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Pathology Liver & Biliary Tract 7528d025

False about Criggler najar syndrome?

A
Absence of UDPG enzyme
B
Normal liver biopsy
C
Conjugated hyperbilirubinemia
D
Type 1 is more severe
High-Yield Explanation
Ans. is 'c' i.e., Conjugated hyperbilirubinemia Criggler-Najjar Syndrome* CN syndrome is a congenital unconjugated hyperbilirubinemia. Basic defect is either reduced or absent UDPG transferase.* There are two types of CN syndrome:-1) Type I# It is an autosomal recessive disorder in which UDPG transferase is absent. Thus phenobarbitone has no effect (as enzyme is absent).# It is more severe hyperbilirubinemia than type II. There may be kernicterus. LFTs and liver histology are normal.2) Type II# It is an autosomal dominant disorder in which UDPG transferase is reduced. Phenobarbitone can reduce unconjugated Bilirubin by inducing UDPG transferase.# LFTs and liver histology are normal and there is no kernicterus

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