Petersen Claus
Department of Pediatric Surgery, Hannover Medical School, Carl-Neuberg Strasse 1, 30625 Hannover, Germany.
Clin Liver Dis. 2006 Feb;10(1):73-88, vi. doi: 10.1016/j.cld.2005.10.001.
Biliary atresia is the single most common cause of chronic cholestasis in children and is the leading indication for liver transplantation worldwide. It results from an inflammatory and fibrosing obstruction of the extrahepatic bile ducts in the first few months of life. Early diagnosis and timely surgical portoenterostomy are necessary for improved biliary drainage, but the liver disease progresses to end-stage biliary cirrhosis in most patients. Although the pathogenesis of disease is largely unknown, recent patient- and animal-based experiments indicate interactions between infectious agents and inflammatory circuits may be important pathogenic mechanisms of disease.
胆道闭锁是儿童慢性胆汁淤积最常见的单一病因，也是全球肝移植的主要指征。它是由出生后最初几个月肝外胆管的炎症性和纤维化性梗阻所致。早期诊断和及时进行手术门静脉肠吻合术对于改善胆汁引流是必要的，但大多数患者的肝病会进展为终末期胆汁性肝硬化。尽管该病的发病机制在很大程度上尚不清楚，但最近基于患者和动物的实验表明，感染因子与炎症通路之间的相互作用可能是该病重要的致病机制。
