Imaging brain vascular function in Cystic Fibrosis: an MRI study of cerebral blood flow and brain oxygenation [0.03%]
囊性纤维化患者脑血管功能的影像学研究：关于脑血流和脑氧合水平的MRI研究
H L Chandler,M Germuska,T M Lancaster et al.
H L Chandler et al.
Background: Cystic fibrosis (CF) is a progressive inherited disorder that primarily affects the lungs. With recent breakthroughs in effective treatments for CF that increase life-expectancy, a higher prevalence of age-rel...
Correlation between patient-derived intestinal organoids and clinical responses to CFTR modulators in people with cystic fibrosis homozygous for F508del [0.03%]
囊性纤维化患者同源F508del突变与临床CFTR调节剂反应的类器官相关性分析
Noelia Rodriguez Mier,Senne Cuyx,Kris De Boeck et al.
Noelia Rodriguez Mier et al.
Background: Patient-derived intestinal organoids (PDIOs) are an in vitro tool used to predict clinical responses to CFTR modulators in people with cystic fibrosis (pwCF). Although strong genotype-based correlations have b...
Neutrophil store-operated Ca2+ entry: A correctable biomarker of cystic fibrosis lung disease progression [0.03%]
中性粒细胞钙离子释放操作电流进入：囊性纤维化肺病进展可修复生物标志物
Joe A Wrennall,Matthew Gs Biggart,Charles D Bengtson et al.
Joe A Wrennall et al.
Rationale: People with cystic fibrosis (pwCF) exhibit chronic and hyperactive neutrophilia which results in a progressive loss of lung function. CF neutrophils have elevated store operated Ca2+ entry (SOCE) relative to he...
Severe hepatotoxicity is uncommon following the introduction of Elexacaftor/Tezacaftor/Ivacaftor: A real-world two-years follow-up study of the Danish cystic fibrosis cohort [0.03%]
伊莱卡福维尔/特扎卡福维尔/艾伏卡福维尔引入后严重肝毒性的发生率极低：丹麦囊性纤维化队列的两年真实世界随访研究
Christine Højte,Mette Frahm Olsen,Daniel Faurholt-Jepsen et al.
Christine Højte et al.
Background: Elexacaftor/Tezacaftor/Ivacaftor (ETI) has raised concerns about liver-related side effects. This study evaluated changes in liver function tests (LFTs) and the prevalence of hepatotoxicity over two years of E...
Qiwei Xiao,Goutham Mylavarapu,James F Chmiel et al.
Qiwei Xiao et al.
Background: Morphological changes of the pulmonary vasculature in cystic fibrosis (CF) with advancing age, disease progression and modulator therapy are not fully understood. ...
Impact of long-term elexacaftor/tezacaftor/ivacaftor therapy on oxidative stress in cystic fibrosis: early insights [0.03%]
长期使用elexacaftor/tezacaftor/ivacaftor治疗对囊性纤维化氧化应激的影响：初步见解
Zoltán Örlős,Imre Barta,Csilla Páska et al.
Zoltán Örlős et al.
Background: Treatment with elexacaftor/tezacaftor/ivacaftor (ETI) has been shown to improve clinical outcomes in people with cystic fibrosis (pwCF). Here, we investigated whether these benefits are associated with the mod...
Feasibility of multiple-breath washout in the clinical setting and prediction of its duration in children and adults with cystic fibrosis [0.03%]
多重气溶胶吸入检测在囊性纤维化患者中的可行性及其持续时间的预测因素研究
Christine Allomba,Leonie Busack,Niklas Ziegahn et al.
Christine Allomba et al.
Background: Pulmonary function tests play an important role in diagnosis, management and treatment of people with cystic fibrosis (pwCF). Multiple-breath washout (MBW) is a sensitive method to detect ventilation inhomogen...
Hepatitis E virus seroprevalence is increased amongst pancreatic insufficient adults with cystic fibrosis - A single centre study [0.03%]
囊性纤维化相关胰腺外分泌功能不全成人戊型肝炎病毒感染的血清流行病学研究
Barbara J Waddell,Christina S Thornton,Yasaman Ghazi et al.
Barbara J Waddell et al.
Validation of an artificial intelligence-based automated PRAGMA and mucus plugging algorithm in pediatric cystic fibrosis [0.03%]
基于人工智能的自动PRAGMA和粘液嵌塞算法在儿童囊性纤维化中的验证研究
Pranali Raut,Yuxin Chen,Ahmad Taleb et al.
Pranali Raut et al.
Background: PRAGMA-CF is a clinically validated visual chest CT scoring method, quantifying relevant components of structural airway damage in CF. We aimed to validate a newly developed AI-based automated PRAGMA-AI and Mu...
"Time out for an hour, every day, my whole life": understanding treatment burden in cystic fibrosis in the era of Elexacaftor/Tezacaftor/Ivacaftor (ETI) [0.03%]
“每天休息一个小时，贯穿终生”——透析囊性纤维化患者对Elexacaftor/Tezacaftor/Ivacaftor(ETI)治疗负担的理解
Jennifer L Butcher,Christopher Siracusa,Hannah Grabowski et al.
Jennifer L Butcher et al.
Background: Reducing treatment burden is a priority within the CF community. Improved outcomes for many people with CF (PWCF) following treatment with Elexacaftor/Tezacaftor/Ivacaftor (ETI) have led to considering whether...