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Multiple-Breath Washout as a Marker of Lung Disease in Preschool Children with Cystic Fibrosis

Portex Respiratory Unit, Institute of Child Health; Cardiorespiratory and Critical Care Division, Great Ormond Street Hospital for Children; Department of Paediatric Respiratory Medicine, Royal Brompton Hospital; Department of Paediatrics, Kings' College Hospital; Department of Paediatrics, Lewisham University Hospital; and Department of Paediatrics, Royal London Hospital, London, United Kingdom; and Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Göteborg, Sweden

Correspondence and requests for reprints should be addressed to Paul Aurora, Portex Respiratory Unit, Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK. E-mail: p.aurora{at}ich.ucl.ac.uk

Sensitive measures of lung function applicable to young subjects are needed to detect early cystic fibrosis (CF) lung disease. Forty children with CF aged 2 to 5 years and 37 age-matched healthy control subjects performed multiple-breath inert gas washout, plethysmography, and spirometry. Thirty children in each group successfully completed all measures, with success on first visit being between 68 and 86% for all three measures. Children with CF had significantly higher lung clearance index (mean [95% CI] difference for CF control 2.7 [1.9, 3.6], p < 0.001) and specific airway resistance (1.65 z-scores [0.96, 2.33], p < 0.001), and significantly lower forced expired volume in 0.5 seconds (–0.49 z-scores [–0.95, –0.03], p < 0.05). Abnormal lung function results were identified in 22 (73%) of 30 children with CF by multiple-breath washout, compared with 14 (47%) of 30 by plethysmography, and 4 (13%) of 30 by spirometry. Children with CF who were infected with Pseudomonas aeruginosa had significantly higher lung clearance index, but no significant difference in other lung function measures, when compared with noninfected children. Most preschool children can perform multiple-breath washout, plethysmography, and spirometry at first attempt. Multiple-breath washout detects abnormal lung function in children with CF more readily than plethysmography or spirometry.

Key Words: child • cystic fibrosis • lung function • preschool

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