Predictive 5-year survivorship model of cystic fibrosis
Open accessAbstract
The objective of this study was to create a 5-year survivorship model to identify key clinical features of cystic fibrosis. Such a model could help researchers and clinicians to evaluate therapies, improve the design of prospective studies, monitor practice patterns, counsel individual patients, and determine the best candidates for lung transplantation. The authors used information from the Cystic Fibrosis Foundation Patient Registry (CFFPR), which has collected longitudinal data on approximately 90% of cystic fibrosis patients diagnosed in the United States since 1986. They developed multivariate logistic regression models by using data on 5,820 patients randomly selected from 11,630 in the CFFPR in 1993. Models were tested for goodness of fit and were validated for the remaining 5,810 patients for 1993. The validated 5-year survivorship model included age, forced expiratory volume in 1 second as a percentage of predicted normal, gender, weight-for-age z score, pancreatic sufficiency, diabetes mellitus, Staphylococcus aureus infection, Burkerholderia cepacia infection, and annual number of acute pulmonary exacerbations. The model provides insights into the complex nature of cystic fibrosis and supplies a rigorous tool for clinical practice and research.
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Weight in pwCF is an independent predictor of survival.
“Weight and BMI in pwCF are closely correlated with improvements in lung function and are independent predictors of survival.24,25”
Pulmonary exacerbations in pwCF are associated with a greater rate of lung function decline.
“Pulmonary exacerbations are important life events for pwCF and are associated with a greater rate of lung function decline and decreased survival.24,26”
BMI in pwCF is closely correlated with improvements in lung function.
“Weight and BMI in pwCF are closely correlated with improvements in lung function and are independent predictors of survival.24,25”
Pulmonary exacerbations in pwCF are associated with decreased survival.
“Pulmonary exacerbations are important life events for pwCF and are associated with a greater rate of lung function decline and decreased survival.24,26”
BMI in pwCF is an independent predictor of survival.
“Weight and BMI in pwCF are closely correlated with improvements in lung function and are independent predictors of survival.24,25”
Weight in pwCF is closely correlated with improvements in lung function.
“Weight and BMI in pwCF are closely correlated with improvements in lung function and are independent predictors of survival.24,25”
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- Authors
- Liou, T. G., Adler, F. R., FitzSimmons, S. C., Cahill, B. C., Hibbs, J. R., & Marshall, B. C.
- Affiliations
- University of Utah (US)
- Cystic Fibrosis Foundation (US)
- New York State Department of Health (US)
- Venue
- American Journal of Epidemiology
- Publisher
- Oxford University Press (OUP)
- Published
- 2001-02-15
- Volume
- 153
- Issue
- 4
- Pages
- 345-352
- ISSN
- 0002-9262 (ISSN-L)
- 1476-6256
- PMC
- PMC2198936
- OpenAlex
- W2144947606
- Cited by
- 749
- References
- 2
- Citations by year
- 2026: 7 · 2025: 9 · 2024: 20 · 2023: 31 · 2022: 22 · 2021: 33 · 2020: 59 · 2019: 30 · 2018: 44 · 2017: 50 · 2016: 57 · 2015: 39 · 2014: 32 · 2013: 24 · 2012: 31
- FWCI
- 15.52
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- American Journal of Epidemiology (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Funders
- Cystic Fibrosis Foundation
- University of Utah
- National Heart, Lung, and Blood Institute (K08HL003656)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cystic Fibrosis Research Advances
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Chronic Obstructive Pulmonary Disease (COPD) Research
- Health Sciences › Medicine › Surgery › Transplantation: Methods and Outcomes
- Keywords
- cystic fibrosis, five-year survival, prognostic model, FEV1, pancreatic sufficiency, diabetes mellitus, Staphylococcus aureus infection, pulmonary exacerbations, weight-for-age z-score, lung transplantation candidates, patient counseling
- MeSH terms
- Adolescent, Adult, Age Factors, Bacterial Infections, Body Weight, Child, Child, Preschool, Cystic Fibrosis, Female, Forced Expiratory Volume, Humans, Male, Middle Aged, Pancreatic Diseases, Predictive Value of Tests, Sex Factors, Multivariate Analysis, Logistic Models, Proportional Hazards Models, Survival Analysis
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