课件：胆汁淤积和黄疸.ppt

4．原发性胆汁性肝硬化(primary biliary cirrhosis,PBC) 本病又称肝内阻塞性胆汁性肝硬化，较少见，多发于中年妇女。 由于伴有许多免疫异常，如：血清IgM增高、90%以上患者抗线粒体抗体及抗线粒体M2抗体阳性，此外还常伴有许多其他自身免疫性疾病，如：类风关、硬皮病、Hashimoto甲状腺肿等。故目前认为本病是一种自身免疫性疾病。 肝活检可见胆管炎症、小胆管阻塞、胆汁淤滞、纤维增生等，晚期发展为小结节型肝硬化。 （四）阻塞性黄疸 属外科性黄疸，是由于感染、结石、寄生虫、肿瘤等造成肝内外胆管的机械性阻塞所致。又称肝后性黄疸。其病因包括：肝内外胆管的炎症、结石、肿瘤，胰腺的炎症、结石、肿瘤等亦可引起。 衰老红细胞破坏 血红蛋白 “旁路性”胆红素 非衰老红细胞来源 与血浆白 蛋白结合 肝窦与白蛋白分离 小胆管阻塞 总胆管阻塞 肠道原胆元 减少或缺如 尿胆原减 少或无 体循环 尿胆素减少 胆红素阳性 大便色变浅 Z蛋白 Y蛋白 高尔基体 微粒体内多种酶 UDGT 毛细胆管 酯化胆红素 肾脏尿胆元减少 或无胆红素增加 尿胆元 光面内质网 非酯型胆红素 酯型胆红 素排泌正常 阻塞性黄疸 逆流入血 黄疸 毛细胆管、小胆管破裂 肝细胞 概况 生理 Bile acids Bile Bilirubin Bilirubin 代谢 病人 Pruritis Scleral icterus Jaundice 病理 Cholestasis Hyperbilirubinemia 病因 Many liver diseases Medications Obstruction of bile excretion THANK YOU SUCCESS * * 可编辑 * * Waste product of hemoglobin, bilirubin is toxic * * * Figure 3. Diagnosis of Drug-Related Hepatotoxicity. There is no single test, including liver biopsy, that can be used to diagnose drug-related hepatotoxicity. Other causes of liver injury must first be considered with the use of a combination of serologic tests, imaging studies, and clues from the patients history. CT denotes computed tomography, MRI magnetic resonance imaging, MRCP magnetic resonance cholangiopancreatography, ERCP endoscopic retrograde cholangiopancreatography, AST aspartate aminotransferase, ALT alanine aminotransferase, TIBC total iron-binding capacity, and A1AT alpha1-antitrypsin.  * Figure 2. Liver-Biopsy Specimens Showing Common Histologic Features of Drug-Related Hepatotoxicity. Panel A shows microvesicular steatosis, in which small fat droplets (arrow) are present within the hepatocytes and do not displace the nucleus. Examples of drugs that can induce such an injury include valproic acid and tetracycline. A typical hepatic enzyme pattern in such a reaction includes moderate elevations of the alanine aminotransferase and aspartate aminotransferase levels. Panel B shows acute hepatitis, with hepatocellular swelling, infl