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USMLE
Fetal and Neonatal Medicine IV
Crigler-Najjar syndrome characterized by all the following EXCEPT
Explanation
ExplanationCrigler-Najjar syndrome is a serious, rare, autosomal recessive, permanent deficiency of glucuronosyltransferase that results in severe indirect hyperbilirubinemia. Type II responds to enzyme induction by phenobarbital, producing an increase in enzyme activity and a reduction of bilirubin levels. Type I do not respond to phenobarbital and manifests as persistent indirect hyperbilirubinemia, often leading to kernicterus.
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