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An overview of the pathogenesis of cystic fibrosis lung disease.

机译：囊性纤维化肺病的发病机理概述。

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摘要

The pathogenesis of cystic fibrosis (CF) lung disease is reviewed, focusing on an overview of the physiologic mechanisms that regulate mucus transport. A major emphasis is placed on the active transport systems that regulate the airway surface liquid (ASL) volume and, particularly, regulate the volume of the periciliary liquid (PCL) layer. A sequence is developed for CF whereby there is a depletion of the PCL that reflects the combined dysfunctions of accelerated Na(+)-dependent volume absorption and failure to secrete Cl(-). Both dysfunctions are a direct consequence of missing cystic fibrosis transmembrane conductance regulator (CFTR) at the apical membrane of airway epithelial cells. PCL depletion leads to failure of mucus transport, which is associated with persistent mucin secretion and formation of adherent mucus plaques and plugs. These plugs become the nidus for persistent bacterial airway infections that ultimately lead to a markedly anaerobic luminal environment.
机译：综述了囊性纤维化（CF）肺病的发病机理，重点是调节粘液运输的生理机制的概述。主要重点放在主动运输系统上，该系统调节气道表面液体（ASL）的体积，尤其是调节周液（PCL）层的体积。为CF开发了一个序列，其中PCL耗竭，反映了Na（+）依赖的加速体积吸收和无法分泌Cl（-）的综合功能障碍。两种功能障碍都是气道上皮细胞根尖膜囊性纤维化跨膜电导调节剂（CFTR）缺失的直接结果。 PCL耗竭导致粘液运输失败，这与粘液持续分泌以及粘液斑块和栓塞形成有关。这些堵塞物成为持久性细菌气道感染的病菌，最终导致明显的厌氧腔环境。

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