【摘要】 胆道闭锁患儿胆汁酸代谢异常可损伤肝细胞及胆管上皮细胞。熊去氧胆酸作为目前胆道闭锁手术后常规用药,其疗效及使用方法仍存在争议;胆汁酸甾体类核受体激动剂(奥贝胆酸)及非甾体类核受体激动剂(LJN-452)、肠道胆汁酸重吸收相关蛋白调控药物(A4250)、肝脏内胆汁酸转运蛋白、肾脏转运蛋白相关药物的开发研究近年来发展迅速,不少药物已开始临床Ⅱ、Ⅲ期试验,将来可能对胆道闭锁肝门空肠吻合术后逐步恢复胆流的患儿有一定帮助,成为胆道闭锁肝门空肠吻合术后新辅助用药。更多还原

【Abstract】 Abnormal bile acid metabolism in biliary atresia( BA) may injure hepatocytes and bile duct epithelial cells. Ursodeoxycholic acid is currently a standard postoperative drug for BA. However,its efficacy and dosing have remained controversial. Steroidal bile acid nuclear receptor activation( obeticholic acid,etc),nonsteroidal nuclear receptor agonist( LJN-452,etc),intestinal bile acid reabsorption related protein regulating drug( A4250,etc),liver bile acid transporter and kidney transporter related drugs have been developed rapidly in recent years. Many candidates have entered phaseⅡ/Ⅲ clinical trials. In the future,these promising drugs may benefit children gradually recovering bile flow after Kasai procedure. They may become new adjuvant drugs for BA.更多还原