原发性胆汁性肝硬化指南解读概述发现史病因学遗传因素环境因素免疫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ImmunologicalabnormalityHistology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:ClinicalmanifestationPBC5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:Differentialdiagnosis特殊情况治疗治疗目标PromotebilesecretionCa++influx,PKC,MAPK：Erk-1,2Anti-apoptosisopposecytotoxicityofhydrophobicbileacidsImmuneregulation?inhibitHLA-I,IIexpressioninhepatocytes,bileductepitheliuminhibitproductionofIL-2,IL-4,IFNgbyPBMCcorticosteriod-likeaction?BeuersU,etal.Hepatology1998;28;28(6):1449-53.UDCAimprovessurvialofPBCpatientsPBC-10year’sUDCAUDCA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OLTimprovessurvivals随访ThankYou!