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先天性胆道闭锁:东西方比较。

Biliary atresia: East versus west.

机构信息

Division of Paediatric Surgery, Department of Surgery, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong, China.

Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect, Shanghai, China.

出版信息

Semin Pediatr Surg. 2020 Aug;29(4):150950. doi: 10.1016/j.sempedsurg.2020.150950. Epub 2020 Jul 23.

DOI:10.1016/j.sempedsurg.2020.150950
PMID:32861448
Abstract

Biliary atresia (BA) is a common cause of surgical jaundice during the neonatal period. It is currently considered as a spectrum of diseases with a common final pathology characterized by obliteration of the extrahepatic biliary tract and the absence of normally branching intrahepatic ducts. Though it is a global disease that can be found in all ethnicities there are some clear differences between BA arising in the East and the West. This is likely to be related to different genetic, environmental and cultural factors. BA is more frequently found in Far Eastern infants (both Chinese and Japanese) though the syndromic associations are much less common. Many Eastern countries have national screening programmes not seen in the West possibly due to debate over its cost effectiveness in countries where incidence is low. Kasai portoenterostomy (KPE) is considered as the primary treatment of BA but its outcome still remains unsatisfactory across the region. Given the complexity of BA, it is unlikely that strategic advances could be made by the sole effort of individual countries and we believe that collaboration between the East and West is the way forward.

摘要

先天性胆道闭锁(BA)是新生儿期外科性黄疸的常见原因。目前认为该病是一种具有共同终末病理表现的疾病谱,其特征为肝外胆管闭塞和正常分支的肝内胆管缺失。尽管该病是一种在所有种族中都可发现的全球性疾病,但东方和西方的 BA 之间存在一些明显的差异。这可能与不同的遗传、环境和文化因素有关。BA 在远东婴儿(包括中国和日本婴儿)中更为常见,尽管综合征相关性要少得多。许多东方国家都有全国性的筛查计划,而西方则没有,这可能是因为在发病率较低的国家,其成本效益存在争议。葛西手术(KPE)被认为是 BA 的主要治疗方法,但该手术在该地区的效果仍然不尽如人意。鉴于 BA 的复杂性,仅靠个别国家的努力不太可能取得战略进展,我们认为东方和西方之间的合作是前进的方向。

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