Oxygen-enhanced MRI and multiple breath washout with Short extension reveal cystic fibrosis lung disease progression despite triple modulator therapy.

Christopher Short^1,2,3, Tom Semple^4,2,5, Mary Abkir^4,2,3, Constantinos Efthyvoulou⁴, Simon Padley^4,2, Mark Rosenthal^4,2, Paul McNally^6,7, Harm Tiddens^8,9,10, Daan Caudri^8,9, Marta Tibiletti¹¹, Geoff J Parker^11,12, Jane Carolyn Davies^4,2,3,5

¹National Heart and Lung Institute, Imperial College London, London, England, UK christopher.Short@imperial.ac.uk.

²Royal Brompton and Harefield Hospitals, Guy's and St Thomas' Hospitals NHS Trust, London, England, UK.

³Lung Clearance Index Core Facility, European Cystic Fibrosis Society, London, UK.

⁴National Heart and Lung Institute, Imperial College London, London, England, UK.

⁵Centre for Paediatrics and Child Health, Imperial College London, London, England, UK.

⁶Department of Paediatrics, Royal College of Surgeons in Ireland, Dublin, Ireland.

⁷Department of Respiratory Medicine, Children's Health Ireland, Dublin, Ireland.

⁸Department of Paediatric Pulmonology and Allergology, Erasmus MC -Sophia Children's Hospital, Rotterdam, Netherlands.

⁹Department of Radiology and Nuclear Medicine, Erasmus MC, Rotterdam, Netherlands.

¹⁰Thirona, Rotterdam, Netherlands.

¹¹Bioxydyn Ltd, Manchester, UK.

¹²Department of Medical Physics & Biomedical Engineering, University College London, London, England, UK.

PubMed DOI

Abstract

INTRODUCTION: The current cystic fibrosis (CF) care era, while hugely welcome, raises new challenges, particularly the need for more sensitive pulmonary outcome measures. Seeking further optimisation, we previously developed a Short extension to multiple breath washout measure (MBWShX) which captures previously overlooked, under-ventilated lung units but lacks regional information. Functional lung…

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