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期刊名:Journal of cystic fibrosis

缩写:J CYST FIBROS

ISSN:1569-1993

e-ISSN:1873-5010

IF/分区:6.0/Q1

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共收录本刊相关文章索引2548
Clinical Trial Case Reports Meta-Analysis RCT Review Systematic Review
Classical Article Case Reports Clinical Study Clinical Trial Clinical Trial Protocol Comment Comparative Study Editorial Guideline Letter Meta-Analysis Multicenter Study Observational Study Randomized Controlled Trial Review Systematic Review
Tim Lee,Kate Hill,Daan Caudri et al. Tim Lee et al.
Cystic fibrosis is a lifelong progressive disease in which lung disease is the main prognostic factor, where starting early treatment is crucial for improving long-term outcomes. Therefore, new treatment should be available as early as poss...
Nora C Burdis,Miranda C Bradford,Sonya L Heltshe et al. Nora C Burdis et al.
Background: Elexacaftor/tezacaftor/ivacaftor (ETI) has dramatically improved pulmonary and extrapulmonary manifestations of cystic fibrosis (CF). ETI clinical trials excluded lung transplant (LTx) recipients and current p...
Daniela Dolce,Silvia Campana,Cristina Fevola et al. Daniela Dolce et al.
Background: Pseudomonas aeruginosa chronic infection can cause lung function decline in people with cystic fibrosis, and early antibiotic treatment (EAT) is crucial to prevent it. While culture methods are standard for mo...
Sophie Gautier,Bénédicte Coulm,Marie-Andrée Thompson-Bos et al. Sophie Gautier et al.
Background: Clinical data regarding in utero exposure to CFTR modulators (CFTRm) are limited. Our objective was to describe pregnancy outcomes, with particular attention to malformations, and neonatal adverse outcomes amo...
William T Harris,Elizabeth H Baker,Jennifer S Guimbellot et al. William T Harris et al.
Background: Tobacco smoke exposure (TSE) is associated with diminished benefit of ivacaftor and tezacaftor/ivacaftor in people with CF (PwCF). This study assessed the association of TSE with clinical benefit from elexacaf...
Jennifer T Duong,Hillary S Hayden,Adrian J Verster et al. Jennifer T Duong et al.
Background: People with cystic fibrosis (PwCF) often have fecal dysbioses relative to those without CF, characterized by increased pro-inflammatory microbiota and gastrointestinal (GI) inflammation as measured by fecal ca...
Mélanie Auvray,Nolwenn Laborde,Marie Mittaine et al. Mélanie Auvray et al.
Background: There are great changes in cystic fibrosis (CF) disease following introduction of modulator treatments. We aimed to focus on the evolution of hepatobiliary involvement following lumacaftor-ivacaftor (LI) and e...
A Akerø,E Edvardsen,P L Finstad et al. A Akerø et al.
Background: Air travel may cause significant hypoxemia in patients with cystic fibrosis (CF). A pre-flight algorithm has previously been validated for patients with chronic obstructive pulmonary disease (COPD). No such to...