Caroli's syndrome associated with polycystic kidney disease
Marmara Medical Journal, vol.10, no.4, pp.206-210, 1997 (Scopus)
- Publication Type: Article / Article
- Volume: 10 Issue: 4
- Publication Date: 1997
- Journal Name: Marmara Medical Journal
- Journal Indexes: Scopus
- Page Numbers: pp.206-210
- Keywords: Acute pyelonephritis, Caroli's syndrome, Children, Poliycystic kidney disease
- Ankara Yıldırım Beyazıt University Affiliated: No
Abstract
Congenital intrahepatic biliary duct dilatation associated with congenital hepatic fibrosis (CHF), referred to as Caroli's syndrome, is a rare condition. Caroli's syndrome is generally associated with autosomal recessive polycystic kidney disease (ARPKD) or rarely autosomal dominant polycystic kidney disease (ADPKD). In this case report, we describe a thirteen and a nine-year-old two brothers with Caroli's syndrome and polycystic kidney disease. There was no parental consanguinity. The elder brother had a history of jaundice. However, the little one had only a history of periodic abdominal pain and on follow up, he had acute pyelonephritis.