Serial lung function and responsiveness in cystic fibrosis during early childhood

Am J Respir Crit Care Med. 2004 Jun 1;169(11):1209-16. doi: 10.1164/rccm.200303-347OC. Epub 2004 Mar 17.


In a 4-year prospective study, we evaluated specific airway resistance (sRaw) by whole-body plethysmography, respiratory resistance by the interrupter technique, and respiratory resistance and reactance at 5 Hz by the impulse oscillation technique combined with measurement of responsiveness to bronchodilators and cold air in 30 children (mean [range] age 5.7 [2 to 8] years) with cystic fibrosis (CF). Spirometry was done at school age. Mean sRaw was consistently abnormal: the mean z score (SD) was 2.52 (2.02) (p < 0.001) at the start and was unchanged 36 months later at 2.74 (2.02). Mean z score (SD) for FEV(1) at first satisfactory measurement, at a mean age (range) of 6.1 (4.9-7.5) years was -1.2 (1.2) and was further reduced to -1.85 (1.2) 4 years from inclusion at a mean age (range) of 9.9 (6.8-12) years. Neither respiratory resistance by the interrupter technique nor the impulse oscillation technique demonstrated consistent abnormal levels. Patients with CF as a group did not differ from healthy subjects in responsiveness to bronchodilators and cold air. sRaw may be a useful tool in CF during early childhood. Reduced lung function was documented from consistently abnormal levels of sRaw and FEV1 during the study. Bronchodilator responsiveness and response to cold air challenge were normal.

Publication types

  • Comparative Study
  • Evaluation Study

MeSH terms

  • Airway Resistance / drug effects
  • Airway Resistance / physiology
  • Bronchial Provocation Tests
  • Bronchodilator Agents / therapeutic use
  • Child
  • Child Welfare
  • Child, Preschool
  • Cold Temperature
  • Cystic Fibrosis / drug therapy
  • Cystic Fibrosis / microbiology
  • Cystic Fibrosis / physiopathology*
  • Female
  • Forced Expiratory Volume / drug effects
  • Forced Expiratory Volume / physiology
  • Humans
  • Longitudinal Studies
  • Lung / physiopathology*
  • Male
  • Prospective Studies
  • Pseudomonas aeruginosa
  • Reproducibility of Results
  • Spirometry
  • Staphylococcus aureus
  • Statistics as Topic
  • Treatment Outcome


  • Bronchodilator Agents