JEAN-LOUIS DUPOND, M.D.; JEAN-PHILIPPE MIGUET, M.D.; JEAN-PIERRE CARBILLET, M.D.; YVES SAINT-HILLIER, M.D.; CLAUDE PEROL, M.D.; ROBERT LeCONTE-des-FLORIS, M.D.
Congenital hepatic fibrosis and the adult type of polycystic disease of liver and kidneys are regarded as different entities (1). [a] Congenital hepatic fibrosis is characterized by fibrous thickening of the portal tracts and an excess of small bile ducts. The cysts of congenital hepatic fibrosis, which are in fact dilated bile ducts, are small, generally microscopic, and communicate with the bile ducts. The hepatic cysts in polycystic disease are large and do not communicate with the biliary passages. [b] In congenital hepatic fibrosis, dilatation of the collecting tubules is considered as the most common associated renal lesion (1). Multiple
DUPOND J, MIGUET J, CARBILLET J, SAINT-HILLIER Y, PEROL C, LeCONTE-des-FLORIS R. Kidney Polycystic Disease in Adult Congenital Hepatic Fibrosis. Ann Intern Med. ;88:514–515. doi: 10.7326/0003-4819-88-4-514
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Published: Ann Intern Med. 1978;88(4):514-515.
Gastroenterology/Hepatology, Liver Disease, Nephrology.
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