Clues to the etiology of bile duct injury in biliary atresia.
Clues to the etiology of bile duct injury in biliary atresia.
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DOI：
10.1055/s-0032-1329899
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发表时间：
2012-11
影响因子：
4.2
通讯作者：
Sokol RJ
中科院分区：
文献类型：
--
作者：
Mack CL;Feldman AG;Sokol RJ
胆道闭锁（BA）是一种病因不明的婴儿梗阻性胆管病，治疗效果不佳，占所有小儿肝移植的40 - 50%。尽管BA中胆管损伤的病因不明，但据推测，产前或围产期病毒感染启动胆管细胞凋亡和抗原释放，触发Th 1免疫应答，导致进一步的胆管损伤、炎症和阻塞性纤维化。体液免疫和先天免疫系统的激活也可能在这一过程中发挥关键作用。此外，最近的研究表明，从小鼠BA模型和人类的数据表明，调节性T细胞和遗传易感性因子可能协调自身免疫机制。是什么控制了这些事件的协调，为什么这种疾病只发生在生命的最初几个月，以及为什么少数围产期病毒感染的婴儿会发展为BA，这些都是有待回答的问题。
Biliary atresia (BA) is an infantile obstructive cholangiopathy of unknown etiology with suboptimal therapy, which is responsible for 40 to 50% of all pediatric liver transplants. Although the etiology of bile duct injury in BA in unknown, it is postulated that a pre- or perinatal viral infection initiates cholangiocyte apoptosis and release of antigens that trigger a Th1 immune response that leads to further bile duct injury, inflammation, and obstructive fibrosis. Humoral immunity and activation of the innate immune system may also play key roles in this process. Moreover, recent investigations from the murine BA model and human data suggest that regulatory T cells and genetic susceptibility factors may orchestrate autoimmune mechanisms. What controls the coordination of these events, why the disease only occurs in the first few months of life, and why a minority of infants with perinatal viral infections develop BA are remaining questions to be answered.
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影响因子：
3.1
作者：
Arikan, Cigdem;Berdeli, Afig;Aydogdu, Sema
通讯作者：
Aydogdu, Sema
影响因子：
2.4
作者：
Davenport, M;Gonde, C;Howard, ER
通讯作者：
Howard, ER
DOI：
10.1046/j.0106-9543.2002.01647.x
发表时间：
2002-06-01
期刊：
LIVER
影响因子：
--
作者：
Donaldson, PT;Clare, M;Kelley, D
通讯作者：
Kelley, D
DOI：
10.1097/01.mpg.0000192247.55583.fa
发表时间：
2006-01-01
影响因子：
2.9
作者：
Arikan, C;Berdeli, A;Aydogdu, S
通讯作者：
Aydogdu, S
影响因子：
29.4
作者：
CHANG, MH;HUANG, HH;LEE, CY
通讯作者：
LEE, CY