| A case of Primary Sclerosing Cholangitis with Ulcerative Colitis |
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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 |
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김정수1, 최연호1, 김종재2, 김우선3, 김인원3, 서정기1 |
1서울대학교 의과대학 소아과학교실
2서울대학교 의과대학 해부병리과학교실
3서울대학교 의과대학 진단방사선과학교실 |
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| Abstract |
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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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