原发性胆汁性肝硬化指南解读PPT.ppt

原发性胆汁性肝硬化 Primary Biliary Cirrhosis （PBC） ——指南与进展;概述;发现史;病因学;遗传因素;环境因素;;原发性胆汁性肝硬化（PBC）;流行病学;自然史;;诊断;生化检测;Immunological abnormality;Histology;;组化：PBC病人的Mallory （马洛里）小体 （ × 820）;;临床表现;胆汁淤积的临床和生化表现;PBC: Clinical manifestation;PBC5期;1、前驱期; 2、少症状期; 3、有症状无黄疸期; 分别出现什么现象; 5、后期; ;PBC: Differential diagnosis;特殊情况;;;;;治疗;治疗目标;;;;Promote bile secretion ?Ca++ influx, PKC, MAPK：Erk-1, 2 Anti-apoptosis oppose cytotoxicity of hydrophobic bile acids Immune regulation ? inhibit HLA-I, II expression in hepatocytes, bile duct epithelium inhibit production of IL-2, IL-4, IFNg by PBMC corticosteriod-like action?;Beuers U, et al. Hepatology 1998; 28; 28(6):1449-53.;;;;UDCA improves survial of PBC patients;PBC- 10 year’s UDCA;UDCA应答欠佳患者的治疗;;;;对症治疗;胆汁淤积瘙痒常用药物;;;;;肝移植;;OLT improves survivals;随访;;Thank You !