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

COVID-19 meets Cystic Fibrosis: for better or worse?
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DOI:
10.1038/s41435-020-0103-y
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发表时间:
2020-07-01
期刊:
影响因子:
5
通讯作者:
Mehta, Anil
Mehta, Anil
中科院分区:
医学3区
文献类型:
--
作者:
Peckham, Daniel;McDermott, Michael F.;Mehta, Anil

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囊性纤维化(CF)是影响高加索人的最常见的常染色体隐性遗传性生命限制性疾病之一。囊性纤维化跨膜传导调节蛋白(CFTR)的缺陷导致氯化物和碳酸氢盐分泌缺陷,以及上皮钠通道(ENaC)失调。这些变化导致粘膜纤毛清除缺陷、气道表面液体减少和部分由感染驱动的过度促炎反应。在这篇简短的文章中,我们探讨了CF和COVID-19感染的病理生理学的重叠,并讨论了如何理解这两种疾病之间的相互作用可能会为未来的治疗提供启示。
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.