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

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

  • Michael W. Konstan, MD

      Affiliations

    • Department of Pediatrics, Rainbow Babies and Children’s Hospital and Case Western Reserve University School of Medicine, Cleveland, Ohio
    • Corresponding Author InformationReprint requests: Michael W. Konstan, MD, Rainbow Babies and Children’s Hospital, 11100 Euclid Ave, Cleveland, OH 44106.
    • ,
  • Wayne J. Morgan, MD

      Affiliations

    • Department of Pediatrics and Physiology, University of Arizona, Tucson, Arizona
    • ,
  • Steven M. Butler, PhD

      Affiliations

    • Specialty Biotherapeutics, Genentech, Inc., South San Francisco, California
    • ,
  • David J. Pasta, MS

      Affiliations

    • Ovation Research Group, San Francisco, California
    • ,
  • Marcia L. Craib, MS

      Affiliations

    • Specialty Biotherapeutics, Genentech, Inc., South San Francisco, California
    • ,
  • Stefanie J. Silva, MS

      Affiliations

    • Ovation Research Group, San Francisco, California
    • ,
  • Dennis C. Stokes, MD

      Affiliations

    • Department of Pediatrics, Children’s Hospital at Dartmouth and Dartmouth Medical School, Lebanon, New Hampshire
    • ,
  • Mary Ellen B. Wohl, MD

      Affiliations

    • Dept. of Pediatrics, Children’s Hospital and Harvard Medical School, Boston, Massachusetts
    • ,
  • Jeffrey S. Wagener, MD

      Affiliations

    • Specialty Biotherapeutics, Genentech, Inc., South San Francisco, California
    • Department of Pediatrics, The Children’s Hospital and University of Colorado School of Medicine, Denver, Colorado
    • ,
  • Warren E. Regelmann, MD

      Affiliations

    • Department of Pediatrics, University of Minnesota, Minneapolis, Minnesota.
    • ,
  • Charles A. Johnson, MBChB

      Affiliations

    • Specialty Biotherapeutics, Genentech, Inc., South San Francisco, California
    • ,
  • Scientific Advisory Group and the Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis

Received 30 June 2006; received in revised form 1 December 2006; accepted 5 March 2007. published online 26 June 2007.

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.

Abbreviations: CF, Cystic fibrosis, ESCF, Epidemiologic Study of Cystic Fibrosis, FEV1, Forced expiratory volume in 1 second

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

PII: S0022-3476(07)00245-4

doi:10.1016/j.jpeds.2007.03.006

Refers to article:

  • Pacing the Marathon: Rate of Decline of Pulmonary Function in Cystic Fibrosis

    Pamela B. Davis
    The Journal of Pediatrics August 2007 (Vol. 151, Issue 2, Pages 111-113)

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

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