Case Report: PDF OnlySokal Etienne M.; de Ville de Goyet, Jean; Buts, Jean Paul; Habets, Sophie; Gosseye, Serge; Clapuyt, Philippe; Claus, Dominique; Otte, Jean BernardJournal of Pediatric Gastroenterology and Nutrition: August 1990 - p 268-274 Free Abstract Summary: A 4-year-old boy and a 14-year-old girl referred for a cholestatic syndrome were found to have a unifocal stricture of the extrahepatic bile duet. Both radiological (endoseopic-retrograde-choledocopancreatography) and histological findings were suggestive of primary sclerosing cholangitis. There was no past history of chronic inflammatory bowel disease nor any other disorder. Serum autoimmune markers were negative. Complete remission was observed after surgical drainage, and both patients remained symptom-free after a follow-up of 12 months and 8 years, respectively. Such unifocal form of the disease may have a better prognosis than the plurifocal disease. The absence of associated disorders and autoimmunity may also be an indicator of better prognosis in these patients. © Lippincott-Raven Publishers.