Immunology of Biliary Atresia

IF 2.5 3区 医学 Q3 PEDIATRICS Seminars in Pediatric Surgery Pub Date : 2024-12-01 DOI:10.1016/j.sempedsurg.2025.151474
Phoebe N Miller , Suruthi Baskaran , Amar Nijagal
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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.
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胆道闭锁的免疫学。
胆道闭锁是一种进行性新生儿胆管疾病,可导致肝功能衰竭。以炎症介导的肝损伤为特征,免疫系统在该病的发病机制中起关键作用。虽然几种类型的免疫细胞和介质与胆道闭锁的动物模型有关,但新兴文献反映了免疫反应组成部分的复杂相互作用,有助于人类疾病的进展。需要针对免疫系统的新疗法来减轻胆道闭锁的破坏性影响。本综述重点介绍了目前有关胆道闭锁的免疫系统组成部分的文献,以及免疫系统主要分支(先天免疫和适应性免疫)之间丰富的相互作用,这些相互作用导致了胆道闭锁的高度病态后果。
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来源期刊
Seminars in Pediatric Surgery
Seminars in Pediatric Surgery PEDIATRICS-SURGERY
CiteScore
2.80
自引率
5.90%
发文量
57
审稿时长
>12 weeks
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.
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