CYSTIC FIBROSIS

Early detection of cystic fibrosis lung disease: multiple-breath washout versus raised volume tests

Sooky Lum¹, Per Gustafsson², Henrik Ljungberg¹, Georg Hülskamp¹, Andrew Bush³, Siobhán B Carr⁴, Rosemary Castle¹, Ah-fong Hoo⁵, John Price⁶, Sarath Ranganathan⁵, John Stroobant⁷, Angie Wade⁸, Colin Wallis⁵, Hilary Wyatt⁶, Janet Stocks¹ on behalf of the London Cystic Fibrosis Collaboration

¹ Portex Anaesthesia, Intensive Therapy and Respiratory Medicine Unit, UCL, Institute of Child Health, London, UK
² Department of Pediatric Clinical Physiology, Queen Silvia Children’s Hospital, Göteborg, Sweden
³ Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London, UK
⁴ Department of Child Health, Royal London Hospital, London, UK
⁵ Department of Paediatric Respiratory Medicine, Great Ormond Street Hospital for Children NHS Trust, London, UK
⁶ Department of Child Health, King’s College Hospital, London, UK
⁷ Department of Child Health, University Hospital Lewisham, London, UK
⁸ Centre for Paediatric Epidemiology and Biostatistics, UCL, Institute of Child Health, London, UK

Correspondence to:
Dr S Lum
Portex Anaesthesia, Intensive Therapy and Respiratory Medicine Unit, UCL, Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK; s.lum{at}ich.ucl.ac.uk

Background: Lung clearance index (LCI), a measure of ventilation inhomogeneity derived from the multiple-breath inert gas washout (MBW) technique, has been shown to detect abnormal lung function more readily than spirometry in preschool children with cystic fibrosis, but whether this holds true during infancy is unknown.

Objectives: To compare the extent to which parameters derived from the MBW and the raised lung volume rapid thoraco–abdominal compression (RVRTC) techniques identify diminished airway function in infants with cystic fibrosis when compared with healthy controls.

Methods: Measurements were performed during quiet sleep, with the tidal breathing MBW technique being performed before the forced expiratory manoeuvres.

Results: Measurements were obtained in 39 infants with cystic fibrosis (mean (SD) age 41.4 (22.0) weeks) and 21 controls (37.0 (15.1) weeks). Infants with cystic fibrosis had a significantly higher respiratory rate (38 (10) vs 32 (5) bpm) and LCI (8.4 (1.5) vs 7.2 (0.3)), and significantly lower values for all forced expiratory flow-volume parameters compared with controls. Girls with cystic fibrosis had significantly lower forced expiratory volume (FEV_0.5 and FEF_25–75 ) than boys (mean (95% CI girls–boys): –1.2 (–2.1 to –0.3) for FEV_0.5 Z score; FEF_25–75: –1.2 (–2.2 to –0.15)). When using both the MBW and RVRTC techniques, abnormalities were detected in 72% of the infants with cystic fibrosis, with abnormalities detected in 41% using both techniques and a further 15% by each of the two tests performed.

Conclusions: These findings support the view that inflammatory and/or structural changes in the airways of children with cystic fibrosis start early in life, and have important implications regarding early detection and interventions. Monitoring of early lung disease and functional status in infants and young children with cystic fibrosis may be enhanced by using both MBW and the RVRTC.

Abbreviations: FEF, forced expiratory flow; FEFV, forced expiratory flow-volume; FEV, forced expiratory volume; FRC, functional residual capacity; FVC, forced vital capacity; LCI, lung clearance index; MBW, multiple-breath washout; RR, respiratory rate; RVRTC, raised lung volume rapid thoraco-abdominal compression; SF₆, sulphur hexafluoride

Keywords: LCI; lung clearance index; MBW; multiple-breath wash out; RVRTC; raised lung volume rapid thoraco–abdominal compression; FEV, forced expiratory volume

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