initial dose of 5,000 U and continuous infusion to
maintain PTT approximately two times control.
2. Intermittent regimens can be employed.
3. With recurrent embolization or contraindication to anticoagulant
therapy, venal caval filter or plication is effective in prevention of
further emboli.
4. If severe hemodynamic compromise occurs, thrombolytic therapy
with streptokinase (initial bolus of 250,000 U and 24-hour infu-
sion) is favored over acute embolectomy.
A. Aneurysms
Pathologic dilatation of a segment of a blood vessel. A true
aneurysm involves all three layers of the vessel wall and is distin-
guished from a pseudoaneurysm, which involves only the intima and
media. Classified by their location and gross appearance: thoracic
versus abdominal, and fusiform versus saccular.
H&P Keys
Most aneurysms are asymptomatic. Symptoms of pain may be pro-
duced by expanding aneurysms; often a harbinger of rupture and an
acute medical emergency. Acute rupture may occur without warning
and is always life threatening. Compression of contiguous blood ves-
sels may result in other symptoms, including stroke from compres-
sion of carotid vessels in thoracic aortic aneurysms and impairment
of lower-extremity blood flow in expanding abdominal aortic
aneurysms.
Abdominal Aortic Aneurysms—Presence of palpable, pulsatile, and
tender abdominal mass with abdominal bruit.
Thoracic Aortic Aneurysms—Tracheal deviation, hoarseness, and CHF
because of aortic dilatation and resulting aortic regurgitation. Pa-
tients with abdominal aneurysms may also have distal arterial em-
bolization and lower-extremity claudication. Most abdominal
aneurysms occur distal to the renal arteries.
Diagnosis
Radiography including CXR and abdominal films may demonstrate
the enlarged aorta sometimes outlined by calcium. More definitive
studies include thoracic and abdominal ultrasonography, CT, MRI,
or invasive angiography.
Disease Severity
Determined by location, size of aneurysm, and rate of dilatation. For
abdominal aneurysms exceeding 6 cm in diameter, the 2-year mor-
35
Treatment Steps
1. Location and extent of the aneurysm using radiographic imaging
techniques.
2. Followed by operative excision of the aneurysm and replacement
with graft and reimplantation of branch vessels.
B. Aortic Dissection
Caused by a transverse or circumferential tear of the aortic intima in
areas with high shear forces (left subclavian artery and right lateral
ascending aortic wall).
• Type 1: Dissection in proximal aorta, may extend into the arch.
• Type 2: Dissection limited to aortic arch.
• Type 3: Dissection begins distal to left subclavian artery and ex-
tends for variable distance inferiorly.
H&P Keys
Acute dissection is characterized by a sudden onset of severe and
tearing pain associated with diaphoresis. Pain usually from front of
chest to interscapular area. May be associated with syncope, dyspnea,
or weakness. Blood pressure may be high or low. Other signs include
differential pulses, pulmonary edema, neurologic symptoms (stroke
or spinal cord compression), aortic regurgitation producing CHF.
Abdominal aortic dissection may be accompanied by bowel ischemia
and hematuria, whereas thoracic dissection may be accompanied by
superior vena caval syndrome, hoarseness, airway compromise, dys-
phagia, inferior MI with hemopericardium and cardiac tamponade.
Diagnosis
CXR, ECG, CT scan, MRI, and aortography.
Disease Severity
Physical examination for involvement of brain, peripheral arteries,
kidneys, spinal cord, heart. Continued pain or symptoms of compro-
mise to major arterial branches indicate active propagation of dissec-
tion. Hypotension or shock, oliguria, mentation.
Treatment Steps
1. Emergency operation is indicated for patients with symptoms in-
dicating propagating dissection. Emergency operation carries a
36
CARDIOVASCULAR MEDICINE Diseases of the Aorta
C. Aortic Occlusion
Chronic occlusive disease that generally involves the distal abdomi-
nal aorta below the renal arteries.
H&P Keys
Claudication, impotence in males (Leriche’s syndrome). Symptoms
vary depending on presence and adequacy of collateral blood flow.
Physical findings include absent or depressed femoral and distal
pulses and presence of bruits over abdominal aorta and femoral ar-
teries. Lower-extremity skin loss, atrophic skin changes, cool extrem-
ities, peripheral skin ulcerations.
Diagnosis
Physical examination, noninvasive Doppler evaluation of arterial
blood flow, ankle-brachial index (ABI) (sphygmomanometry). Ab-
dominal aortography to define anatomy prior to revascularization.
on rounds
C
CARDIOLOGY AT A GLANCE
Myocardial Infarction
• Chest pain, diaphoresis, 20% without pain
• Transmural or Q wave has ST segment elevation and T wave inversions
• Nontransmural has ST segment elevation and T wave inversions.
• CK-MB peaks at 24 hours
• AST or SGOT peaks at 48–72 hours
• LHD peaks at 3–5 days
Prinzmetal’s or Variant Angina
• Angina at rest
• Younger patients
• ECG positive for ST elevation during symptoms
• Gold standard diagnostic test: Spasm on angiography
• Treatment: Nitrates, calcium channel blockers, stop smoking, avoid cocaine
Signs of Heart Failure
Left Side, Low Output
• Fatigue, dyspnea, PND, orthopnea
• S3 or S4, displaced cardiac impulse, CXR, echo
Left Side, High Output
• Brisk pulse, dyspnea, orthopnea, hyperdynamic circulation
Right Side
• Fatigue, RV heave, JVD, hepatomegaly, atrial arrhythmias
• ECG may show RV or RA hypertrophy
• Echo may show RV dilation and hypokinesis
37
D. Aortitis
Syphilitic, rheumatic.
1. Syphilitic Aortitis
Usually affecting the proximal aorta, resulting in aortic root dilata-
tion and aneurysm formation.
H&P Keys
Often asymptomatic. As it progresses, may cause compression and
erosion into adjacent structures; rupture may occur.
Diagnosis
CXR, 2-D echo, cardiac catheterization, immunologic screening,
rapid plasma reagin (RPR), Venereal Disease Research Laboratory
(VDRL).
Disease Severity
Long latency period: 15–30 years after initial infection. Symptoms
may occur from aortic regurgitation or narrowing of coronary ostia
or from compression to adjacent structures (esophagus), or rupture.
Serologic tests confirm diagnosis. Evaluation includes CXR, ultra-
sonography, CT scan, MRI, and aortography.
Concept and Application
Destruction of collagen elastic tissue, leading to dilatation of aorta
with scar formation and calcification, is due to obliterative endarteri-
tis of the vasa vasorum in the adventitia. This is an inflammatory re-
sponse to invasion of the adventitia by spirochetes.
Treatment Steps
1. Antibiotic treatment (penicillin).
2. Surgical excision and repair.
2. Rheumatic Aortitis
Includes rheumatoid arthritis, ankylosing spondylitis, psoriatic
arthritis, Reiter’s syndrome, Behçet’s syndrome, relapsing polychon-
dritis, and inflammatory bowel disorders.
BIBLIOGRAPHY
ACC/AHA Guidelines
1. Guidelines for the management of patients with ST-elevation myocardial infarc-
tion––executive summary. Circulation 2004;110:588–636.
2. Guideline update for the management of patients with unstable angina and
non-ST segment elevation myocardial infarction. J Am Coll Cardiol 2002;40:1366.
3. Guidelines for the evaluation and management of chronic heart failure in the
adult. Circulation 2001;104:2996.
38
CARDIOVASCULAR MEDICINE Diseases of the Aorta
4. Implications of recent clinical trials for the National Cholesterol Education Pro-
gram Adult Treatment Panel III Guidelines. Circulation 2004;110:227–239.
Braunwald E. Heart Disease: A Textbook of Cardiovascular Medicine, 7th ed. Philadelphia:
W.B. Saunders, 2005.
Fuster V, et al (eds.). Hurst’s The Heart, 11th ed. New York: McGraw-Hill, 2004.
Loh E, McClellan JR. Congestive heart failure. In: Conn RB, et al (eds.). Current Diag-
nosis. Orlando, FL: W.B. Saunders, 1997.
McClellan JR. Clinical approach to the patient in shock. In Chizner M (ed.). Classic
Teachings in Clinical Cardiology. Cedar Grove, NJ: Laennec Publishing, 1996.
Dermatology 2
I. ACUTE EXANTHEMS / 40
A. Varicella (Chickenpox) / 40
B. Varicella Zoster (Shingles) / 41
BIBLIOGRAPHY / 69
39
Copyright © 2004, 2001 by The McGraw-Hill Companies, Inc. Click here for terms of use.
U
40
DERMATOLOGY Acute Exanthems
A. Varicella (Chickenpox)
In smallpox, the vesicular
H&P Keys
lesions are in the same
stage of development. Rash begins on face and scalp and rapidly spreads to trunk, with rel-
ative sparing of extremities. Typical lesions are vesicles with a pink
base, but presence of lesions at all stages of development (macules,
diagnostic
decisions d papules, vesicles, pustules, crusted lesions) is characteristic. Fever,
chills, malaise, and headache may accompany. Mucous membranes
may be affected.
2. Candidiasis, Cutaneous
H&P Keys
B. Bacterial Infections
1. Cellulitis, Abscess, or Other Local Infections
H&P Keys
Diagnosis
Culture, Gram stain.
Disease Severity
Systemic toxicity suggests bacteremia.
Concept and Application
Diagnosis
Bacterial culture, Gram stain.
Disease Severity
Spread by close contacts. Secondary infection of preexisting skin le-
sions is common.
Concept and Application
Highly communicable infection. Crowding, poor hygiene, neglected
wounds, and minor trauma contribute to spread. Bullous variant
caused by phage group II type 71 Staphylococcus.
Treatment Steps
Topical antibiotic ointments, including mupirocin. β-Lactamase–
resistant antibiotics for bullous type.
C. Viral Infections
1. Verrucae (Warts)
H&P Keys
Common wart (verruca vulgaris), palmoplantar wart (verruca pal-
maris/plantaris), flat wart (verruca plana), genital wart (condyloma
acuminatum); spread by trauma.
Diagnosis
History and physical exam; biopsy confirmatory.
Disease Severity
Disseminated lesions associated with immunodeficiency; certain viral
types (16, 18, 31, 33) associated with squamous cell carcinoma.
Concept and Application
Human papillomavirus (HPV) infection. Most treatment modalities
involve destruction of involved tissue. Imiquimod induces a specific
immune response locally.
Treatment Steps
1. Topical acids (e.g., salicylic acid) daily. Imiquimod for genital
warts.
2. Liquid nitrogen, paring, topical acids.
3. Excision, laser.
2. Molluscum Contagiosum
H&P Keys
Discrete umbilicated pearly papules in children and adults; may be
transmitted sexually.
44
DERMATOLOGY Other Skin Infections
Diagnosis
History and physical exam, biopsy confirmatory.
Disease Severity
Numerous, large, and disfiguring in acquired immune deficiency
syndrome (AIDS) patients.
Concept and Application
DNA poxvirus infection.
Treatment Steps
Same as for warts.
3. Herpes Simplex
H&P Keys
Grouped vesicles on base of erythema. Occurs anywhere on the skin,
but mostly perioral or genital. Local discomfort and possible pro-
drome or systemic symptoms (Fig. 2–3).
Diagnosis
History and physical exam. Vesicle for culture or direct fluorescent
antibody assay, polymerase chain reaction (PCR). Tissue biopsy.
Disease Severity
Herpetic keratoconjunctivitis can lead to blindness. Erythema multi-
forme minor usually related to outbreak of orolabial herpes. Dissem-
ination to central nervous system (CNS) seen mostly in immunosup-
pressed.
Concept and Application
Herpes simplex virus (HSV) is a DNA virus spread by physical con-
tact. HSV-1 causes most orolabial disease and almost all adults are
seropositive. HSV-2 associated with genital herpes with 20% seropos-
itivity.
Treatment Steps
1. Acyclovir or derivative PO at first sign of outbreak. Local care.
2. Suppressive therapy with daily acyclovir if more than six episodes
per year.
D. Secondary Syphilis
H&P Keys
Lesions appear 6–12 weeks after onset of chancre (primary stage).
Associated lymphadenopathy. Great imitator—many cutaneous ex-
pressions: macules, brownish-red papules, variable scale. Palms and
soles often involved (Fig. 2–4).
Diagnosis
History and physical exam, darkfield microscopy, serology, biopsy.
Disease Severity
Associated findings: mucous patches, condylomalta, pharyngitis, iri-
tis, periostitis, arthralgias, hepatosplenomegaly.
Concept and Application
U
Sexually transmitted disease caused by the spirochete Treponema pal-
lidum. Untreated, it may progress to tertiary phase (granulomas,
gummas). cram facts
Treatment Steps
1. Benzathine penicillin (tetracycline or doxycycline in penicillin al- Secondary syphilis Rx:
lergic). Beware of Jarisch–Herxheimer reaction (acute exacerba- benzathine penicillin 2.4
tion of disease with treatment). million units IM × 3 doses
2. Sexual partners should be screened. administered q 7 days.
3. HIV test recommended for all patients with syphilis.
management
decisions d bows, knees, scalp, buttocks, nails. Variants: pustular, erythrodermic,
guttate, palmoplantar.
Diagnosis
PSORIASIS History and physical exam, biopsy to confirm.
U
ers Strep. If pustular, start acitretin.
2. Add phototherapy. If pustular disease unresponsive to ac-
cram facts itretin, consider other systemic agent.
3. Start systemic agent.
2. Seborrheic Dermatitis
The nail changes of
psoriasis are pitting, “oil H&P Keys
spot” macule, The mildest form is flaking of scalp (dandruff). Scale is yellowish
onychogryphosis, and and greasy. Variable erythema. Also affects external ear canal, eye-
onycholysis.
brows, nasal crease. Occasionally involves presternal area, axillae,
umbilicus, and groin. In infant, referred to as cradle cap.
47
4. Atopic Dermatitis
H&P Keys
Personal or family history of atopy. Marked pruritus. May begin in
infancy. Erythematous, vesicular or crusted plaques (see Fig. 2-7)
in antecubital and popliteal fossae, posterior neck. Redundant eye-
lid fold, hyperlinear palmar creases, dry skin, and cataracts are asso-
ciated findings.
Diagnosis
History and physical exam, biopsy, blood eosinophilia, immunoglob-
ulin E (IgE) levels.
Disease Severity
May generalize (erythroderma). Staphylococcus aureus is a frequent
colonizer and may trigger flares. Increased susceptibility to herpes
infection (Kaposi’s varicelliform eruption) and fungal infection.
Concept and Application
U
A type of eczematous dermatitis with an unknown cause. In some pa-
tients, allergens (e.g., foods) may provoke itching, dermatitis, and
cram facts bronchospasm.
Treatment Steps
Nummular dermatitis is 1. Moisturizers, topical corticosteroids, sedating antihistamines,
another eczematous avoidance of irritants. Antibiotics if secondary infection present
dermatitis that often arises or if staphylococcal-related flare suspected.
in patients with dry skin or 2. Tar preparations, higher-potency topical steroids. Topical
a history of atopic
tacrolimus and pimecrolimus.
dermatitis (see Fig. 2–8).
3. UVB, PUVA, systemic steroids only rarely.
5. Urticaria (Hives)
H&P Keys
Acute or chronic (> 6 weeks). Lesions are fleeting. Pruritic, pink,
edematous papules (wheals).
Diagnosis
History and physical. Biopsy in chronic cases to rule out vasculitis.
Differentiate from dermographism (hives occurring after scratch-
ing).
Disease Severity
May be associated with laryngeal spasm or bronchospasm. An-
gioedema (deep swelling in skin) may be associated (if prominent
feature, rule out hereditary angioedema associated with C1 esterase
inhibitor deficiency).
Concept and Application
Numerous causes. Both type I and II hypersensitivity implicated.
Mast cell degranulation leads to edema and vascular permeability.
Treatment Steps
1. Elimination of cause if known (drugs, foods, insect bites most
common), oral antihistamines, avoid systemic corticosteroids.
2. Switch antihistamine to H1/H2 blocker (doxepin) or add
H2-blocking antihistamine; consider patch testing.
6. Drug Reactions
H&P Keys
Variable presentation. Disseminated pruritic pink macules and
papules (morbilliform) are typical (e.g., mononucleosis patient
given amoxicillin). Time course of rash usually corresponds to of-
fending medication. Other presentations: urticaria, erythema multi-
forme (targetoid lesions), photosensitivity, vasculitis, fixed (lesions
recur in same spot with rechallenge).
Diagnosis
History and physical exam. Biopsy. Some drugs are more frequent
offenders (trimethoprim–sulfamethoxazole, phenytoin, thiazides,
penicillins).
Disease Severity
May generalize (erythroderma). Itching may be severe. Erythema
multiforme (Stevens–Johnson syndrome, toxic epidermal necrolysis)
may be life threatening. Systemic vasculitis may be associated with
cutaneous lesions (palpable purpura).
Treatment Steps
1. Cessation of offending medication.
2. Antihistamines, soothing topical emollient lotions, topical
steroids. Systemic corticosteroids should be used with caution.
B. Blistering Diseases
1. Bullous Pemphigoid
H&P Keys
Elderly patients; pruritus; large, tense blisters and urticarial plaques;
negative Nikolsky’s sign (cannot induce blister with blunt pressure).
Diagnosis
Biopsy for routine studies and direct immunofluorescence, serum for
indirect immunofluorescence (to detect circulating autoantibody).
Disease Severity
Blisters can be large and leave large eroded areas; mucosal lesions
are painful; pruritus can be severe.
Concept and Application
Autoimmune mechanisms with immunoglobulin G (IgG) and com-
plement infiltrating skin; immunosuppressives used for treatment.
Rarely, may be caused by drugs (furosemide).
Treatment Steps
1. Local skin care; superpotent topical corticosteroids for limited
disease.
2. Systemic steroids, tetracyclines, or other immunosuppressives for
generalized disease.
3. Taper as tolerated.
2. Herpes Gestationis
H&P Keys
Clinical presentation identical to bullous pemphigoid except occurs
in second or third trimester of pregnancy.
Diagnosis
History and physical exam, skin biopsy and immunofluorescence
(differentiate from pruritic urticarial papules and plaques of preg-
nancy [PUPPP], which begins on abdomen [usually striae] and oc-
curs late in pregnancy).
Disease Severity
May increase fetal mortality or premature delivery; fetus may be
born with skin lesions.
Concept and Application
Autoimmune mechanism. Oral contraceptives may exacerbate dis-
ease in patients with documented disease.
Treatment Steps
Some cases of mild disease can be managed with antihistamines and
topical steroids. Most patients require systemic steroids.
3. Pemphigus
H&P Keys
Two types: superficial (foliaceus) and common (vulgaris). Vulgaris
type shows fragile blisters and Nikolsky’s sign. Oral lesions are com-
51
Disease Severity
Itching usually severe. Associated steatorrhea, anemia.
Concept and Application
Strong human leukocyte antigen (HLA) predisposition identical to
that seen in celiac disease. Immunoglobulin A (IgA) in skin proba-
bly has gut origin.
Treatment Steps
1. Dapsone. Gluten-free diet is beneficial and may reduce need for
dapsone.
2. Taper dapsone as tolerated.
Acute—Excoriations.
Chronic—Nodules (prurigo nodularis) or scaly plaques (lichen
simplex chronicus).
Diagnosis
History and physical exam, biopsy will confirm.
54
DERMATOLOGY Skin Eruptions
Disease Severity
Disfiguring scars. In factitial dermatitis (self-inflicted), bizarre con-
figuration of lesion is characteristic.
Concept and Application
In some cases, an inciting event (insect bite, rash) leads to self-
perpetuating itch–scratch cycle.
Treatment Steps
1. Break the itch–scratch cycle, corticosteroids (triamcinolone ace-
tonide) injected into nodules. Superpotent topical cortico-
steroids for lichen simplex chronicus.
2. Reinject as necessary. Psychological counseling in severe cases.
E. Ectoparasites
1. Scabies
H&P Keys
Marked pruritus (especially in the evening); papules, vesicles, and
burrows in typical sites (interdigital web spaces, volar wrists, axillae,
areolae, umbilicus, genitals, knees, ankles) (Fig. 2–11).
Diagnosis
Skin scraping with a drop of mineral oil on slide will demonstrate
mites or their products.
Disease Severity
Variant (Norwegian scabies) produces crusted, scaly lesions and is
found in elderly, mentally retarded, or immunocompromised pa-
tients.
Concept and Application
May be sexually transmitted.
Treatment Steps
1. Permethrin 5% cream or Lindane 1% (associated with neurotoxi-
city in kids) lotion. Antihistamines and topical steroids for itch-
ing. Treat contacts. Consider HIV testing if sexually transmitted.
2. Repeat course if necessary or ivermectin PO.
A. Chloracne
H&P Keys
History of occupational/environmental exposure to halogenated
aromatic hydrocarbons (e.g., dioxin).
Diagnosis
History and physical exam. See multiple closed comedones and
straw-colored cysts over malar crescents and retroauricular folds.
Disease Severity
As toxicity increases, more skin is involved. Cosmetic disfigurement;
scarring.
Concept and Application
There is a delay of 2 weeks to 1 month before the appearance of le-
sions following exposure. Liver disease, peripheral neuropathy, hy-
perlipidemia, porphyria cutanea tarda have been reported depend-
ing on the level of chemical exposure. Possible increased risk of soft
tissue sarcoma.
Treatment Steps
1. Keratolytics (e.g., salicylic acid), topical retinoids, oral antibiotics,
and topical benzoyl peroxide.
2. Oral isotretinoin if recalcitrant, acne surgery.
B. Rosacea
H&P Keys
Adult onset, women predominate, associated with flushing; telang-
iectasia (dilated blood vessels), papules, pustules symmetrically dis-
tributed on face (Fig. 2–13); no comedones.
Diagnosis
History and physical exam; may resemble malar erythema of lupus
erythematosus.
Disease Severity
Keratitis may be associated. Rhinophyma (hypertrophy of sebaceous
glands) may result. Figure 2–13. Rosacea.
56
DERMATOLOGY Hair and Hair Follicle Disorders
Concept and Application
cram facts U Flushing with increase in skin temperature provoked by hot liquids,
spicy foods, and alcohol.
Treatment Steps
1. Reduction or elimination of provoking factors. Topical metro-
ACNE VULGARIS nidazole or sulfur lotions.
2. Add oral tetracycline, rarely, isotretinoin.
Keratolytics (salicylic acid)
and topical retinoids control C. Alopecia Areata
comedones (beware
photosensitivity while using H&P Keys
retinoids––sunscreen Localized round or oval patches of hair loss without visible skin in-
important), mild soaps flammation (see Fig. 2–14); children or adults; any hair-bearing area
minimize irritation. If there is can be affected; can be stress provoked.
an inflammatory
component, add antibiotic, Diagnosis
topical (clindamycin, History and physical exam, biopsy.
erythromycin, benzoyl
peroxide) if mild, oral Disease Severity
(tetracycline or derivative, Can involve entire scalp (alopecia totalis) or entire body (alopecia
erythromycin) if more universalis); associated with other autoimmune disorders
severe. If acne is
(Hashimoto’s thyroiditis, vitiligo).
persistent, consider
hormonal workup/ Concept and Application
treatment. Severe cystic
Autoimmune lymphocytes react against hair follicles, treatment diffi-
acne usually responds well
to a course of oral cult.
isotretinoin. Need to
Treatment Steps
monitor triglycerides and
liver function tests (LFTs). 1. Superpotent topical steroid.
2. If no response, intralesional triamcinolone, consider topical mi-
noxidil, anthralin.
A B
Figure 2–15. (A) Squamous cell carcinoma of lower lip. (B) Basal cell carcinoma.
Disease Severity
Locally invasive. Metastasis rare.
Concept and Application
Malignant tumor of skin related to chronic sun injury.
Treatment Steps
1. Excision, destruction (electrical, freezing).
2. Mohs’ surgery if cosmetically sensitive site.
3. Surveillance for recurrence, new lesions.
4. Malignant Melanoma
H&P Keys
Enlarging, asymmetric, irregularly bordered, variably colored, large
(> 6 mm) pigmented patch. Black color is suspicious. Initially is flat
(radial growth), then becomes nodular (vertical growth) (Fig.
2–16). May bleed and ulcerate. Family history and/or history of blis-
tering sunburns.
Diagnosis
Excisional biopsy.
Disease Severity
Aggressive tumor with high propensity to metastasize widely. Progno-
sis related to depth of skin invasion (Clark’s and Breslow’s levels).
Figure 2–16. Melanoma.
Lesions < 1 mm have good prognosis, > 3 mm have poor prognosis.
Concept and Application
Can develop from congenital nevi or chronic sun exposure (lentigo
maligna). Superficial spreading is most common; nodular is worst
prognostically; acrolentiginous (hands, feet, mucosae) is most com-
mon in blacks.
59
B. Benign Neoplasms
1. Lipoma
H&P Keys
Single or multiple, rubbery or compressible subcutaneous masses,
most often on trunk, posterior neck, or forearms. Angiolipomas are
often multiple and painful.
Diagnosis
Physical exam, excisional biopsy.
Disease Severity
Rarely infiltrates into skeletal muscle. May occur in Gardner’s syn-
drome. Midline back lesions can be associated with spinal dys-
raphism.
Concept and Application
Benign tumor of adipose tissue.
Treatment Steps
None if asymptomatic, excision.
2. Atypical (Dysplastic) Nevus
H&P Keys
Familial or sporadic, large, irregularly bordered, pigmented macules
and papules. Family history.
Diagnosis
History and physical exam; biopsy.
Disease Severity
Multiple familial atypical nevi associated with greatly increased risk
of melanoma. Lesions themselves are not necessarily precursors.
Concept and Application
Proliferation of melanocytes with varying cytologic atypia.
Treatment Steps
Excision if melanoma is a diagnostic consideration; sun precaution
and sunscreens; semiannual complete skin exams.
3. Hemangioma
H&P Keys
Capillary or strawberry nevus (congenital), cavernous (bluish-purple
subcutaneous), senile (red papules on trunk of elderly person).
Diagnosis
History and physical exam.
Disease Severity
Multiple cutaneous lesions may be associated with internal organ in-
volvement (CNS, gastrointestinal [GI] tract, liver). Large cavernous
60
DERMATOLOGY Tumors of the Skin
Treatment Steps
Excision, laser.
5. Seborrheic Keratosis
H&P Keys
Most common benign skin lesion. Warty, light to dark brown stuck-
on plaques. Usually multiple, in various stages of development (Fig.
2–18).
Disease Severity
May be sign of internal malignant neoplasm (esophagus). May ap-
pear at menopause (keratoderma climacterium).
Treatment Steps
1. Lubrication, elimination of aggravating factors.
2. Add keratolytic agents (salicylic acid, urea) if insufficient re-
sponse.
Diagnosis
Clinical exam; biopsy in atypical lesions.
Disease Severity
Multiple or large ulcers may occur; secondary cellulitis.
Treatment Steps
1. Bed rest, leg elevation, hydrocolloid dressings, compression
stockings, diuresis.
2. Compression bandage, surgical treatment for varicosities.
62
DERMATOLOGY Other Conditions
2. Arteriosclerotic Ulcer
H&P Keys
Markedly painful, dry, shallow necrotic ulcer on foot or lateral leg.
Foot is cold and hypoesthetic. Rest pain, claudication, or other signs
and symptoms of atherosclerosis.
Diagnosis
Palpation of peripheral pulses, Doppler studies, arteriography.
Disease Severity
Significant associated large and small arterial disease.
Concept and Application
Chronic obstruction of small and large vessels by atheromas.
Treatment Steps
1. Address underlying disease, smoking cessation, antiplatelet
agents, pentoxifylline.
2. Check ankle-brachial index.
3. Pyoderma Gangrenosum
H&P Keys
Painful nodule or pustule that rapidly ulcerates, with a tender, un-
dermined border. Associated with ulcerative colitis, Crohn’s disease,
arthritis, paraproteinemia, and leukemia.
Diagnosis
History and phyiscal exam, biopsy, rule out associated underlying
diseases.
63
C. Cutaneous Manifestations
of Systemic Disease
1. Lupus Erythematosus
H&P Keys
Three types: acute (systemic), subacute, chronic (discoid) (Fig.
2–20).
Acute—Malar erythema (butterfly rash), Raynaud’s phenomenon,
oral ulcers, alopecia, vasculitis.
Subacute—Annular or polycyclic (resembling tinea or psoriasis),
photosensitivity.
Chronic—Scarring, red, scaling plaques, primarily on sun-exposed
areas. Review-of-systems check.
Diagnosis
History and physical exam; laboratory studies may include: complete
blood count, antinuclear antibody (ANA), urinalysis, SMA, Ro (SS-
A), La (SS-B); skin biopsy, immunofluorescence.
Disease Severity
Acute may have serious renal or CNS manifestations. Subacute is as-
sociated with a low risk of CNS or renal disease. Chronic is usually
limited to the skin.
Disease Severity
In adults may herald internal malignant neoplasms.
Treatment Steps
Topical and systemic steroids, rest, cytotoxic agents.
3. Scleroderma
H&P Keys
Localized: morphea. Diffuse: progressive systemic sclerosis. Latter
also associated with Raynaud’s phenomenon, dysphagia, masklike
face. Skin is tight, woody, bound down.
Diagnosis
History and physical exam, skin biopsy, ANA, anticentromere anti-
body, Scl-70 (anti-topoisomerase).
Disease Severity
Progressive systemic sclerosis associated with multisystem involve-
ment: renal, lung, esophagus. Variant: CREST = calcinosis, Ray-
naud’s, esophageal dysfunction, sclerodactyly, telangiectasia. Better
prognosis; associated with anticentromere antibody.
Treatment Steps
Symptomatic, physical therapy, immunosuppressives. Penicillamine
(controversial).
4. Livedo Reticularis
H&P Keys
Mottled, netlike vascular erythema; aggravated by cold exposure;
most common in women < 40 years old.
Diagnosis
History and physical exam.
65
Treatment Steps
Avoidance of cold exposure, treatment of associated medical condi-
tions.
5. Amyloidosis, Systemic
H&P Keys
Diagnosis
Clinical exam.
Disease Severity
Associated findings: arthritis, retinal vasculitis, cardiovascular and
neurologic effects.
Concept and Application
No known etiology; more frequent in men. Lesions develop at sites
of trauma (pathergy).
Treatment Steps
Systemic steroids, colchicine, azathioprine, chlorambucil, cyclophos-
phamide.
7. Tuberous Sclerosis
H&P Keys
Autosomal dominant, seizures, retardation. Skin lesions: white spots
(ash-leaf macule, earliest lesion), adenoma sebaceum (Fig. 2–21),
connective tissue nevi (shagreen patch), periungual fibrous tumors.
66
DERMATOLOGY Other Conditions
Diagnosis
History and physical exam, biopsy of adenoma sebaceum.
Disease Severity
Variable expression. Some patients may show cutaneous features
only.
Concept and Application
Genetic multisystem disease.
Treatment Steps
Supportive management, magnetic resonance imaging (MRI) of the
head to rule out CNS involvement.
8. Necrobiosis Lipoidica
H&P Keys
Yellow-brown atrophic plaques on shins, may ulcerate.
Diagnosis
History and physical exam, biopsy.
Disease Severity
Most cases associated with diabetes.
Concept and Application
Necrosis of the lower dermis, with granulomatous inflammation and
vasculitis.
Treatment Steps
Topical and intralesional corticosteroids, antiplatelet agents, check
glucose.
9. Porphyria Cutanea Tarda
H&P Keys
Photosensitivity, blisters on backs of hands, scars, increased hair
growth (werewolf), hyperpigmentation.
Diagnosis
Urine will fluoresce under Wood’s light; blood, urine, stool studies
for porphyrins; skin biopsy.
67
Figure 2–23. Beau’s lines (patient had ruptured berry aneurysm 6 months previously).
BIBLIOGRAPHY
Fitzpatrick TB, et al. Dermatology in General Medicine, 6th ed. New York: McGraw-Hill,
2003.
Odom RB, James WD, Berger TG. Andrews’ Diseases of the Skin, Clinical Dermatology, 9th
ed. Philadelphia: W.B. Saunders, 2000.
Wolf K, et al. Fitzpatrick’s Color Atlas & Synopsis of Clinical Dermatology, 5th ed. New York:
McGraw-Hill, 2005.
Endocrinology 3
I. THYROID GLAND DISORDERS / 72
A. Hyperthyroidism / 72
B. Hypothyroidism / 73
C. Neoplasms of the Thyroid Gland / 74
BIBLIOGRAPHY / 87 71
Copyright © 2004, 2001 by The McGraw-Hill Companies, Inc. Click here for terms of use.
72
ENDOCRINOLOGY Thyroid Gland Disorders
d
A. Hyperthyroidism
diagnostic H&P Keys
decisions Most commonly autoimmune etiology (Graves’ disease). Can also be
toxic adenoma, toxic multinodular goiter, iodine induced or facti-
HYPERTHYROIDISM tious.
Weight loss, palpitations, nervousness, muscle weakness, heat in-
Graves’ Disease tolerance, dyspnea, increased bowel movements, change in men-
Family history of thyroid
strual function, eye symptoms (burning, tearing, diplopia). Family
disease, other autoimmune
diseases, diffusely enlarged
history of thyroid disease.
gland with bruit. Elevated Thyroid enlargement common, thyroid bruit, proptosis, tremor,
T4, T3RU, suppressed TSH. tachycardia, hyperactive reflexes, systolic hypertension, pretibial
Increased 123I uptake. myxedema, onycholysis, proximal myopathy, ophthalmopathy (Fig.
Subacute Thyroiditis 3–1).
Recent viral illness, painful Hyperthyroidism in the elderly often presents with apathetic
thyroid. Elevated T4 and rather than hyperkinetic symptoms. Cardiac manifestations are com-
T3RU, suppressed TSH. mon (atrial fibrillation, congestive failure).
Suppressed 123I uptakes.
Toxic Adenoma Diagnosis
Older age, solitary nodule Elevated levels of total or free thyroxine (T4), triiodothyronine (T3),
on exam. Elevated T4, and resin uptake (T3RU). Suppressed levels of thyrotropin (TSH).
T3RU, and TSH. Single, Estrogen in pills or pregnancy increases T4 binding globulin: in this
“hot” nodule on 123I scan.
situation the T4 is increased and the T3 uptake is decreased. Assay of
thyroid antibodies may be performed to exclude Hashimoto’s thy-
roiditis. An iodine (123I) uptake and scan can be performed if the di-
agnosis is not secure or if radioactive iodine therapy is considered.
Figure 3–1. Photographs of three patients with ophthalmopathy due to Graves’ disease. A, B. A young woman with moderate asymmetric proptosis, with
marked left eyelid retraction. This patient has few manifestations of ocular inflammatory disease. C. A young woman with marked periorbital edema, bilateral lid re-
traction, and proptosis. D. A middle-aged woman with marked infraorbital edema, conjunctival injection and chemosis, and muscle restriction of the right eye, but lit-
tle proptosis. (Reproduced, with permission, from Felig P. Endocrinology and Metabolism, 4th ed. New York: McGraw-Hill, 2001.)
73
B. Hypothyroidism
H&P Keys
Usually secondary to chronic autoimmune (Hashimoto’s) thyroidi-
tis, radioactive iodine therapy, head or neck irradiation, or thyroid
surgery. Family history of thyroid disease, fatigue, weakness, cold in-
tolerance, sleepiness, dry skin, hoarseness, constipation, depression,
slow mentation, menstrual irregularities, infertility, weight gain.
On physical examination, a firm goiter with multiple nodules
(Hashimoto’s disease) or a nonpalpable gland; bradycardia; slow,
hoarse speech; cool, dry, thick skin; delayed relaxation of deep-
tendon reflexes; yellow skin (carotenemia); loss of scalp hair and
eyebrows (see Fig. 3–2).
Diagnosis
Elevated level of TSH, low total or free T4 level; T3 levels and 123I up-
take and scans not helpful in diagnosis. Assays for thyroid peroxi-
dase antibodies (previously antimicrosomal antibodies) or antithy-
roglobulin antibodies to confirm Hashimoto’s thyroiditis. If positive,
these patients and their families have an increased incidence of
other autoimmune diseases such as Graves’, pernicious anemia, vi-
tiligo, rheumatoid arthritis, adrenal insufficiency, and premature
menopause.
Disease Severity
Mental status: confusion, dementia, stupor, or coma; decreased ven-
tilation and abnormal blood gases; hypothermia, cardiomyopathy,
ataxia.
Treatment Steps
Uncomplicated Hypothyroidism
1. Begin L-thyroxine replacement at 0.075–0.150 mg daily. El-
derly patients should be started on 0.0125–0.025 mg daily.
2. Check TSH level in 6 weeks after any dose adjustment.
3. Avoid concomitant use of cholestyramine, antacids, and iron
supplements that interfere with T4 absorption in the gastroin-
testinal tract.
Myxedema Coma
1. Respiratory support.
2. Hydocortisone 100 mg IV every 8 hours; first dose must pro-
ceed T4 replacement.
3. L-thyroxine IV 2 µg/kg load, followed by 100 µg every 24
hours until respiratory and mental status improve.
Diagnosis
Fine-needle aspiration for histocytopathologic examination. If speci-
men inadequate, repeat the aspiration. Assay of T4, TSH, and thy-
roid antibodies to determine if patient has Hashimoto’s thyroiditis
with a lumpy thyroid. Baseline thyroid ultrasonogram to check nod-
ule size and rule out a cyst. 123I thyroid uptake and scan will reveal if
the nodule is “cold” (nonfunctioning), or “hot” (hyperfunctioning)
and less likely to be malignant. A thyroglobulin level is occasionally
helpful as a tumor marker. If elevated, it can be rechecked after
surgery, and followed for recurrence of the tumor. Elevated calci-
tonin levels are seen in medullary thyroid carcinoma.
75
A. Hypercalcemia
H&P Keys
Family or personal history of hypercalcemia, renal stones, multiple
endocrine neoplasia (MEN) type 1 or 2, or malignancy. Many are
asymptomatic. Common symptoms: fatigue, lethargy, nocturia, weak-
ness, constipation, depression, or renal colic from kidney stones.
History of calcium, vitamin D or vitamin A intake, use of thiazides or
lithium.
Diagnosis
Elevation of calcium levels corrected for albumin. Primary hyper-
parathyroidism will have low levels of phosphorus and normal to ele-
vated parathyroid hormone (PTH) assays (intact PTH assays most re-
liable). Sestamibi parathyroid scan to localize adenoma or
hyperplasia. Suppression of PTH levels in face of hypercalcemia sug-
gests nonparathyroid source. Chest x-ray (CXR), parathyroid-related
protein assay in smoker with hypercalcemia. Serum protein elec-
trophoresis to evaluate for multiple myeloma. 1,25-dihydroxyvitamin
D levels elevated in lymphoma and granulomatous diseases (sar-
coidosis, leprosy). T4 and TSH to exclude hyperthyroidism.
Disease Severity
Calcium levels > 12 mg/dL. Kidney stones, osteoporosis, pancreati-
tis, lethargy, confusion, stupor or coma.
Concept and Application
Overproduction of parathyroid hormone in primary hyperparathy-
roidism (single adenoma or hyperplasia of all four glands), may be
familial (autosomal dominant), part of MEN 1, which includes tu-
mors of the pituitary and pancreas (insulinoma, gastrinoma), or
MEN 2, which includes hyperparathyroidism, pheochromocytoma,
and medullary thyroid carcinoma.
In malignancy-associated hypercalcemia, production of humoral
bone resorbing factors (i.e., PTH-related protein, or direct bone de-
struction by tumor). Excess vitamin D intake or production.
Treatment Steps
Severe Hypercalcemia
1. Intravenous hydration with 0.9% (normal) saline solution.
2. Intravenous furosemide to promote calciuresis.
76
ENDOCRINOLOGY Pituitary Gland Disorders
d
3. Salmon calcitonin subcutaneously every 6–12 hours for rapid
management lowering of Ca2+ level.
decisions 4. Intravenous bisphosphonates (pamidronate or etidronate) for
prolonged control.
HYPERCALCEMIA 5. Treatment of underlying source.
Primary
Hyperparathyroidism III. PITUITARY GLAND DISORDERS
Surgery indicated if
osteoporosis or kidney A. Anterior Pituitary
stones are present; if Ca2+
> 12 mg/dL, or if patient 1. Overproduction Syndromes
< 50 years of age. Familial
H&P Keys
hypercalcemic
hypocalciuria must be
Galactorrhea, amenorrhea, infertility (prolactinomas); enlargement
excluded prior to surgery. of hands, jaw, feet (acromegaly); moon facies, dorsocervical and
Hypercalcemia of
supraclavicular fat pads, striae (Cushing’s disease). May be a non-
Malignancy functioning adenoma presenting with visual changes, hypogo-
Intravenous hydration and nadism, fatigue, loss of axillary and pubic hair.
furosemide, followed by
salmon calcitonin and Diagnosis
bisphosphonates. Assays of T4, TSH (rule out primary hypothyroidism, which may ele-
Treatment of the underlying vate prolactin levels). Prolactin level (exclude use of medications
malignancy if possible. that act on the central nervous system and elevate prolactin, e.g.,
Vitamin D Intoxication phenothiazines).
Removal of vitamin D Obtain a growth hormone and insulin-like growth factor-1
source if possible, (IGF-1) level (somatomedin C) to evaluate for acromegaly. (See Fig.
hydration, glucocorticoids.
3–3.) Corticotropin (ACTH)-producing tumors can be screened for
with an overnight dexamethasone suppression test (AM cortisol level
Figure 3–3. Facial appearance of a 43-year-old woman with acromegaly whose disease had been present for 15 years. Soft tissue overgrowth about the
eyes, nose, and mouth has resulted in coarsening of the features. Lacrimal overgrowth is evident, as is thickening of the skin folds and the presence of fibroma mol-
luscum (acrochordon). (Reproduced, with permission, from Felig P. Endocrinology and Metabolism, 4th ed. New York: McGraw-Hill, 2001.)
77
Treatment Steps
1. Bromocriptine or cabergoline for prolactinomas and some
growth hormone/prolactin producing tumors. Microprolactino-
mas (< 10 mm) need only medical management.
2. Transsphenoidal surgery for macroprolactinomas with visual
changes, and all other tumors.
3. Postoperative radiation therapy for residual tumor.
4. Octreotide for partially treated/recurrent acromegaly.
2. Underproduction Syndromes
H&P Keys
History of prior pituitary surgery or irradiation, history of sinus irra-
diation, postpartum hemorrhage. Severe headache (acute), inability
to breast-feed, fatigue, amenorrhea, sexual dysfunction, testicular at-
rophy, signs of hypothyroidism, hypotension.
Diagnosis
Measurement of serum T4 and TSH levels. TSH will be low or nor-
mal in face of a low T4 level. Measurement of estrogen or testos-
terone and FSH/LH. Low morning cortisol level, inappropriate
adrenal hormone stimulation with insulin or metyrapone challenge.
Insufficient stimulation of growth hormone with hypoglycemia. MRI
of pituitary with gadolinium showing hemorrhage, necrosis, tumor,
or granulomatous infiltration (sarcoidosis, tuberculosis).
Treatment Steps
1. Hydrocortisone replacement with split dosing morning and
evening, or long-acting glucocorticoid (prednisone, dexametha-
sone) daily.
2. L-thyroxine replacement; cannot follow TSH levels to determine
adequacy of dose, follow free T4.
3. Estrogen replacement (oral or transdermal) or testosterone re-
placement (intramuscular or transdermal).
4. Growth hormone (GH) replacement in children; controversial in
adults.
B. Posterior Pituitary
1. Diabetes Insipidus
H&P Keys
Failure to concentrate urine, hypernatremia with thirst, polydipsia,
polyuria.
78
ENDOCRINOLOGY Adrenal Gland
Diagnosis
Measure volume of fluid intake and urine output, serum elec-
trolytes, urine and serum osmolality; water deprivation test.
Concept and Application
Deficiency of vasopressin caused by brain trauma, neurosurgery, sar-
coidosis, brain tumors (pinealoma, craniopharyngioma), his-
tiocytosis. Nephrogenic diabetes insipidus (no response to vaso-
pressin) should be ruled out.
Treatment Steps
1. Hydration and normalization of electrolytes.
2. Aqueous vasopressin injections or nasal spray desmopressin
(DDAVP). Chlorpropamide in mild cases.
2. Syndrome of Inappropriate Antidiuretic
Hormone Secretion (SIADH), Hyponatremia
H&P Keys
Mental confusion. History of cerebrovascular accident, tumor; use of
chlorpropamide, phenothiazines; malignancies, pulmonary disease.
Diagnosis
Urine hypertonic to plasma. Exclude adrenal insufficiency, diuretic
therapy, nephrosis, cirrhosis, hypothyroidism, compulsive water
drinking.
Concept and Application
Inappropriate release of vasopressin causing an inability to dilute
urine despite hyponatremia, extracellular fluid expanded without
edema.
Treatment Steps
Acute
1. 3% saline solution intravenously until Na+ 125 mEq/L.
2. Intravenous furosemide.
3. Normal saline solution (NSS) if further hydration required.
Chronic
1. Fluid restriction 800–1,200 mL/day.
2. Demeclocycline.
A. Adrenal Insufficiency
H&P Keys
Fatigue, weight loss, anorexia, nausea, vomiting, abdominal pain, hy-
perpigmentation, and volume depletion.
Diagnosis
Electrolyte determination for hyponatremia, hyperkalemia, hypo-
glycemia. Low morning cortisol level. Cosyntropin (Cortrosyn) stim-
ulation test.
Disease Severity
Hypotension or circulatory collapse, hypoglycemia, hyperkalemia,
fever.
79
Acute
1. Intravenous 5% dextrose/NSS.
2. Intravenous hydrocortisone 100 mg, followed by 100 mg every
8 hours.
3. If diagnosis not secure, IV dexamethasone pending cortisol
testing.
Chronic
1. Hydrocortisone split dose morning and evening, or long-
acting glucocorticoid (prednisone, dexamethasone) daily.
2. Florinef started at 0.1 mg daily, dose titrated to normalization
of electrolytes and plasma renin activity.
3. Increased glucocorticoid dosing with febrile illness, surgery,
or trauma.
4. Increase F and NaCl in hot weather.
B. Cushing’s Syndrome
H&P Keys
Central obesity, violaceous striae > 1 cm wide, general and proximal
muscle weakness, easy bruising, dorsocervical and supraclavicular
fatty deposition, moon facies, plethora, menstrual irregularities, hir-
sutism, sexual dysfunction, hyperpigmentation (Fig. 3–4).
Diagnosis
Elevated late afternoon cortisol levels. Baseline 24-hour urinary free-
cortisol elevation. Normal or elevated ACTH level. Dexamethasone
suppression tests: overnight: 1 mg at 11 PM, serum cortisol at 8 AM;
low-dose: 0.5 mg every 6 hours for 48 hours, with 24-hour urine col-
lection for cortisol and 17-hydroxysteroids: high-dose: 2 mg every 6
hours for 48 hours, with 24-hour urine collection for cortisol and 17-
hydroxysteroids. Computed tomographic (CT) scan or MRI of pitu-
itary. CT of chest and abdomen. Petrosal sinus sampling.
Disease Severity
Rapidity of symptom onset, hypokalemia, congestive heart failure,
paper-thin skin, and osteoporosis.
Concept and Application
Overproduction of cortisol: ACTH-producing pituitary or carcinoid
tumor, cortisol-producing adrenal adenoma, and exogenous steroid
use.
Treatment Steps
Acute
1. Removal of source of excess hormone (transsphenoidal hy-
pophysectomy, adrenal adenectomy, removal of carcinoid tu-
mor).
2. Pituitary irradiation for residual or recurrent pituitary ade-
noma.
3. Replacement dose steroids until adrenal pituitary axis re-
sumes function.
Chronic
d
Ketoconazole, aminoglutethimide, metyrapone, or mitotane for
diagnostic nonoperative cases.
decisions
C. Hirsutism
CUSHING’S SYNDROME H&P Keys
Age of onset, rate of progression, family history. Terminal hair
Cushing’s Disease growth in central location (upper lip, chin, neck, chest), cli-
Basophilic adenoma of
toromegaly, male pattern baldness, menstrual irregularities, male
pituitary, normal to slightly
elevated ACTH, elevated body habitus.
serum/urine cortisol.
Suppresses with high-dose Diagnosis
but not low-dose Cosyntropin-stimulated 17-hydroxyprogesterone, and 17-hydroxy-
dexamethasone. Unilateral pregnenolone levels, dehydroepiandrosterone sulfate (DHEAS),
ACTH elevation with testosterone, cortisol levels. Thyroid function tests. CT of abdomen.
petrosal sinus sampling.
Adrenal Adenoma Disease Severity
Elevated urine/serum Rapid onset of hair growth, menstrual irregularities, virilization, pre-
cortisol, suppressed ACTH, pubertal or older age of onset.
adrenal nodule on MRI/CT.
No suppression with high- Concept and Application
dose dexamethasone.
Overproduction of androgens of adrenal or gonadal origin (hyper-
Ectopic ACTH plasia, tumor, exogenous); hypersensitivity of hair follicles to normal
Rapid onset symptoms,
levels of androgens.
elevated cortisol levels and
ACTH levels. No
Treatment Steps
suppression with low-dose
dexamethasone, rarely 1. Removal of androgen source if possible.
suppressed with high dose. 2. Oral contraceptives.
ACTH levels equal 3. Spironolactone or cyperoterone acetate (contraindicated if
bilaterally with petrosal chance of becoming pregnant).
sinus sampling. 4. Replacement hydocortisone if congenital adrenal hyperplasia.
5. Electrolysis, bleaching.
81
Acute
1. Phentolamine or nitroprusside for hypertensive crisis.
2. α-Blockade: phenoxybenzamine (dose adjusted to cessation of
paroxysms and hypertension) for 10–14 days.
3. β-Blockade added only after establishment of α-blockade.
4. Surgery to remove tumor.
Chronic
1. Phenoxybenzamine.
2. β-Blockers.
3. Metyrosine (catecholamine synthesis inhibitor).
H&P Key
Family history of congenital adrenal hyperplasia, dehydration, hy-
potension, and salt wasting (in infants), ambiguous genitalia, viriliza-
tion, and hypertension.
Diagnosis
Elevated levels of adrenal androgens, cortisol precursors, or miner-
alocorticoid precursors (depending on the specific enzyme abnor-
mality).
Disease Severity
Hypotension, salt wasting, failure to thrive in infants; severe genital
ambiguity.
Concept and Application
Complete or partial dysfunction of one of the enzymes used in the
production of cortisol from cholesterol (21-hydroxylase,11β-hydrox-
ylase, 17α-hydroxylase, 3β-hydroxysteroid dehydrogenase, or choles-
terol side-chain cleavage enzyme). May be due to an absolute or rela-
tive absence of the enzyme or abnormal enzyme structure of
function. Backup of enzyme substrates causes the clinical sequelae
associated with each specific enzyme abnormality.
82
ENDOCRINOLOGY Clinical Lipoprotein Disorders
Treatment Steps
Acute
1. Aggressive intravenous fluid repletion.
2. Hydrocortisone 100 mg intravenously every 8 hours.
Chronic (Infants and Children)
1. Assignment of gender.
2. Replacement dose steroids (lowest possible dose to ensure ad-
equate hormone concentrations and minimize growth-retard-
ing potential of steroid therapy).
3. Surgical reconstruction of genitalia toward assigned gender.
Chronic (Adult: Partial Enzyme Blocks)
1. Glucocorticoids to decrease adrenal androgen production if
menstrual irregularities interfere with fertility.
2. Spironolactone for hirsutism (cannot use if pregnancy
planned).
B. Type II Hyperlipoproteinemia
H&P Keys
Premature coronary artery disease (CAD), tuberous and tendinous
xanthomas, corneal arcus.
Diagnosis
Lipoprotein measurements with elevated low-density lipoprotein
(LDL) alone (type IIa pattern) or elevated LDL and triglycerides
(type II b).
Disease Severity
Family history, age of onset of CAD, degree of lipid elevation.
Concept and Application
Three distinct diseases: familial hypercholesterolemia (defect in
LDL receptor), familial combined hyperlipidemia (overproduction
of apoprotein B100), polygenic hypercholesterolemia.
83
D. Type IV Hyperlipoproteinemia
H&P Keys
May be obese, presence of CAD, gallstones.
Diagnosis
Elevated triglycerides, elevated VLDL, decreased high-density
lipoproteins (HDL); rule out renal disease and diabetes with appro-
priate tests.
Disease Severity
Premature CAD.
Concept and Application
Autosomal dominant inheritance as familial hypertriglyceridemia or
familial combined hyperlipidemia.
Treatment Steps
1. Low cholesterol, low saturated fat diet.
2. Weight loss.
3. Alcohol and estrogen avoidance.
4. Gemfibrozil or fenofibrate, niacin.
E. Type V Hyperlipoproteinemia
H&P Keys
Obesity, history of pancreatitis, eruptive xanthomas (Fig. 3–5), hep-
atosplenomegaly.
84
ENDOCRINOLOGY Clinical Lipoprotein Disorders
Diagnosis
Elevated triglycerides, cloudy plasma, increased chylomicrons, and
VLDL.
Disease Severity
History of recurrent pancreatitis and/or diabetes.
Concept and Application
Multiple molecular defects.
Treatment Steps
1. Low-fat diet.
2. Weight loss.
3. Gemfibrozil or fenofibrate, niacin.
U
VI. DIABETES MELLITUS
Diabetic Ketoacidosis
1. Respiratory support.
2. Intravenous NSS 1–2 L first 1–2 hours, then 150–500
mL/hour until hemodynamically stabilized.
3. K+ replacement if serum K+ normal or low.
4. IV insulin by continuous infusion, initial bolus 10 units fol-
lowed by 10 units/hour.
5. Monitor blood glucose levels every hour and adjust insulin
drip to maintain decrease of 75–100 mg/dL per hour.
6. Change fluids to D5NSS when serum glucose 250.
7. Continue drip until anion gap normalized.
Maintenance
1. Insulin: split mixed regimen (NPH/Regular, lispro, or insulin
aspart) before breakfast (two-thirds of total daily dose) and
dinner (one-third of total daily dose). Also can use intermedi-
ate-acting or long-acting insulin 1–2 times daily with lispro or
insulin aspart before each meal. Some patients prefer insulin
pump.
86
ENDOCRINOLOGY Diabetes Mellitus
B. Type 2
d
Adult/maturity onset, noninsulin dependent, nonketosis prone. Ac-
management counts for 90% of diabetes cases.
decisions H&P Keys
Family history, obesity, limited exercise, polyuria, polyphagia, poly-
NEW-ONSET DIABETES dipsia, blurry vision, weakness, confusion, coma. Usually occurs over
MELLITUS age of 40; rapidly increasing prevalence in children and teenagers.
DKA Diagnosis
IV hydration; IV insulin Fasting blood sugar ≥ 126 mg/dL on two occasions; 2-hour post-
continuous infusion;
prandial blood sugar 200 mg/dL; random glucose 200 mg/dL with
electrolyte management;
search for precipitating
symptoms. Exclude secondary causes, e.g., Cushing’s syndrome, hy-
event. perthyroidism, growth hormone excess, steroid use, medications,
HHNK
pancreatitis, cystic fibrosis, and pregnancy. HbA1c > 7% consistent
IV hydration; search for with diagnosis.
precipitating event; IV
insulin not necessary. Disease Severity
Type 2, symptomatic,
Retinopathy, neuropathy, nephropathy/end-stage renal disease, pe-
blood sugars > 250–300 ripheral vascular disease, coronary artery disease. Hyperosmolar hy-
mg/dL perglycemic nonketotic coma (HHNK).
Subcutaneous insulin; meal
planning and exercise. Concept and Application
Consider switch to oral Multiple defects. Patients typically overweight; impaired insulin se-
agents if blood sugar cretion and peripheral insulin resistance; increased glucose produc-
control improves in few tion in the liver and decreased glucose uptake in muscle and adi-
months.
pose tissue.
Type 2, symptomatic,
blood sugars < 250 Treatment Steps
Meal planning and 1. ADA diet. Weight reduction as needed. Regular exercise.
exercise; sulfonylurea or
2. Diabetes education.
metformin, consider
combination therapy if
3. Insulin secretagogues (glipizide, glyburide, repaglinide, etc.); in-
goals not met. sulin sensitizers (metformin, pioglitazone, rosiglitazone); acar-
Type 2, asymptomatic,
bose anhydrase inhibitors; insulin.
blood sugars < 200 4. Control of lipid abnormalities: LDL goal < 100.
Meal planning and 5. Angiotensin-converting enzyme (ACE) inhibitors for hyperten-
exercise. Consider addition sion control or if micro/macro proteinuria present.
of oral agent if blood sugar 6. Yearly dilated ophthalmologic exam.
goals not met.
C. Hyperosmolar Hyperglycemic Nonketotic
Coma (HHNK)
H&P Keys
Usually an elderly patient with infection, myocardial infarction (MI),
or cerebrovascular accident (CVA). Lethargy, confusion, coma.
Diagnosis
Blood sugars often > 1,000 mg/dL. Low bicarbonate due to lactic
acidosis; ketones normal to minimally elevated. Serum osmolality
high. Average fluid deficit is 10 L.
87
D. Hypoglycemia
H&P Keys
Anxiety, sweating, intense hunger, headache, palpitations, blurred
vision, irritability, pallor, nausea, diminished mental acuity, convul-
diagnostic
decisions d
sions, syncope. Can occur fasting or after eating (reactive). Past his-
tory of gastrointestinal surgery, malabsorbtion, hyperparathyroidism, HYPOGLYCEMIA
or pituitary tumor. Medications (insulin, sulfonylureas, pentamidine,
etc.), alcohol consumption. Liver or renal disease. Insulinoma
Whipple’s triad, blood
Diagnosis glucose < 50 mg/dL,
Blood sugars < 45 mg/dL in men and < 35 in women with symptoms elevated C-peptide and
that improve with increase in plasma glucose. insulin levels, negative
Simultaneous measurement of glucose, insulin, C-peptide, and screen for sulfonylureas.
urine sulfonylurea metabolites during an episode of hypoglycemia; Insulin Overdose
may need hospitalization for a 72-hour fast. Insulin antibodies can Whipple’s triad, blood
glucose < 50 mg/dL,
be determined.
suppressed C-peptide
Disease Severity level, elevated insulin level.
Lethargy, coma, seizures, syncope, and death. In diabetes, symptoms Sulfonylurea Induced
may be masked if autonomic neuropathy is present or if the patient Whipple’s triad, blood
glucose < 50 mg/dL,
is on β-blockers.
elevated C-peptide and
insulin levels, positive
Concept and Application
screen for sulfonylureas.
Imbalances between hepatic production and glucose utilization. In-
creased utilization (insulinoma, exogenous insulin, sulfonylureas).
Glucose overutilization by other tumors (sarcoma, fibroma, hep-
atoma, etc.). Diminished glucose production from alcoholism, liver
disease, adrenal or pituitary deficiency.
Treatment Steps
1. Identification and treatment of underlying cause. If decreased
production, frequent meals and snacks. Decrease insulin or sul-
fonylurea dose.
2. If severe (coma, stupor), IV dextrose 50 mL of 50%, the infusion
of 10% glucose to keep plasma glucose > 100 mg/dL.
3. Glucagon injection (1 mg), not effective if hepatic glycogen
stores are depleted.
4. Surgery for insulinoma or nonpancreatic carcinoma.
5. Diazoxide and octreotide in refractory cases.
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ENDOCRINOLOGY Diabetes Mellitus
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Thompson JS. Genectics in Medicine. Philadelphia: W.B. Saunders, 1986.
Wilson J, Foster D, Kronenberg H, Larsen PR (eds.). Williams Textbook of Endocrinology,
10th ed. Philadelphia: W.B. Saunders, 2002.
Diseases and Disorders
of the Digestive System 4
I. ESOPHAGUS / 91
A. Malignant Neoplasms / 91
B. Gastroesophageal Reflux Disease (GERD) / 92
C. Motor Disorders of the Esophagus / 93
II. STOMACH / 95
A. Gastric Cancer / 95
B. Acid-Related Disorders of the Stomach / 96
C. Disorders of Gastric Motility / 98
V. RECTUM / 118
A. Malignant Neoplasm of Rectum / 118
B. Hemorrhoids / 118
C. Anal Fissure / 119
D. Anorectal Abscess / 120
89
Copyright © 2004, 2001 by The McGraw-Hill Companies, Inc. Click here for terms of use.
90 Diseases and Disorders of the Digestive System
BIBLIOGRAPHY / 138
91
A. Malignant Neoplasms
H&P Keys
Symptoms that imply advanced disease include dysphagia, chest
pain, weight loss, regurgitation, pulmonary symptoms, and iron-
deficiency anemia. Endoscopy with biopsy is almost always diagnos-
tic.
Disease Severity (Staging Techniques)
1. Endoscopic ultrasonography (EUS).
2. Chest and abdominal computed tomography (CT) to assess dis-
tant organ involvement.
Concept and Application
Diagnosis
Patients with typical symptoms of GERD (without alarm symptoms)
do not require any specific tests initially. In mild cases, simply giving a
trial of a proton pump inhibitor (PPI) is diagnostic of GERD. En-
doscopy reliably demonstrates the presence or absence of erosive
esophagitis. However, severity of heartburn correlates poorly with
presence or degree of erosive esophagitis. Endoscopy is useful in de-
termining the presence or absence of Barrett’s esophagus. A 24-hour
pH probe and manometry are used if treatment is refractory to phar-
macology or if surgery is contemplated.
Pathophysiology
Incompetence of the lower esophageal sphincter (LES) through in-
appropriate or transient relaxations or through fixed low LES pres-
sure (hypotensive LES). Anatomic disruption of the LES and the
crural diaphragm, as occurs with hiatus hernia where two high pres-
sure areas, the LES and the crural diaphragm with an intervening
low-pressure area, the intrathoracic hiatal hernia, leads to impaired
clearance of gastric acid.
Treatment Steps
1. Initial goals are to relieve symptoms and heal esophagitis.
Lifestyle modifications include reduction of dietary fat, weight re-
duction, smoking cessation, avoidance of large-volume meals and
alcohol, avoidance of tight-fitting clothes, elevation of the head of
bed, remaining upright for 2–3 hours after eating, and avoidance
of reflux-promoting medications.
2. Medications include antacids and antacid/alginate combinations
used for short-term symptom relief; and histamine2 receptor an-
tagonists (H2RAs): cimetidine, ranitidine, famotidine, nizatidine,
available over the counter, are useful for controlling symptoms in
mild grades of GERD. PPIs are effective in relieving symptoms
93
Diagnosis
If symptoms suggest oropharyngeal disorder, video esophagram. Al-
ternatively, a barium swallow can be done.
Esophageal dysphagia: endoscopy to visualize any mucosal or
structural abnormality, define strictures, and obtain biopsies. If no
obstructive lesions are detected, motility disorders enter the differ-
ential diagnosis and esophageal manometry may be helpful (Fig.
4–1).
cinoma that infiltrates the LES, typically gastric cardia (most com-
mon), breast, prostate, and pancreas.
Treatment Steps
1. Pharmacological approaches include calcium channel blockers
and nitrates, which provide transient, short-term relief.
2. Botulinum toxin injected directly into the LES at endoscopy pro-
vides symptomatic improvement, but long-term studies indicate
relapse of symptoms and no improvement in esophageal empty-
ing.
3. Pneumatic dilatation and surgical myotomy are the main thera-
peutic options and are equally effective. A major advance in ther-
apy is thoracoscopic and laparoscopic approaches to myotomy al-
lowing for minimally invasive Heller myotomy.
2. Diffuse Esophageal Spasm (DES)
Patients present with intermittent episodes of chest pain and dyspha-
gia.
Manometric features include episodic or intermittent simultane-
ous, nonpropagated, and high-amplitude contractions separated by
periods of normal esophageal peristalsis. Radiographic features may
be absent, but a “corkscrew” appearance of the esophagus indicates
multiple simultaneous contractions. Radiographic and manometric
features can be simulated by gastroesophageal reflux. Therefore, re-
flux can be considered as one of the causes of “spasm.”
Treatment Steps
Symptomatic: nitrates and calcium channel blockers via sublingual
administration prior to meals.
3. Scleroderma
Esophageal manometry is a useful screening test for detection of vis-
ceral involvement of scleroderma and mixed connective tissue disor-
ders. Visceral and systemic manifestions may not parallel; severe vis-
95
II. STOMACH
A. Gastric Cancer
H&P Keys
Early symptoms are nonspecific: abdominal pain, dyspepsia, weight
loss, early satiety, and anemia. Systemic symptoms suggest advanced
disease.
Diagnosis
Endoscopy with biopsy and cytology. Tumor cells spread through
lymphatic and along vascular pathways to liver, lung, bone, and
brain. Some gastric tumors in women spread intraperitoneally to in-
volve both ovaries (Krukenberg tumors).
Disease Severity (Staging Techniques)
Endoscopic ultrasound is superior to CT for defining the depth of
penetration (T-stage) and perigastric lymph nodes, with a limited
view of the liver. Abdominal CT +/− laparoscopy to evaluate visceral,
peritoneal, liver, and regional and distant lymph nodes (Virchow’s
node, Sister Mary Joseph’s node).
Concepts and Applications
4-1
ENVIRONMENTAL FACTORS ASSOCIATED WITH GASTRIC CANCER
Diet
Nitrites derived from nitrates
Smoked foods
Pickled vegetables
Excessive salt intake
Decreased fresh vegetables and fruits
Infection with H. pylori
Chronic gastritis with intestinal metaplasia
Pernicious anemia
Chronic gastritis with intestinal metaplasia
Subtotal distal gastrectomy (Billroth I, II)
Nonendoscopic
In-office Ab test Whole blood 67–88% 74–91% Initial diagnosis
In-office Ab test Serum 86–94% 75–88% Initial diagnosis
Lab Ab test ELISA 86–94% 78–95% Initial diagnosis
Stool antigen 88–94% 89–92% Initial diagnosis and follow-up
Urea breath 90–96% 88–98% Initial diagnosis and follow-up
Endoscopic
Biopsy urease 88–95% 95–100% Initial diagnosis and follow-up
Histology 90–95% 98–99% Initial diagnosis and follow-up
Culture 60–95% 100% Initial diagnosis and follow-up
Treatment Steps
1. Ulcers unrelated to H. pylori:
• Long-term acid suppression.
• Search for a cause (e.g., NSAIDs, Crohn’s disease, Zollinger–
Ellison syndrome) (Table 4–3).
2. Ulcers related to H. pylori (most ulcer patients have H. pylori infec-
tion):
• All ulcer patients with H. pylori should receive treatment for H.
pylori regardless of whether the ulcer was newly diagnosed or
recurrent.
• H. pylori–infected patients taking NSAIDs should also receive
treatment for H. pylori and, if possible, the NSAIDs are stopped.
• H. pylori treatment is indicated for all infected patients with a
history of duodenal or gastric ulcer, including those with a his-
tory of ulcer complication (bleeding, perforation, obstruction).
3. H. pylori treatment regimes: See Table 4–4.
4-3
ETIOLOGIES OF GASTRIC AND DUODENAL ULCERS
Most common
H. pylori
NSAIDs
Less common
Gastric malignancy
Stress ulceration
Viral infections (HSV-1, CMV)
Uncommon
Zollinger–Ellison syndrome
Drug-induced
Crohn’s disease
Treponema pallidum
Systemic mastocytosis
Idiopathic (non–H. pylori) hypersecretory duodenal ulcer
CMV, cytomegalovirus; HSV-1, herpes simplex virus type 1; NSAIDs, nonsteroidal anti-inflammatory drugs.
98
DISEASES AND DISORDERS OF THE DIGESTIVE SYSTEM Stomach
4-4
FDA-APPROVED COMBINATION REGIMENS FOR H. PYLORI
INFECTION IN PATIENTS WITH PEPTIC ULCER
Outcomes
Mechanical obstruction
Pylorus
Duodenum
Small intestine
Post gastric surgery
Vagotomy/antrectomy
Roux-en-Y
Fundoplication
Metabolic/endocrine
Diabetes mellitus
Hypothyroidism
Hyperthyroidism
Adrenal insufficiency
Smooth muscle disorders
Scleroderma
Hollow visceral myopathy
Post viral gastroparesis
Medications
Anticholinergics
Narcotics
L-Dopa
Progesterone, estrogen
Calcium channel blockers
Chronic mesenteric ischemia
Naturopathic disorders
Parkinson’s disease
Paraneoplastic syndrome
Shy–Drager syndrome
Hollow visceral neuropathy
Idiopathic
Treatment Steps
1. The goal of treatment is to correct the underlying motility disor-
der and provide symptomatic relief. For diabetics, optimize glu-
cose control and correct ketoacidosis and dehydration. In all pa-
tients:
• Avoid medications and dietary factors that may delay gastric
emptying.
• Avoid indigestible solids and fats.
• Gastroparesis diet (Table 4–6).
• If dumping is present, separate liquids and solids.
4-6
NUTRITIONAL MAINTENANCE OF GASTROPARESIS
AND CHRONIC INTESTINAL PSEUDOOBSTRUCTION
Test Comments
Stool culture Not needed in 90% of acute diarrheal illnesses. Although bacterial infection rarely causes chronic
diarrhea, it can be excluded by stool culture, including culture on special media for Aeromonas and
Pleisiomonas, Yersinia, and Campylobacter. Candida in stool may cause acute or chronic diarrhea,
both nosocomial and community acquired, even in immunocompetent individuals.
Fecal leukocytes The presence of white blood cells in the stool suggests an inflammatory diarrhea. This is assessed
by Wright’s stain or during a stool ova and parasite examination. A latex agglutination test for the
neutrophil granule protein lactoferrin may also be useful.
Stool lactoferrin A recently developed latex agglutination test for the neutrophil product lactoferrin is highly sensitive
and specific for the detection of neutrophils in stool.
Stool ova and parasites Positive and negative predictive value is dependent on observer skill. Biopsy may be needed.
Special techniques required to detect cryptosporidia and microporidia.
Fecal ELISA for More sensitive and specific for the detection of Giardia.
Giardia-specific antigen
Normalization of stool weight Common with osmotic diarrhea.
with fasting
Stool weight (g/24 hr) Provides objective data on severity
> 500 common with secretory diarrhea
< 500 common with osmotic diarrhea
Stool electrolytes Use with plasma osmolality to calculate stool osmotic gapa
2(stool [Na+] + [K+]) = Posm common with secretory diarrhea
2(stool [Na+] + [K+]) < Posm common with osmotic diarrhea
Osmotic gap (mOsm/kg) Large (> 125 mOsm/kg) in osmotic diarrhea in which nonelectrolytes account for most of the
osmolality of stool water.b
290 − {[Na] + [K] × 2} Small (< 50 mOsm/Kg) in secretory diarrhea in which electrolytes account for most of the stool
osmolality.
Stool C. difficile toxin Toxin B assay is gold standard, requires tissue culture ELISA-based tests detect toxin A, B, or,
occasionally, both. For highest sensitivity, send three stool samples.
Sigmoidoscopy, or colonoscopy When the differential diagnosis includes ulceration, polyps, tumors, Crohn’s disease, ulcerative
with mucosal biopsy colitis, amebiasis, microscopic colitis, amyloidosis, granulomatous infections, and chronic
schistosomiasis and endoscopic biopsy of the proximal small bowel mucosa. A small bowel follow-
through examination is preferable to an enteroclysis study for the radiographic evaluation of patients
with chronic diarrhea.
Upper endoscopy with Useful if small intestinal malabsorptive disorder, such as Whipple’s disease, intestinal lymphoma,
mucosal biopsy eosinophilic gastroenteritis, and celiac disease. Aspirate of small intestinal contents for quantitative
aerobic and anaerobic bacterial culture is useful if small bowel bacterial overgrowth is suspected.
Computerized tomography Useful in patients with chronic diarrhea when the differential diagnosis includes: chronic pancreatitis
or pancreatic cancer, inflammatory bowel disease, chronic infections, intestinal lymphoma, carcinoid
syndrome.
Stool pH Values of < 5.6 are consistent with carbohydrate malabsorption.
Stool test for cathartics A panel of tests to detect laxative abuse. Usually includes a test for phenolphthalein (pink color
upon alkalization), Mg+2, SO4, and PO4.
Stool for fat Performed during the ingestion of a high-fat diet. The presence of excess stool fat should be
evaluated by means of a Sudan stain or by direct measurement. The presence of excessively large
and numerous fat globules by stain or measured stool fat excretion > 14 g/24 hr suggests
malabsorption or maldigestion. Stool fat concentration of > 8% strongly suggests pancreatic
exocrine insufficiency.
Fecal occult blood testing A positive test result suggests the presence of inflammatory bowel disease, neoplastic diseases, or
celiac sprue or other spruelike syndromes.
aNa and K are the major cations in fecal fluid. To simplify calculation of the contribution of electrolytes to stool amorality, anions are not directly measured, but assumed to equal the
measured cations, hence the doubling of the cation concentration.
bOsmolality (mOsm/kg): Measured osmolality of stool water can vary widely and is generally an artifact of the collection process. Because the gut epithelium cannot maintain an
osmotic gradient, in vivo stool water amorality is arbitrarily defined as 290–300.
B. Celiac Sprue
H&P Keys
Common in persons of western European heritage, where preva-
lence is 1 in 250. Prevalence in relatives of celiac patients is 15%.
Certain disorders are associated with increased prevalence of celiac
4-8
REPLACEMENT OF DIARRHEAL FLUID LOSS
• Replace diarrheal fluid loss with oral standard solutions containing balanced electrolytes, minerals,
and nutrients.
• Several are available on the market.
104
DISEASES AND DISORDERS OF THE DIGESTIVE SYSTEM Small Intestine
Disease Severity
Complications include chronic ulcerative jejunitis, enteropathy-
associated T-cell lymphoma, and refractory sprue.
Treatment Steps
1. Patient education is important, as celiac disease is a lifelong disor-
der.
2. Every patient needs education about the physiology of the disease
and the importance of diet and potential consequences, includ-
ing anemia, osteopenia, and enteropathy-associated T-cell lym-
phoma (EATCL).
3. Refer every celiac patient to a dietitian and to a patient support
group.
4. Primary treatment is a gluten-free diet.
5. Clinical response to gluten-free diet should be apparent within
weeks.
6. EMA levels should fall if gluten is being avoided.
105
IV. COLON
A. Invasive Diarrhea
H&P Keys
Crampy lower abdominal pain, tenesmus, stool bloody or mucoid,
volume < 1 L/day; fecal leukocytes are usually seen. History of prior
administration of antibiotics suggests C. difficile as causative agent.
Systemic symptoms may provide a clue to the diagnosis: Hemolytic
uremic syndrome (hemolytic anemia, uremia, renal failure, and dis-
seminated intravascular coagulation [DIC]) occurs with both
Shigella and enterohemorrhagic E. coli. Reiter’s syndrome (arthritis,
urethritis, and uveitis) occurs after Salmonella, Shigella, Campylobacter,
and Yersinia infections.
Diagnosis
Stool studies for bacterial pathogens, ova, and parasites; stool cyto-
toxin assay for C. difficile. Proctosigmoidoscopy may show erythema,
ulceration, hemorrhage, or pseudomembranes, yellow-white, raised
plaques characteristic of pseudomembranous colitis associated with
C. difficile.
Disease Severity
Fever, tachycardia, volume depletion, leukocytosis, abdominal dis-
tention, guarding, tenderness, decreased bowel signs, signs of tox-
emia; development of peritoneal signs suggests toxic megacolon.
Concept and Application
Invasive organisms cause histologic damage and may also produce
signs and symptoms of systemic infection. Species of Shigella, Salmo-
nella, Campylobacter, Yersinia, Clostridium, and E. histolytica produce in-
vasive diarrhea.
Treatment Steps
1. Avoid barium enema (could increase morbidity).
2. Oral rehydration in mild disease or intravenous hydration for the
more severely ill.
3. Antibiotic therapy with vancomycin or metronidazole for C. diffi-
cile, ciprofloxacin or trimethoprim–sulfamethoxazole for Shigella.
4. Other enteric infections, such as Campylobacter and intestinal Sal-
monella, are self-limited and usually don’t require antibiotics.
5. Antidiarrheals, which may delay clearance of the pathogen,
should be avoided.
4-9
ROME CRITERIA FOR DEFINING IRRITABLE BOWEL SYNDROME
Diagnosis
Identify symptoms complex and the compatible with IBS, associa-
tions of symptoms with factors that produce gut hyperactivity, and
exclusion of organic cause of symptoms.
Evaluation
Use diagnostic tests based on presenting symptoms (diarrhea or con-
stipation). Colonoscopy with mucosal biopsy is used to exclude mi-
croscopic or collagenous colitis on histologic examination. Com-
plete blood count (CBC), laxative screening, stool O&P, and small
bowel radiography. Obtain a detailed dietary history to identify fac-
tors that may aggravate or cause symptoms, especially diarrhea, gas
bloating, or constipation. Dietary factors include lactose, fructose,
sorbitol, carbonated beverages, legumes, other gas-producing foods,
and caffeinated beverages.
Concept and Application
Almost 50% of patients who see American physicians in the United
States for bowel symptoms do not have any organic cause for their
symptoms. A chronic functional gastrointestinal disorder manifested
by abdominal pain and altered bowel habits, which occurs chroni-
cally or recurrently at times of life stress, change in diet, menses, and
emotional tension.
Treatment Steps
1. Establish a good physician–patient relationship.
2. Educate patients about their condition.
3. Emphasize the excellent prognosis and benign nature of the ill-
ness.
4. Employ therapeutic interventions centering on dietary modifica-
tions, pharmacotherapy, and behavioral intervention.
5. Dietary fiber increases stool bulk, either by water retention or by
serving as a substrate for microbial growth in the large intestine.
6. Pharmacological treatment:
• Synthetic opioids such as loperamine and diphenoxylate are ef-
fective in those with diarrhea.
• Smooth-muscle relaxants (anticholinergics and calcium chan-
nel blockers) relieve GI symptoms by inhibiting smooth muscle
contractions.
• Tegaserod is engineered specifically for women with constipa-
tion-component IBS refractory to standard treatment.
• Tricyclic antidepressants in low doses are advocated for chronic
somatic or visceral pain.
107
4-10
ENVIRONMENTAL FACTORS THAT MAY INFLUENCE COLORECTAL CARCINOGENESIS1
4-11
HEREDITARY NONPOLYPOSIS COLORECTAL CANCER
Three or more relatives with colorectal cancer (one must be a first-degree relative
of the other two)
Colorectal cancer involving at least two generations
One or more colorectal cancer cases before age 50 years
Used by permission from Friedman SL, McQuaid KR, Grendell JH. Current Diagnosis & Treatment in
Gastroenterology, 2nd ed. New York: McGraw-Hill, 2003.
109
Age to
Setting Begin Test Interval
Diagnosis
Colonoscopy is the preferred examination for patients with positive
screening tests (FOBT, sigmoidoscopy), asymptomatic iron defi-
ciency anemia, or colonic symptoms. The goal of colonoscopy is to
detect cancer, remove adenomas, and, in the case of ulcerative coli-
tis, to search for dysplastic lesions that might indicate a higher risk.
Disease Severity
Tumor stage is the most important prognostic factor. Determinants
of stage are the depth of penetration through the bowel wall and the
presence and number of lymph nodes pathologically. Method of
staging includes endoscopic ultrasound. It is the staging procedure
of choice for rectal cancers to assess depth of invasion and lymph
node metastasis. Surgical staging correlates with 5-year survival
(Table 4–13). Five-year survival for node-negative patients varies
from 85–95% if the tumor has not penetrated the muscularis pro-
pria; and 60–80% if the tumor has penetrated the muscularis pro-
pria. With positive nodes, 5-year survival is < 60%.
4-13
SURGICAL STAGE AND SURVIVAL
Survival (%)
TNM Stage AJCC Stage Dukes’ Stage Five-Year
Disease Severity
Inability to function in environment because of inability to detect
crucial odors, loss of appetite, malnutrition secondary to loss of ap-
petite, psychic trauma because of loss of sense of taste and smell.
Treatment Steps
1. Use of topical steroids for inflammatory process, removal of ob-
structive lesions of nose and nasal vault.
2. Removal from environment containing noxious and polluting
substances.
3. Treatment of infectious processes when appropriate.
4. Return of olfactory function may take from 3 weeks to 18
months.
Diagnosis
Physical examination, determination of febrile state.
Disease Severity
Degree of nasal obstruction, ear discomfort, throat pain, dysphagia,
musculoskeletal symptoms.
Treatment Steps
1. In acute phase, nasal and oral decongestants, steam or cool-mist
vaporization, antihistamines, antipyretics, and anti-inflammatory
548
OTOLARYNGOLOGY AND RESPIRATORY SYSTEM DISEASES Diseases of the Respiratory System
I. Wegener’s Granulomatosis
H&P Keys
Lesion of upper respiratory tract may involve ear, nose, sinus, soft
d
palate, hard palate, tongue, and larynx—most often close to mid-
management line. Patients generally have systemic symptoms, including cough,
decisions and often renal symptoms.
Diagnosis
RECOMMENDED MEDICAL CT scans of sinuses and ear to determine presence of lesion, biopsy
TREATMENT FOR of specific lesions showing Wegener’s granulomas, chest x-ray (CXR)
WEGENER’S
GRANULOMATOSIS
to determine presence of vasculitis. Biopsies of pulmonary lesions
and renal biopsies also are indicated.
Nonsystemic Localized
Disease Severity
Disease
Upper airway: nasal
Wegener’s granulomatosis may progress rapidly and may involve the
steroids, saline ear, with both facial and auditory nerve involvement; may involve
irrigations, antibiotics for the sinuses and eyes, with changes in vision; and may involve the up-
superimposed bacterial per airway, with airway compromise.
infections
Lower airway: systemic Concept and Application
antibiotic therapy, Wegener’s granulomatosis is a disease of unknown etiology mani-
systemic steroid therapy fested by the involvement of upper respiratory, pulmonary, and re-
Systemic Disease nal systems. Biopsies show classic granulomas and vasculitis.
Cyclophosphamide therapy
Steroid therapy Treatment Steps
Systemic antibiotic therapy Use of cyclophosphamide and steroids in combination for long-term
and supportive systemic therapy.
J. Cystic Fibrosis
H&P Keys
Chronic recurrent upper and lower respiratory dysfunction with
nasal obstruction, nasal purulence, loss of pulmonary function, dysp-
nea, chronic cough, production of purulent secretions with cough.
Examination reveals debilitated child or adolescent; watery nasal
polypoid tissue can be seen intranasally, often extending into the na-
sopharynx.
Diagnosis
Direct examination shows multiple polypoid changes in young chil-
dren; CT scans show polypoid and polycystic changes in all sinuses.
Sweat colloid study and CXR.
Disease Severity
The degree of nasal obstruction and purulence and their impact on
patient’s pulmonary status with increased dyspnea, cough, cyanosis,
and recurrent infection.
551
Copyright © 2004, 2001 by The McGraw-Hill Companies, Inc. Click here for terms of use.
552
INDEX
INDEX
Central nervous system (CNS), Cholecystitis, acute, 121–122, 517 intestinal obstruction, 115–116
infectious diseases of, Choledochal cyst, 517–518 invasive diarrhea, 105
259–264 Choledocholithiasis, 122–123, 518 irritable bowel syndrome (IBS),
abscesses, 263 Cholelithiasis, 122 105–107
meningitis, 260–263 Chorionic villus sampling, 308 Rome criteria for defining, 106
aseptic, 260–261 Chronic lymphocytic leukemia (CLL), ischemic bowel disease, 115
bacterial (septic), 261–262 151–152 peritonitis, 117–118
fungal, 262 Chronic myelogenous leukemia Colonic aganglionosis (Hirschsprung’s
tuberculous, 262–263 (CML), 151 disease), 376–377
spirochetes, 263–264 Chronic obstructive pulmonary Colorectal carcinoma (CRC), 107–110
Lyme disease, 263–264 diseases, 429–430 familial adenomatosis polyposis
viruses, 259–260 bronchiectasis, 430 (FAP), 107
herpes simplex virus (HSV), chronic bronchitis, 429 hereditary nonpolyposis colorectal
259–260 cystic fibrosis (CF), 136–137, 430 cancer (HNPCC), 107–108
human immunodeficiency virus emphysema, 429–430 risk factors for, 108
(HIV), 259 Churg–Strauss syndrome, 439 screening guidelines, 109
Cerebrovascular accident (CVA), 159 Chvostek’s sign, 475 surgical stage and survival, 109
Cerebrovascular disorders, 271–275 Chylomicronemia, type I familial, 82 Compartment syndrome, 255
cardioembolic strokes, 273–274 Cirrhosis, 131–133 Concussion, 184–185
intracerebral hemorrhage, 274 Cleft lip or palate, 373 Conduct disorder, 402–403
ischemic thrombotic strokes, Clonorchis, 135 Condyloma acuminatum (genital
271–273 Clostridium botulinum, 357 wart), 43
subarachnoid hemorrhage, 274–275 Clostridium difficile, 100, 103, 105, 113 Congenital adrenal hyperplasia, 81–82
Cerumen (earwax) impaction, Clostridium perfringens, 102 Congenital dislocation of the hip
532–533 Coagulopathies, 159 (CDH), 234
Cervix, 292–294, 316–317 Coarctation of the aorta, 24 Congestive heart failure (cardiogenic
abnormal Pap smear, management Cocaine poisoning, 194, 196, 276 pulmonary edema), 426
of, 294 Coccidioides immitis, 426 Conjunctivitis, 168–169, 331–333, 334
cancer of, 211, 292–293 Coccidioidomycosis, 211, 426 associated systemic diseases in, 333
cervicitis and sexually transmitted Cogan’s syndrome, 349, 533 management of, 334
diseases (STDs), 293–294 Coin lesion (solitary pulmonary signs of, 332
dysplasia, 294 nodule), 444 symptoms of, 331
incompetent, 316–317 Collagen vascular disease, 65 Constipation, 117
Chalazion, 335 Colles’ fracture, 198 Contact dermatitis, 47, 175–176
Chancroid, 220, 221, 293, 294 Colon, 105–118 Contraception, general counseling for,
Charcot’s joint arthropathy, 233, 234 appendicitis, 110–111 304
Chest and abdominal injury, 186–189 benign neoplasm of, 511–512 Contusions, 249. 282
epidemiology and prevention of, 189 colorectal carcinoma (CRC), Conversion disorder, 400
flail chest, 188 107–110, 512–513 Cor pulmonale, 439–440
hemothorax, 187–188 familial adenomatosis polyposis Cornea
pelvic fractures, 189 (FAP), 107 corneal abrasion, 186
perforation of viscus, 188 hereditary nonpolyposis corneal laceration, 186
pneumothorax, 187 colorectal cancer (HNPCC), disorders of, 347–349
rib fracture, 186–187 107–108 corneal ulcer, 332, 347–348
Chest pain, 442–443 risk factors for, 108 herpes simplex keratitis, 348
Chickenpox (varicella), 40, 223, screening guidelines, 109 interstitial keratitis, 349
369–370 surgical stage and survival, 109 ophthalmic herpes zoster,
Child abuse, 203, 206 constipation, 117 348–349
Child–Turcotte–Pugh scale, 132 diverticulitis, 116–117 Corynebacterium diphtheriae, 541
Child’s classification in liver failure, familial Mediterranean fever Cough, 441–442
506 (recurrent polyserositis), 118 Cowden’s syndrome (multiple
Chlamydia, 219, 293, 294, 452 inflammatory bowel disease (IBD), hamartoma syndrome), 67
Chlamydia trachomatis, 218, 219, 221, 111–115 Cradle cap, 46
222, 293, 303, 310, 321, 451 Crohn’s disease, 113–115, Cranial injury, 183–186, 491–492
Chloracne, 55 507–508 auditory injury, 186
Cholangitis, acute suppurative, ulcerative colitis (UC), 111–113, concussion, 184–185
122–123 507 epidural hematoma, 185
554
INDEX
INDEX
Erectile impotence, 456–457 Fractures, 183–184, 186–187, 189, German measles (rubella), 223, 371
Erythema chronicum migrans (“bull’s- 197–199, 249–256 congenital, 372
eye” rash), 230, 263 and dislocations, 249–255 Giant cell arteritis, 32
Erythema multiforme, 49, 52–53 cervical spine, 249–250 Giardia lamblia, 102, 103
Erythema nodosum, 53, 65 extremities, 197–199 Glasgow Coma Scale, 491
Erythema toxicum neonatorum, 68 Colles’, 198 Glaucoma, 331, 343, 349–351, 359
Erythrocytosis (polycythemia), femur, 198 acute angle-closure, 332, 349–351
156–157 fibula, 197–198 congenital, 359
Escherichia coli, 101, 102, 105, 303, humerus, 199 open-angle, 343, 349–351
449 radius, 198 Glioblastoma, 276–277
Esophagus, 91–95 tibia, 197 Glomerulonephritis, 467–468
Barrett’s esophagus, 91 ulna, 198–199 Glucagonoma syndrome, 68
gastroesophageal reflux disease facial (frontal bone, mandible, Gonadal dysgenesis 45,XO (Turner’s
(GERD), 91–92 maxilla, orbits, or nose), syndrome), 378
malignant neoplasms, 91–92 183–184 Gonococcal tenosynovitis, 230
motor disorders of, 93–95 femur, neck of, 252–253 Gonorrhea, 218–219, 293, 294,
achalasia, 93–94 foot and leg, 253 451–452
diffuse esophageal spasm (DES), hand phalanges, closed fracture of, Goodpasture’s syndrome, 439
94 251–252 Gout, 237
scleroderma, 94–95 lumbar spine, 251 Graves’ disease, 72, 74
varices, 505–506 open, 255 Grey Turner’s sign, 134
Essential thrombocythemia, 161 pelvic, 189 Guillain–Barré syndrome (acute
Eye, diseases of, 347 rib, 186–187 inflammatory demyelinating
Eyelids, disorders of, 333–337 skull, 184 polyneuropathy), 265
blepharitis, 333–334 Smith’s, 198 Gunshot wounds, 206
blepharoptosis, 336–337 thoracic spine, 250–251 Gynecologic examination and
chalazion, 335 vascular compromise following, 255 screening, 303–304
ectropion, 335–336 vertebral column, 197
entropion, 335 Fragile X syndrome, 378
epicanthus, 336 French–American–British (FAB) H
stye, 334 classification, 149–150 Haemophilus ducreyi, 221, 293
Frontal bone fracture, 183–184 Haemophilus influenzae, 42, 229, 261,
Frostbite, 203 368. 369, 419, 541
F Frozen shoulder, 245 pneumonia, 421–422
Facial fracture, 183–184 Fungal infections, 41 Haemophilus parainfluenzae, 541
Factitious disorder, 400, 401 Hair and hair follicle disorders, 55–56
Falls, 206–207 alopecia areata, 56
Familial adenomatosis polyposis (FAP), G chloracne, 55
107 Galactorrhea, 76 rosacea, 55–56
Family planning, 304 Gallbladder, 121–123 Hashimoto’s thyroiditis, 73, 74, 85
Febrile seizures, 357, 366 acute cholecystitis, 121–122 Head injury, 205
Felty’s syndrome, 162 carcinoma of, 518–519 Headaches, 270–271
Femur fractures, 198 choledocholithiasis and acute Hearing loss, 186, 534–537
Fibroadenoma, 300 suppurative cholangitis, noise-induced, 186
Fibrocystic breast disease, 301 122–123 sudden, 536–537
Fibromyalgia, 243 cholelithiasis, 122 Heart disease, congenital, 22–24
Fibula fractures, 197–198 Gardner’s syndrome, 59, 67 atrial septal defect (ASD), 23
FIGO staging Gastric volvulus, 506–507 coarctation of the aorta, 24
1985, 292 Gastrinoma, 75, 522–523 tetralogy of Fallot, 23–24
1988, 289, 295 Gastroesophageal reflux disease ventricular septal defect (VSD),
Fire prevention, 206 (GERD), 91–92 22–23
Flail chest, 188, 492 Gastroparesis diet, 99 Heart failure (HF), 6–9, 36
Folic acid deficiency, 143–144, 147 Generalized anxiety disorder, 394 left-sided, 6–7, 36
Foreign bodies, 190–191 Genital herpes, 222, 293, 294 high output, 7, 36
aspiration, 190–191 Genital ulcer disease, 220 low output, 6–7, 36
eye, ear, and nose, 190 Genital wart (condyloma right-sided, 8–9, 36
swallowed, 191 acuminatum), 43 Heatstroke, 204
556
INDEX
Heavy metal poisoning, 195, 196 Hoarseness, 540–541 Hypotension and acute circulatory
Helicobacter pylori, 91, 95, 96, 97, 98 Hodgkin’s disease, 152–153, 210 collape (shock), 9–11
HELLP syndrome, 314 Ann Arbor staging of, 153 cardiogenic shock, 9–10
Hemangioma, 59–60, 480–481 Hordeolum, 334 distributive shock, 11
Hemochromatosis, 126, 128, 131 Howell–Jolly bodies, 104, 148 hypovolemic shock, 10
Hemophilia A and B, 158 Human immunodeficiency virus obstructive shock, 10–11
Hemolytic anemia, 147 (HIV), 159, 210–218, 259, Hypothermia, 203–204
Hemolytic–uremic syndrome, 295, 371–372 Hypothyroidism, 73–74
congenital, 373 congenital, 371–372
Hemoptysis, 443 elements to follow in, 213
Hemorrhage, exsanguinating, 492 revised classification for, 211 I
Hemorrhoids, 118–119, 513 wasting syndrome caused by, 211 Idiopathic pulmonary fibrosis, 431–432
Hemothorax, 187–188 Human papillomavirus (HPV), 43, 91, Idiopathic thrombocytopenic purpura
Henoch–Schönlein purpura, 360 292, 293, 294 (ITP), 160–161
Hepatic adenoma, 519 Humerus fracture, 199 Immunodeficiency, 171–172
Hepatic fibrosis, 131–133 Huntington’s disease, 280–281 cellular (T-cell), 172
Hepatitis, 123–129 Hutchinson’s sign, 348 humoral, 171–172
alcohol-induced injury, 125, 128 Hutchinson’s teeth, 349, 371 Imperforate anus, 516
alcoholic, 124 Hyaline membrane disease (newborn Impetigo, 42–43
autoimmune, 124–129 respiratory distress Impotence, erectile, 456–457
chronic, 124 syndrome), 437 Incompetent cervix, 316–317
clinical situations/syndromes Hydrocele, 455–456 Infantile botulism, 357
associated with HBV, 124 Hydrocephalus, 359 Infantile polycystic disease, 362
drug-induced hepatotoxicity, 124 Hyperbilirubinemia, neonatal, 366 Infections, congenital (STORCH),
industrial and environmental toxic Hypercalcemia, 75–76, 475 371–373
causes of, 26 Hypercoagulable states, 159–160 cytomegalovirus (CMV), 372
viral, 123, 125, 127 Hyperemesis gravidarum, 321–322 hemolytic–uremic syndrome, 373
Hereditary angioedema, 172–173, 175 Hypereosinophilic syndrome, herpes simplex virus (HSV),
Hereditary nonpolyposis colorectal 178–179 372–373
cancer (HNPCC), 107–108 Hyperkalemia, 472 human immunodeficiency virus
Hernia, 523–524 Hyperlipoproteinemia, 27–28 (HIV), 371–372
femoral, 523 type II, 82–83 rubella (German measles), 372
incisional, 524 type IV, 83 syphilis, 371
inguinal, 523 type V, 83–84 toxoplasmosis, 371
with obstruction, 524 Hypernatremia, 471–472 Infertility, 301–303, 455
umbilical, 524 Hyperosmolar hyperglycemic Inflammatory bowel disease (IBD),
Herpes gestationis, 50 nonketotic coma (HHNK), 111–115
Herpes simplex keratitis, 348 86–87 Crohn’s disease, 113–115
Herpes simplex virus (HSV), 44, 211, Hyperparathyroidism, 75, 486–487 ulcerative colitis (UC), 111–113
217–218, 220, 259–260, 371, Hypersensitivity angiitis, 32 Influenza pneumonia, 423–424
372–373 Hypersensitivity pneumonitis, 177–178, Infrainguinal aneurysmal disease, 502
congenital, 371, 372–373 433–434 Insect bites, 201–202
of the oral cavity, 538–539 Hypertension, 25–27 Insulin types, 85
type 2 (genital herpes), 222, 293, essential, 25–26 Insulinoma, 75
294, 452–453 malignant, 27 Interferon, side effects of, 128
Herpes zoster, ophthalmic, 348–349 secondary, 26–27 Intersex, 454
High-density lipoprotein, decreased, Hyperthyroidism, 72–73 Interstitial keratitis, 349
84 Hyphema, 186 Intestinal obstruction, 115–116
Hip disorders, congenital, 234 Hypocalcemia, 475 Intoeing, 236
Hirschsprung’s disease (colonic Hypochondriasis, 401 Intoxication, 408
aganglionosis), 376–377, 516 Hypoglycemia, 87 Intra-aortic balloon counterpulsation
Histoplasma, 210 Hypokalemia, 472 (IABP), 4
Histoplasma capsulatum, 425 Hypomagnesemia, 474–475 Intracerebral hemorrhage, 274
Histoplasmosis, 211, 424–425 Hyponatremia, 78, 471 Intussusception, 375
HIV. See Human immunodeficiency Hypopharynx, malignant neoplasms Iridocyclitis, 332
virus of, 540 Iron deficiency anemia, 143, 147
Hives (urticaria), 48–49, 170–171, 175 Hypospadias, 361, 457 Iron poisoning, 196
557
INDEX
Irritable bowel syndrome (IBS), Laryngomalacia (congenital laryngeal Lp(a) lipoprotein, elevated, 84–85
105–107 stridor), 378–379 Lumbar disk disease, 232–233
Rome criteria for defining, 106 Laryngotracheitis (croup), 368, 369, Lupus arthritis, 238–239, 255
Ischemia, silent, 6 419 Lupus erythematosus, 63–64
Ischemic bowel disease, 115 Larynx, cancer of, 561–562 Lupus nephritis, 471
Ischemic heart disease, 3–6 Latrodectus mactans (black widow Lyme disease, 224, 230, 263–264
acute, 3–5 spider), 202 Lymphogranuloma venereum (LGV),
myocardial infarction, 4–5 Legionnaire’s disease, 422–423 220, 293, 294
spasm (Prinzmetal’s or variant Legionella, 103, 135, 422 Lymphoma, 210, 211
angina), 5 Legg–Calvé–Perthes disease, 235 Burkitt’s, 154, 211
unstable angina, 3–4 Leiomyomata uteri, 289–290 intermediate- or high-grade,
chronic, 5–6 Lens, disorders of, 349–353 154–155
silent ischemia, 6 cataracts, 352–353, 360 low-grade, 153–154
stable angina pectoris, 5–6 classification of, 352
Isoimmunization, 319 congenital, 360
Isospora belli, 103 glaucoma, 331, 343, 349–351, 359 M
Isosporiasis, 211 acute angle-closure, 332, 349–351 Major (unipolar) depression, 390–391
Ixodes dammini (deer tick), 224, 230, congenital, 359 Malignant melanoma, 58–59
264 open-angle, 343, 349–351 Malingering, 400, 401
Leptospirosis, 135 Mandible fracture, 183–184
Leser–Trélat, sign of, 67 Mania, 386–387
J Leukemia Maxilla fracture, 183–184
Jarisch–Herxheimer reaction, 45 acute lymphocytic (ALL), 150–151 Measles (rubeola), 223
Joint, effusion of, 247–248 acute myelogenous (AML), 149–150 Meconium aspiration, 365
Joint infections, 255 chronic lymphocytic (CLL), 151–152 Mees’ lines, 195
chronic myelogenous (CML), 151 Melanoma, 482
French–American–British (FAB) Ménière’s disease, 533
classification of, 149–150 Meningioma, 277
K Lipoma, 59, 480 Meningitis, 260–263, 370–371
Kaposi’s sarcoma, 96, 210, 211 Lipoprotein abnormality types, 28 aseptic, 260–261
Kaposi’s varicelliform eruption, 48 Listeria monocytogenes, 371 bacterial (septic), 261–262, 370–371
Kawasaki disease, 361, 366 Livedo reticularis, 64 fungal, 262
Keratitis, 331 Liver, 123–133, 517–521 tuberculous, 262–263
Keratoderma, acquired (tylosis), 61, focal nodular hyperplasia, 519 Menopause, 298–299
67, 91 hepatic adenoma, 519 Menstrual disorders, 296–298
Keratoderma climacterium, 61 hepatic fibrosis and cirrhosis, amenorrhea, 297–298
Keratoconjunctivitis sicca (dry-eye 131–133 dysmenorrhea, 296–297
syndrome), 337 hepatitis, 123–129 premenstrual syndrome (PMS),
Kidney disease, multicystic, 362–363 alcohol-induced injury, 125, 128 297
Kimmelstiel–Wilson lesions, 467 alcoholic, 124 Metabolic acidosis, 473
Klebsiella pneumoniae, 422 autoimmune, 124–129 Metabolic alkalosis, 473
Klinefelter’s syndrome, 378 chronic, 123–124 Metabolic and nutritional disorders,
Korsakoff’s psychosis, 267 clinical situations/syndromes 236–238, 267–268
Korsakoff’s syndrome, 408 associated with HBV, 124 gout, 237
Krukenberg tumor, 95 drug-induced hepatotoxicity, 124 metabolic encephalopathy, 268
Kussmaul’s respirations, 85 industrial and environmental osteoporosis, 236–237
Kussmaul’s sign, 19 toxic causes of, 26 rickets, 237–238
Kyphosis, 380 viral, 123, 125, 127 thiamine deficiency, 267–268
hepatocellular carcinoma, 129 vitamin B12 deficiency, 267
metastasis, 520–521 Metabolic encephalopathy, 268
L primary hepatobiliary cancer, Metacarpal phalangeal (MCP) sprain,
Lacerations, 189–190 519–520 200
Lacrimal system, disorders of, 337–338 transplantation, 133 Metastases
acute dacryocystitis, 338 “Looser’s lines,” 237 neurologic, 277–278
undersecretion, 337 Low back pain, 232 to bone, 243–244
Lactation, 308–309 Loxosceles reclusa (brown recluse Metastatic malignant tumors,
Large-bowel obstruction, 510–511 spider), 202 pulmonary, 441
558
INDEX
INDEX
Papillary necrosis, 469 odd, eccentric, 398 pneumococcal, 421
Papilledema, 339 paranoid, 398 recurrrent, 211
Papillitis, 339–341 schizoid, 398 staphylococcal, 421
Parathyroid gland disorders, 75–76 schizotypal, 398 Pneumonitis, hypersensitivity, 177–178
Parkinson’s disease, 280 Pertussis, 420 Pneumothorax, 187, 435, 492
Paroxysmal disorders, 268–271 Peutz–Jeghers syndrome, 67 open, 492
headaches, 270–271 Peyronie’s disease, 456 tension, 492
seizures, 268–269 Pharyngitis, 370, 541 Poisoning, 192–196. See also Toxic
trigeminal neuralgia, 269–270 Pheochromocytoma, 75, 81, 487–488 disorders
Parrot’s pseudoparalysis (periostitis), Phobias, 396–397 acetaminophen, 192–193, 196
371 Phycomycosis, 427 alcohol, 194
Parvovirus B19, 148 Pilonidal cyst, 514 aspirin, 196
PCP (phencyclidine) poisoning, 194, Pilonidal disease, 121 barbiturates, 196
196 Pituitary gland disorders, 76–78 carbon monoxide, 195
Pediculosis, 54 anterior pituitary, 76–77 cocaine, 194, 196
Pelvic fractures, 189 overproduction syndromes, 76–77 diethyl-m-toluamide (DEET),
Pelvic inflammatory disease (PID), underproduction syndromes, 77 195–196
221, 303 posterior pituitary, 77–78 drugs visible on x-ray, 196
Pelvic pain, chronic, 303 diabetes insipidus, 77–78 heavy metals and arsenic, 195, 196
Pelvic relaxation/urinary syndrome of inappropriate ingestion of poisonous and toxic
incontinence, 296 antidiuretic hormone agents, 205–206
Pemphigus, 50–51 secretion (SIADH), iron, 196
Penile carcinoma, 464 hyponatremia, 78 management overview, 196
Peptostreptococcus, 303 Pityrosporum ovale, 47, 334 narcotics, 196
Percutaneous transluminal coronary Placenta previa, 312–313, 326 PCP (phencyclidine), 194, 196
angioplasty (PTCA), 4 Platelet dysfunction, 162 sedatives, 193, 196
Perforation of viscus, 188 Pleural effusion, 434–435 selected antidotes, 196
Pericardial diseases, 17–19 Pleurisy (pleuritis), 435 solvent sniffing, 195
acute pericarditis, 17–18 Plummer–Vinson syndrome, 91 stimulants, 193–194, 196
cardiac tamponade, 18–19 Pneumococcal pneumonia, 421 symptoms at a glance, 196
constrictive pericarditis, 19 Pneumoconiosis, 432–434 theophylline, 196
pericardial effusion, 18 asbestosis, 432–433 tricyclic antidepressants, 193
Pericardial effusion, 18 coal workers’, 433 Polyarteritis nodosa, 32
Pericardial tamponade, 188 hypersensitivity pneumonitis, Polycystic disease, infantile, 362
Pericarditis 177–178, 433–434 Polycystic kidney disease, 465, 469–470
acute, 17–18 silicosis, 423 Polycystic ovarian disease (PCOD), 298
constrictive, 19 Pneumocystis jiroveci (formerly carinii) Polycythemia (erythrocytosis), 156–157
Peripheral arterial vascular disease, pneumonia, 210, 211, Polymyalgia rheumatica, 238, 255
30–32, 497–498 212–213, 423 Polymyositis–dermatomyositis, 239–240
acute arterial occlusion, 31–32 Pneumonia, 495 Porphyria cutanea tarda, 66–67
chronic atherosclerotic occlusion, atypical, 422–424 Port-wine stain (nevus flammeus), 68
30–31 influenza, 423–424 Posterior urethral valves, 361–362
vasculitis syndromes, 32 Legionnaire’s disease, 422–423 Postherpetic neuralgia, 41
Perirectal abscess, 514 Mycoplasma pneumoniae infection, Postnasal drip, 441
Peritonitis, 117–118 423 Postoperative infections, 494–495
Pernicious anemia, 96 Pneumocystis jiroveci (formerly Postpartum hemorrhage, 323
Personality disorders (Axis II), carinii), 210, 211, 212–213, Postpartum sepsis, 323–324
397–399 423 Posttraumatic stress disorder (PTSD),
anxious, fearful, inhibited, 398 fungal, 424–427 395–396
avoidant, 398 blastomycosis, 425–426 Preeclampsia, 314–315, 326, 469–470
dependent, 398 coccidioidomycosis, 211, 426 Pregnancy
obsessive–compulsive, 398 cryptococcosis, 426–427 complicated, 310–326
dramatic, emotional, 398 histoplasmosis, 424–425 abnormalities of labor, dystocia,
antisocial (ASP), 398 invasive aspergillosis, 427 322–323
borderline, 398 phycomycosis, 427 abruptio placentae, 313–314, 326
histrionic, 398 gram-negative bacillary, 422 adolescent pregnancy, 310
narcissistic, 398 Haemophilus influenzae, 421–422 asthma, 325–326
560
INDEX
INDEX
Shoulder–hand (frozen shoulder) Sprains and dislocations, 199–201 alcohol-induced persisting
syndrome, 245 ankle, 200–201 dementia, 409
Sick sinus syndrome, 14 elbow dislocation, 201 alcoholic encephalopathy
Sickle cell anemia, 148–149 hand, 199–200 (Wernicke’s
Silent ischemia, 6 distal interphalangeal (DIP) encephalopathy), 408
Silicosis, 423 sprain, 199–200 drug dependence, 409–411
Sinus tachycardia, 15 metacarpal phalangeal (MCP) anabolic steroid abuse, 411
Sinusitis, 542–544 sprain, 200 caffeine, 411
acute, 542 proximal interphalangeal (PIP) cannabinoids, 410
chronic, 543 sprain, 200 hallucinogens, 410
fungal, 543–544 shoulder dislocation or injury, 201 inhalants, 411
Sitophilus granarius, 177 Squamous cell carcinoma, 57, 481–482 nicotine, 411
Sjögren’s syndrome, 337 Stab wounds, 206 opioids, 409
Skin cancers, 57 Staphylococcal pneumonia, 421 phencyclidine (PCP), 410–411
Skin, inflammatory conditions of, Staphylococcus, 42, 43, 148, 430 sedative-hypnotics, 410
45–50 Staphylococcus aureus, 48, 102, 174, 229, stimulants, 409
dermatitis, 46–48 308, 334, 421 intoxication, 408
atopic, 48 Staphylococcus epidermidis, 334 Sudden infant death syndrome (SIDS),
contact, 47 Stasis ulceration, 61 25, 416
seborrheic, 46–47 Status epilepticus, 269 Suicide risk assessment, 391
drug reactions, 49–50 “Steakhouse syndrome,” 93 Supraglottitis (epiglottitis), 368, 369,
psoriasis, 45–46 Sterilization, 304 419
urticaria (hives), 48–49 Stevens–Johnson syndrome, 49, 52, 335 Sweet’s syndrome, 67
Skull trauma or fracture, 184 Stimulant poisoning, 193–194, 196 Syndrome of inappropriate
Sleep disorders, 284–285 Stomach, 95–100 antidiuretic hormone
narcolepsy, 284–285 acid-related disorders of, 96–98 secretion (SIADH), 78
sleep apnea, 284, 444–445 disorders of gastric motility, 98–100 Syphilis, 210, 219–220, 293, 294, 371,
Slipped capital femoral epiphysis, 235, gastric cancer, 95–96, 506 452
380 carcinoid tumors of the stomach, congenital, 371
Small intestine, 100–105 96 secondary, 45
acute enteric infections, 100–103 environmental factors associated
benign neoplasm of, 511 with, 96
celiac sprue, 103–105 Kaposi’s sarcoma, 96 T
malrotation of, 374, 515–516 lymphomas, 95–96 Tachyarrhythmias, 15–16
obstruction, 509 gastric volvulus, 506–507 Takayasu’s arteritis, 32
Smith’s fracture, 198 Strabismus, 344–345 Tardive dyskinesia, 275
Snake bites, 201–202 Strep throat (acute tonsillitis, Temporal arteritis, 32
Solar keratosis (actinic keratosis), pharyngitis), 541 Tendinitis, 242–243
57 Streptococcus, 42, 229, 370 Tenosynovitis, gonococcal, 230
Solitary pulmonary nodule (coin Streptococcus pneumoniae, 368, 371, 421, Tension pneumothorax, 492
lesion), 444 541 Terry’s nails, 68
Solvent sniffing, 195 Streptococcus pyogenes, 541 Testicular carcinoma, 464, 488
Somatoform disorders, 399–401 Stridor, 443–444 Testicular torsion, 454
body dysmorphic disorder, 401 Strongyloides stercoralis, 102 Tetralogy of Fallot, 23–24
conversion disorder, 400 Stroke, 271–274, 495 Theophylline poisoning, 196
factitious disorders, 400, 401 cardioembolic, 273–274 Thermal injuries, 203–204, 206
hypochondriasis, 401 ischemic thrombotic, 271–273 frostbite, 203
malingering, 400, 401 Sturge–Weber syndrome, 68, 359 heatstroke, 204
pain disorder, 399–400 Stye, 334 hypothermia, 203–204
psychosomatic disorders, 401 Subarachnoid hemorrhage, 274–275 Thiamine deficiency, 267–268
somatization disorder, 399 Subdural hematoma, 185, 281–282 Thoracic aorta, aneurysms of, 498–499
Spina bifida, 358 Substance-related disorders, 405–411 Thoracic outlet syndrome (TOS),
Spinal cord injury, 205 alcohol dependence, 407 501
Spinal stenosis, 248–249 alcohol withdrawal syndromes, 408 Thromboangiitis obliterans, 32
Spontaneous abortion (SAB), 311–312 alcohol-induced persisting amnestic Thrombophlebitis, superficial, 504
Spontaneous bacterial peritonitis, 132 disorder (Korsakoff’s Thrombotic thrombocytopenic
Spouse abuse, 206 syndrome), 408 purpura (TTP), 161
562
INDEX
Thrush, oral (candidiasis, moniliasis), Tubulointerstitial disease, 466 Varicella (chickenpox), 40, 223,
211, 539 Turner’s syndrome (gonadal 369–370
Thyroid gland disorders, 72–75 dysgenesis 45,XO), 378 Varicella zoster (shingles), 41
hyperthyroidism, 72–73 Tylosis (acquired keratoderma), 61, 67 Varicocele, 455–456
hypothyroidism, 73–74 Tympanic membrane perforation, 186 Varicose veins, 503–504
neoplasms, 74–75, 486 Tzanck smear, 40 Vasculitis, pulmonary, 438–439
Thyroiditis, subacute, 72 Vasculitis syndromes, 32
Tibia fractures, 197 Vasectomy, 304
Tinea U Venous thrombosis, 32–33
capitis, 41 Uhthoff’s phenomenon, 283 Venous ulceration, 483–484
corporis, 41 Ulcer of lower limbs, 61–63 Ventricular fibrillation (VF), 16–17
manuum, 41 arteriosclerotic, 62 Ventricular septal defect (VSD), 22–23
pedis, 41 Ventricular tachycardia (VT), 16–17
pyoderma gangrenosum, 62–63
Tinnitus, 538 Verrucae (warts), 43
stasis ulceration, 61
Tonsillitis, 541 Vertebral column fractures, 197
Ulcerative colitis (UC), 111–113, 507
Toxic adenoma, 72
Ulna fractures, 198–199 Vertigo, 533–534
Toxic disorders, 275–276
Undescended testis, 363 Vesicoureteral reflux, 362, 457–458
antipsychotic drugs, 275–276
Upper respiratory infection, acute, Vesicular eruptions, 40
barbiturates, 275
547–548 Vibrio cholerae, 101
benzodiazepines, 275
Ureaplasma urealyticum, 451 Vibrio parahaemolyticus, 102
cocaine, 276
Ureteropelvic junction obstruction, Visual pathways, disorders of, 341–343
opioids, 275
459–460 bitemporal defects, 342
Toxic epidermal necrolysis (TEN), 49,
Urethral carcinoma, 464 homonymous defects, 342–343
52
Urethral stricture, benign, 456 monocular defects (central
Toxoplasma, 210, 216–217
Urethral syndromes, 449 scotomas), 341–342
encephalitis, 216–217
Urethral valves, posterior, 361–362 Viscus, perforation of, 188
Toxoplasma gondii, 216, 371
Urethritis, 450–451 Vitamin B12 deficiency, 145–146, 147,
Toxoplasmosis, 211, 371
Urinary incontinence, 296, 459 267
congenital, 371
stress-related, 459 Vitamin K deficiency, 159
Tracheoesophageal fistula (TEF), 374
Urinary tract infection (UTI), 449, 495 Volume depletion, 472–473
Transfusion reactions, 157
Urolithiasis, 458 Volume excess, 473
Transient ischemic attack (TIA), 495
Urologic trauma, 460 von Willebrand disease, 158–159
Transient tachypnea of the newborn
Urticaria (hives), 48–49, 170–171, 175 Vulvar carcinoma, 294–295
(TTN), 363
Uterus, 289–291
Traumatic injury, 203, 491–493
endometrial cancer, 289
abdominal injury, 492–493
endometriosis, 290–291
cranial injury, 491–492 W
fibroids, common types of, 290
multiple injuries, 491 Warts (verrucae), 43
leiomyomata uteri, 289–290
Treponema pallidum, 45, 97, 220, 293, Wegener’s granulomatosis, 439, 548
Uveitis, 331
371 Wernicke’s encephalopathy, 267, 408
Trichinella spiralis, 102 Western blot, 210
Trichomonas, 451 Wheezing, 443–444
Trichomoniasis, 295–296 V
Whiplash, 205
Tricyclic antidepressant poisoning, 193 Vagina and vulva, 294–296
Whipple’s triad, 87
Trigeminal neuralgia, 269–270 candidiasis of, 295
Wilms’ tumor, 463
Trisomy, 317–319 pelvic relaxation/urinary
Wilson’s disease, 126, 128, 131
13, 378 incontinence, 296
Wound infection, 494
18, 377–378 vaginal cancer, 295
21 (Down syndrome), 377, 378 vaginitis and vulvovaginitis, 295–296
Trousseau’s sign, 475 vulvar carcinoma, 294–295
Tubal obstruction, 302 Vaginismus, 412 Y– Z
Tuberculosis, 210, 214–215, 424 Valvular diseases, 20–21 Yersinia, 105
pulmonary, 424 acute, 20–21 Zenker’s diverticulum, 504–505
Tuberous sclerosis, 65–66 chronic, 21 Zollinger–Ellison syndrome, 96, 97