Changes in pulmonary mechanics with increasing disease severity in children and young adults with cystic fibrosis.
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.[1]References
- Changes in pulmonary mechanics with increasing disease severity in children and young adults with cystic fibrosis. Hart, N., Polkey, M.I., Clément, A., Boulé, M., Moxham, J., Lofaso, F., Fauroux, B. Am. J. Respir. Crit. Care Med. (2002) [Pubmed]
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