PEDIATRICS Vol. 65 No. 4 April 1980, pp. 782-788
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Familial Cholestatic Cirrhosis Associated with Kayser-Fleischer Rings

Chaim Kaplinsky MD1, Irmin Sternlieb MD1, Norman Javitt MD, PhD1, and Yaacov Rotem MD1

1 Department of Pediatrics C, Chaim Sheba Medical Center, Tel Hashomer, Israel; the Division of Genetic Medicine and Liver Research Center, Albert Einstein College of Medicine and Bronx Municipal Hospital Center, Bronx, New York; and the Division of Gastroenterology, New York Hospital, Cornell Medical Center, New York

A brother and sister who suffered from pruritus since infancy developed hepatic cirrhosis early in life. Although this clinical picture has never been seen in Wilson's disease, Kayser-Fleischer rings in the boy made further studies necessary. Oral radiocopper loading tests administered to both children and to their parents served to exclude Wilson's disease conclusively. Determinations of the concentrations and patterns of bile acids in the serum indicated that the abnormalities observed in these children are not related to errors in bile acid synthesis. Although a defect in bile acid transport is present, it appears to have occurred as a consequence of the liver disease.

Submitted on March 16, 1979
Accepted on June 28, 1979




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