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Cystic fibrosis
Lung Clearance Index and HRCT are complementary markers of lung abnormalities in young children with CF
  1. C M Owens1,
  2. P Aurora2,3,
  3. S Stanojevic3,4,
  4. A Bush5,
  5. A Wade4,
  6. C Oliver2,3,
  7. A Calder1,
  8. J Price6,
  9. S B Carr7,
  10. A Shankar8,
  11. Janet Stocks3 on behalf of the London Cystic Fibrosis Collaboration
  1. 1Department of Paediatric Radiology, Great Ormond Street Hospital for Children NHS Trust, London, UK
  2. 2Respiratory Medicine, Great Ormond Street Hospital for Children NHS Trust, London, UK
  3. 3Portex Unit: Respiratory Physiology and Medicine, UCL Institute of Child Health, London, UK
  4. 4Centre for Paediatric Epidemiology & Biostatistics, UCL Institute of Child Health, London, UK
  5. 5Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London, UK
  6. 6Department of Child Health, Kings College Hospital, London, UK
  7. 7Department of Respiratory Paediatrics, Barts and The London Children's Hospital, London, UK
  8. 8Department of Child Health, University Hospital Lewisham, London, UK
  1. Correspondence to Professor Janet Stocks, Portex Respiratory Unit, UCL, Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK; j.stocks{at}ich.ucl.ac.uk

Abstract

Rationale High resolution computed tomography (HRCT) is a more sensitive tool for detecting early cystic fibrosis (CF) lung disease than either spirometry or plain radiography, but its relationship to other measures of lung function has not been established in young children.

Objectives 1) To assess whether the lung clearance index (LCI) derived from multiple breath inert-gas washout (MBW) is as effective as HRCT in identifying pulmonary abnormalities; and 2) explore the relationships between abnormalities detected by HRCT and by spirometry, plethysmography and MBW (collectively, LFTs) in young children with CF.

Methods Children with CF underwent LFTs and volumetric HRCT on the same day. Healthy age-matched controls underwent identical LFTs without HRCT. Scans were anonymised, and scored using the Brody-II CT scoring system, to assess for presence and extent of bronchiectasis, airway wall thickening, mucus plugging, and parenchymal opacities.

Results Assessments were undertaken in 60 children with CF (mean (SD) 7.8 (1.3) years) and 54 healthy controls (7.9 (1.2) y). Among children with CF, 84% (47/56) had abnormal LCI, 58% (27/47) abnormal plethysmographic lung volumes (FRCpleth or RV), 35% (21/60) abnormal sRaw and 47% (28/60) abnormal spirometry (FEV1 or FEF25–75); whereas HRCT scans were abnormal in 85% (51/60): median total Brody-II score: 9.5% (range 0–51%). Total CT score correlated more strongly with LCI (Spearman correlation=0.77) than with spirometry (R=−0.43) or any other marker of lung function. Of the nine children with normal LCI, five had abnormalities on HRCT, whereas five children with normal HRCT had raised LCI.

Conclusions These results suggest that while LCI and HRCT have similar sensitivity to detect CF lung disease, complimentary information may be gained in individual patients.

  • Pulmonary function tests
  • child
  • lung imaging
  • CT
  • cf
  • multiple breath wash out
  • cystics fibrosis
  • imaging/CT MRI

https://doi.org/10.1136/thx.2010.150375

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    Footnotes

    • Members of the London Cystic Fibrosis Collaboration P Aurora, I Balfour Lynn, A Bush, S Carr, J Davies, R Dinwiddie, A-F Hoo, W Kozlowska, S Lum, C Oliver, C Owens, A Prasad, J Price, S Ranganathan, M Rosenthal, G Ruiz, A Shankar, S Stanojevic, J Stocks, J Stroobant, R. Suri, A Wade, C Wallis and H Wyatt

    • Funding This study was funded by the CF Trust, Smiths Medical and Asthma UK.

    • Competing interests None.

    • Provenance and peer review Not commissioned; externally peer reviewed.