Nutrition and cardiometabolic health in cystic fibrosis: Emerging evidence and research gaps [0.03%]
囊性纤维化患者的营养和心代谢健康：新证据与研究缺口
Veronica Lynch,Joanne Barrett,Sarah Collins et al.
Veronica Lynch et al.
Cardiometabolic risk is increasingly recognised as a significant and evolving health concern within the cystic fibrosis (CF) population. Although nutrition has long been central to CF management, its broader influence on cardiometabolic hea...
Antiviral defenses are diminished at birth in cystic fibrosis pig airways [0.03%]
囊性纤维病猪呼吸道在出生时抗病毒防御功能减弱
Jennifer A Bartlett,Miguel E Ortiz,Linda S Powers et al.
Jennifer A Bartlett et al.
Background: There is evidence that antiviral defenses are impaired in airway epithelia derived from the lungs of people with cystic fibrosis (CF), however it is unclear whether this phenotype is an intrinsic feature of CF...
Real-world population pharmacokinetic modeling of elexacaftor, tezacaftor, and ivacaftor [0.03%]
基于真实世界数据的elexacaftor、tezacaftor和ivacaftor群体药代动力学模型研究
A K Kunzelmann,S Naehrig,C Shad et al.
A K Kunzelmann et al.
Background: Cystic fibrosis transmembrane conductance regulator modulators have revolutionized therapy for people with cystic fibrosis, yet marked interindividual variability in drug exposure limits targeted individualize...
Oliver J McElvaney,Sonya L Heltshe,Don B Sanders et al.
Oliver J McElvaney et al.
Background: Forced expiratory volume in one second (FEV₁) is routinely used to assess pulmonary exacerbations (PEx) in cystic fibrosis (CF) but underrepresents air trapping and small airways dysfunction. Forced vital cap...
Abnormalities of the distal terminal ileum in people with cystic fibrosis assessed using magnetic resonance imaging [0.03%]
应用磁共振成像评估囊性纤维化患者的远端回肠异常
Faiz Alqarni,Soma Kumasaka,Madeleine Taylor et al.
Faiz Alqarni et al.
Background: The terminal ileum (TI) is commonly affected in people with cystic fibrosis (pwCF), but limited quantitative information is available on its appearance and dimensions. This study aimed to use magnetic resonanc...
Current diabetes therapy and use of diabetes technology in people living with cystic fibrosis-related diabetes (CFRD): Insights from the DPV network [0.03%]
糖尿病患者当前的糖尿病治疗和糖尿病技术的应用：来自DPV网络囊性纤维化相关糖尿病患者的见解
Thomas Hörtenhuber,Stefanie Schmid,Claudia Boettcher et al.
Thomas Hörtenhuber et al.
Background: Cystic fibrosis-related diabetes (CFRD) is a common comorbidity in cystic fibrosis (CF), significantly impacting morbidity and mortality. Recent advancements in therapy and diabetes technology have transformed...
Timing and rate of reimbursement for cystic fibrosis modulator therapies in EU countries [0.03%]
欧盟各国囊性纤维化调节剂治疗的报销时间和速度
Enrico Costa,Silvia Girotti,Saif Elayan et al.
Enrico Costa et al.
Background: In the EU, Cystic Fibrosis modulator therapies (CFTR modulators) are centrally licensed, but reimbursement is determined at the national level, resulting in variations in access. This study examined cross-coun...
Improvement in functional exercise performance and quadriceps muscle strength after two years treatment with elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis: A prospective observational study [0.03%]
两年的艾沙卡福维克/泰萨卡福维克/伊维卡福维克治疗对囊性纤维化患者功能运动能力和股四头肌力量影响的前瞻性观察研究
Anne-Sophie Aubriot,Catherine Song,Clémence Gonçalvès et al.
Anne-Sophie Aubriot et al.
Background: As reduced exercise capacity and muscle weakness are common in people with cystic fibrosis (pwCF) and associated with worse life prognosis and quality of life, we aimed to evaluate the changes in functional ex...
Real-world experience with a generic formulation of elexacaftor/tezacaftor/ivacaftor in children with cystic fibrosis [0.03%]
适用于囊性纤维化患儿的仿制埃勒司凯福塔司/泰泽凯福塔司/伊伐 Krakendale治疗的现实世界经验
María Macarena Oneglia,Hilda Giugno,Ariela Agustinho et al.
María Macarena Oneglia et al.
Background: Cystic fibrosis (CF) is caused by mutations in the gene encoding the CF transmembrane conductance regulator protein. Since 2021, Argentina has provided a generic formulation of the triple modulator therapy ele...