Department of Pharmacology, National Research Institute for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo 157-8538, Japan.
J Hepatobiliary Pancreat Sci. 2013 Jun;20(5):459-64. doi: 10.1007/s00534-013-0604-4.
Biliary atresia (BA) is a progressive fibro-obliterative cholangiopathy affecting the extra- and intrahepatic biliary tree to various degrees and resulting in obstructive bile flow, cholestasis and icterus in neonates. It is the most common cause of pediatric liver transplantation. The etiology of BA is still unclear, although there is some evidence pointing to viral, toxic, and multiple genetic factors. For new therapeutic options other than liver transplantation to be developed, a greater understanding of the pathogenesis of BA is indispensable. The fact that the pathology of BA develops during a period of biliary growth and remodeling suggests an involvement of developmental anomalies. Recent studies indicate an association of the etiology of BA with some genetic factors such as laterality genes, epigenetic regulation and/or microRNA function. In this paper, we present an overview of recent advances in the understanding of the disease focusing on bile duct developmental anomaly.
先天性胆道闭锁（BA）是一种进行性纤维性阻塞性胆管病，可不同程度地影响肝内外胆管，导致新生儿胆汁流出受阻、胆汁淤积和黄疸。它是小儿肝移植最常见的原因。BA 的病因尚不清楚，尽管有一些证据表明与病毒、毒性和多种遗传因素有关。为了开发除肝移植以外的新治疗方法，必须更深入地了解 BA 的发病机制。BA 的病理学是在胆道生长和重塑过程中发展起来的，这表明其涉及发育异常。最近的研究表明，BA 的病因与一些遗传因素有关，如左右基因、表观遗传调控和/或 microRNA 功能。本文主要关注胆管发育异常，综述了近年来对该病发病机制的认识进展。
