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OBJECTIVE A case of hepatic fibrosis in adult polycystic liver disease is reported. This association, commonly present in infancy, is exceptional in the adult polycystic liver disease. METHODS We report a 69-years-old female with adult polycystic kidney and liver disease with fatal hepatic failure, portal hypertension and renal failure. An autopsy was performed showing hepatic and renal polycystic disease with hepatic fibrosis. CONCLUSIONS Hepatic fibrosis is a rarely associated phenomenon with adult polycystic liver disease. This data may suggest a relation of this entity with infancy fibropolycystic liver disease.
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