Caroli's syndrome associated with polycystic kidney disease
Marmara Medical Journal, cilt.10, sa.4, ss.206-210, 1997 (Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 10 Sayı: 4
- Basım Tarihi: 1997
- Dergi Adı: Marmara Medical Journal
- Derginin Tarandığı İndeksler: Scopus
- Sayfa Sayıları: ss.206-210
- Anahtar Kelimeler: Acute pyelonephritis, Caroli's syndrome, Children, Poliycystic kidney disease
- Ankara Yıldırım Beyazıt Üniversitesi Adresli: Hayır
Özet
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.