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[Two cases of primary sclerosing cholangitis (author's transl)]
Abstract:
Two patients with symptoms of progressive obstructive jaundice, a history of vague pains in the right upper quadrant and laboratory evidence of biliary obstruction underwent laparotomy. A stone-free, but extremely thick-walled gallbladder was found in both patients. Intraoperative cholangiography showed diffusely-stenosed extrahepatic bile ducts suggestive of chronic inflammatory changes in the biliary system. The correct diagnosis was made only on histological examination, which revealed primary sclerosing cholangitis with secondary cholestatic changes of the liver. Postoperative treatment included long-time corticosteroid therapy. Both patients have remained jaundice-free for periods of one and two years, respectively, to date, but the eventual prognosis is poor. The diagnosis, which can be made only surgically, therapy and prognosis are discussed.