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原发性硬化性胆管炎(PSC)是一种肝外胆管的炎性闭塞性纤维化,肝内胆管可同时被累及或不被累及。病情缓慢地进展,终于发展成肝硬化、门脉高压症和肝衰竭而死亡。本文报告Mayo Clinic 1970～1977年间经手术和/或放射线检查证实的50例PSC病情分析。1970～1973年每年发病约4例,1974年起应用内窥镜逆行胰胆管造影术后,本病的诊断数加倍。 PSC最多见于30～40岁,50岁以下占82％,40岁以下占60％;70％是男性。90％患者都有症状,主要是黄疸(68％)、瘙痒(62％)和非特异性腹痛(50％)。胆管炎最常见的典型症状如右上腹反复疼痛、发热、寒战和
Primary sclerosing cholangitis (PSC) is an inflammatory obliterative fibrosis of the extrahepatic bile duct that can be involved with or without involvement of the intrahepatic bile duct. The disease progressed slowly and eventually developed into cirrhosis, portal hypertension and liver failure. This article reports 50 patients with PSC confirmed by surgery and / or radiology from Mayo Clinic between 1970 and 1977. From 1970 to 1973, about 4 cases were reported annually. After endoscopic retrograde cholangiopancreatography was performed in 1974, the number of diagnosis of this disease doubled. PSC most often seen in 30 to 40 years of age, 82% of those under 50 years of age, 60% under 40 years of age; 70% of men. Ninety percent of patients have symptoms, mainly jaundice (68%), pruritus (62%), and nonspecific abdominal pain (50%). The most common typical symptoms of cholangitis, such as repeated pain in the right upper quadrant, fever, chills and