胆道闭锁肝纤维化病因学研究进展  

Advances in etiology of hepatic fibrosis in biliary atresia

作　　者：孔萌 陈帅[1] 张利红[2] 白月霞[2] 刘红真[1] 刘婷婷[1] 贾金华 刘传阳 刘威[1] 张士松[1] Kong Meng;Chen Shuai;Zhang Lihong;Bai Yuexia;Liu Hongzhen;Liu Tingting;Jia Jinhua;Liu Chuanyang;LiuWei;Zhang Shisong(Department of Pediatric Surgery,Affiliated Hospital of Shandong University Jinan Children's Hospital,Jinan 250022,China;Department of Pathology,Affiliated Hospital of Shandong University Jinan Children's Hospital,Jinan 250022,China)

机构地区：[1]山东大学附属儿童医院济南市儿童医院小儿外科,济南250022 [2]山东大学附属儿童医院济南市儿童医院病理科,济南250022

出　　处：《中华实验外科杂志》2024年第7期1621-1625,共5页Chinese Journal of Experimental Surgery

基　　金：山东省自然科学基金面上项目(ZR2022MH229);济南市卫生健康委员会科技计划项目(2023-1-53、2022-2-157);山东大学附属儿童医院2023年高层次科研计划项目(SDFE-GCC2023003)。

摘　　要：胆道闭锁(BA)是引起婴儿阻塞性黄疸最常见的原因, 其显著特征是肝外胆道进行性梗阻和肝纤维化。尽管葛西(Kasai)手术可以改善部分患儿的预后, 但并不能有效阻止肝纤维化的进展, 大多数患儿仍需肝移植才能长期存活。目前关于胆道闭锁肝纤维化的发生机制尚未完全明确。最新研究结果显示胆道闭锁肝纤维化的发生可能与遗传基因、胆道发育异常、病毒感染、自身免疫和炎性反应、上皮间质转化、血管系统等因素有关, 本文就胆道闭锁肝纤维化的发病机制进行综述, 对未来改善胆道闭锁患儿术后的长期生存率和减少肝移植具有重要意义。Biliary atresia is the most common cause of obstructive jaundice in infants,which is characterized by progressive extrahepatic biliary obstruction and liver fibrosis.Although Kasai procedure can improve the prognosis of some patients,it cannot effectively prevent the progression of liver fibrosis,and most patients still need liver transplan-tation for long-term survival.At present,the mechanism of liver fibrosis in biliary atresia is not completely clear.A new study has found that the occurrence of biliary atresia of liver fibrosis may be related to genetic,biliary dysplasia,virus infection,autoimmune,inflammation response,transformation of epithelial mesenchymal and vascular system and so on factors.This paper summarized the pathogenesis of biliary atresia of liver fibrosis,for future improving the long-term survival of patients with biliary atresia and reducing liver transplantation are of great significance.

关 键 词：胆道闭锁 肝纤维化 发病机制 遗传基因 

分 类 号：R575.2[医药卫生—消化系统]