ArticleJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society2023
Cystic fibrosis prevalence in the United States and participation in the Cystic Fibrosis Foundation Patient Registry in 2020.
Article in Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 35 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
35 citing papers in PubMed, 56 citations in OpenAlex.
- TIDES 2.0: A protocol for examining the prevalence and longitudinal course of depression, anxiety and behaviour problems in children with cystic fibrosis under 12 years of age.BMJ open respiratory research · 2026Article
- Feasibility and Impact of a Coached Home-Based Exercise Program on Cardiorespiratory Fitness in Adults With Cystic Fibrosis on Highly Effective Modulator Therapy.CHEST pulmonary · 2026Article
- The Impact of Personal-Level Social Networks on Treatment Adherence, Depression, Anxiety, and Perceived Stigma Among Adults with Cystic Fibrosis: An Egocentric Network Analysis.International journal of behavioral medicine · 2026Article
- Kidney Failure in Persons With Cystic Fibrosis.Kidney medicine · 2026Article
- Effect of Elexacaftor/Tezacaftor/Ivacaftor Treatment on Extrapulmonary Complications of Cystic Fibrosis.Advances in therapy · 2026Article
- Chasing the ghost in the code: highlighting the mystery of a rare genetic condition.American journal of physiology. Cell physiology · 2026Review
- Challenges to Assessing the Prevalence of Cystic Fibrosis in the Caribbean.Pediatric pulmonology · 2026Review
- Microbial Ecological Signatures Predict Pathogen Emergence and Multidrug Resistance in Cystic Fibrosis Airways up to a Year in Advance.medRxiv : the preprint server for health sciences · 2026Article
- Beyond the Surface: Novel Therapy Approach for Pancreatitis in the Setting of CFTR Dysfunction.Case reports in gastrointestinal medicine · 2026Article
- Postpartum Tubal Sterilization in Sickle Cell Disease in the 2012-2019 National Inpatient Sample.Journal of women's health (2002) · 2025Article
- Challenges and opportunities for drug development in rare pulmonary diseases like cystic fibrosis: an industry perspective.Current opinion in pulmonary medicine · 2025Review
- Efficacy of elexacaftor/tezacaftor/ivacaftor in non-cystic fibrosis bronchiectasis patients with a single pathogenicERJ open research · 2025Article
- Elexacaftor/tezacaftor/ivacaftor prescription in lung transplant recipients with cystic fibrosis in the US.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2025Article
- Review of Disordered Eating Behaviors in Cystic Fibrosis.Life (Basel, Switzerland) · 2025Review
- ACE-tRNAs are a platform technology for suppressing nonsense mutations that cause cystic fibrosis.Nucleic acids research · 2025Article
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- The Real-World Effectiveness of Antifungals in People with Cystic Fibrosis andAnnals of the American Thoracic Society · 2025Article
- Cystic fibrosis-related bone disease: an update on screening, diagnosis, and treatment.Therapeutic advances in endocrinology and metabolism · 2025Review
- Validating the Data Completeness and Accuracy of the Canadian Cystic Fibrosis Registry.Canadian respiratory journal · 2025Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
8 authors at 2 institutions in 1 country.
Funding
Abstract
backgroundThe Cystic Fibrosis Foundation Patient Registry (CFFPR) collects data on individuals with cystic fibrosis (CF) in the United States (US). In 2012, the US CF population was estimated at 33,292 to 34,327 individuals, with 81-84% CFFPR participation.
methodsIn this study, we update these estimates via simulation to account for uncertainty in CF incidence by race or Hispanic ethnicity, initiation of CF newborn screening (NBS) programs by state, and updated cumulative survival for CF births 1968-2020. We defined registry participation as the proportion of individuals alive as of 2020 with any prior CFFPR participation as well as the proportion with contributing data in 2019 or 2020; we summarize CFFPR participation for those born prior to 1968.
resultsWe estimated the 2020 prevalent CF population between 1968-2020 to be 38,804 (95% Uncertainty Interval (UI): 38,532 to 39,065) individuals, with 77% of the prevalent CF population contributing recent data. CFFPR participation differs by age (54% of those born in 1968) and exceeds >90% of the population born in 2009 or later.
conclusionsWe demonstrate that the CFFPR remains a valid data source generalizable to the CF population. High participation among younger individuals may reflect the success of newborn screening programs and early referral to CF care. If engagement can be sustained, the percentage of individuals participating in the CFFPR will grow over time and there is an opportunity to identify factors associated with loss to follow up among older individuals to optimize the quality of the CFFPR data.
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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.