Department of Pediatrics, Division of Pulmonary and Sleep Medicine, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
Department of Pediatric Pulmonology, Children's Wisconsin, Milwaukee, Wisconsin, USA.
Pediatr Pulmonol. 2022 Feb;57(2):347-360. doi: 10.1002/ppul.25459. Epub 2021 May 25.
The outlook for those with cystic fibrosis (CF) has never been brighter with ever increasing life expectancy and the approval of the highly effective CFTR modulators, such as elexacaftor/tezacaftor/ivacaftor. With that being said, the progressive pulmonary decline and importance of lung health, infection, and inflammation in CF remains. This review is the second part in a three-part CF Year in Review 2020. Part one focused on the literature related to CFTR modulators while part three will feature the multisystem effects related to CF. This review focuses on articles from Pediatric Pulmonology, including articles from other journals that are of particular interest to clinicians. Herein, we highlight studies published during 2020 related to CF pulmonary disease, infection, treatment, and diagnostics.
囊性纤维化(CF)患者的前景从未如此光明,他们的预期寿命不断延长,而且 CFTR 调节剂(如 elexacaftor/tezacaftor/ivacaftor)也获得了批准。话虽如此,CF 患者的肺部进行性下降以及肺部健康、感染和炎症的重要性仍然存在。这篇综述是 2020 年 CF 年度回顾的第三部分中的第二部分。第一部分重点介绍了与 CFTR 调节剂相关的文献,第三部分将介绍与 CF 相关的多系统影响。这篇综述主要关注《儿科肺病学》上的文章,包括对临床医生特别有兴趣的其他期刊上的文章。在此,我们重点介绍了 2020 年期间发表的与 CF 肺部疾病、感染、治疗和诊断相关的研究。