Biliary atresia: East versus west

Biliary atresia: East versus west
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DOI:
10.1016/j.sempedsurg.2020.150950
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发表时间:
2020-08-01
影响因子:
1.7
通讯作者:
Tam, Paul Kwong Hang
Tam, Paul Kwong Hang
中科院分区:
医学4区
文献类型:
--
作者:
Chung, Patrick Ho Yu;Zheng, Shan;Tam, Paul Kwong Hang

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胆道闭锁(BA)是新生儿期手术黄疸的常见原因。目前,它被认为是一系列具有共同最终病理学特征的疾病,其特征是肝外胆道闭塞和缺乏正常分支的肝内胆管。尽管它是一种全球性疾病,在所有种族中都有发现,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(中国和日本)中更为常见,尽管这种综合征的关联性要少得多。许多东方国家都有西方国家没有的国家筛查计划,这可能是由于在发病率低的国家对其成本效益存在争议。葛西门肠造口术 (KPE) 被认为是 BA 的主要治疗方法,但其结果在整个地区仍不令人满意。鉴于BA的复杂性,仅靠个别国家的努力不可能取得战略进展,我们相信东西方之间的合作才是前进的方向。 (C) 2020 Elsevier Inc. 保留所有权利。
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. (C) 2020 Elsevier Inc. All rights reserved.