Cystic fibrosis (CF) lung disease reflects the failure of airways defense against chronic bacterial infection. Studies of CF cultures, transgenic mice, and CF patients suggest that the initiating event in CF airways disease pathogenesis is reduced airway surface liquid (ASL) volume, i.e., dehydration. CF ASL volume regulation depends on a single extracellular signaling system, ATP, which renders CF airways more vulnerable to disease-causing insults (e.g., viruses) than are normal airways, which regulate ASL volume by dual ATP and adenosine signaling pathways. Clinical studies have explored the hypothesis that treating the dehydration of CF airways will be therapeutically beneficial. Inhaled hypertonic saline osmotically draws water onto airway surfaces, improves mucus clearance and pulmonary function, and reduces acute exacerbations in CF patients. Thus, rehydration therapies may slow the progression of CF lung disease in patients with established bacterial infection and may prevent the onset of CF lung disease if initiated early in life.

中文翻译：

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囊性纤维化中气道表面脱水：发病机理和治疗方法。

囊性纤维化（CF）肺部疾病反映了针对慢性细菌感染的气道防御能力下降。对CF培养物，转基因小鼠和CF患者的研究表明，CF气道疾病发病机制中的起始事件是气道表面液（ASL）体积减少，即脱水。CF ASL的体积调节取决于单个细胞外信号系统ATP，这使CF气道比通过双ATP和腺苷信号通路调节ASL体积的正常气道更容易遭受致病性侵害（例如病毒）。临床研究已经探索了以下假设：治疗CF气道脱水将对治疗有益。吸入高渗盐水将水渗透到气道表面，改善粘液清除率和肺功能，并减少CF患者的急性加重。