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KMID : 0614619950270040488
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Korean Journal of Gastroenterology
1995 Volume.27 No. 4 p.488 ~ p.492
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A Case of Type 2 Crigler-Najjar Syndrome
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이돈행
최정현/안홍석/윤일국/장태정
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Abstract
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Type 2 Crigler-Najjar syndrome is a rare hereditary disorder characterized by chronic, nonhemolytic and modest unconjugated hyperbilirubinemia. There are currently reported three types of syndrome: Gilbert, Type 1 and 2 Crigler-Najjar syndrome.
Type 2
Crigler-Najjar syndrome is caused by hereditary glucuronosyl transferase deficiency and it can be differentiated from the Gilbert and Type 1 Crigler-Najjar syndrome by the severity of hyperbilirubinemia and its response to phenobarbital
administration.
We expierienced a 22 year-old male patient who had shown persistent jaundice and unconjugated hyperbilirubinemia from adolescence. It was noted that several persons had shown jaundice among his maternal family. The plasma bilirubin concentration
was
elevated after fasting for 48 hours and was decreased dramatically by phenobarbital treatment. (Korean J Gastroenterol 1995; 27: 488-492)
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