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首页> 外文期刊>American Journal of Physiology >Understanding the cellular mechanism for inhaled hyperosmotic saline therapy for patients with cystic fibrosis. Focus on 'effect of apical hyperosmotic sodium challenge and amiloride on sodium transport in human bronchial epithelial cells from cystic fibrosis donors'
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Understanding the cellular mechanism for inhaled hyperosmotic saline therapy for patients with cystic fibrosis. Focus on 'effect of apical hyperosmotic sodium challenge and amiloride on sodium transport in human bronchial epithelial cells from cystic fibrosis donors'

机译：了解囊性纤维化患者吸入高渗盐水疗法的细胞机制。专注于“根尖性高渗钠挑战和阿米洛利对囊性纤维化供体的人支气管上皮细胞钠运输的影响”

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摘要

since the first clinical trials on inhaled hyperosmotic saline therapy (HS) in cystic fibrosis (CF) patients (3, 9), HS has proven to substantially improve a number of critical measures of lung function and, thus, is now considered a first-line therapy for these patients (2). One mechanism believed to link CF genotype to phenotype is abnormalities in properties of the airway surface liquid (ASL). Understanding how important ASL is to airway hydra-tion and mucociliary clearance has constituted a driver for extensive study of airway epithelia. Airway surfaces are covered with ASL, a thin layer of fluid consisting of periciliary liquid or sol layer with a viscous gel layer above. ASL functions to trap particulate matter in the mucus layer and move the contents cephalad by ciliary activity so that ultimately the contents are swallowed or expectorated. In normal airways, the surface epithelium and submucosal glands generate ASL, which contains, in addition to ions, antimicrobial and immune defense molecules (Fig. 1). In normal airways, the volume of ASL is a function of the highly water-permeable airway epithelia and active transport processes that control NaCl mass on airway surfaces. Isotonic ASL is maintained by absorbing Na~+ through sodium channels (ENaC) and Cl~ via the paracellular pathway with water moving passively by osmosis through airway epithelial water channels.
机译：自从首次在囊性纤维化（CF）患者中进行吸入性高渗盐水治疗（HS）的临床试验以来（3，9），HS已被证明可以显着改善许多关键的肺功能指标，因此，现在被认为是首例-这些患者的线疗法（2）。认为将CF基因型与表型联系起来的一种机制是气道表面液（ASL）的性质异常。了解ASL对气道水化和粘膜纤毛清除的重要性已成为广泛研究气道上皮细胞的动力。气道表面被ASL覆盖，ASL是一薄层流体，由胶质液体或溶胶层组成，上面有粘性凝胶层。 ASL的功能是将颗粒物捕获在粘液层中，并通过纤毛活动将内容物移至头侧，从而最终将内容物吞咽或吐出。在正常的气道中，表面上皮和粘膜下腺会产生ASL，ASL除含有离子外还含有抗菌和免疫防御分子（图1）。在正常的气道中，ASL的体积是高度透水的气道上皮和控制气道表面NaCl质量的主动转运过程的函数。等渗的ASL通过经由钠离子通道（ENaC）吸收Na〜+和经由细胞旁途径的Cl〜来维持，水通过渗透作用通过气道上皮水通道被动地移动。

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