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iLIVER 2022, 1(3): 159-168 https://doi.org/10.1016/j.iliver.2022.08.003
Review | Open Access | Issue | Published: 30 August 2022

Advanced therapies for congenital biliary tract malformation: From bench to bedside

Show Author's Information Yixuan Shaoa,1,  Xinyu Yanga,c,d,1,  Hao Chena,c,d,  Di Lua,c,d,  Yonggang Huanga,b( ),  Xiao Xua,c,d ( )
Key Laboratory of Integrated Oncology and Intelligent Medicine of Zhejiang Province, Department of Hepatobiliary and Pancreatic Surgery, Affiliated Hangzhou First People's Hospital, Zhejiang University School of Medicine, Hangzhou, 310006, China
Department of Hernia and Abdominal Wall Surgery, Affiliated Hangzhou First People's Hospital, Zhejiang University School of Medicine, Hangzhou, China
Institute of Organ Transplantation, Zhejiang University, Hangzhou, 310003, China
Westlake Laboratory of Life Sciences and Biomedicine, Hangzhou, 310024, China

1 These authors contributed equally to this work.

Keywords:
Immunotherapy, Biliary tract malformation, Biliary atresia, Alagille syndrome, Pediatric liver transplantation, Organoid
Cite this article:
Shao Y, Yang X, Chen H, et al. Advanced therapies for congenital biliary tract malformation: From bench to bedside. iLIVER, 2022, 1(3): 159-168. https://doi.org/10.1016/j.iliver.2022.08.003
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Abstract Full text About this article

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 failure. In most cases, supportive or clinically specific therapies cannot achieve satisfactory outcomes. Therefore, liver transplantation (especially living donor liver transplantation) may be required. As 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 feasible. These 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 therapies. This review article discusses the potential pathogenesis of and promising therapeutic strategies for biliary tract malformations.


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About this article

Advanced therapies for congenital biliary tract malformation: From bench to bedside

Show Author's information Yixuan Shaoa,1, Xinyu Yanga,c,d,1, Hao Chena,c,d, Di Lua,c,d, Yonggang Huanga,b( ), Xiao Xua,c,d ( )
Key Laboratory of Integrated Oncology and Intelligent Medicine of Zhejiang Province, Department of Hepatobiliary and Pancreatic Surgery, Affiliated Hangzhou First People's Hospital, Zhejiang University School of Medicine, Hangzhou, 310006, China
Department of Hernia and Abdominal Wall Surgery, Affiliated Hangzhou First People's Hospital, Zhejiang University School of Medicine, Hangzhou, China
Institute of Organ Transplantation, Zhejiang University, Hangzhou, 310003, China
Westlake Laboratory of Life Sciences and Biomedicine, Hangzhou, 310024, China

1 These authors contributed equally to this work.

Abstract

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 failure. In most cases, supportive or clinically specific therapies cannot achieve satisfactory outcomes. Therefore, liver transplantation (especially living donor liver transplantation) may be required. As 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 feasible. These 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 therapies. This review article discusses the potential pathogenesis of and promising therapeutic strategies for biliary tract malformations.

Keywords: Immunotherapy, Biliary tract malformation, Biliary atresia, Alagille syndrome, Pediatric liver transplantation, Organoid

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Publication history

Received: 22 June 2022
Revised: 19 July 2022
Accepted: 02 August 2022
Published: 30 August 2022
Issue date: September 2022

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© 2022 The Author(s). Published by Elsevier Ltd on behalf of Tsinghua University Press.

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