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Detection of early subclinical lung disease in children with cystic fibrosis by lung ventilation imaging with hyperpolarised gas MRI
  1. Helen Marshall1,
  2. Alex Horsley1,2,
  3. Chris J Taylor3,
  4. Laurie Smith1,3,
  5. David Hughes3,
  6. Felix C Horn1,
  7. Andrew J Swift1,
  8. Juan Parra-Robles1,
  9. Paul J Hughes1,
  10. Graham Norquay1,
  11. Neil J Stewart1,
  12. Guilhem J Collier1,
  13. Dawn Teare4,
  14. Steve Cunningham5,
  15. Ina Aldag3,
  16. Jim M Wild1
  1. 1POLARIS, Academic Radiology, University of Sheffield, Sheffield, UK
  2. 2Centre for Respiratory Medicine and Allergy, Institute of Inflammation and Repair, Manchester Academic Health Science Centre, The University of Manchester and University Hospital of South Manchester NHS Foundation Trust, Manchester, UK
  3. 3Sheffield Children's Hospital, Sheffield, UK
  4. 4School of Health and Related Research, University of Sheffield, Sheffield, UK
  5. 5Department of Respiratory and Sleep Medicine, Royal Hospital for Sick Children, Edinburgh, UK
  1. Correspondence to Professor Jim M Wild, Department of Academic Radiology, Floor C, Royal Hallamshire Hospital, Glossop Road, Sheffield S10 2JF, UK; j.m.wild{at}sheffield.ac.uk

Abstract

Hyperpolarised 3He ventilation-MRI, anatomical lung MRI, lung clearance index (LCI), low-dose CT and spirometry were performed on 19 children (6–16 years) with clinically stable mild cystic fibrosis (CF) (FEV1>−1.96), and 10 controls. All controls had normal spirometry, MRI and LCI. Ventilation-MRI was the most sensitive method of detecting abnormalities, present in 89% of patients with CF, compared with CT abnormalities in 68%, LCI 47% and conventional MRI 22%. Ventilation defects were present in the absence of CT abnormalities and in patients with normal physiology, including LCI. Ventilation-MRI is thus feasible in young children, highly sensitive and provides additional information about lung structure–function relationships.

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Footnotes

  • Helen Marshall and Alex Horsley are joint first authors.

  • Contributors HM: MRI acquisition and analysis, overall data analysis, co-wrote the manuscript. AH: study design, MBW set-up and over-read of analysis, co-wrote the manuscript. CJT: study design, patient recruitment, reviewed the manuscript. LS: performed and analysed lung function and MBW measurements, reviewed the manuscript. DH: scored CT images and reviewed all MRI, reviewed the manuscript. FCH: performed MBW measurements, MRI acquisition, reviewed the manuscript. AJS: scored CT images, reviewed the manuscript. JP-R: polarised gas. PJH: MR image registration. GN, NJS and GJC: polarised gas, technical imaging support. DT: statistical support. SC: study design, reviewed the manuscript. IA: patient recruitment and consent, reviewed the manuscript. JMW: study design, MRI acquisition design, data analysis, co-wrote the manuscript, underwrites the work. The corresponding author had access to all the data in the study and accepts responsibility for its validity.

  • Funding This article presents independent research funded by the Cystic Fibrosis Trust and the National Institute of Health Research (NIHR).

  • Disclaimer The funders had no role in the study design, data collection, analysis, interpretation or preparation of this report. The views expressed are those of the authors and not necessarily those of the NHS, the NIHR or the Department of Health.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Ethics approval National Research and Ethics Committee.

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

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