False about Criggler najar syndrome?
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
Absence of UDPG enzyme
Normal liver biopsy
Conjugated hyperbilirubinemia
Type 1 is more severe
Liver & Biliary Tract