Recent advances in understanding biliary atresia.
Recent advances in understanding biliary atresia.
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DOI：
10.12688/f1000research.16732.1
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发表时间：
2019-01-01
期刊：
影响因子：
--
通讯作者：
Wells, Rebecca G
中科院分区：
文献类型：
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作者：
Wehrman, Andrew;Waisbourd-Zinman, Orith;Wells, Rebecca G
胆道闭锁（BA）是一种新生儿肝脏疾病，其特征是肝外胆管树进行性阻塞和纤维化以及肝实质纤维化和炎症。最近的研究发现，患有胆管炎的婴儿在出生后的最初几天内直接胆红素水平会升高，这表明这种疾病是在子宫内开始的。然而，BA 的病因和发病机制仍不清楚。在这里，我们讨论最近研究检查 BA 的潜在发病机制，包括遗传易感性、免疫系统的参与以及病毒和毒素等环境损伤，尽管可能不是单一的病因，而是一大群损伤性损伤导致肝外胆管梗阻和肝纤维化的最终共同途径。在过去十年中，BA 的管理和诊断并没有取得显着进展，但鉴于最近在了解 BA 发生时间和潜在发病机制方面取得的进展，我们希望未来十年能够带来早期诊断和新颖的治疗方法。
Biliary atresia (BA) is a neonatal liver disease characterized by progressive obstruction and fibrosis of the extrahepatic biliary tree as well as fibrosis and inflammation of the liver parenchyma. Recent studies found that infants who will go on to develop BA have elevated direct bilirubin levels in the first few days of life, suggesting that the disease starts in utero. The etiology and pathogenesis of BA, however, remain unknown. Here, we discuss recent studies examining potential pathogenetic mechanisms of BA, including genetic susceptibility, involvement of the immune system, and environmental insults such as viruses and toxins, although it is possible that there is not a single etiological agent but rather a large group of injurious insults that result in a final common pathway of extrahepatic bile duct obstruction and liver fibrosis. The management and diagnosis of BA have not advanced significantly in the past decade, but given recent advances in understanding the timing and potential pathogenesis of BA, we are hopeful that the next decade will bring early diagnostics and novel therapeutics.