胆道闭锁
医学
吻合
黄疸
胆汁淤积
胆管
胃肠病学
内科学
新生儿胆汁淤积症
胎儿
闭锁
外科
肝移植
怀孕
移植
生物
遗传学
作者
David C. Trampert,Ulrich Beuers
标识
DOI:10.1016/j.jhep.2023.12.018
摘要
Biliary atresia (BA) is a neonatal fibro-obliterative disease of the extrahepatic bile ducts. BA manifests in affected children as early as a few days to weeks after birth with (sub)total biliary obstruction leading to cholestasis and – when left untreated – hepatic failure and ultimately death from end-stage liver disease.1 Clinical presentation includes neonatal jaundice with pale stools, conjugated hyperbilirubinemia and elevated plasma liver enzymes. Thus far, treatment is primarily surgical with the objective being to restore bile flow through a hepatoportoenterostomy with a Roux-en-Y anastomosis, known as the Kasai procedure.
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