Balistreri W F, Grand R, Hoofnagle J H, Suchy F J, Ryckman F C, Perlmutter D H, Sokol R J
Children's Hospital Medical Center, Cincinnati, OH 45229-3039, USA.
Hepatology. 1996 Jun;23(6):1682-92. doi: 10.1002/hep.510230652.
Biliary atresia (BA) is the end result of a destructive, inflammatory process that affects intra- and extrahepatic bile ducts, leading to fibrosis and obliteration of the biliary tract with the development of biliary cirrhosis. It is the commonest cause of chronic cholestasis in infants and children, and therefore is the most frequent indication for liver transplantation in this age group. The disease occurs worldwide, affecting an estimated 1 in 8,000 to 12,000 live births. At present, there is no specific therapy for BA; however, sequential surgical therapy begins with creation of a hepatoportoenterostomy (HPE); in those with end-stage liver disease, liver transplantation is indicated. Since most candidates are young children of small size, there is a shortage of size-matched donors for liver transplantation. At present, an increased awareness to ensure early diagnosis and development of methods to prevent progressive fibrosis are needed. These considerations are dependent on detailed studies of the pathogenesis of BA. Recent studies have focused on normal and altered bile duct morphogenesis and the role of various factors (infectious or toxic agents and metabolic insults) in isolation or in combination with a genetic or immunologic susceptibility in the etiology of BA.
胆道闭锁（BA）是一种破坏性炎症过程的最终结果，该过程会影响肝内和肝外胆管，导致纤维化以及胆道闭锁，并伴有胆汁性肝硬化的发展。它是婴幼儿慢性胆汁淤积最常见的原因，因此也是该年龄组中最常见的肝移植指征。该疾病在全球范围内均有发生，估计每8000至12000例活产中就有1例受影响。目前，尚无针对BA的特异性治疗方法；然而，序贯性手术治疗始于肝门空肠吻合术（HPE）的创建；对于终末期肝病患者，则需进行肝移植。由于大多数候选者是体型较小的幼儿，因此肝移植时缺乏尺寸匹配的供体。目前，需要提高对确保早期诊断的认识，并开发预防进行性纤维化的方法。这些考量取决于对BA发病机制的详细研究。最近的研究集中在正常和异常的胆管形态发生，以及各种因素（感染性或毒性因子以及代谢损伤）在BA病因中单独或与遗传或免疫易感性相结合所起的作用。
