Caroli's disease and adult polycystic kidney disease: a rarely recognized association.
Caroli's disease is a rare form of fibropolycystic disease of the hepatobiliary system characterized by segmental cystic dilatation of intrahepatic ducts. It is associated with intrahepatic cholelithiasis, cholangitis and hepatic abscesses. Like other forms of fibropolycystic disease of the liver, Caroli's disease is often accompanied by cystic renal disease, specifically renal tubular ectasia or medullary sponge kidney. Adult-type polycystic kidney disease associated with Caroli's disease is rare, only one such case having appeared in the literature to our knowledge. We present a second instance of this association.
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Lillian and Henry M. Stratton-Hans Popper Department of Pathology, Mount Sinai School of Medicine, City University of New York, New York.
Harpaz
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Thung
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D001649:Bile Duct Diseases / Q000150:complications / Q000473:pathology; D001653:Bile Ducts, Intrahepatic / Q000473:pathology*; D003560:Cysts / Q000150:complications* / Q000473:pathology; D006801:Humans; D008099:Liver / Q000473:pathology; D008103:Liver Cirrhosis / Q000151:congenital; D008297:Male; D008875:Middle Aged; D007690:Polycystic Kidney Diseases / Q000150:complications*