Biliary Atresia in Children: Update on Disease Mechanism, Therapies, and Patient Outcomes.
Biliary Atresia in Children: Update on Disease Mechanism, Therapies, and Patient Outcomes.
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DOI：
10.1016/j.cld.2022.03.001
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发表时间：
2022-08
影响因子：
5.1
通讯作者：
中科院分区：
文献类型：
--
作者：
胆道闭锁是一种罕见的疾病，但仍然是儿科肝移植最常见的适应症，因为诊断后没有有效的药物治疗来减缓进展。遗传、免疫和环境因素的不同作用导致胆道闭锁患者的疾病异质性。对疾病机制的深入了解将有助于开发有针对性的药物治疗并改善患者的预后。
Biliary atresia is a rare disease but remains the most common indication for pediatric liver transplantation as there are no effective medical therapies to slow progression after diagnosis. Variable contribution of genetic, immune, and environmental factors contributes to disease heterogeneity among patients with biliary atresia. Developing a deeper understanding of the disease mechanism will help develop targeted medical therapies and improve patient outcomes.