ReviewEuropean journal of pediatrics2021
A new era for people with cystic fibrosis.
Review in European journal of pediatrics, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 42 papers, 2 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
42 citing papers in PubMed, 2 syntheses or guidelines pooled it.
- Effectiveness of Probiotics, Prebiotics, and Symbiotic Supplementation in Cystic Fibrosis Patients: A Systematic Review and Meta-Analysis of Clinical Trials.Medicina (Kaunas, Lithuania) · 2025Pooled it
- Caregiver burden in cystic fibrosis: a systematic literature review.Therapeutic advances in respiratory diseasePooled it
- Ecological Patterns of Gut Microbiota During Elexacaftor/Tezacaftor/Ivacaftor Therapy in Cystic Fibrosis.Microorganisms · 2026Article
- Mental health, sleep, and quality of life following CFTR modulator therapy: a longitudinal study of children with cystic fibrosis and caregivers.European journal of pediatrics · 2026Observational
- Prediction of Antimicrobial Resistance in People Living With Cystic Fibrosis Using Machine Learning.MedComm · 2026Article
- Early effect of CFTR modulators on the mental health of patients with cystic fibrosis and parents.European journal of pediatrics · 2026Observational
- A qualitative exploration of the experiences of adults with cystic fibrosis unable to benefit from triple combination therapy.Journal of health psychology · 2026Article
- L467F;F508del Complex Allele in a Heterozygous State with CFTRdele2,3: What to Expect from CFTR Modulators?International journal of molecular sciences · 2025Article
- Article
- Physical Training and Pulmonary Rehabilitation in Patients with Cystic Fibrosis: A Systematic Review and Meta-Analysis of Clinical Trials.Healthcare (Basel, Switzerland) · 2025Review
- Burkholderia Cenocepacia MALDI-ToF Mass Spectra in the Assessing the Course of Lower Respiratory Tract Infection in Patients with Cystic Fibrosis.Indian journal of microbiology · 2025Article
- Clinical Disorders in Cystic Fibrosis That Affect Emergency Procedures-A Case Report and Review.Journal of clinical medicine · 2025Review
- Lung Organoids from hiPSCs Can Be Efficiently Transduced by Recombinant Adeno-Associated Viral and Adenoviral Vectors.Biomedicines · 2025Article
- Lived experiences for individuals with cystic fibrosis who have undergone lung transplantation: a qualitative study.BMC nursing · 2025Article
- Towards effective cystic fibrosis gene therapy by optimizing prime editing and pulmonary-targeted LNPs.Frontiers in systems biology · 2025Article
- Proteomics profiling of inflammatory responses to elexacaftor/tezacaftor/ivacaftor in cystic fibrosis.Frontiers in immunology · 2025Article
- Rheological comparison of sputum and reconstituted airway epithelium mucus.Scientific reports · 2024Article
- Review
- Review
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Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
4 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Cystic fibrosis is the most prevalent inherited disease caused by a defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The impaired electrolyte homeostasis caused by the mutated or absent protein leads to symptoms in multiple organ systems. However, the pulmonary manifestation with chronic infections and eventually respiratory failure remains the most important threat. Until one decade ago, only symptomatic treatment was available. However, since 2012, different combinations of CFTR modulators are available for people with cystic fibrosis (pwCF) that carry different mutations. The advent of these drugs has impressively changed life expectancy and quality of life in people with cystic fibrosis and raised new challenges regarding long-term complications and tapering of conventional therapies.Conclusion: In this review, we provide an update on the latest developments around diagnostics, treatment, and prognosis of pwCF. What is Known: • Cystic fibrosis is an incurable and life-shortening disease asking for life-long symptomatic treatment. • Three combination CFTR modulating drugs has gained marked approval over the last 10 years. What is New: • The emerge of new (modulating) therapies contribute to the increasing life expectancy. • A high unmet need to develop new therapies for people with CF who cannot access or benefit from these drugs remains. This review gives an update on the current status.
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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.