Abstract
Two cases with primary sclerosing cholangitis (PSC) in adolescence were reported. Case 1(a 19-year-old male) and Case 2 (a 16-year-old male) were admitted to our hospital because of liver dysfunction. In both cases, serum transaminases and alkaline phosphatase were moderately elevated. Endoscopic retrograde cholangiography of the first case showed sclerotic wall and local narrowing of the common bile duct. That of the second case showed dilatation of the common bile duct and pruned tree appearance of the right branch as well as beaded appearance of the left branch of the intrahepatic ducts. PSC is a rare disease in the adolescence and only 18 cases (including our cases) have been reported in patients younger than 20 years old in Japan. We reviewed these cases and contrasted clinical features of the adolescence and adult of PSC. Characteristics of PSC in adolescence were (1) less common in association with jaundice, (2) mainly with the intrahepatic type, and (3) more frequently associated with ulcerative colitis than PSC in adulthood.
