{"title":"Immunology of Biliary Atresia.","authors":"Phoebe N Miller, Suruthi Baskaran, Amar Nijagal","doi":"10.1016/j.sempedsurg.2025.151474","DOIUrl":null,"url":null,"abstract":"<p><p>Biliary atresia is a progressive neonatal cholangiopathy that leads to liver failure. Characterized by inflammation-mediated liver injury, the immune system plays a critical role in the pathogenesis of this disease. Though several types of immune cells and mediators have been implicated in animal models of biliary atresia, emerging literature reflects the complex interplay of components of the immune response that contributes to disease progression in humans. Novel therapies targeting the immune system are needed to mitigate the devastating effects of biliary atresia. This review highlights the current literature on the components of the immune system that have been in implicated in biliary atresia and the rich interplay between the major arms of the immune system- innate and adaptive immunity- to cause the highly morbid consequences of this disease.</p>","PeriodicalId":49543,"journal":{"name":"Seminars in Pediatric Surgery","volume":"33 6","pages":"151474"},"PeriodicalIF":1.4000,"publicationDate":"2025-01-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Seminars in Pediatric Surgery","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1016/j.sempedsurg.2025.151474","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"PEDIATRICS","Score":null,"Total":0}
引用次数: 0
Abstract
Biliary atresia is a progressive neonatal cholangiopathy that leads to liver failure. Characterized by inflammation-mediated liver injury, the immune system plays a critical role in the pathogenesis of this disease. Though several types of immune cells and mediators have been implicated in animal models of biliary atresia, emerging literature reflects the complex interplay of components of the immune response that contributes to disease progression in humans. Novel therapies targeting the immune system are needed to mitigate the devastating effects of biliary atresia. This review highlights the current literature on the components of the immune system that have been in implicated in biliary atresia and the rich interplay between the major arms of the immune system- innate and adaptive immunity- to cause the highly morbid consequences of this disease.
期刊介绍:
Seminars in Pediatric Surgery provides current state-of-the-art reviews of subjects of interest to those charged with the surgical care of young patients. Each bimontly issue addresses a single topic with articles written by the experts in the field. Guest editors, all noted authorities, prepare each issue.