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原发性硬化性胆管炎(PSC)是一种少见的胆道疾患,诊断亦较困难。近年来随着胆道检查技术的改进,特别是逆行胰胆管造影术(ERCP)和经皮经肝胆道造影术(PTC)的广泛应用,PSC的诊断率有所提高。临床上,原发性胆管狭窄、胆管癌、原发性胆汁性肝硬化等疾病作出诊断时常需与PSC进行鉴别。本文就近年文献作一简要综述。病因与发病原发性硬化性胆管炎是一种原因不明的以肝内、外胆管慢性纤维性狭窄和闭塞为特征的综合征。这种狭窄既非由损伤引起,亦非恶性肿瘤所致。不少病例合并有炎性肠道疾患,多数是慢性溃疡性结肠炎(CUC)。自Delbet 1924年发现本病以来,至今已有60多年,但PSC的确切发病率仍不清楚。各年龄均可
Primary sclerosing cholangitis (PSC) is a rare biliary disease, diagnosis is more difficult. In recent years, with the improvement of biliary tract examination techniques, especially the extensive application of retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC), the diagnostic rate of PSC has been increased. Clinically, primary biliary stricture, cholangiocarcinoma, primary biliary cirrhosis and other diseases to make diagnosis often need to be identified with the PSC. In this paper, a brief review of recent literature. Etiology and pathogenesis of primary sclerosing cholangitis is an unknown cause of the liver, extrahepatic bile duct chronic fibrous stenosis and occlusion is characterized by syndrome. This stenosis is neither caused by injury nor malignant. Many cases with inflammatory bowel disease, most are chronic ulcerative colitis (CUC). It has been more than 60 years since Delbet discovered this disease in 1924, but the exact incidence of PSC remains unclear. All ages can be