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Changes in Pulmonary Mechanics with Increasing Disease Severity in Children and Young Adults with Cystic Fibrosis

Pediatric Pulmonary Department, Armand Trousseau Hospital-AP-HP INSERM E 0213, Paris; Department of Clinical Physiology, Raymond Poincaré Hospital AP-HP, Garches, France; Respiratory Muscle Laboratory, Royal Brompton Hospital; and Department of Respiratory Medicine and Allergy, Guy's, King's and St. Thomas' School of Medicine, King's College Hospital, London, United Kingdom

Correspondence and requests for reprints should be addressed to Dr. Brigitte Fauroux, Pediatric Pulmonary Department, Armand Trousseau Hospital, 28 Avenue du Docteur Arnold Netter, 75012 Paris, France. E-mail: brigitte.fauroux{at}trs.ap-hop-paris.fr

As forced expiratory volume in 1 second (FEV₁) is a major predictor of outcome in patients with cystic fibrosis (CF), we investigated the effect of FEV₁ 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 (FEV₁ range: 12–49% predicted). We observed correlations between FEV₁ and Pa_O2 (r = 0.76, p < 0.0001) and Pa_CO2 (r = -0.70, p < 0.0001), FEV₁ and respiratory rate/tidal volume (r = -0.41, p = 0.02), FEV₁ and dynamic lung compliance (r = 0.64, p < 0.0001), and FEV₁ 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 FEV₁ 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 FEV₁ 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.

Key Words: cystic fibrosis • forced expiratory volume in 1 second • pulmonary mechanics

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