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KMID : 0882419780210010075
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Korean Journal of Medicine
1978 Volume.21 No. 1 p.75 ~ p.81
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Congential Non-hemolytic Unconjugated Hyperbilirubinemais in a Family
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Yoon Yong-Bum
Park Seon-Yang
Kim Noe-Kyeong
Kim Chung-Yong
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Abstract
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Congenital non-hemolytic unconjugated hyperbiliru-binemia is a relatively, common disorder which is caused by a deficiency of hepatic bilirubin uridine diphosphate glucuronyl transferase activity, and the extent of the deficiency of the enzyme and the degree of jaundice attained are very variable so that the patients have been divided into three groups(i.e., Crigler-Najjar Type I; Crigler-Najjar Type II and Gilbert´s syndrome).
In Korea, however, none of them with definite family history has been reported so far, and the authors have found and treated a patient with Type II Crigler-Najjar syndrome and two with Gilbert´s syndrome in a family.
They showed typical responses to the low-calorie and phenobarbital administration in the serum bilirubin level as expected in Type II Crigler-Najjar syndrome and in Gilbert´s syndrome. They also had normal histology in the liver biopsy specimens, and there could be found no evidence of hemolysis or structural disorders of the hepatobiliary system.
The occurrence of Type II Crigler-Najjar syndrome and Gilbert´s syndrome in a family in these cases suggests that the former is the homozygous state and the latter the heterozygous state of the same hereditary disorder.
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