Boucher Richard C
Cystic Fibrosis Pulmonary Research and Treatment Center and the UNC Virtual Lung Group, The University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
Trends Mol Med. 2007 Jun;13(6):231-40. doi: 10.1016/j.molmed.2007.05.001. Epub 2007 May 23.
Cystic fibrosis (CF) lung disease involves chronic bacterial infection of retained airway secretions (mucus). Recent data suggest that CF lung disease pathogenesis reflects the vulnerability of airway surfaces to dehydration and collapse of mucus clearance. This predisposition is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, resulting in (i) the absence of CFTR-mediated Cl- secretion and regulation of epithelial Na+ channel (ENaC) function; and (ii) the sole dependence on extracellular ATP to rebalance these ion transport processes through P2 purinoceptor signaling. Recent clinical studies indicate that inhalation of hypertonic saline osmotically draws sufficient water onto CF airway surfaces to provide clinical benefit.
囊性纤维化（CF）肺部疾病涉及呼吸道潴留分泌物（黏液）的慢性细菌感染。近期数据表明，CF肺部疾病的发病机制反映出气道表面易于脱水以及黏液清除功能衰退。这种易感性是由囊性纤维化跨膜传导调节因子（CFTR）基因突变引起的，导致（i）缺乏CFTR介导的氯离子分泌以及上皮钠通道（ENaC）功能的调节；（ii）完全依赖细胞外ATP通过P2嘌呤受体信号来重新平衡这些离子转运过程。近期临床研究表明，吸入高渗盐水可通过渗透作用将足够的水分吸引至CF气道表面，从而带来临床益处。
