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Early detection of cystic fibrosis lung disease: multiple-breath washout versus raised volume tests
  1. Sooky Lum1,
  2. Per Gustafsson2,
  3. Henrik Ljungberg1,
  4. Georg Hülskamp1,
  5. Andrew Bush3,
  6. Siobhán B Carr4,
  7. Rosemary Castle1,
  8. Ah-fong Hoo5,
  9. John Price6,
  10. Sarath Ranganathan5,
  11. John Stroobant7,
  12. Angie Wade8,
  13. Colin Wallis5,
  14. Hilary Wyatt6,
  15. Janet Stocks1,
  16. on behalf of the London Cystic Fibrosis Collaboration
  1. 1Portex Anaesthesia, Intensive Therapy and Respiratory Medicine Unit, UCL, Institute of Child Health, London, UK
  2. 2Department of Pediatric Clinical Physiology, Queen Silvia Children’s Hospital, Göteborg, Sweden
  3. 3Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London, UK
  4. 4Department of Child Health, Royal London Hospital, London, UK
  5. 5Department of Paediatric Respiratory Medicine, Great Ormond Street Hospital for Children NHS Trust, London, UK
  6. 6Department of Child Health, King’s College Hospital, London, UK
  7. 7Department of Child Health, University Hospital Lewisham, London, UK
  8. 8Centre for Paediatric Epidemiology and Biostatistics, UCL, Institute of Child Health, London, UK
  1. 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

Abstract

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 (FEV0.5 and FEF25–75 ) than boys (mean (95% CI girls–boys): –1.2 (–2.1 to −0.3) for FEV0.5 Z score; FEF25–75: –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.

  • 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
  • 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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Footnotes

  • Funding: Portex Ltd (SL and JS); European Respiratory Society long-term research fellowship (HL); Innovative Medizinische Forschung, University of Münster and the Gesellschaft für Pädiatrische Pneumologie, Germany (GH) and Cystic Fibrosis Trust (SR). Research at the Institute of Child Health and Great Ormond Street Hospital for Children NHS Trust benefits from R&D funding received from the NHS Executive.

    Contributors: JS was responsible for the study design; SR set up the recruitment/referral process; HL, GH, SL and A-fH recruited and measured infants and together with JS and PG, calculated and interpreted lung function parameters; RC was responsible for the database; SL and AW were responsible for statistical analysis; SL, JS and PG drafted the manuscript. AB, SBC, JP, SR, JStr, CW and HW were LCFC collaborators and recruited infants with CF to the study. All authors contributed to interpretation and commented on the manuscript.

  • Competing interests: None.

  • This paper was presented in part at the American Thoracic Society Conference in Seattle, 2003.

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