Journal of the Korean Pediatric Society 1996;39(10):1448-1454.
Published online October 15, 1996.
A case of Primary Sclerosing Cholangitis with Ulcerative Colitis
Jung Sue Kim1, Yon Ho Choe1, Chong Jai Kim2, Woo Sun Kim3, In-One Kim3, Jeong Kee Seo1
1Department of Pediatrics, Seoul National University, College of Medicine, Seoul, Korea
2Department of Pathology, Seoul National University, College of Medicine, Seoul, Korea
3Department of Radiology, Seoul National University, College of Medicine, Seoul, Korea
궤양성 대장염 환아에서 발생한 원발성 경화성 담관염 1례
김정수1, 최연호1, 김종재2, 김우선3, 김인원3, 서정기1
1서울대학교 의과대학 소아과학교실
2서울대학교 의과대학 해부병리과학교실
3서울대학교 의과대학 진단방사선과학교실
An association between primary sclerosing cholangitis and ulcerative colitis is well known. But, primary sclerosing cholangitis with ulerative colitis has been rarely reported in children. The prevalence of primary sclerosing cholangitis among ulcerative colitis patiens is 3% in children. Primary sclerosing cholangitis is characterised by inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts. The diagnosis of primary sclerosing cholangitis based on biochemical, histologic and cholangiographic criteria. A twofold or greater elevation of serum alkaline phosphatase is required to suspect this diagnosis. The definitive diagnosis of primary sclerong cholangitis can usually made by cholangiography. The prognosis varies. No adequate treatment exists although a number of potential treatments have been evaluated. We experienced a case of primary sclerosing cholangitis with ulcerative colitis in a 14 year old girl. She was admitted with a history of intermittent bloody diarrhea and jaundice over a two year period. Hepatosplenomegaly and cholestasis had been noted. Abnormal liver function tests were noted. AST was 117U/l, ALT 179U/l, alkaline phosphatase 603U/l, γ-GT 366U/l, total bilirubin 5.5mg/dl. An endoscopic retrograde cholecystopancreatography showed evidence of strictures, beading, and irregularities of intra and extrahepatic biliary system. Liver biopsy showed histologic findings compatible with a sclerosing cholangitis and evidence of periductular fibrosis. She sufferred from persistent cholestasis and sign of portal hypertension. She had developed recurrent episodes of variceal hemorrhages which had been successfully managed several times endoscopic variceal ligations.
Key Words: Primary sclerosing cholangitis, Ulcerative colitis, Cholangiography

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