ReviewEuropean respiratory review : an official journal of the European Respiratory Society2020
Impact of CFTR modulator use on outcomes in people with severe cystic fibrosis lung disease.
Review in European respiratory review : an official journal of the European Respiratory Society, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 59 papers, 3 of them syntheses that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
59 citing papers in PubMed, 3 syntheses or guidelines pooled it, 105 citations in OpenAlex.
- Health-related quality of life following lung transplantation for cystic fibrosis: A systematic review.Clinics (Sao Paulo, Brazil) · 2023Pooled it
- Dornase alfa for cystic fibrosis.The Cochrane database of systematic reviews · 2021Pooled it
- From Ivacaftor to Triple Combination: A Systematic Review of Efficacy and Safety of CFTR Modulators in People with Cystic Fibrosis.International journal of molecular sciences · 2020Pooled it
- Red blood cell deformability as a mechanobiological marker of CFTR dysfunction in cystic fibrosis.iScience · 2026Article
- Divergent neurobehavioral effects of CFTR modulators elexacaftor and ivacaftor in mice.Acta psychologica · 2026Article
- The clinical, psychosocial, and economic burden of cystic fibrosis lung disease in the era of CFTR modulator therapy.Annals of the American Thoracic Society · 2026Review
- A novel drug series optimized to address cystic fibrosis and other CFTR deficiency diseases of human airways.npj drug discovery · 2025Article
- Cardiometabolic insights in cystic fibrosis and highly effective modulator therapies: a narrative review.Endocrine connections · 2025Review
- Cystic fibrosis airway epithelial cells have an exaggerated response to hypoxia that is partially reversible through transmembrane receptor modulation.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2025Article
- Provider Practices in Pulmonary Exacerbations of Cystic Fibrosis in the Era of Highly Effective Modulator Therapy.Annals of the American Thoracic Society · 2025Article
- Clinical Disorders in Cystic Fibrosis That Affect Emergency Procedures-A Case Report and Review.Journal of clinical medicine · 2025Review
- Elexacaftor-tezacaftor-ivacaftor pharmacokinetics with concurrent tacrolimus administration after lung transplant.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2025Observational
- Improving ototoxicity monitoring in patients receiving aminoglycosides using a novel digital approach: a quality improvement project.BMJ open quality · 2025Article
- Impact of elexacaftor-tezacaftor-ivacaftor in lung transplantation for cystic fibrosis in the United States.JHLT open · 2025Article
- Article
- Societal Economic Burden of Cystic Fibrosis in Iran: A Cost-of-Illness Study.Journal of health economics and outcomes research · 2025Article
- Clinical and research innovations in childhood interstitial lung disease (chILD).Pediatric pulmonology · 2024Article
- Definitions of pulmonary exacerbation in people with cystic fibrosis: a scoping review.BMJ open respiratory research · 2024Article
- Neutrophil serine proteases in cystic fibrosis: role in disease pathogenesis and rationale as a therapeutic target.European respiratory review : an official journal of the European Respiratory Society · 2024Review
- Microbial community organization designates distinct pulmonary exacerbation types and predicts treatment outcome in cystic fibrosis.Nature communications · 2024Observational
Corrections and comments
- Commented on by
Authors and funding
2 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Drug compounds that augment the production and activity of the cystic fibrosis (CF) transmembrane regulator (CFTR) have revolutionised CF care. Many adults and some children with CF suffer advanced and severe lung disease or await lung transplantation. While the hope is that these drug compounds will prevent lung damage when started early in life, there is an ongoing need to care for people with advanced lung disease. The focus of this review is the accumulating data from clinical trials and case series regarding the benefits of CFTR modulator therapy in people with advanced pulmonary disease. We address the impact of treatment with ivacaftor, lumacaftor/ivacaftor, tezacaftor/ivacaftor and elexacaftor/tezacaftor/ivacaftor on lung function, pulmonary exacerbations, nutrition and quality of life. Adverse events of the different CFTR modulators, as well as the potential for drug-drug interactions, are discussed.
Indexed as
Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.