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Congenital bile duct dilatation (Caroli's disease--Grumbach's disease).

Johannes Lämmer, R. Fötter, H Becker

Abstract

Congenital bile duct dilatation is an autosomal recessive inherited disease. Pathologically tortuous dilated, dysplastic intrahepatic bile ducts were found. It may be associated with hepatic fibrosis (Grumbach's disease), or without (Caroli's disease). A common additional finding is an infantile polycystic kidney. Three cases are reported and the radiological and sonographic findings are discussed.

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Available abstract

Key concepts: Medicine, Intrahepatic bile ducts, Congenital hepatic fibrosis, Autosomal Recessive Polycystic Kidney Disease, Bile Duct Diseases, Polycystic disease, Gastroenterology, Bile duct