BILIRUBIN SECRETION AND CONJUGATION IN THE CRIGLER-NAJJAR SYNDROME TYPE II

摘要: Features characteristic of the Crigler-Najjar syndrome (type II) are described in an adolescent boy with severe congenital unconjugated hyperbilirubinemia. Bilirubin encephalopathy developed only early puberty after surgery and fasting, coincident a dramatic rise serum bilirubin, which responded to intensive therapy. Fasting was later shown increase markedly bilirubin levels probably major factor precipitating initial acute event. One year later, while patient metabolic steady state, secretion rate measured by duodenal marker-perfusion technique, nature secreted conjugates characterized. Total rates were low, 4.39 mg per hr 4.44 on two separate studies. The pigment detected bile monoglucuronide. diglucuronide comprised minor fraction pigments, other not detected. present study documents reduced biliary suggests that addition second glucuronic acid moiety molecule may be defective II).