天津医药 ›› 2015, Vol. 43 ›› Issue (1): 4-7.doi: 10.3969/j.issn.0253-9896.2015.01.002

• 专题研究-胆道闭锁 • 上一篇    下一篇

胆道闭锁肝纤维化研究进展

丁美云1,詹江华2   

  1. 1. 天津医科大学研究生院
    2. 天津市儿童医院外科
  • 收稿日期:2014-08-04 修回日期:2014-11-10 出版日期:2015-01-15 发布日期:2015-01-30
  • 通讯作者: 詹江华 E-mail:zhanjianghuatj@163.com
  • 基金资助:
    天津市卫生局重点攻关项目

The Advances of Liver Fibrosis In Biliary Atresia Research

  • Received:2014-08-04 Revised:2014-11-10 Published:2015-01-15 Online:2015-01-30
  • Contact: zhanjianghua E-mail:zhanjianghuatj@163.com

摘要: 胆道闭锁(biliary atresia, BA)是严重危及婴幼儿生命健康的消化系统疾病之一,晚期肝脏纤维化是导致患儿死亡的主要原因。在胆道闭锁发病过程中,病毒感染可诱导一系列免疫和炎症反应,导致调节性T细胞(Treg细胞)减少,CD14高表达,多种炎症通路以及转化生长因子-β(TGF-β)/ Smad2/3促纤维化通路被激活。激活的通路产生大量炎性介质损伤肝细胞和胆管细胞,释放各种促炎因子、氧代谢物质和细胞因子,进一步加重肝、胆系统损伤造成肝细胞内环境失衡,失衡的内环境伴随肝实质细胞、肝巨噬细胞、肝内聚集的炎性细胞等发生适应性变性、坏死、增生,导致肝星形细胞(HSCs)激活,HSCs转化为成纤维细胞,促进肝纤维化进程。免疫、炎症损伤、促纤维化通路是导致胆道闭锁肝纤维化肝硬化的三大重要因素。

关键词: 胆道闭锁, 肝硬化, T淋巴细胞, 调节性, 转化生长因子β, 抗原, CD14, Smad蛋白质类

Abstract: Biliary atresia is one of the most serious digestive system diseases, which is threatening the health of infants, and liver fibrosis is a major cause of death in children. In the process of the pathogenesis of biliary atresia, virus damage the body induced a series of immune and inflammatory reaction results in a decrease of regulatory T cells (Treg cells); high expression of CD14, activating a variety of inflammatory pathways and TGF-β/Smad2/3 pro-fibrogenic pathway, produced a large number of medium damage of liver cells and bile duct cells, release of proinflammatory factor, oxygen metabolism matter and cytokines, etc., which further damage hepatobiliary system; Imbalance of internal environment with liver parenchyma cells, hepatic macrophages, gathered in the liver of inflammatory cells, being such as adaptive degeneration, necrosis, hyperplasia, activating hepatic satellite cells (HSCs) and then HSCs into fibroblasts promote the process of liver fibrosis. Immune and inflammatory lesions, pro-fibrogenic pathway are the important factors in contributing to liver fibrosis and cirrhosis of biliary atresia.

Key words: biliary atresia, liver cirrhosis, T-lymphocytes, regulatory, transforming growth factor beta, antigens, CD14, Smad proteins