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

Konstan, M., Morgan, W., Butler, S., Pasta, D., Craib, M., Silva, S., Stokes, D., Wohl, M., Wagener, J., Regelmann, W. & Johnson, C.

The purpose of this study was to identify risks factors associated with  FEV1 decline in children and adolescents with Cystic Fibrosis (CF) and to characterize it's rate. The authors determined the rate of decline predicted over 3-6 years in 3 different age groups and compared and identified risk factors for this within these age groups as a function of disease severity; with repeated measures, mixed model regression.

Mean (±SD) baseline FEV1% 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 FEV1% predicted/year was ?1.12, ?2.39, and ?2.34, respectively. Across all age groups, high baseline FEV1 and persistent crackles were significant independent risk factors for decline. Also identified were the female sex, an infection of Pseudomonas aeruginosa, low weight for their age, wheezing, sinusitis, pulmonary exacerbations treated with intravenous antibiotics, elevated liver test results, and pancreatic insufficiency.

In conclusion, the study identifies risk factors for FEV1 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 FEV1.

Journal of Pediatrics 2007. 151:(2), 134-139

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