CAJ | 학술논문

胆道闭锁（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表达增高，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、氧代谢产物和细胞因子，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。
담도폐쇄（BA）시엄중위급영유인생명건강적소화계통질병지일，만기간장섬유화시도치환인사망적주요원인。재담도폐쇄발병과정중，병독감염가유도일계렬면역화염증반응，도치조절성T세포（Treg세포）감소，CD14표체증고，다충염증통로이급전화생장인자-β（TGF-β）/Smad2/3촉섬유화통로피격활。격활적통로산생대량염성개질손상간세포화담관세포，석방각충촉염인자、양대사산물화세포인자，진일보가중간、담계통손상조성간세포내배경실형，실형적내배경반수간실질세포、간거서세포、간내취집적염성세포등발생괄응성변성、배사、증생，도치간성형세포（HSCs）격활，HSCs전화위성섬유세포，촉진간섬유화진정。면역、염증손상、촉섬유화통로시도치담도폐쇄간섬유화간경화적삼대중요인소。
Biliary atresia (BA) is one of the most serious digestive system diseases, which threatens the health of infants. Liver fibrosis is a major cause of death in children with BA. In the process of the pathogenesis of BA, virus infection can in?duce a series of immune and inflammatory reaction, result in a decrease of regulatory T cells (Treg cells) and high expression of CD14, activating a variety of inflammatory pathways and TGF-β/Smad2/3 pro-fibrogenic pathway, which produces a large number of medium damage of liver cells and bile duct cells, releases proinflammatory factor, oxygen metabolism matter and cytokines. These changes further aggravate damage of hepatobiliary system and cause the internal environment imbalance of liver parenchyma cells. The imbalance of internal environment with adaptive degeneration and necrosis in liver parenchyma cells, hepatic macrophages and gathered inflammatory cells leads to the activation of hepatic stellate cells (HSCs). HSCs can be converted into fibroblast cells, and 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.