Evidence for airway surface dehydration as the initiating event in CF airway disease
Evidence for airway surface dehydration as the initiating event in CF airway disease
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DOI：
10.1111/j.1365-2796.2006.01744.x
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发表时间：
2007-01-01
影响因子：
11.1
通讯作者：
Boucher, R. C.
中科院分区：
文献类型：
--
作者：
Boucher, R. C.
囊性纤维化（CF）肺部疾病反映气道管腔持续细菌感染。已经提出了几种假设，将CFTR基因突变与CF肺保护自身免受细菌感染的失败联系起来。目前最有成效的假设之一是“气道表面液体（ASL）体积低”或“脱水”假设。这一假设预测气道表面脱水会产生CF的粘液粘连、炎症和细菌生物膜形成特征。吸入高渗盐水的临床试验已经证明了为CF气道表面补水的操作的治疗效果。
Cystic fibrosis (CF) lung disease reflects persistent bacterial infection of airway lumens. Several hypotheses have been advanced to link mutations in the CFTR gene to the failure of the CF lung to defend itself against bacterial infection. Amongst the most productive hypotheses at present is the ''low airway surface liquid (ASL) volume'' or ''dehydration'' hypothesis. This hypothesis predicts that airway surface dehydration produces the mucus adhesion, inflammation, and bacterial biofilm formation characteristic of CF. Clinical trials of inhaled hypertonic saline have demonstrated therapeutic benefit of manoeuvres designed to rehydrate CF airway surfaces.