Biliary atresia (BA) is a fibroinflammatory cholangiopathy and portal venopathy. It is of unknown etiology and is associated with systemic immune dysregulation, in which the first insult begins before birth. Maternal microchimerism is a naturally occurring phenomenon during fetal life in which maternal alloantigens promote the development of tolerogenic fetal regulatory T-cells in utero. However, maternal cells may alter the fetus’s response to self-antigens and trigger an autoimmune response under certain histocompatibility combinations between the mother and the fetus. A recent report on a set of dizygotic discordant twins with BA, one of whose placentae showed villitis of unknown etiology, implies a certain immune-mediated conflict between the fetus with BA and the mother. Maternal chimeric cells persist postnatally for various time spans and can cause cholangitis, which ultimately leads to liver failure. In contrast, patients who eliminate maternal chimeric cells may retain their liver function.

胆道闭锁 (BA) 是一种纤维炎症性胆管病和门静脉病。它的病因不明，与全身免疫失调有关，其中第一次损伤在出生前就开始了。母体微嵌合体是胎儿生命中自然发生的现象，其中母体同种异体抗原促进耐受性胎儿调节性 T 细胞的发育在子宫内. 然而，母体细胞可能会改变胎儿对自身抗原的反应，并在母体和胎儿之间的某些组织相容性组合下引发自身免疫反应。最近关于一组异卵双胎 BA 的报告，其中一个胎盘显示病因不明的绒毛炎，这意味着患有 BA 的胎儿与母亲之间存在某种免疫介导的冲突。母体嵌合细胞在出生后持续存在不同的时间跨度，并可能导致胆管炎，最终导致肝功能衰竭。相反，消除母体嵌合细胞的患者可能保留其肝功能。