Long-term outcomes in people with CF lacking FEV1 response to elexacaftor/tezacaftor/ivacaftor therapy [0.03%]
对elxacaftor/tezacaftor/ivacaftor治疗无FEV₁反应的囊性纤维化患者长期结果的探究
Mohamad Hadhud,Dan Halevy,Joel Reiter et al.
Mohamad Hadhud et al.
Background: Real-world data demonstrate variability in the response to elexacaftor/tezacaftor/ivacaftor (ETI) treatment among people with CF (pwCF). The aim of this study was to evaluate long-term outcomes in pwCF that ha...
Intra-individual diversity of bacteriophage susceptibility in Burkholderia cultured from cystic fibrosis sputum [0.03%]
铜绿假单胞菌产生囊胞的机理研究进展及意义展望
Ortal Yerushalmy,Abby M Korn,Guichun Yao et al.
Ortal Yerushalmy et al.
The use of bacteriophage (phage) to treat bacterial infection of airways in persons with cystic fibrosis (CF) is gaining interest. However, phenotypic diversification of bacteria during chronic airway infection presents a potential challeng...
Cannabinoid receptor 2 agonist, lenabasum, for the treatment of pulmonary exacerbations in cystic fibrosis [0.03%]
大麻素受体2激动剂Lenabasum治疗囊性纤维化肺部感染恶化
Natalie E West,Michael W Konstan,Patrick A Flume et al.
Natalie E West et al.
Background: Lenabasum is a cannabinoid receptor 2 (CB2) agonist under development for cystic fibrosis (CF), targeting inflammation. We evaluated the efficacy and safety of lenabasum in people with CF (pwCF). ...
Pancreatic islet autoantibodies and their association with glycemic status in cystic fibrosis patients: A comprehensive meta-analysis [0.03%]
囊性纤维化患者胰岛自身抗体及其与血糖状态的关系：一项全面的meta分析研究
Regina Molnár,Blanka Rebeka Bódy,Boróka Varga et al.
Regina Molnár et al.
Background: The role of autoimmune beta-cell damage in cystic fibrosis-related glucose abnormalities remains unclear. This study evaluates the prevalence of pancreatic islet autoantibodies (AABs) by glycemic status and ag...
Characteristics and outcomes of people with cystic fibrosis on the Eurotransplant liver transplantation waiting list [0.03%]
欧洲移植联盟器官分配系统中囊性纤维化肝移植受者的特征和预后研究
Marissa I van der Spek,Henkjan J Verkade,Johan W Jonker et al.
Marissa I van der Spek et al.
Background: Advanced cystic fibrosis (CF) liver disease can necessitate liver transplantation. This study aims to investigate characteristics, waiting list dynamics, and waiting list mortality of people with CF (pwCF) reg...
Be it resolved airway clearance cannot and should not be replaced by exercise in the era of CFTR modulators-Summary of a Pro/Con debate [0.03%]
CFTR调节剂时代不能也不应由运动取代支气管引流祛痰疗法——摘要一场正方和反方的辩论
G Stanford,K von Berg,C Smith et al.
G Stanford et al.
Airway clearance to clear excessive sputum has long been a key part of cystic fibrosis care, however the introduction of highly effective modulator medications where many people with CF are experiencing reduced sputum loads, has raised a qu...
LONGITUDE: An observational study of the long-term effectiveness of elexacaftor/tezacaftor/ivacaftor in people aged ≥12 years with cystic fibrosis using data from the United Kingdom Cystic Fibrosis Registry - 2-year analysis [0.03%]
基于英国囊性纤维化登记数据的经度研究：伏 Roxacaftor/Tezacaftor/Ivacaftor 治疗≥12岁囊性纤维化患者的长期疗效观察研究-两年数据分析
Gabriela Vega-Hernandez,Gordon MacGregor,Andrew Wilfin et al.
Gabriela Vega-Hernandez et al.
Background: The cystic fibrosis (CF) transmembrane conductance regulator modulator (CFTRm) elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) has demonstrated efficacy and safety in clinical trials and emerging observational ...
Pulmonary arteriole narrowing in end-stage cystic fibrosis lungs occurs with and without small airway disease [0.03%]
终末期囊性纤维化肺动脉狭窄的发生与是否存在小气道疾病有关
Astrid Vermaut,Gitte Aerts,Lynn Willems et al.
Astrid Vermaut et al.
Background: Pulmonary hypertension (PH) is an important, life-limiting co-morbidity in cystic fibrosis (CF), where multiple mechanisms such as hypoxia, inflammation and primary CF-transmembrane regulator (CFTR) dysfunctio...
Physical well-being and burden of care in adults on modulator therapy: A mixed methods study of patient-reported experiences from the Well-ME survey [0.03%]
调核素治疗成人患者的身体健康和照护负担：来自Well-ME调查的混合研究方法中患者的报告体验
Cynthia D Brown,Carla Frederick,Elizabeth Yu et al.
Cynthia D Brown et al.
Background: Despite widespread availability of modulator therapies and improved lung function in many people with cystic fibrosis (CF), physical symptoms may remain burdensome for some people with CF (PwCF). This study id...
Association between inhaled antibiotic use and treatment-emergent organisms among Canadian people with cystic fibrosis [0.03%]
吸入式抗生素使用与加拿大囊性纤维化患者治疗过程中出现的病原体之间的关系
Jonathan D Cogen,Eric Zhang,Jane She et al.
Jonathan D Cogen et al.
Background: Inhaled antibiotics are frequently used as chronic Pseudomonas aeruginosa (Pa) suppressive therapy among people with cystic fibrosis (PwCF). However, their use might increase the risk of developing treatment-e...