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Eur Respir J. 2019 Feb 28. pii: 1802188. doi: 10.1183/13993003.02188-2018. [Epub ahead of print]

Hyperpolarised 129Xe MRI to monitor treatment response in children with cystic fibrosis.

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Division of Respiratory Medicine, Department of Paediatrics, Hospital for Sick Children, Toronto, ON Canada.
Department of Paediatrics, University of Toronto, Toronto, ON, Canada.
Translational Medicine Program, SickKids Research Institute, Toronto, ON, Canada.
Department of Medical Biophysics, University of Toronto, Toronto, ON, Canada.
Department of Diagnostic Imaging, Hospital for Sick Children, Toronto, ON, Canada.


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.

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