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Vol. 10, No. 3, 1990
Issue release date: 1990
Am J Nephrol 1990;10:237–241
(DOI:10.1159/000168088)

Polycystic Kidney of Autosomal Dominant Inheritance, Polycystic Liver and Congenital Hepatic Fibrosis in a Single Kindred

Matsuda O. · Ideura T. · Shinoda T. · Shiigai T. · Takeuchi H. · Wei-Chia C. · Miyake S.
Departments of aInternal Medicine and fPathology, Musashino Red Cross Hospital, Musashino-City, Tokyo; bDepartment of Internal Medicine, Showa University, Fujigaoka Hospital, Yokohama; cDepartment of Geriatrics, Shinshu University School of Medicine, Matsumoto-City, Nagano; dDepartment of Internal Medicine, Toride-Kyodo Hospital, Toride-City, Ibaraki; eDepartment of Urology, Mishima Hospital, Mishima-City, Shizuoka, Japan

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Abstract

A family with clear evidence of concominant congenital hepatic fibrosis, polycystic liver and adult-type polycystic kidney is reported. The autosomal dominant inheritance of polycystic kidney in association with either liver cyst or hepatic fibrosis in this family suggests that various combinations of hepatic and renal fibrocystic lesions are possible to occur. Additionally, in the proband a very rare association of congenital hepatic fibrosis and cerebral aneurysm was present.



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