Pescaru Camelia Corina, Crișan Alexandru Florian, Marițescu Adelina, Cărunta Vlad, Marc Monica, Dumitrache-Rujinski Ștefan, Laitin Sorina, Oancea Cristian
Center for Research and Innovation in Personalized Medicine of Respiratory Diseases (CRIPMRD), "Victor Babes" University of Medicine and Pharmacy Timisoara, Eftimie Murgu Square 2, 300041 Timisoara, Romania.
Pulmonary Rehabilitation Center, Clinical Hospital of Infectious Diseases and Pulmonology, "Victor Babes", Gheorghe Adam Street 13, 300310 Timisoara, Romania.
Infect Dis Rep. 2025 Jul 7;17(4):80. doi: 10.3390/idr17040080.
: Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Modulator therapies have the ability to improve CFTR function in CF patients, but despite the clear evidence of benefits regarding CFTR modulator therapy, including improved lung function, the reduced rate of exacerbations, and an overall improved quality of life, studies focusing on the reduction rates of infections during modulator therapy expressed the need for future research on this topic. : This study aimed to evaluate the impact of CFTR modulator therapies on the prevalence, density, and persistence of infection in CF patients and to explore the mechanisms involved. : A systematic literature review was performed by searching five major databases (PubMed, Cochrane Library, Scopus, Google Scholar, and Web of Science), and 21 relevant articles investigating the link between CFTR therapy and infections were selected following the PRISMA guidelines. : The data indicated that Ivacaftor and the combination Elexacaftor/Tezacaftor/Ivacaftor (ETI) can reduce total bacterial load and markers of systemic inflammation. However, clonal lines of persist in most cases, and complete eradication is rare, mainly due to biofilm formation and antimicrobial resistance. : Although CFTR-modulating therapies help to improve clinical condition and reduce inflammation, they do not consistently lead to the elimination of .
囊性纤维化（CF）是一种常染色体隐性疾病，由囊性纤维化跨膜传导调节因子（CFTR）基因突变引起。调节剂疗法有能力改善CF患者的CFTR功能，但尽管有明确证据表明CFTR调节剂疗法有益，包括改善肺功能、降低病情加重率以及总体提高生活质量，但专注于调节剂疗法期间感染减少率的研究表明需要对该主题进行未来研究。本研究旨在评估CFTR调节剂疗法对CF患者感染的患病率、密度和持续性的影响，并探索其中涉及的机制。通过搜索五个主要数据库（PubMed、Cochrane图书馆、Scopus、谷歌学术和科学网）进行了系统的文献综述，按照PRISMA指南选择了21篇研究CFTR疗法与感染之间联系的相关文章。数据表明，依伐卡托以及依列卡托/替扎卡托/依伐卡托组合（ETI）可降低总细菌负荷和全身炎症标志物。然而，在大多数情况下感染的克隆株持续存在，完全根除很少见，主要是由于生物膜形成和抗菌耐药性。尽管CFTR调节疗法有助于改善临床状况并减轻炎症，但它们并不能始终导致感染的消除。
