<Originals>Pathophysiological aspects of Gilbert's syndrome and Crigler-Najjar syndrome type II

概要

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To determine the common pathophysiological basis in Gilbert's syndrome and Crigler-Najjar syndrome type II, biliary bilirubin moieties and hepatic bilirubin UDP-glucuronosyltransferase activity were measured. The bilirubin UDP-glucuronosyltransferase activity in the liver homogenate decreased to 29% and 11% of the normal (mean) value in Gilbert's syndrome and Crigler-Najjar syndrome type II, respectively. In Gilbert's syndrome and Crigler-Najjar syndrome type II, the mean biliary bilirubin diglucuronide fraction decreased to 55.3% and 9.7% (normal : 76.5%), while the mean bilirubin monoglucuronide fraction increased to 28.0% and 63.1% (normal 9.8%), in Gilbert's syndrome and Crigler-Najjar syndrome type II, respectively. In Crigler-Najjar syndrome type II, a marked increase in bilirubin XIα in bile was also observed. These findings suggest that, stepwise reduction of hepatic bilirubin conjugating capacity is a feature of these syndromes.
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