Advanced therapies for congenital biliary tract malformation:From bench to bedside
作者机构：People's HospitalZhejiang University School of MedicineHangzhou310006China Department of Hernia and Abdominal Wall SurgeryAffiliated Hangzhou First People's HospitalZhejiang University School of MedicineHangzhouChina Institute of Organ TransplantationZhejiang UniversityHangzhou310003China Westlake Laboratory of Life Sciences and BiomedicineHangzhou310024China
出 版 物：《iLIVER》 (国际肝胆健康（英文）)
年 卷 期：2022年第1卷第3期
页 面：159-168页
学科分类：1002[医学-临床医学] 100201[医学-内科学(含：心血管病、血液病、呼吸系病、消化系病、内分泌与代谢病、肾病、风湿病、传染病)] 10[医学]
基 金：supported by the State Key Program of National Natural Science Foundation of China(no.81930016) Major Research Plan of the National Natural Science Foundation of China(no.92159202) National Key Research and Development Program of China(no.2021YFA1100500) Young Program of National Natural Science Funds(no.82000617)
主 题：Biliary tract malformation Biliary atresia Alagille syndrome Pediatric liver transplantation Organoid Immunotherapy
摘 要：Congenital biliary tract malformations are a series of rare but extremely serious diseases that mainly include biliary atresia and biliary hypoplasia(referred to as Alagille syndrome).The rapid progression of biliary atresia and Alagille syndrome results in jaundice,cholestatic liver disease,cirrhosis,and even liver *** most cases,supportive or clinically specific therapies cannot achieve satisfactory ***,liver transplantation(especially living donor liver transplantation)may be *** many studies have elucidated the role of genetic factors and the molecular mechanism of congenital biliary tract malformations,experimental therapies such as organoid transplantation,cell therapy,and immunotherapy have been proved to be *** advanced methods have shown outstanding advantages,particularly in patients with end-stage biliary tract malformations,surgery failure,and other problems that cannot be solved by conventional *** review article discusses the potential pathogenesis of and promising therapeutic strategies for biliary tract malformations.
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