Miller Phoebe N, Baskaran Suruthi, Nijagal Amar
Department of Surgery, University of California San Francisco, 505 Parnassus Avenue, San Francisco, CA 94143, USA.
Department of Surgery, University of Texas Health Science Center, 7703 Floyd Curl Drive San Antonio, TX 78229, USA.
Semin Pediatr Surg. 2024 Dec;33(6):151474. doi: 10.1016/j.sempedsurg.2025.151474. Epub 2025 Jan 18.
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.
胆道闭锁是一种导致肝衰竭的进行性新生儿胆管病。其特征为炎症介导的肝损伤，免疫系统在该疾病的发病机制中起关键作用。尽管在胆道闭锁动物模型中已涉及多种免疫细胞和介质，但新出现的文献反映了免疫反应各成分之间复杂的相互作用，这种相互作用导致了人类疾病的进展。需要针对免疫系统的新型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免疫系统中与胆道闭锁相关的成分的文献，以及免疫系统的主要分支——固有免疫和适应性免疫——之间丰富的相互作用，正是这种相互作用导致了该疾病的高度病态后果。
