Hyperglycemia differentially affects neutrophil transmigration across cystic fibrosis and wildtype bronchial epithelia [0.03%]
高血糖对囊性纤维化和野生型支气管上皮中性粒细胞迁移的差异化影响
Guiying Cui,Analía Vazquez Cegla,Jonica Brown et al.
Guiying Cui et al.
Background: Hallmarks of Cystic Fibrosis (CF) lung disease are chronic obstruction, infection, and inflammation dominated by lifelong, excessive influx of neutrophils (PMNs) into airways which is further exacerbated by CF...
Letter to the editor: False reassurance following single gene non-invasive prenatal testing for cystic fibrosis [0.03%]
致编辑的信：单一基因无创产前检测囊性纤维化假阳性后的误报安慰现象
Edith T Zemanick,Manesha Putra,Hannah Elfman et al.
Edith T Zemanick et al.
Functional CFTR may be required for Prevotella melaninogenica regulation of epithelial cell defense against Staphylococcus aureus [0.03%]
功能性CFTR可能是Prevotella melaninogenica调节上皮细胞防御金黄色葡萄球菌所必需的
Maksym Goryachok,Ana Fairbanks-Mahnke,Sam Fulte et al.
Maksym Goryachok et al.
Background: Prevotella melaninogenica is enriched in the lungs of people with cystic fibrosis (pwCF), yet its functional impact on respiratory tract homeostasis remains incompletely understood. Prior studies identified im...
Persistent plasma and RBC fatty acid abnormalities in children and adolescents with cystic fibrosis on highly effective CFTR modulators [0.03%]
囊性纤维化患者在接受高效CFTR调节剂治疗后血脂和红细胞膜脂肪酸的持续异常
Tatiana Yuzyuk,Catherine M McDonald,Kayode Balogun et al.
Tatiana Yuzyuk et al.
Background: Essential fatty acid deficiency (EFAD) is a common complication in people with cystic fibrosis (pwCF). While CFTR modulators (CFTRm) have become the standard of care, their effect on EFAD has been minimally ex...
Peak nasal inspiratory flow as an adjunct measure of respiratory function in paediatric cystic fibrosis [0.03%]
鼻峰吸气流量作为儿童囊性纤维化呼吸功能的辅助测量指标
Pezzella Paolo,Russo Mario Brandon,Sepe Angela et al.
Pezzella Paolo et al.
Background: Chronic rhinosinusitis (CRS) is common in cystic fibrosis (CF) and affects quality of life (QoL), especially in children. Objective tools like Peak Nasal Inspiratory Flow (PNIF) may aid in assessing nasal obst...
Research and development spending versus revenues after approval for CFTR modulators [0.03%]
囊性纤维化跨膜转导调节剂获批后的研发支出去向探究
Jonathan Guo,Grace Hennessy,Benedict Young et al.
Jonathan Guo et al.
Background: The high prices of CFTR modulators present barriers to access for people with cystic fibrosis (CF), especially those in low- and middle-income countries. Costs of research and development (R&D) are often cited...
The treatment with Elexacaftor/Tezacaftor/Ivacaftor significantly increases serum bilirubin and decreases blood platelets in children and adolescents with cystic fibrosis homozygous or double heterozygous for the F508del CFTR variant [0.03%]
Elexacaftor/Tezacaftor/Ivacaftor治疗可显著增加囊性纤维化F508del突变纯合或双杂合患儿的血清胆红素和减少血小板
Alice Castaldo,Chiara Cimbalo,Cristina Fevola et al.
Alice Castaldo et al.
The Elexacaftor/Tezacaftor/Ivacaftor (ETI) combination of cystic fibrosis transmembrane regulator modulators is safe and effective even in children with at least one F508del variant. However, cases of liver damage have been reported, and we...
Nasal irrigation as an alternate method to monitor airway microbiology in cystic fibrosis [0.03%]
鼻腔冲洗作为囊性纤维化患者监测呼吸道微生物学的替代方法
Raynuka Lazarus,Cassandra Thompson,Jennifer Bishop et al.
Raynuka Lazarus et al.
Background: The sinus cavity may be an alternative sampling site for microbial pathogens in people with cystic fibrosis. However, the congruence between sinus and cough-based sputum sampling is unknown. This study aimed t...
Global prevalence of CFTR variants with respect to their responsiveness to elexacaftor-tezacaftor-ivacaftor [0.03%]
CFTR突变体对elexacaftor-tezacaftor-ivacaftor的响应性及其全球分布
P-R Burgel,A Orenti,E Cromwell et al.
P-R Burgel et al.
Background: Elexacaftor-tezacaftor-ivacaftor (ETI) is mostly approved in people with cystic fibrosis (pwCF) with a p.Phe508del CFTR variant. The US Food and Drug Administration (FDA) approved ETI for an additional 177 rar...
Impact of glucagon-like-peptide-1 receptor agonist therapy on pulmonary function in people with cystic fibrosis who achieve normal body mass index [0.03%]
葡萄糖酸胜肽-1受体激动剂治疗对囊性纤维化患者肺功能的影响——仅体重指数正常的患者
Andrew Horvit,Katie Kaput,Amber Neece et al.
Andrew Horvit et al.
Background: Glucagon-like-peptide-1 receptor agonists (GLP-1 RA) and glucose-dependent insulinotropic polypeptide/glucagon-like peptide-1 receptor agonists (GIP/GLP-1 RA) are being explored for use in people with CF (pwCF...