Elsevier

The Journal of Pediatrics

Volume 151, Issue 2, August 2007, Pages 134-139.e1
The Journal of Pediatrics

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

https://doi.org/10.1016/j.jpeds.2007.03.006Get rights and content

Objectives

To characterize the rate of decline of forced expiratory volume in 1 second (FEV1) in children and adolescents with cystic fibrosis and to identify and compare risk factors associated with FEV1 decline.

Study design

The rate of decline in FEV1% 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.

Results

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. High baseline FEV1 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.

Conclusions

This 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.

Section snippets

Methods

Patients 6 to 17 years old were included if they had at least 3 spirometry measurements obtained at times of clinical stability after enrollment in ESCF. The first measurement was defined as the baseline spirometry. A 1-year lead-in period followed, during which baseline clinical characteristics were assessed. The subsequent 5.5 years comprised the observation period. To ensure adequate long-term follow-up, spirometry must have been performed at times of clinical stability at least once during

Results

Of the 4923 patients who met the inclusion criteria, 4866 (98.8%) had data available for all of the final risk factors and were included in the final model. Baseline and lead-in year characteristics are shown in Table I. Progression of lung disease is evident on the basis of a cross-sectional analysis of baseline FEV1, which was highest in the 6- to 8-year-old group and lowest in the 13- to 17-year-old group. The percentage of patients with a positive respiratory tract culture for P aeruginosa

Discussion

In this analysis, we characterized the rate of FEV1 decline in children and adolescents with CF who participated in a large, prospective observational study. The rates of FEV1 decline that we estimated were either of the same magnitude or less than those previously published from studies assessing FEV1 decline in individuals with CF.1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19 Moreover, we identified many of the same risk factors for FEV1 decline. Both of these observations

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    Supported by Genentech, Inc., South San Francisco, Calif.

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