Primary Sclerosing Cholangitis in Children

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Abstract
Primary sclerosing cholangitis (PSC) in eight children, five males and three females between the ages of 4 and 13 years, presented with minimal clinical symptoms and few signs of liver disease. Diagnosis was made by a highly characteristic histology in all and additional endoscopic retrograde cholangiography (ERC) in four patients. Six children suffered from concomitant inflammatory bowel disease (IBD). Laboratory abnormalities consisted of mild elevation of transaminases and alkaline phosphatase, with marked elevation of immunoglobulin G in seven and detection of anti-nuclear antibodies in four of the eight children. In all cases, needle liver biopsy specimens revealed portal tracts considerably expanded by edema and chronic inflammation diagnostic of nonsuppurative cholangitis and nonsuppurative fibrosing pericholangitis. ERC showed decreased arborization in the whole biliary tree. In two patients, changes were confined to the intrahepatic portion of the biliary tract. No specific drug therapy was given to our patients. Those with concomitant IBD were treated with salazosulfapyridine (SASP). The clinical course has been mild in all patients up to now. It is concluded that PSC may be much more frequent in childhood than was considered before, especially in children with IBD.