Abstract
Congenital indirecthyperbilirubinemiaoccurs due to the relative deficiency or absence of the primary conjugating enzyme uridinediphosphate-glucuronyltransferase.The enzyme UDP glucuronyltransferase is responsible for the conjugation of bilirubin in liver. The enzyme is also essential for transport of many other substrates including drugs, hormones, toxins and neurotransmitters. Thus, anaesthetic management of such a case is quite challenging. Avoiding drugs which use this enzyme for its metabolism or excretion during the perioperative period allows safe conduct of anaesthesia for these patients. We report a case of inherited unconjugated hyperbilirubinemiafor an incidental surgery and review the different types of congenital indirect hyperbilirubinemias.
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