Crigler-Najjar syndrome

Summary of Crigler-Najjar syndrome
Crigler–Najjar syndrome is a rare inherited disorder affecting the metabolism of bilirubin. It is caused by an absent UDP-glucuronosyltransferase enzyme. The disorder results in a form of nonhemolytic jaundice, which results in high levels of unconjugated bilirubin and often leads to brain damage in infants. The disorder is autosomal recessive.

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Pathology

Gastrointestinal system

Peritoneum and peritoneal cavity disorders
Upper gastrointestinal tract disorders
Lower gastrointestinal tract disorders
Liver, gallbladder and pancreas disorders
Gastrointestinal system pathology review

Assessments
Crigler-Najjar syndrome

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High Yield Notes
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Crigler-Najjar syndrome

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Crigler-Najjar syndrome results in a(n) (conjugated/unconjugated) hyperbilirubinemia.

Questions

USMLE® Step 1 style questions USMLE

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USMLE® Step 2 style questions USMLE

2 questions
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A 20-hour-old female infant is reviewed on the ward because of neonatal jaundice. She was born at term following an uncomplicated pregnancy. She has passed meconium. Physical examination shows she is alert, well perfused, feeding normally, and afebrile. She is jaundiced over face, trunk, and limbs, and has no organomegaly. Laboratory studies show an elevated unconjugated bilirubin of 426 μmol/L (25mg/dL) (reference range  2–14 μmol/L [0.1-0.8 mg/dL]), normal liver function tests, and no evidence of hemolysis. Which of the following diagnoses is the most likely cause of jaundice in this newborn? 

External References