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Laurence-Moon-Biedl syndrome accompanied by congenital hepatic fibrosis
F Nakamura1, H Sasaki, H Kajihara
1Department of Internal Medicine, Hiroshima Memorial Hospital, Japan.
Journal of Gastroenterology and Hepatology
|March 1, 1990
Abstract:
A 33 year old woman with mental deficiency, retinitis pigmentosa, obesity, and parental consanguinity (heredity) was diagnosed as having Laurence-Moon-Biedl syndrome. She also had diabetes mellitus, slight hepatic dysfunction, and hepatomegaly. Liver biopsy showed perilobular fibrosis and bile duct proliferation with cystic dilatation; these findings are consistent with those of congenital hepatic fibrosis.