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S/S 99%
Western Blot direct visualization of virion proteins Confirmatory
FTT or atypical or difficult to iradicate infections =
Immunosupression
HIV typically acquired through vertical transmission or
breastmilk or secretions at delivery
Neonates test at birth and for 6 months
Give 6 weeks of zidovudine starting in first few hours of life
HIV POSITIVE give TMP for PCP prophylaxis at 6
weeks of age
Diabetes hyperglycemia promotes neutrophil
dysfunction, circulatory insufficiency ineffective
chemotaxis
LAD NEUTROPHILIA IS COMMON more than 50,000 cells
SCID Xlinked or AR
Di George No T cells, characteristic facies,
velocardiaiofacial defects, VSD, TOF, THYMIC/PARATHYROID
dysgenesis
Hypocalcemia, seizures
5 Kleinfeltter syndrome
Immature, insecure, gynecomastia, longer extrimities,
mental delay
@ 13 Tanner Stage 1
Nondisjunction XXY 1/600-800
Mental retardation diagnosed before 18 years of age
Ultimate diagnosis require IQ testing below 80
Kleinfeltters struggle with reading, spelling, math
although iQ may be normal
Most kleinfelter males go undiagnosed until
PUBERTY think about this diagnosis when presenting
with mental retardation/psychosocial or adjustment
problems at puberty
Elevated incidence of breast cancer, heme cancers in
kleinfelter
XYY explosive tembers, long and asymmetrical ears,
increased length vs breadth for hands, feet, cranium,
pectus excavatum
Tend to be taller and display aggressive or defiant
behavior
Large teeth, accelerated growth
Turner widely space nipples, broad chest, cubitis valgus
(increased carrying angle of arms), edema of hands and
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o Prognosis
Girls have better prognosis
AAs and Hispanics have worse prognosis
Under 1, older than 10 have worse prognosis
Higher leukocyte count above 50,000 worse
prognosis
T-cell ALL Worse than B-cell ALL
9:22 ALL and 4:11 AML
Blasts in CSF is worse prognosis
CXR mediastinal mass
Prednisoine, vincristine, apsparignase Induction
6MP, vincristine, 2-3 years to prevent replse
Therapy is discontinued for children after 2-3
years in remission
5 year survival better than 80%
Leukemia more common in
Kleinfelter, bloom, fanconi, ataxia
telangiectasia, NF, down syndrome
Live vaccines contraindicated in child with ALL
until at least 6 months after completion of
treatment
ITP - AFTER VIRAL INFECTION
Case 18 Diabetic mother
o Large newborn with resp depression DO ABCs
Check for hypoglycemia over next 24 hours
o Fetal hyperinsulin from hyperglycemia (maternal)
increased o2 requirements = neonatal distress
o Blood glucose 25-40 FEED THE BABy
Less than 25 IV GLUCOSE
o Resp distress, polycythemia and hyperviscosity,
hypocalcemia, hypomagenisum, hyperbilirubin
o Women screened for diabetes between 24-28 weeks
o VENOUS THROMBOSIS kidney and sinus, stroke, NEC,
persistent hypertension
o HYPOCALCEMIA IS COMMON causes irritability, sweating,
seizures
o Heart disease, small left colon, NTDs, caudal regression
o Infants born to diabetics neonatal RDS!
o Jaundice liver immature + polycythemia
Do Total bili and HCT
o RENAL VEIN THROMBOSIS can present as
abdominall mass kidney becomes congested
Gross hematuria, thrombocytopenia, oliguria
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Case 29 Postinfectoius GN
o Lab studies LOW C3 NORMAL C4, ASO+, Anti-DNase
B
o 98% o children recover
o ACUTE PROCESS
o Brown urine DDX
PIGN
Strenouous activity Rhabdomyolosis muscle
aches, fatigue, nausea, vomiting
IgA nephropathy painless hematuria, preceded by
URI
HSP
Lupus C3 DOES NOT NORMALIZE IN 6-12 WEEKS
o GN = glomerular inflammation hematuria, proteinuria,
hypertension
RBC casts marker for glomerular injury
o Rheumatic fever and PIGN RARELY occur together
o Abx use during initial GABHS infection reduces risk of
rheumatic fever
BUT HAS NOT AFFECT ON ACUTE PSGN
o Interval between GABHS pharyngitis and PSGN is 12 weeks
o Most have microscopic hematuria
85% have edema
60-80% HTN
o MOST IMP LAB TEST LOW C3 NORMAL C4
o Renal biopsy is NO LONGER routine
o HTN easily controld with CCBs
o Resolution is rapid and complete 12 days
o C3 levels normalize in 2-3 months
o Microcopic hematuria may persist for 1-2 years
o Everybody I nthe family has blood in urine BENIGN
FAMILIAR HEMATURIA
AD condition THIN MEMBRANE DISEASE
Case 30 Precocious puberty
o Most common cause idiopathic
o Do serum FSH/LH and bone radiographs
o Delayed pybtery no signs by 13 in girls or 14 in boys
o Precicoius pubtery, before 6-8 girls before 9 in boys
o True precocious puberty secretion of GnRH
o Questions to ask:
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o Age 6mo-6years
Duration <15, no lateralizing signs, no focal seizures,
not recurrent Siple febrile OBSERVE
Copmlex NEED WORK UP
<1 yr old LP
>1 consider CT, EEG+/- LP
o younger than 1 50-65% recurrence, older have 20-30%
o Increased risk for epiplepsy with febrile seizures
o Seizure after trauma
Case 42 Musuclar dystrophy
o 4 year old boy has delayed walking, waddling gait,
clumsiness, proximal muscle weakness
o Test DNA Peripherap blood analysis
immunohistochemical detection of abnormal dystrophin on
a muscle biopsy
o Duchene X linked recessive
o Initial tests CK and DNA of peripheral blod
o ONGOING CARDIAC EVALUATION FOR CARDIOMYOPATHY
o DMD most common heridetary NEUROMUSCULAR
DEGENERATIVE DISEASES
1/330 30% are new mutations
o Sx frequent falling, difficulty climbing stairs, hip waddle,
proximal muscle weakness (gower sign)
Muscle enlargement, caused by hypertrophy of
muscle fibers and infiltration of fat and collagen
proliferation
Pseudohypertrophy and a woody feel in affected
area
Cardiomyopathy TALL R WAVES, and RIGHT and
DEEP Q WAVES on the left
Intellectual impairment
Brain atrophy can be seen on CT
o Patinet in wheel chair by 10-13 years of age have rapid
progression of scoliosis after loss of ambulation
o Distal muscles remain functional permitting manual
dexterity
o Diminsed pulm function due to muscle involvement
recurrent infections, can lead to aspiration
o DNA Blod nalysis is diagnostic in 2/3 cases = if its not do
muscle biopsy
Endomysial connetive tissue proliferation,
inflammatory cell infiltrates, areas of regeneration
interspersed with areas of degeneration, and areas of
necrosis
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58 child abuse
Next step obtain skeletal survery
Lack of trauma history in infant who is not mobile
Mother seeks treatment for 2 days concerning
Neglect is most common form of child maltreatment
Muchausen by proxy repeated hospitlizations with
vague and unfiagnose conditions
Caretaken who takes great interest in the
medical staff and intervension, and often times
has some type of medical background
Caretakes forms relationship with healthcare and
often noted to be an exemplary parent
Skeletal srvery
Accidental bruises found over bony surfaces
However, abdomen, butt, thigs, inner arms are
abuse
Intentional hot water stocking glove
Cigarette burns may look lke impetigo or insect bites
Ddx
Hemophilia, ITP, HSP, DIC
OI, scurvy, cortical hyperostosis, Menkes kinky hair
disease
Cupping/coining folk mediine
59 foreign body aspiration
2 year old previously healthy acute onset RD
next step: evaluate childs airway with
bronchoscopy
IV access for fluids and sedation
TAKE NOTHING BY MOUTH until distress rsolves
SpO2 monitored closely
Stridor monophonic (one pitch)
Wheezing polyphonic (multiple pitches)
Foreign body aspiration most common cause of RD in
hildren major cause of morbidity and mortality
BRONCH the patient even if Xray is normal and you
suspect
Ddx
Croup
Epiglottitis
DROOLING, Preference to sit in tripond or
upright (sniffing position), hoarseness, absnce
of cough
Bacterial trachieitis staph 5-7 days after croup*
Retropharyngeal abscess
Angioedema
Tracheomalacia
Aortic/vascular ring compression of airway, tumor
compressing airway
Intraluminal obstruction (papilloma, hemangioma)
Coins in trachea xray show up as LINES due to the
cartilagienous rings forcing it into that position
Coins in esophagus show up as circles
Objects small than carina right mainstem
May not be visualized but air trapping, mediastinal
shift towards normal lung
Hyperextended neck, tachypnea, incosonolable, cold
TAKE TO OR and KEEP INFANT CALM intubate and
tracheostomy
Spasmodic croup appear well during day, but
develop symptoms at night rough night cause is
unknown
Croup aerosolized epinephrine and steroids
60 Kawasaki syndrome
LOOKS LIKE INFECTION MEASLES
High skpiking fever, irritable, CONNJUNCTIVITIS,
LYMPHADENOPATHY, EDEMA HANDS AND FEET,
RASH, OROPHARGEAL ERYTHEMA< RED CRACKED
LIPS
No Labs do echo to monior heart
ESR and CRP will be elevated
Normocytic anemia
Theombocytosis supportdiagnosis
GIVE IVIG and ASPIRIN
INTERMITTENT FEVER FOR 4 days
Hydrops of gallbladder wall of gallbladder ecoems
acutely distended WITHOUT STONES OR
INFLAMMATION
Kwasaki, leptospirosis, HSP
STRAWBERRY TONGUE
Children younger than 4
Coronary artery disease***
COnjuncitivits in 90% of cases
Sterule pyuria, CSF pleocytsis, elevated hepatic
transimases
Abdominal pain, RUQ pain, vomiting (Hydrops of
gallbladder)
Arthlagias, arthritis, anterior uveisis
Can have effusion, myocarditis, coronary arery disease
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