CF 肺生理学病理概述。

Overview of CF lung pathophysiology.

机构信息

Department of Life Sciences and Biotechnology, University of Ferrara, Ferrara, Italy; Center of Innovative Therapies for Cystic Fibrosis, University of Ferrara, Ferrara, Italy.

Department of Medical Sciences, University of Ferrara, Ferrara, Italy; Center of Innovative Therapies for Cystic Fibrosis, University of Ferrara, Ferrara, Italy.

出版信息

Curr Opin Pharmacol. 2022 Jun;64:102214. doi: 10.1016/j.coph.2022.102214. Epub 2022 Apr 19.

DOI:10.1016/j.coph.2022.102214

PMID:35453033

Abstract

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.

摘要

囊性纤维化跨膜电导调节因子（CFTR）蛋白缺陷影响气道表面液体中的氯离子、碳酸氢根离子、钠离子和水的动态平衡，影响黏液的组成和黏度，从而导致 CF 患者整个生命周期中严重的感染和炎症。CFTR 调节剂的引入，即直接干预以挽救 CFTR 蛋白功能的新型药物，开创了实验研究的新纪元。本文综述了最近在理解 CF 肺部感染和炎症病理特征方面的进展。