Risk Factors For Rate of Decline in Forced Expiratory Volume in One Second in Children and Adolescents with Cystic Fibrosis

• Published in: The Journal of pediatrics Vol. 151; no. 2; pp. 134 - 139.e1
• Main Authors: Konstan, Michael W., Morgan, Wayne J., Butler, Steven M., Pasta, David J., Craib, Marcia L., Silva, Stefanie J., Stokes, Dennis C., Wohl, Mary Ellen B., Wagener, Jeffrey S., Regelmann, Warren E., Johnson, Charles A.
• Format: Journal Article
• Language: English
• Published: New York, NY Mosby, Inc 01.08.2007; Elsevier
• Subjects: Adolescent; Age Distribution; Biological and medical sciences; Child; Cohort Studies; Cystic Fibrosis - diagnosis; Cystic Fibrosis - epidemiology; Disease Progression; Epidemiology; Errors of metabolism; Female; Forced Expiratory Volume; General aspects; Humans; Incidence; Lung Diseases - diagnosis; Lung Diseases - epidemiology; Male; Medical sciences; Metabolic diseases; Miscellaneous hereditary metabolic disorders; Pediatrics; Prognosis; Public health. Hygiene; Public health. Hygiene-occupational medicine; Risk Factors; Severity of Illness Index; Sex Distribution; Spirometry - methods; Time Factors; Vital Capacity; ESCF; CF; FEV 1; Epidemiologic Study of Cystic Fibrosis; Cystic fibrosis; Forced expiratory volume in 1 second; Human; Pediatrics; Respiratory disease; Metabolic diseases; Epidemiology; Genetic disease; Risk rate; Volume; Adolescent; Risk factor; Digestive diseases; Child; Pancreatic disease
• ISSN: 0022-3476; 1097-6833; 1097-6833
• DOI: 10.1016/j.jpeds.2007.03.006

To characterize the rate of decline of forced expiratory volume in 1 second (FEV 1) in children and adolescents with cystic fibrosis and to identify and compare risk factors associated with FEV 1 decline. The rate of decline in FEV 1% predicted over 3 to 6 years in 3 different age groups was determined. Risk factors for decline were identified and compared among and within age groups as a function of disease severity with repeated-measures, mixed-model regression. Mean (±SD) baseline FEV 1% predicted was 88.4% ± 20.5% for 6- to 8-year-olds (n = 1811), 85.3% ± 20.8% for 9- to 12-year-olds (n = 1696), and 78.4% ± 22.0% for 13- to 17-year-olds (n = 1359). Decline in FEV 1% predicted/year was −1.12, −2.39, and −2.34, respectively. High baseline FEV 1 and persistent crackles were significant independent risk factors for decline across all age groups. Female sex, Pseudomonas aeruginosa infection, low weight-for-age, sputum, wheezing, sinusitis, pulmonary exacerbations treated with intravenous antibiotics, elevated liver test results, and pancreatic insufficiency were also identified as independent risk factors in some age groups. This study identifies risk factors for FEV 1 decline in children and adolescents with cystic fibrosis. Clinicians should not be reassured by high lung function, particularly in young children, because this factor, among others, is independently associated with steeper decline in FEV 1.