Abstract
Pulmonary magnetic resonance imaging using hyperpolarised 129Xe gas (XeMRI) can quantify ventilation inhomogeneity by measuring the percentage of unventilated lung volume (ventilation defect percent; VDP). While previous studies have demonstrated its sensitivity to detect early cystic fibrosis (CF) lung disease, the utility of XeMRI to monitor response to therapy in CF is unknown. The aim of this study was to assess the ability of XeMRI to capture treatment response in paediatric CF patients undergoing inpatient antibiotic treatment for a pulmonary exacerbation.
15 CF patients age 8–18 years underwent XeMRI, spirometry, plethysmography, and multiple breath nitrogen washout at the beginning and end of inpatient treatment of a pulmonary exacerbation. VDP was calculated from XeMRI images obtained during a static breath hold using semi-automated k-means clustering and linear binning approaches.
XeMRI was well tolerated. VDP, lung clearance index and the forced expiratory volume in one second all improved with treatment, however, response was not uniform in individual patients. Of all outcome measures, VDP showed the largest relative improvement (−42.1%; 95%CI −52.1, −31.9, p<0.0001).
These data support further investigation of XeMRI as a tool to capture treatment response in CF lung disease.
Footnotes
This manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.
Conflict of interest: Dr. Rayment has nothing to disclose.
Conflict of interest: Dr. Couch reports grants from Siemens Healthcare, during the conduct of the study.
Conflict of interest: Dr. McDonald has nothing to disclose.
Conflict of interest: Dr. Kanhere has nothing to disclose.
Conflict of interest: Dr. Manson has nothing to disclose.
Conflict of interest: Dr. Santyr has nothing to disclose.
Conflict of interest: Dr. Ratjen has nothing to disclose.
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