Risk factors for rate of decline in FEV1 in adults with cystic fibrosis
Open accessAbstract
Background Previously we assessed risk factors for FEV(1) decline in children and adolescents using the Epidemiologic Study of Cystic Fibrosis (J Pediatr 2007;151:134-139); the current study assessed risk factors in adults. Methods Risk factors for FEV(1) decline over 3-5.5 years for ages 18-24 and ≥25 years were assessed using mixed-model regression. Results Mean rates of FEV(1) decline (% predicted/year) were -1.92 for ages 18-24y (n=2793) and -1.45 for ages ≥25y (n=1368). For the 18-24y group, B. cepacia, pancreatic enzyme use, multidrug-resistant P. aeruginosa, cough, mucoid P. aeruginosa, and female sex predicted greater decline; low baseline FEV(1) and sinusitis predicted less decline. For the ≥25y group, only pancreatic enzyme use predicted greater decline; low baseline FEV(1) and sinusitis predicted less decline. Conclusions Risk factors for FEV(1) decline in adults <25 years are similar to those previously identified in children and adolescents; older adults had few statistically significant risk factors.
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Pulmonary exacerbations are important clinical events associated with disease progression.
“Pulmonary exacerbations are important clinical events associated with disease progression21-24; elexacaftor–tezacaftor–ivacaftor resulted in a lower rate of pulmonary exacerbations, including severe events leading to hospitalization or treatment with intravenous antibiotics, than placebo.”
From Elexacaftor–Tezacaftor–Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele (2019) · cites this paper as [23]
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Research works printed but not linked: 18
- [1]Corey M Edwards L Levison H Knowles M Longitudinal analysis of pulmonary function decline in patients with cystic fibrosis J Pediatr 1997 131 809 814 9427882
- [2]Schluchter MD Konstan MW Davis PB Jointly modeling the relationship between survival and pulmonary function in cystic fibrosis patients Stat Med 2002 21 1271 1287 12111878
- [3]Konstan MW Morgan WJ Butler SM Risk factors for rate of decline in forced expiratory volume in one second in children and adolescents with cystic fibrosis J Pediatr 2007 151 134 139 17643762
- [4]Morgan WJ Butler SM Johnson CA Epidemiologic study of cystic fibrosis: design and implementation of a prospective, multicenter, observational study of patients with cystic fibrosis in the U.S. and Canada Pediatr Pulmonol 1999 28 231 41 10497371
- [5]Hankinson JL Odencrantz JR Fedan KB Spirometric reference values from a sample of the general U.S. population Am J Respir Crit Care Med 1999 159 179 187 9872837
- [6]The Cystic Fibrosis Genotype-Phenotype Consortium Correlation between genotype and phenotype in patients with cystic fibrosis New Engl J Med 1993 329 1308 1313 8166795
- [7]Kristidis P Bozon D Corey M Markiewicz D Rommens J Tsui LC Genetic determination of exocrine pancreatic function in cystic fibrosis Am J Hum Genet 1992 50 6 1178 1184 1376016 PMC1682557
- [8]Corey M Levison H Crozier D Five- to seven-year course of pulmonary function in cystic fibrosis Am Rev Respir Dis 1976 114 1085 1192 1008344
- [9]Davis PB Byard PJ Konstan MW Identifying treatments that halt progression of pulmonary disease in cystic fibrosis Pediatr Res 1997 41 161 165 9029632
- [10]Konstan MW Wagener JS Pasta DJ Clinical use of dornase alfa is associated with a slower rate of FEV1 decline in cystic fibrosis Pediatr Pulmonol 2011 46 545 553 21438174 PMC4109161
- [11]Umetsu DT Moss RB King VV Lewiston NJ Sinus disease in patients with severe cystic fibrosis; relation to pulmonary exacerbation Lancet 1990 335 1077 1078 1970379
- [12]Kerem E Corey M Gold R Levison H Pulmonary function and clinical course in patients with cystic fibrosis after pulmonary colonization with Pseudomonas aeruginosa J Pediatr 1990 116 714 719 2109790
- [13]Lewin LO Byard PJ Davis PB Effect of Pseudomonas cepacia colonization on survival and pulmonary function of cystic fibrosis patients J Clin Epidemiol 1990 43 125 131 2303842
- [14]Demko CA Byard PJ Davis PB Gender differences in cystic fibrosis: Pseudomonas aeruginosa infection J Clin Epidemiol 1995 48 1041 1049 7775991
- [15]Steinkamp G Wiedemann B Relationship between nutritional status and lung function in cystic fibrosis: cross sectional and longitudinal analyses from the German CF quality assurance (CFQA) project Thorax 2002 57 596 601 12096202 PMC1746376
- [16]Schaedel C de Monestrol I Hjelte L Predictors of deterioration of lung function in cystic fibrosis Pediatr Pulmonol 2002 33 483 491 12001283
- [17]Liou TG Adler FR Cox DR Cahill BC Lung transplantation and survival in children with cystic fibrosis N Engl J Med 2007 357 2143 2152 Erratum in: N Engl J Med. 2008; 359:e6 18032764 PMC2522236
- [18]Konstan MW Wagener JS Yegin A Millar SJ Pasta DJ VanDevanter DR Design and powering of cystic fibrosis clinical trials using rate of FEV1 decline as an efficacy endpoint J Cyst Fibros 2010 9 332 338 20646968 PMC4079112
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- Authors
- Konstan, M. W., Wagener, J. S., VanDevanter, D. R., Pasta, D. J., Yegin, A., Rasouliyan, L., & Morgan, W. J.
- Affiliations
- Rainbow Babies & Children's Hospital (US)
- University of Colorado Anschutz (US)
- Genentech (US)
- University of Arizona (US)
- Corresponding author
- Konstan, Michael W.
- Venue
- Journal of Cystic Fibrosis
- Publisher
- Elsevier BV
- Published
- 2012-09
- Volume
- 11
- Issue
- 5
- Pages
- 405-411
- ISSN
- 1569-1993 (ISSN-L)
- PMC
- PMC4086189
- OpenAlex
- W1995117300
- Cited by
- 115
- References
- 0
- Citations by year
- 2026: 3 · 2025: 5 · 2024: 7 · 2023: 5 · 2022: 7 · 2021: 12 · 2020: 14 · 2019: 7 · 2018: 7 · 2017: 15 · 2016: 7 · 2015: 12 · 2014: 8 · 2013: 5 · 2012: 1
- FWCI
- 6.48
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Journal of Cystic Fibrosis (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- Genentech
- National Institute of Diabetes and Digestive and Kidney Diseases (P30DK027651)
- 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
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Kidney Cyst Diseases
- Keywords
- FEV1 decline, risk factors, adults, Pseudomonas aeruginosa, Burkholderia cepacia, multidrug-resistant Pseudomonas aeruginosa, mucoid Pseudomonas aeruginosa, pancreatic enzyme replacement therapy, sinusitis, cough, female sex, lung function
- MeSH terms
- Adult, Age Factors, Cystic Fibrosis, Female, Humans, Male, Nutritional Status, Pancreas, Pseudomonas aeruginosa, Pseudomonas Infections, Regression Analysis, Respiratory Function Tests, Respiratory System, Respiratory Tract Infections, Risk Factors, Sex Factors, Disease Progression, Enzyme Replacement Therapy
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