ArticleAmerican journal of respiratory and critical care medicine2010
Effects of gender and age at diagnosis on disease progression in long-term survivors of cystic fibrosis.
Article in American journal of respiratory and critical care medicine, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT02262871 (Randomized Cross-over Physiologic Study of High Flow Nasal Oxygen Cannula Versus Non-invasive Ventilation in Cystic Fibrosis. The HIFEN Study), which is not on this map. Cited by 41 papers.
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.
Randomized Cross-over Physiologic Study of High Flow Nasal Oxygen Cannula Versus Non-invasive Ventilation in Cystic Fibrosis. The HIFEN Study
Who cites it
41 citing papers in PubMed, 125 citations in OpenAlex.
- Cystic Fibrosis Mortality Trends 1999-2024-A CDC Wonder Study.Advances in respiratory medicine · 2026Article
- Transition from pediatric to adult care in thoracic transplantation-Challenges and opportunities.JHLT open · 2025Article
- Aging with CF: Characteristics of people with CF aged 40 and older in the United States.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2025Article
- Sex differences in airway disease: estrogen and airway surface liquid dynamics.Biology of sex differences · 2024Review
- Article
- Cystic fibrosis-related mortality in the United States from 1999 to 2020: an observational analysis of time trends and disparities.Scientific reports · 2023Observational
- Risk factors for forced expiratory volume in 1 s decline in European patients with cystic fibrosis: data from the European Cystic Fibrosis Society Patient Registry.ERJ open research · 2023Article
- Cystic fibrosis associated with Wernicke's encephalopathy in an older adult.BMJ case reports · 2022Article
- Just breathe: a review of sex and gender in chronic lung disease.European respiratory review : an official journal of the European Respiratory Society · 2022Review
- Differences in Nutritional Status and Inflammatory Biomarkers between Female and Male Patients with Bronchiectasis: A Large-Cohort Study.Biomedicines · 2021Article
- Fighting diagnostic confirmation bias: Cystic fibrosis, allergic bronchopulmonary aspergillus, or both?Clinical case reports · 2021Article
- Sex and Gender Differences in Lung Disease.Advances in experimental medicine and biology · 2021Article
- Review
- Microbial clues lead to a diagnosis of cystic fibrosis in late adulthood.BMJ case reports · 2020Article
- Cystic Fibrosis Patients Infected With EpidemicFrontiers in cellular and infection microbiology · 2020Article
- The demographics of adverse outcomes in cystic fibrosis.Pediatric pulmonology · 2019Review
- Article
- Disparities in Mortality of Hispanic Patients with Cystic Fibrosis in the United States. A National and Regional Cohort Study.American journal of respiratory and critical care medicine · 2018Article
- Gender differences in bronchiectasis: a real issue?Breathe (Sheffield, England) · 2018Review
- Up-to-date and projected estimates of survival for people with cystic fibrosis using baseline characteristics: A longitudinal study using UK patient registry data.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2018Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
16 authors at 3 institutions in 1 country.
Funding
Abstract
rationaleLong-term survivors of cystic fibrosis (CF) (age > 40 yr) are a growing population comprising both patients diagnosed with classic manifestations in childhood, and nonclassic phenotypes typically diagnosed as adults. Little is known concerning disease progression and outcomes in these cohorts.
objectivesExamine effects of age at diagnosis and gender on disease progression, setting of care, response to treatment, and mortality in long-term survivors of CF.
methodsRetrospective analysis of the Colorado CF Database (1992-2008), CF Foundation Registry (1992-2007), and Multiple Cause of Death Index (1992-2005). MEASUREMENTS AND MAIN
resultsPatients with CF diagnosed in childhood and who survive to age 40 years have more severe CFTR genotypes and phenotypes compared with adult-diagnosed patients. However, past the age of 40 years the rate of FEV(1) decline and death from respiratory complications were not different between these cohorts. Compared with males, childhood-diagnosed females were less likely to reach age 40 years, experienced faster FEV(1) declines, and no survival advantage. Females comprised the majority of adult-diagnosed patients, and demonstrated equal FEV(1) decline and longer survival than males, despite a later age at diagnosis. Most adult-diagnosed patients were not followed at CF centers, and with increasing age a smaller percentage of CF deaths appeared in the Cystic Fibrosis Foundation Registry. However, newly diagnosed adults demonstrated sustained FEV(1) improvement in response to CF center care.
conclusionsFor patients with CF older than 40 years, the adult diagnosis correlates with delayed but equally severe pulmonary disease. A gender-associated disadvantage remains for females diagnosed in childhood, but is not present for adult-diagnosed females.
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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.