Recent advancements in laboratory screening, diagnosis, and prognosis of biliary atresia: a literature review

B. Sun, Sarah Kelleher, Celia Short, Patricio Arias Valencia, J. Zagory
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引用次数: 0

Abstract

Biliary atresia (BA) is a neonatal cholangiopathy associated with fibrotic obliteration of the extrahepatic biliary tree leading to profound cholestasis and progressive liver failure (1,2). The incidence of BA is variable, estimated to range from 1 in 3,000 to 1 in 18,000 live births, and is more common in Taiwan, China, and Japan (3-6). Although relatively rare, BA is the most common cause of liver failure in children and is the leading indication for pediatric liver transplant (5,7-11). Unfortunately, without treatment, BA is universally lethal by 2 years of age (6,12). Aside from liver transplantation, the only treatment for BA is the Kasai portoenterostomy (KPE), a surgical procedure Review Article
胆道闭锁实验室筛查、诊断及预后的最新进展:文献综述
胆道闭锁(BA)是一种新生儿胆管疾病,与肝外胆道树纤维化闭塞相关,可导致重度胆汁淤积和进行性肝功能衰竭(1,2)。BA的发病率是可变的,估计范围从1 / 3000到1 / 18000活产婴儿,在台湾、中国大陆和日本更为常见(3-6)。虽然相对罕见,但BA是儿童肝功能衰竭的最常见原因,也是儿童肝移植的主要适应症(5,7-11)。不幸的是,如果不进行治疗,BA在2岁时普遍是致命的(6,12)。除了肝移植,治疗BA的唯一方法是Kasai门肠造口术(KPE),这是一种外科手术
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