ArticleAmerican journal of respiratory and critical care medicine2017
Phenotypes of Rapid Cystic Fibrosis Lung Disease Progression during Adolescence and Young Adulthood.
Article in American journal of respiratory and critical care medicine, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers.
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Who cites it
29 citing papers in PubMed, 60 citations in OpenAlex.
- Patterns of weight gain during the first half of pregnancy and risk of large newborns in women with insulin-dependent diabetes mellitus.Pregnancy (Hoboken, N.J.) · 2026Article
- Hypercubes to identify geomarkers of rapid cystic fibrosis lung disease progression.BMC medical informatics and decision making · 2025Article
- Predicting return of lung function after a pulmonary exacerbation using the cystic fibrosis respiratory symptom diary-chronic respiratory infection symptom scale.BMC pulmonary medicine · 2024Observational
- Clinical implications of innate immune exhaustion in cystic fibrosis.ERJ open research · 2024Article
- Predicting lung function decline in cystic fibrosis: the impact of initiating ivacaftor therapy.Respiratory research · 2024Article
- Social-environmental phenotypes of rapid cystic fibrosis lung disease progression in adolescents and young adults living in the United States.Environmental advances · 2023Article
- Built environment factors predictive of early rapid lung function decline in cystic fibrosis.Pediatric pulmonology · 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
- Ventilatory Threshold and Risk of Pulmonary Exacerbations in Cystic Fibrosis.Respiratory care · 2023Article
- Patient and Caregiver Perceptions of Airway Clearance Methods Used for Cystic Fibrosis.Canadian respiratory journal · 2023Article
- Organoid Technology and Its Role for Theratyping Applications in Cystic Fibrosis.Children (Basel, Switzerland) · 2022Review
- Ivacaftor Alters Macrophage and Lymphocyte Infiltration in the Lungs Following Lipopolysaccharide Exposure.ACS pharmacology & translational science · 2022Article
- Lung clearance index to characterize clinical phenotypes of children and adolescents with cystic fibrosis.BMC pulmonary medicine · 2022Article
- Rapid cystic fibrosis lung-function decline and in-vitro CFTR modulation.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2021Article
- Long term clinical effectiveness of ivacaftor in people with the G551D CFTR mutation.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2021Observational
- An Animated Functional Data Analysis Interface to Cluster Rapid Lung Function Decline and Enhance Center-Level Care in Cystic Fibrosis.Journal of healthcare engineering · 2021Article
- A Volatile and Dynamic Longitudinal Microbiome Is Associated With Less Reduction in Lung Function in Adolescents With Cystic Fibrosis.Frontiers in cellular and infection microbiology · 2021Article
- Integrating latent classes in the Bayesian shared parameter joint model of longitudinal and survival outcomes.Statistical methods in medical research · 2020Article
- Sleep-disordered breathing in cystic fibrosis.Sleep medicine · 2020Article
- Functional data analysis and prediction tools for continuous glucose-monitoring studies.Journal of clinical and translational science · 2020Article
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Authors and funding
7 authors at 6 institutions in 1 country.
Funding
Abstract
rationaleIndividuals with cystic fibrosis are at risk for prolonged drops in lung function, clinically termed rapid decline, during discreet periods of the disease.
objectivesTo identify phenotypes of rapid pulmonary decline and determine how these phenotypes are related to patient characteristics.
methodsA longitudinal cohort study of patients with cystic fibrosis aged 6-21 years was conducted using the Cystic Fibrosis Foundation Patient Registry. A statistical approach for clustering longitudinal profiles, sparse functional principal components analysis, was used to classify patients into distinct phenotypes by evaluating trajectories of FEV MEASUREMENTS AND MAIN
resultsThree distinct phenotypes of rapid decline were identified, corresponding to early, middle, and late timing of maximal FEV
conclusionsBy identifying phenotypes and associated risk factors, timing of interventions may be more precisely targeted for subgroups at highest risk of lung function loss.
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