- Marmara Medical Journal
- Vol: 10 Issue: 4
- Caroli's syndrome associated with polycystic kidney disease
Caroli's syndrome associated with polycystic kidney disease
Authors : B. Altuntaş, N. Yaralı, S. Karayalçin, S. Kuyucu, N. Arda, A. Akçayoz, Z. Arslan, Ü. Ertan, A. Öner, T. Teziç
Pages : 206-210
View : 6 | Download : 3
Publication Date : 1997-07-01
Article Type : Research
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.Keywords : Caroli's Syndrome, poliycystic kidney disease, children, acute pyelonephritis