Changes in pulmonary mechanics with increasing disease severity in children and young adults with cystic fibrosis

Am J Respir Crit Care Med. 2002 Jul 1;166(1):61-6. doi: 10.1164/rccm.2112059.


As forced expiratory volume in 1 second (FEV(1)) is a major predictor of outcome in patients with cystic fibrosis (CF), we investigated the effect of FEV(1) on pulmonary mechanics in children and young adults with CF. We measured respiratory rate; tidal volume; minute ventilation; arterial blood gases; sniff esophageal pressure; dynamic lung compliance; total pulmonary resistance; intrinsic positive end expiratory pressure; and total, elastic, and resistive work of breathing in 32 patients (FEV(1) range: 12-49% predicted). We observed correlations between FEV(1) and Pa(O(2)) (r = 0.76, p < 0.0001) and Pa(CO(2)) (r = -0.70, p < 0.0001), FEV(1) and respiratory rate/tidal volume (r = -0.41, p = 0.02), FEV(1) and dynamic lung compliance (r = 0.64, p < 0.0001), and FEV(1) and total work of breathing (r = -0.52, p = 0.002) and elastic work of breathing (r = -0.60. p = 0.0003). No correlations were observed between FEV(1) and sniff esophageal pressure (p = 0.5), minute ventilation (p = 0.9), total pulmonary resistance (p = 0.3), intrinsic positive end expiratory pressure (p = 0.3), or resistive work of breathing (p = 0.1). As FEV(1) declines in children and young adults with CF, there is an increase in the elastic load and work of breathing, resulting in a rapid shallow breathing pattern, that is associated with further impairment of gas exchange.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Cystic Fibrosis / physiopathology*
  • Disease Progression
  • Forced Expiratory Volume
  • Humans
  • Linear Models
  • Pulmonary Gas Exchange
  • Respiratory Mechanics*
  • Work of Breathing