ReviewNature reviews. Disease primers2024
Cystic fibrosis.
Review in Nature reviews. Disease primers, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 123 papers.
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
123 citing papers in PubMed.
- Safety and efficacy of elexacaftor/tezacaftor/ivacaftor in adolescents and adults with cystic fibrosis and F508del-gating and F508del-residual function genotypes: results from an open-label extension study.Annals of the American Thoracic Society · 2026Trial
- Elexacaftor/tezacaftor/ivacaftor in children aged ≥6 years with cystic fibrosis heterozygous forThe European respiratory journal · 2025Trial
- Vanzacaftor-tezacaftor-deutivacaftor for children aged 6-11 years with cystic fibrosis (RIDGELINE Trial VX21-121-105): an analysis from a single-arm, phase 3 trial.The Lancet. Respiratory medicine · 2025Trial
- Suboptimal vaccine coverage for preventable respiratory infections in children with cystic fibrosis in the Cystic Fibrosis Regional Reference Centre of Tuscany: Need for improving preventive strategies.Human vaccines & immunotherapeutics · 2026Article
- Post-exercise hypoglycaemia in adults with cystic fibrosis compared to healthy control adults.Journal of clinical & translational endocrinology · 2026Article
- Long-term effectiveness and safety of cystic fibrosis transmembrane conductance regulator modulators and their impact on the lives of people with cystic fibrosis.American journal of respiratory and critical care medicine · 2026Article
- Anti-inflammatory and phosphorylation effects of CFTR modulator triple therapy in cystic fibrosis.iScience · 2026Article
- Elexacaftor-tezacaftor-ivacaftor for individuals with residual CFTR activity: an open-label, nonrandomized clinical trial.Annals of the American Thoracic Society · 2026Article
- SOLAR: a noninterventional study of outcomes over the long term of people with cystic fibrosis (CF) aged ≥6 years treated with elexacaftor/tezacaftor/ivacaftor using data from the French CF Registry.ERJ open research · 2026Article
- Bridging the Gap in Eastern European Cystic Fibrosis Care: How Newborn Screening and Advanced CFTR Modulation Shape the Clinical Landscape in Western Romania.Children (Basel, Switzerland) · 2026Article
- Pregnancy-related effect on elexacaftor, tezacaftor and ivacaftor pharmacokinetics in women with cystic fibrosis.British journal of clinical pharmacology · 2026Observational
- Sinus Surgery Improves Maxillary Sinus Deformation in Cystic Fibrosis Detected by Magnetic Resonance Imaging.American journal of rhinology & allergy · 2026Article
- Contemporary 0.55 T MRI for Lung Disease Assessment in Children and Young Adults with Cystic Fibrosis: A Proof-of-Concept Study.Tomography (Ann Arbor, Mich.) · 2026Article
- Review
- Patient-Reported Health-Related Quality of Life in Romanian Patients with Cystic Fibrosis in the Era of Highly Effective CFTR Modulator Therapy: A National Cross-Sectional Mixed-Methods Survey.Journal of clinical medicine · 2026Article
- Effects of elexacaftor/tezacaftor/ivacaftor on the nasal microbial metagenome in cystic fibrosis.Microbiology spectrum · 2026Article
- Comparing Multiple Breath Washout Parameters in a Matched Cohort of Children With Cystic Fibrosis and Primary Ciliary Dyskinesia.Pediatric pulmonology · 2026Article
- Developmental Progress and Future Potential for Inhaled Biologics in the Treatment of Respiratory Diseases.Drugs · 2026Review
- Endoscopic sinus surgery for lung transplanted cystic fibrosis patients: a systematic review and meta-analysis.European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery · 2026Review
- Extracellular-Vesicle-Associated Nucleic Acids in the Diagnosis and Treatment of Respiratory Diseases: A Narrative Review.Pharmaceutics · 2026Review
63 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
6 authors.
Funding
Abstract
Cystic fibrosis is a rare genetic disease caused by mutations in CFTR, the gene encoding cystic fibrosis transmembrane conductance regulator (CFTR). The discovery of CFTR in 1989 has enabled the unravelling of disease mechanisms and, more recently, the development of CFTR-directed therapeutics that target the underlying molecular defect. The CFTR protein functions as an ion channel that is crucial for correct ion and fluid transport across epithelial cells lining the airways and other organs. Consequently, CFTR dysfunction causes a complex multi-organ disease but, to date, most of the morbidity and mortality in people with cystic fibrosis is due to muco-obstructive lung disease. Cystic fibrosis care has long been limited to treating symptoms using nutritional support, airway clearance techniques and antibiotics to suppress airway infection. The widespread implementation of newborn screening for cystic fibrosis and the introduction of a highly effective triple combination CFTR modulator therapy that has unprecedented clinical benefits in up to 90% of genetically eligible people with cystic fibrosis has fundamentally changed the therapeutic landscape and improved prognosis. However, people with cystic fibrosis who are not eligible based on their CFTR genotype or who live in countries where they do not have access to this breakthrough therapy remain with a high unmet medical need.
Indexed as
Identifiers
39117676What 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.