先天性胆道闭锁（congenital biliary atresia，CBA）是一种新生儿早期胆管疾病，导致肝内肝外胆管不同程度的 闭锁并造成肝组织纤维化伴肝胆管不可逆性损伤。它是新生儿阻塞性黄疸和小儿肝移植最主要的原因。其发病机制目前 尚未研究清楚，是遗传与环境因素共同作用导致的复杂疾病。Hedgehog信号通路在哺乳动物胚胎发育、器官形成及发育 中有重要作用。近期研究发现，Hedgehog信号通路在CBA的发生过程中也发挥重要作用。Hedgehog通路的激活会影响胚 胎肝脏发育、增强肝胆管纤维化的发生并且阻碍胆管形态的形成，从而导致CBA的发生。文章重点介绍Hedgehog通路在 CBA形成过程中所起的重要作用。

　Congenital biliary atresia (CBA) is an early neonatal biliary disease, which results in different degrees of intrahepatic and extrahepatic biliary atresia and liver fibrosis accompanied by irreversible injury of hepatobiliary duct. It is the main cause of neonatal obstructive jaundice and liver transplantation in children. Its pathogenesis has not yet been delineated clearly, and it is a complex disease caused by the combination of genetic and environmental factors. Hedgehog signaling pathway plays an important role in mammalian embryonic development, organ formation and development. Recent studies have found that Hedgehog signaling pathway also plays an important role in the of CBA. Activation of Hedgehog pathway can affect embryonic liver development, enhance the hepatobiliary fibrosis and hinder the formation of bile duct morphology, leading to the development of CBA. This article focuses on the important role of Hedgehog pathway in the formation of CBA.