ArticleAnnals of internal medicine2014
Longevity of patients with cystic fibrosis in 2000 to 2010 and beyond: survival analysis of the Cystic Fibrosis Foundation patient registry.
Article in Annals of internal medicine, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT04379726 (Characterization of β-cell Function and Insulin Sensitivity in Pre-transplant Patients With Cystic Fibrosis), which is not on this map. Cited by 141 papers, 4 of them syntheses that pooled it.
What it found
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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.
Characterization of β-cell Function and Insulin Sensitivity in Pre-transplant Patients With Cystic Fibrosis
Who cites it
141 citing papers in PubMed, 4 syntheses or guidelines pooled it.
- Carrier Screening Programs for Cystic Fibrosis, Fragile X Syndrome, Hemoglobinopathies and Thalassemia, and Spinal Muscular Atrophy: A Health Technology Assessment.Ontario health technology assessment series · 2023Pooled it
- Physical activity and exercise training in cystic fibrosis.The Cochrane database of systematic reviews · 2022Pooled it
- Interventions for promoting participation in shared decision-making for children and adolescents with cystic fibrosis.The Cochrane database of systematic reviews · 2019Pooled it
- Effectiveness of positive expiratory pressure on patients over 16 years of age with cystic fibrosis: systematic review and meta-analysis.Therapeutic advances in respiratory diseasePooled it
- A Randomized Clinical Trial of Antimicrobial Duration for Cystic Fibrosis Pulmonary Exacerbation Treatment.American journal of respiratory and critical care medicine · 2021Trial
- Ivacaftor in People with Cystic Fibrosis and aAnnals of the American Thoracic Society · 2021Trial
- Ivacaftor in Infants Aged 4 to <12 Months with Cystic Fibrosis and a Gating Mutation. Results of a Two-Part Phase 3 Clinical Trial.American journal of respiratory and critical care medicine · 2021Trial
- Intravenous versus oral antibiotics for eradication of Pseudomonas aeruginosa in cystic fibrosis (TORPEDO-CF): a randomised controlled trial.The Lancet. Respiratory medicine · 2020Trial
- Home Monitoring of Patients with Cystic Fibrosis to Identify and Treat Acute Pulmonary Exacerbations. eICE Study Results.American journal of respiratory and critical care medicine · 2017Trial
- Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.The New England journal of medicine · 2015Trial
- Cystic Fibrosis Mortality Trends 1999-2024-A CDC Wonder Study.Advances in respiratory medicine · 2026Article
- Kidney Failure in Persons With Cystic Fibrosis.Kidney medicine · 2026Article
- D-Amino acids affect Pseudomonas aeruginosa biofilm and quorum sensing molecules in lung infection models developed under a cystic fibrosis environment.Scientific reports · 2025Article
- The Impact of Physical Activity on Clinical Outcomes in Children with Cystic Fibrosis: A Narrative Review.Children (Basel, Switzerland) · 2025Review
- Pancreatic Status Is Not a Risk Factor for Cystic Fibrosis-Related Bone Disease.Pediatric pulmonology · 2025Article
- Global functional genomics reveals GRK5 as a cystic fibrosis therapeutic target synergistic with current modulators.iScience · 2025Article
- Real-life impact of genotype and severity of lung disease on efficacy of elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis.Pulmonary pharmacology & therapeutics · 2025Observational
- Symptom phenotyping in people with cystic fibrosis during acute pulmonary exacerbations using machine-learning K-means clustering analysis.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2024Article
- Allergic Bronchopulmonary Aspergillosis (ABPA) in the Era of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulators.Journal of fungi (Basel, Switzerland) · 2024Review
- Impact of Anxiety, Depression, and Coping Strategies on Health-Related Quality of Life in Patients with Cystic Fibrosis.Thoracic research and practice · 2024Article
81 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
7 authors.
Funding
Abstract
backgroundAdvances in treatments for cystic fibrosis (CF) continue to extend survival. An updated estimate of survival is needed for better prognostication and to anticipate evolving adult care needs.
objectiveTo characterize trends in CF survival between 2000 and 2010 and to project survival for children born and diagnosed with the disease in 2010.
designRegistry-based study.
setting110 Cystic Fibrosis Foundation-accredited care centers in the United States. PATIENTS: All patients represented in the Cystic Fibrosis Foundation Patient Registry (CFFPR) between 2000 and 2010. MEASUREMENTS: Survival was modeled with respect to age, age at diagnosis, gender, race or ethnicity, F508del mutation status, and symptoms at diagnosis.
resultsBetween 2000 and 2010, the number of patients in the CFFPR increased from 21,000 to 26,000, median age increased from 14.3 to 16.7 years, and adjusted mortality decreased by 1.8% per year (95% CI, 0.5% to 2.7%). Males had a 19% (CI, 13% to 24%) lower adjusted risk for death than females. Median survival of children born and diagnosed with CF in 2010 is projected to be 37 years (CI, 35 to 39 years) for females and 40 years (CI, 39 to 42 years) for males if mortality remains at 2010 levels and more than 50 years if mortality continues to decrease at the rate observed between 2000 and 2010. LIMITATIONS: The CFFPR does not include all patients with CF in the United States, and loss to follow-up and missing data were observed. Additional analyses to address these limitations suggest that the survival projections are conservative.
conclusionChildren born and diagnosed with CF in the United States in 2010 are expected to live longer than those born earlier. This has important implications for prognostic discussions and suggests that the health care system should anticipate greater numbers of adults with CF. PRIMARY FUNDING SOURCE: Cystic Fibrosis Foundation.
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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.