Department of Respiratory Medicine, Alfred Health, Melbourne, VIC 3004, Australia.
Department of Medicine, Monash University, Alfred Campus, Melbourne, VIC 3004, Australia.
Int J Mol Sci. 2023 Feb 17;24(4):4052. doi: 10.3390/ijms24044052.
The interplay between airway inflammation and infection is now recognized as a major factor in the pathobiology in cystic fibrosis (CF). A proinflammatory environment is seen throughout the CF airway resulting in classic marked and enduring neutrophilic infiltrations, irreversibly damaging the lung. Although this is seen to occur early, independent of infection, respiratory microbes arising at different timepoints in life and the world environment perpetuate this hyperinflammatory state. Several selective pressures have allowed for the CF gene to persist until today despite an early mortality. Comprehensive care systems, which have been a cornerstone of therapy for the past few decades, are now revolutionized by CF transmembrane conductance regulator (CTFR) modulators. The effects of these small-molecule agents cannot be overstated and can be seen as early as in utero. For an understanding of the future, this review looks into CF studies spanning the historical and present period.
气道炎症和感染之间的相互作用现在被认为是囊性纤维化 (CF) 发病机制中的一个主要因素。在 CF 气道中可以看到一个促炎环境，导致典型的明显和持久的中性粒细胞浸润，不可逆转地损害肺部。尽管这种情况被认为发生在早期，与感染无关，但在不同时间点出现的呼吸道微生物和全球环境使这种过度炎症状态持续存在。一些选择压力使得 CF 基因得以持续存在，尽管早期死亡率很高。过去几十年的治疗基石是综合护理系统，现在 CF 跨膜电导调节剂 (CTFR) 调节剂正在彻底改变这一系统。这些小分子药物的效果怎么强调都不为过，甚至可以在子宫内就观察到。为了了解未来，本综述回顾了跨越历史和现在的 CF 研究。
