Blankenship Sydney, Landis Aaron R, Harrison Williams Emily, Peabody Lever Jacelyn E, Garcia Bryan, Solomon George, Krick Stefanie
Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, The University of Alabama at Birmingham, Birmingham, AL, United States.
Gregory Fleming James Cystic Fibrosis Research Center, The University of Alabama at Birmingham, Birmingham, AL, United States.
Front Med (Lausanne). 2024 Jan 8;10:1340388. doi: 10.3389/fmed.2023.1340388. eCollection 2023.
Cystic fibrosis (CF) is one of the most common genetic diseases with around 70,000 affected patients worldwide. CF is a multisystem disease caused by a mutation in the CF transmembrane conductance regulator gene, which has led to a significant decrease in life expectancy and a marked impairment in the quality of life for people with CF (pwCF). In recent years, the use of highly effective CFTR modulator therapy (HEMT) has led to improved pulmonary function, fewer CF exacerbations, lower symptom burden, and increased weight. This has coincided with an increased life expectancy for pwCF, with mean age of survival being now in the 50s. This being a major breakthrough, which the CF population has hoped for, pwCF are now facing new challenges by growing old with a chronic respiratory disease. In this mini review, we are attempting to summarize the current knowledge of the aging process and its effect on CF disease and its manifestations including new developments, the current research gaps and potential future developments in the field to allow healthy aging for the CF community.
囊性纤维化（CF）是最常见的遗传病之一，全球约有7万名患者受其影响。CF是一种多系统疾病，由囊性纤维化跨膜传导调节基因的突变引起，这导致CF患者（pwCF）的预期寿命显著缩短，生活质量明显受损。近年来，使用高效的CFTR调节剂疗法（HEMT）已使肺功能得到改善，CF病情加重次数减少，症状负担减轻，体重增加。这与pwCF预期寿命的延长相吻合，目前平均生存年龄已达50多岁。这是CF群体一直期待的重大突破，然而，pwCF现在正面临着因患慢性呼吸道疾病而变老带来的新挑战。在这篇小型综述中，我们试图总结目前关于衰老过程及其对CF疾病及其表现的影响的知识，包括新进展、当前研究空白以及该领域潜在的未来发展，以使CF群体能够健康老龄化。
