All of the following are (Jnconjugated Hyperbilirubinemias EXCEPT
Correct Answer: Dubin Johnson syndrome
Description: (C) Dubin Johnson syndrome # Crigler-Najjar syndrome (CNS) is a rare autosomal recessive disorder of bilirubin metabolism. Two distinct forms have been described, as follows: type 1 and type 2.> Type 1 CNS, first described in 1952 by Crigler and Najjar, is associated with neonatal unconjugated hyperbilirubinemia (high levels) and kernicterus.> Type 2 CNS (Arias syndrome), first described in 1962 by Arias, presents with a lower serum bilirubin level and responds to phenobarbital treatment.> CNS is elicited by a lack or deficiency of the enzyme uridine diphosphate glycosyltransferase (UGT).> A markedly elevated unconjugated (indirect) hyperbilirubinemia is observed in inherited disorders such as Gilbert syndrome and CNS. Among the inherited unconjugated hyperbilirubinemias, Gilbert syndrome is believed to affect approximately 3-7% of the adult population.> Dubin Johnson syndrome & Rotor syndromes are inborn errors of metabolism which lead to conjugated hyperbilirubinemia
Category:
Biochemistry
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