Caroli's syndrome associated with polycystic kidney disease

Altuntas B., Yarali N., Karayalcin S., Kuyucu S., Arda N., Akcayoz A., ...More

Marmara Medical Journal, vol.10, no.4, pp.206-210, 1997 (Scopus) identifier

  • 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.