| ECFS best practice guidelines: the 2018 revision |
73 |
| CFTR modulator theratyping: Current status, gaps and future directions |
33 |
| Up-to-date and projected estimates of survival for people with cystic fibrosis using baseline characteristics: A longitudinal study using UK patient registry data |
30 |
| Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines |
22 |
| Multicentre standardisation of chest MRI as radiation-free outcome measure of lung disease in young children with cystic fibrosis |
17 |
| Lumacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease homozygous for F508del-CFTR |
17 |
| Animal and model systems for studying cystic fibrosis |
15 |
| Isogenic cell models of cystic fibrosis-causing variants in natively expressing pulmonary epithelial cells |
14 |
| Epidemiology of CF: How registries can be used to advance our understanding of the CF population |
13 |
| Predictive factors for lumacaftor/ivacaftor clinical response |
13 |
| Prevalence and diversity of filamentous fungi in the airways of cystic fibrosis patients - A Dutch, multicentre study |
13 |
| An open-label extension study of ivacaftor in children with CF and a CFTR gating mutation initiating treatment at age 2-5 years (KLIMB) |
13 |
| Antimicrobial susceptibility testing (AST) and associated clinical outcomes in individuals with cystic fibrosis: A systematic review |
12 |
| Measured fetal and neonatal exposure to Lumacaftor and Ivacaftor during pregnancy and while breastfeeding |
12 |
| Functional characterization and phenotypic spectrum of three recurrent I Check for disease-causing deep intronic variants of the CFTR gene |
11 |
| Lumacaftor/Ivacaftor reduces pulmonary exacerbations in patients irrespective of initial changes in FEV1 |
11 |
| Recent progress in translational cystic fibrosis research using precision medicine strategies |
11 |
| Combination potentiator ('co-potentiator') therapy for CF caused by CFTR mutants, including N1303K, that are poorly responsive to single potentiators |
10 |
| KB001-A, a novel anti-inflammatory, found to be safe and well-tolerated in cystic fibrosis patients infected with Pseudomonas aeruginosa |
10 |
| Therapeutic approaches to CFTR dysfunction: From discovery to drug development |
10 |
| Clofazimine inhalation suspension for the aerosol treatment of pulmonary nontuberculous mycobacterial infections |
10 |
| Audiometric assessment of pediatric patients with cystic fibrosis |
10 |
| Cystic fibrosis screen positive inconclusive diagnosis (CFSPID): Experience in Tuscany, Italy |
10 |
| Dietary intake of energy-dense, nutrient-poor and nutrient-dense food sources in children with cystic fibrosis |
10 |
| Newborn blood spot screening for cystic fibrosis with a four-step screening strategy in the Netherlands |
10 |
| Strategies for newborn screening for cystic fibrosis: A systematic review of health economic evaluations |
9 |
| Defining antimicrobial resistance in cystic fibrosis |
9 |
| Quality of life and mood in children with cystic fibrosis: Associations with sleep quality |
9 |
| The CF Canada-Sick Kids Program in individual CF therapy: A resource for the advancement of personalized medicine in CF |
9 |
| Resolvin D1 regulates epithelial ion transport and inflammation in cystic fibrosis airways |
9 |
| Early life growth patterns persist for 12 years and impact pulmonary outcomes in cystic fibrosis |
9 |
| A phase 3 study of tezacaftor in combination with ivacaftor in children aged 6 through 11 years with cystic fibrosis |
9 |
| CFTR activity is enhanced by the novel corrector GLPG given with and without ivacaftor in two randomized trials |
9 |
| A two-center analysis of hyperpolarized Xe-129 lung MRI in stable pediatric cystic fibrosis: Potential as a biomarker for multi-site trials |
9 |
| Detection of CFTR function and modulation in primary human nasal cell spheroids |
9 |
| Risk factors for persistent Aspergillus respiratory isolation in cystic fibrosis |
8 |
| Theranostics by testing CFTR modulators in patient-derived materials: The current status and a proposal for subjects with rare CFTR mutations |
8 |
| Drugs during pregnancy and breast feeding in women diagnosed with Cystic Fibrosis - An update |
8 |
| Methylomic correlates of autophagy activity in cystic fibrosis |
8 |
| Antisense oligonucleotide eluforsen improves CFTR function in F508del cystic fibrosis |
8 |
| Failure to conceive in women with CF is associated with pancreatic insufficiency and advancing age |
7 |
| Impact of a program ensuring consistent response to acute drops in lung function in children with cystic fibrosis |
7 |
| CFTR rescue with VX-809 and VX-770 favors the repair of primary airway epithelial cell cultures from patients with class II mutations in the presence of Pseudomonas aeruginosa exoproducts |
7 |
| Interleukin-1 is associated with inflammation and structural lung disease in young children with cystic fibrosis |
7 |
| Comparison of lung clearance index determined by washout of N-2 and SF6 in infants and preschool children with cystic fibrosis |
7 |
| Variability of monthly nitrogen multiple-breath washout during one year in children with cystic fibrosis |
7 |
| Variability of lung clearance index in clinically stable cystic fibrosis lung disease in school age children |
7 |
| GLPG a CFTR potentiator, in p.Gly551Asp (G551D)-CF patients: An open-label, single-arm, phase 2a study (SAPHIRA1) |
7 |
| Measuring recovery in health-related quality of life during and after pulmonary exacerbations in patients with cystic fibrosis |
7 |
| Sexual and reproductive health behaviors and experiences reported by young. women with cystic fibrosis |
7 |
