Successful off-label use of elexacaftor/tezacaftor/ivacaftor in an infant with severe CF disease [0.03%]
托伏卡弗/维卡弗/伊娃卡弗.off label.用于治疗重症囊性纤维化患儿取得良好效果
Elizabeth Shevill,Claire E Wainwright,Martha Churchett et al.
Elizabeth Shevill et al.
Cystic fibrosis airway epithelial cells have an exaggerated response to hypoxia that is partially reversible through transmembrane receptor modulation [0.03%]
气道上皮细胞对缺氧的反应过激现象可以通过跨膜受体调节部分逆转
Elsa Brunet-Ratnasingham,Tasha Tsao,Rashmi P Mohanty et al.
Elsa Brunet-Ratnasingham et al.
The pathophysiology of cystic fibrosis (CF) leads to epithelial cell hypoxia, which directly affects epithelial cells. CF is caused by genetic disruption of the CF transmembrane receptor that has important direct impacts on cell signaling a...
Genomic and epidemiologic investigation of Mycobacterium abscessus isolates in a cystic fibrosis center to determine potential routes of transmission [0.03%]
结核分枝杆菌脓肿亚种的基因组和流行病学研究确定潜在传播途径
Jane E Gross,Jason Fullmer,Gregory McCleland et al.
Jane E Gross et al.
Background: Cystic Fibrosis (CF) Centers worldwide have reported healthcare-associated outbreaks of nontuberculous mycobacteria (NTM). We report a retrospective investigation of shared Mycobacterium abscessus strains amon...
PRenatal mOdulator treatment to PrEvent CF complicaTions (PROTECT) workshop report [0.03%]
防治囊性纤维化并发症的孕前调节治疗(PROTECT)研讨会报告
Sylvia Szentpetery,Dara Riva,Yair J Blumenfeld et al.
Sylvia Szentpetery et al.
Background: Data from cystic fibrosis (CF) animal models and case studies suggests that in utero administration of CF transmembrane conductance regulator (CFTR) modulators (variant specific therapies, VST) can rescue CFTR...
Lung clearance index in patients with cystic fibrosis: Can we avoid repeating the test three times? [0.03%]
囊性纤维化患者的肺清除指数：我们可以避免重复进行三次测试吗？
Amany F Elbehairy,Doone Y Boorman,Cassandra MacNaughton et al.
Amany F Elbehairy et al.
Multiple breath washout (MBW) testing and its derived measurements (e.g., lung clearance index (LCI)) are useful for measuring ventilation heterogeneity in patients with cystic fibrosis, monitoring disease progression, and assessing the res...
Results of a clinical trial of ANG003, a non-porcine pancreatic enzyme replacement therapy, in people with cystic fibrosis [0.03%]
一种非猪源性胰酶替代疗法ANG003治疗囊性纤维化患者的临床试验结果
Meghana Sathe,Steven D Freedman,Melissa S Putman et al.
Meghana Sathe et al.
Background: Pancreatic enzyme replacement therapy (PERT) prevents malnutrition in people with exocrine pancreatic insufficiency, including those with cystic fibrosis (CF). We developed a lipase that is stable against prot...
Shallow metagenomic shotgun sequencing improves detection of pathogenic species in cystic fibrosis respiratory samples [0.03%]
低深度的宏基因组鸟枪测序可改善囊性纤维化呼吸样本中病原菌的检测
Eline Cauwenberghs,Ilke De Boeck,Lize Delanghe et al.
Eline Cauwenberghs et al.
Background: Chronic infection and inflammation of the lungs contribute significantly to disease progression in persons with cystic fibrosis (pwCF). Treatment regimens are largely based on isolating the putative causative ...
Elisabeth P Dellon,Mary G Prieur
Elisabeth P Dellon
Elevated serum lipase in infants with cystic fibrosis exposed to prenatal and postnatal elexacaftor/tezacaftor/ivacaftor [0.03%]
接受孕期和产后艾沙卡福韦/-te扎卡福韦/ ivacaftor治疗的囊性纤维化婴儿血清脂肪酶升高
Haley Haskett,Christopher Fortner,Laura Shanley et al.
Haley Haskett et al.
Cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction leads to progressive exocrine pancreatic insufficiency, resulting in difficulty in the secretion of digestive enzymes and subsequent malabsorption of nutrients. Case rep...
"All my food is customized*": Barriers & facilitators concerning nutrition for persons living with cystic fibrosis [0.03%]
“所有食物均个性定制”——囊性纤维化患者营养摄取的影响因素分析
Amanda S Bruce,Sakinah C Suttiratana,Hannah Grabowski et al.
Amanda S Bruce et al.
Background: Maintaining optimal nutrition is often an important concern for persons with cystic fibrosis (PWCF). With the introduction of effective modulators, the focus has now shifted from preventing malnourishment to i...