True about Crigler Najjar type II syndrome is:
Answer is B (Recessive trait) Crigger Najjar syndrome is inherited as an autosomal recessive trait. Crigger Najjar Syndrome (Type H) is associated with a deficient activity of Bilirubin UDP - Glucoronyl Transferase activity (UGITAL) and not with dimlucornide deficiency. Kernincterus is a characteristic features of type 1 Criggler Najjar syndrome and Kernincterus is extremely rare in type 11 Criggler Najjar syndrome. Phenobarbital is extremely effective and useful in type 11 Criggler Najjar syndrome and reduces bilirubin levels by > 25%. Principal Characteristics Criggler-Najjar Syndromes Feature Crigler-Najjar Syndrome Typel Type II Total serum bilirubin, pmol/L (mg/dl) 310-755 (usually > 345) 100-430 (usually <345) Routine liver tests Normal Normal Response to Phenobarbital None Decreases bilirubin by>25% Rare Kernicterus Usual Hepatic histology Normal Normal Bile characteristics Color Pale or colorless Pigmented Bilirubin fractions monoconjugates >90% unconjugated Largest fraction (mean: 57%) Bilirubin UDP-glucuronosyl-transferase Typically absent; traces in some patients Markedly reduced:0-10% of normal Inheritance (all autosomal) Recessive Predominantly recessive
Diglucuronide deficiency
Recessive trait
Kernicterus is seen
Phenobarbitone is not useful