Defects of the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) protein affect the homeostasis of chloride, bicarbonate, sodium, and water in the airway surface liquid, influencing the mucus composition and viscosity, which induces a severe condition of infection and inflammation along the whole life of CF patients. The introduction of CFTR modulators, novel drugs directly intervening to rescue the function of CFTR protein, opens a new era of experimental research. The review summarizes the most recent advancements to understand the characteristics of the infective and inflammatory pathology of CF lungs.

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CF肺病理生理学概述

囊性纤维化 (CF) 跨膜电导调节 (CFTR) 蛋白的缺陷会影响气道表面液体中氯化物、碳酸氢盐、钠和水的稳态，影响粘液成分和粘度，从而导致严重的感染和炎症CF 患者的一生。CFTR调节剂的引入，直接干预以挽救CFTR蛋白功能的新药，开启了实验研究的新纪元。该综述总结了了解 CF 肺感染性和炎症性病理学特征的最新进展。