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新冠疫情遇上囊性纤维化:是好是坏?

COVID-19 meets Cystic Fibrosis: for better or worse?

机构信息

Leeds Institute of Medical Research at St James's, University of Leeds, Leeds, UK.

Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK.

出版信息

Genes Immun. 2020 Aug;21(4):260-262. doi: 10.1038/s41435-020-0103-y. Epub 2020 Jul 1.

Abstract

Cystic fibrosis (CF) is one of the most common autosomal recessive life-limiting conditions affecting Caucasians. The resulting defect in the cystic fibrosis transmembrane conductance regulator protein (CFTR) results in defective chloride and bicarbonate secretion, as well as dysregulation of epithelial sodium channels (ENaC). These changes bring about defective mucociliary clearance, reduced airway surface liquid and an exaggerated proinflammatory response driven, in part, by infection. In this short article we explore the overlap in the pathophysiology of CF and COVID-19 infection and discuss how understanding the interaction between both diseases may shed light on future treatments.

摘要

囊性纤维化(CF)是影响白种人的最常见的常染色体隐性致死性疾病之一。囊性纤维化跨膜电导调节蛋白(CFTR)的缺陷导致氯离子和碳酸氢根离子分泌缺陷,以及上皮钠离子通道(ENaC)的失调。这些变化导致粘液纤毛清除功能障碍、气道表面液体减少和炎症反应过度,部分原因是感染。在这篇短文中,我们探讨了 CF 和 COVID-19 感染的病理生理学重叠,并讨论了理解这两种疾病之间的相互作用如何为未来的治疗提供启示。

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