胆道闭锁肝纤维化与自体肝生存关系的研究进展

Relationship between liver fibrosis and native liver survival in infants with biliary atresia

胆道闭锁是婴儿期最严重的肝胆系统疾病之一。肝门-空肠吻合术(Kasai手术)是治疗胆道闭锁的主要手段,但术后自体肝长期生存效果不佳,大部分患儿需要通过肝移植来挽救生命。肝纤维化是影响胆道闭锁患儿自体肝生存的重要因素之一,其发生机制复杂,涉及多种信号通路及细胞因子的调控。Kasai手术后胆汁引流不畅、胆管炎发作都会导致肝纤维化进展,进而发生肝硬化。在完善早期诊断、早期手术、通畅引流胆汁及控制胆管炎的同时,还应积极应对患儿肝纤维化的持续进展,以期达到自体肝的长期生存。

Biliary atresia(BA) is one of the most serious hepatobiliary diseases during infancy.Kasai portoenterostomy has been a standard treatment for BA.However,its long-term postoperative survival of native liver is poor.Most children require liver transplantation for saving their lives.Liver fibrosis is one of the important influencing factors for native liver survival in BA children.The underlying mechanism is so complex as to involve a large variety of signaling pathways and cytokines.Imperfect biliary drainage and postoperative cholangitis may lead to liver fibrogenesis and even cirrhosis.For achieving long-term native liver survival,we should actively intervene to arrest the progression of liver fibrosis.