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原发性胆汁性肝硬化(PBC)多发于中年女性,具有脏器特异性,该病由免疫介导、多以破坏肝内小胆管为特征的自身免疫性疾病,最终可发展为肝纤维化和肝硬化。PBC的诊断要点有:(1)肝内胆汁淤积的血清酶谱表现;(2)血清IgM升高;(3)免疫荧光法检测的抗线粒体抗体(AMA)阳性;通过ELISA或免疫印迹法检测的PBC特征性抗体AMA的M2阳性;(4)肝内中小胆管的破坏或缺损。在M2阳性患者中,确诊PBC需行
Primary biliary cirrhosis (PBC) is more common in middle-aged women with organ-specific disease that is mediated by immunity and is mostly an autoimmune disorder characterized by the destruction of the intrahepatic bile ducts that eventually develop into liver fibrosis And cirrhosis. Diagnostic criteria for PBC are: (1) serum enzyme profiles of intrahepatic cholestasis; (2) elevated serum IgM; (3) positive for anti-mitochondrial antibodies (AMA) detected by immunofluorescence; as detected by ELISA or Western blotting Of PBC characteristic antibody AMA M2 positive; (4) intrahepatic bile duct destruction or defect. In M2-positive patients, the diagnosis of PBC required