ArticleAnnals of the American Thoracic Society2014
Risk factors for the progression of cystic fibrosis lung disease throughout childhood.
Article in Annals of the American Thoracic Society, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers, 1 of them a synthesis that pooled it.
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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.
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Who cites it
29 citing papers in PubMed, 1 synthesis or guideline pooled it, 67 citations in OpenAlex.
- Academy of Nutrition and Dietetics: 2020 Cystic Fibrosis Evidence Analysis Center Evidence-Based Nutrition Practice Guideline.Journal of the Academy of Nutrition and Dietetics · 2021Guideline
- Reflections on 50 Years of Cystic Fibrosis Newborn Screening Experience with Critical Perspectives, Assessment of Current Status, and Predictions for Future Improvements.International journal of neonatal screening · 2025Article
- Exploring Therapeutic Strategies for Pediatric Cystic Fibrosis: An In-Depth Comparative Review.Cureus · 2024Review
- Gaps in Cystic Fibrosis Care Are Associated with Reduced Lung Function in the U.S. Cystic Fibrosis Foundation Patient Registry.Annals of the American Thoracic Society · 2023Article
- 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
- Defining and identifying early-onset lung disease in cystic fibrosis with cumulative clinical characteristics.Pediatric pulmonology · 2022Article
- CFTR-mediated monocyte/macrophage dysfunction revealed by cystic fibrosis proband-parent comparisons.JCI insight · 2022Article
- Lung inflammation and simulated airway resistance in infants with cystic fibrosis.Respiratory physiology & neurobiology · 2021Article
- Current and Emerging Therapies to Combat Cystic Fibrosis Lung Infections.Microorganisms · 2021Review
- Hyperinflation is associated with increased respiratory rate and is a more sensitive measure of cystic fibrosis lung disease during infancy compared to forced expiratory measures.Pediatric pulmonology · 2021Article
- Quantification of Phenotypic Variability of Lung Disease in Children with Cystic Fibrosis.Genes · 2021Review
- Newborn screening alone insufficient to improve pulmonary outcomes for cystic fibrosis.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2021Article
- Revealing the Metabolic Alterations during Biofilm Development ofMetabolites · 2021Article
- Lung function over the life course of paediatric and adult patients with cystic fibrosis from a large multi-centre registry.Scientific reports · 2020Article
- Influence of pancreatic status, CFTR mutations, Staphylococcus aureus and/or Pseudomonas aeruginosa infection/colonization on lung function in cystic fibrosis during a 2-year follow-up period.Wiener klinische Wochenschrift · 2020Article
- Applying whole-genome sequencing in relation to phenotype and outcomes in siblings with cystic fibrosis.Cold Spring Harbor molecular case studies · 2020Article
- In utero and postnatal VX-770 administration rescues multiorgan disease in a ferret model of cystic fibrosis.Science translational medicine · 2019Article
- Early life growth patterns persist for 12 years and impact pulmonary outcomes in cystic fibrosis.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2018Article
- Impact of social complexity on outcomes in cystic fibrosis after transfer to adult care.Pediatric pulmonology · 2018Article
- Longitudinal development of initial, chronic and mucoid Pseudomonas aeruginosa infection in young children with cystic fibrosis.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2018Article
Corrections and comments
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Authors and funding
8 authors at 5 institutions in 2 countries.
Funding
Abstract
rationalePrevious studies of risk factors for progression of lung disease in cystic fibrosis (CF) have suffered from limitations that preclude a comprehensive understanding of the determinants of CF lung disease throughout childhood. The epidemiologic component of the 27-year Wisconsin Randomized Clinical Trial of CF Neonatal Screening Project (WI RCT) afforded us a unique opportunity to evaluate the significance of potential intrinsic and extrinsic risk factors for lung disease in children with CF.
objectivesDescribe the most important intrinsic and extrinsic risk factors for progression of lung disease in children with CF.
methodsVariables hypothesized at the onset of the WI RCT study to be determinants of the progression of lung disease and potential risk factors previously identified in the WI RCT study were assessed with multivariable generalized estimating equation models for repeated measures of chest radiograph scores and pulmonary function tests in the WI RCT cohort. MEASUREMENTS AND MAIN
resultsCombining all patients in the WI RCT, 132 subjects were observed for a mean of 16 years and contributed 1,579 chest radiographs, and 1,792 pulmonary function tests. The significant determinants of lung disease include genotype, poor growth, hospitalizations, meconium ileus, and infection with mucoid Pseudomonas aeruginosa. The previously described negative effect of female sex was not seen.
conclusionsModifiable extrinsic risk factors are the major determinants of progression of lung disease in children with CF. Better interventions to prevent or treat these risk factors may lead to improvements in lung health for children with CF.
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Registered trials
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