All issues > Volume 39(10); 1996

Case Report

J Korean Pediatr Soc. 1996;39(10):1448-1454. Published online October 15, 1996.

A case of Primary Sclerosing Cholangitis with Ulcerative Colitis

Jung Sue JS Kim¹, Yon Ho YH Choe¹, Chong Jai CJ Kim², Woo Sun WS Kim³, In-One IO Kim³, Jeong Kee JK 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

Abstract

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.

Keywords :Primary sclerosing cholangitis, Ulcerative colitis, Cholangiography