Extract
Treatment response in cystic fibrosis (CF) is traditionally monitored using pulmonary function tests (PFTs), such as spirometry. However, PFTs can be insensitive to treatment, particularly in early CF lung disease [1]. Hyperpolarised xenon-129 magnetic resonance imaging (Xe-MRI) has been shown to be feasible in children [2], more sensitive to early CF lung disease compared to PFTs [3] and captures improvements in ventilation inhomogeneity in paediatric CF patients receiving intravenous antibiotic treatment for a pulmonary exacerbation [4]. However, access to hyperpolarised 129Xe gas is not widely available, and Xe-MRI requires subjects to perform an extended breath-hold (10–15 s), which is challenging for very sick children.
Abstract
Ventilation distributions obtained with PREFUL MRI correlated with Xe-MRI, and both could detect improvements following treatment of a PEx. PREFUL MRI offers an effective and more widely available alternative to Xe-MRI for monitoring treatment. https://bit.ly/3le0TT9
Acknowledgements
The authors would like to thank: Shahideh Safavi, Yonni Friedlander, Robert Grimm, Raymond Hu, Nikhil Kanhere, Krzysztof Kowalik, Andras Lindenmaier, Tammy Rayner, Laura Seed, Elaine Stirrat, Ruth Weiss, David Wilson, and Brandon Zanette for their assistance with data acquisition and analysis.
Footnotes
The work was carried out using REB and Health Canada-approved protocols at the Hospital for Sick Children (clinicaltrials.gov: NCT02740868, for PEx participants, and NCT02606487, for healthy volunteers). Individual participant data will not be shared.
Author contributions: Study design: F. Ratjen and G. Santyr; data collection: M.J. Couch and J.H. Rayment; analysis: S. Munidasa, J.H. Rayment, M.J. Couch and G. Santyr; manuscript drafting, editing and approval: all.
Conflict of interest: M.J. Couch reports that he was supported by a MITACS Elevate Postdoctoral Fellowship, which was funded in part by Siemens Healthcare Limited, and is currently an employee of Siemens Healthcare Limited. This employment began after the conclusion of the study.
Conflict of interest: J.H. Rayment reports consultancy fees from Polarean Inc, outside the submitted work.
Conflict of interest: A. Voskrebenzev has a patent “Method of quantitative magnetic resonance lung imaging” licensed to Siemens.
Conflict of interest: R. Seethamraju is an employee of Siemens Medical Solutions, USA Inc.
Conflict of interest: J. Vogel-Claussen reports grants from Siemens Healthineers, during the conduct of the study; grants and personal fees from Boehringer Ingelheim and AstraZeneca, grants from GSK, outside the submitted work; and has a patent “Method of quantitative magnetic resonance lung imaging” number EP3107066, US-2016-0367200-A1 22.12.2016 licensed to Siemens Healthineers.
Conflict of interest: F. Ratjen has nothing to disclose.
Conflict of interest: G. Santyr reports grants and non-financial support from Siemens Healthineers, grants from Canadian Institutes of Health Research, during the conduct of the study; grants and non-financial support from Siemens Healthineers, outside the submitted work.
Conflict of interest: S. Munidasa reports grants from Cystic Fibrosis Centre, Natural Sciences and Engineering Research Council of Canada and Canadian Institutes of Health Research, during the conduct of the study.
Support statement: The Hospital for Sick Children (Catalyst Grant from the Cystic Fibrosis Centre), Natural Sciences and Engineering Research Council of Canada (NSERC) Discovery grant (RGPIN 217015-2013), Canadian Institutes of Health Research (CIHR) operating and project grants (MOP 123431, PJT 153099, PJT 376120), and The Irwin Fund. Funding information for this article has been deposited with the Crossref Funder Registry.
- Received August 12, 2020.
- Accepted November 22, 2020.
- Copyright ©ERS 2021
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