Unique manifestations of biliary atresia provide new immunological insight into its etiopathogenesis
Unique manifestations of biliary atresia provide new immunological insight into its etiopathogenesis
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DOI：
10.1007/s00383-017-4155-7
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发表时间：
2017-12-01
影响因子：
1.8
通讯作者：
Ieiri, Satoshi
中科院分区：
文献类型：
--
作者：
Muraji, Toshihiro;Ohtani, Haruo;Ieiri, Satoshi
胆道闭锁（BA）是一种独特的新生儿胆汁淤积性疾病，其背景是肝脏免疫反应过度，机制不明。目前已提出三种假说：病毒感染引发的自身免疫型胆管病、与母体微嵌合体相关的免疫介导的移植物抗宿主病和胆管板畸形学说。病毒感染理论的研究者们已经在实验上探索了导致这种疾病的小鼠模型上的胆管病变的免疫过程，而在母体微嵌合体假说中，在BA患者的肝脏中检测到母体细胞，其作用尚未确定。导管板畸形理论是一个有趣的假设，因为它表明这种疾病的发病是在怀孕的头三个月。这一理论可能是次要的这两个免疫相关的侮辱之一。在这篇综述中，四个独特的重点：（1）发病时间，（2）肝炎样病理图片，（3）不均匀的肝段萎缩时，先进的，（4）肿大的淋巴结在肝门。这些独特的临床病理学方面的这种疾病应该很好地解释这些假说。
Biliary atresia (BA) is a unique cholestatic disease of newborns with a background of exaggerated immune response in the liver of unknown mechanism. Three hypotheses have been proposed; autoimmune type of cholangiopathy triggered by virus infection, graft-versus-host disease type of immune-mediated disease associated with maternal microchimerism and ductal plate malformation theory. Researchers on virus infection theory have experimentally explored immune process causing cholangiopathy on murine models of this disease, while in maternal microchimerism hypothesis were detected maternal cells in the BA patients' liver, of which roles are yet to be determined. Ductal plate malformation theory is an intriguing hypothesis in the sense that it suggests the onset of this disease is in the first trimester. This theory can be secondary to either one of these two immune-related insults. In this review, four unique points are focused; (1) the timing of onset, (2) hepatitis-like pathological picture, (3) heterogenous atrophy of the liver segments when advanced, and (4) swollen lymph nodes at the porta hepatis. These unique clinicopahtological aspects of this disease should be well explained by these hypotheses.