Master the board step 3

Pediatrics
If there is prolonged jaundice (> 2 weeks) and NO elevation of conjugated
bilirubin, consider the following:
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UTI or other infection
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Bilirubin conjugation abnormalities (e.g., Gilbert’s syndrome, Crigler-
Najjar syndrome)
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Hemolysis
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Intrinsic red cell membrane or enzyme defects (spherocytosis, elliptocytosis,
glucose-6-phosphate dehydrogenase deficiency, pyruvate kinase deficiency)
Where there is prolonged jaundice (> 2 weeks) AND elevation of conjugated
bilirubin, consider cholestasis:
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Initial diagnostic tests: liver function tests
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Most specific tests: ultrasound and liver biopsy
The most feared
complication of jaundice
results from elevated
indirect (unconjugated)
bilirubin, which can cross
the blood brain barrier,
deposit in the basal ganglia
and brainstem nuclei, and
cause kernicterus.
Watch out for hypotonia,
seizures, opisthotonos,
delayed motor skills,
choreoathetosis, and
sensorineural hearing loss.
Management is immediate
exchange transfusion.
Treatment
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Phototherapy when bilirubin > 10–12 mg/dL (normally decreases by 2 mg/dL
every 4–6 hours)
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Exchange transfusion in any infant with suspected bilirubin encephalopathy
or failure of phototherapy to reduce total bilirubin and risk of kernicterus
Basic Science Correlate
Phototherapy isomerizes bilirubin, making it water-soluble.
Neonatal Sepsis
A 5-week-old infant is brought into the clinic with irritability, weight loss of 3 lbs
over the past week, and “grunting.” Physical examination reveals temperature of
102.5°F. There is a bulging anterior fontanel, delayed capillary refill. What is the
next step in management?
Answer: Next step in management includes transferring the patient to the emergency
room and initiating a full sepsis workup. This includes: 1. CBC with differential, 2. blood
culture, 3. urinalysis/urine culture, and 4. chest x-ray BEFORE antibiotics are given.
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