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Gastroenterology. 1984 Apr;86(4):757-60.

Congenital hepatic fibrosis and asymptomatic familial adult-type polycystic kidney disease in a 19-year-old woman.


Congenital hepatic fibrosis has been associated with a variety of renal malformations, but rarely adult-type polycystic kidneys. The case of a 19-yr-old woman with congenital hepatic fibrosis associated with asymptomatic familial adult-type (autosomal dominant) polycystic kidney disease is described. A literature review revealed seven other reports of this association. Our patient differs because the association between congenital hepatic fibrosis and adult-type polycystic kidney disease is clear, and because her asymptomatic kidney disease accords with the later onset of symptomatic polycystic kidney disease in her family.

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