Hyperpolarised 129Xe magnetic resonance imaging to monitor treatment response in children with cystic fibrosis. Issue 5 (2nd May 2019)

• Record Type: Journal Article
• Authors: Rayment, Jonathan H.; Couch, Marcus J.; McDonald, Nancy; Kanhere, Nikhil; Manson, David; Santyr, Giles; Ratjen, Felix
• Is Part Of: European respiratory journal. Volume 53:Issue 5(2019)
• Publication Date: 2019-05-02
• Subjects: Respiratory organs -- Diseases -- Periodicals; Respiration -- Periodicals; 616.2
• DOI: 10.1183/13993003.02188-2018
• Languages: English
• ISSNs: 0903-1936

Abstract

Pulmonary magnetic resonance imaging using hyperpolarised 129 Xe gas (XeMRI) can quantify ventilation inhomogeneity by measuring the percentage of unventilated lung volume (ventilation defect per cent (VDP)). While previous studies have demonstrated its sensitivity for detecting 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 aged 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 1 s 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. Hyperpolarised 129 Xe MRI is emerging as an important imaging biomarker in cystic fibrosis. Ventilation distribution, measured using this technique, improves after treatment of a pulmonary exacerbation in children with cystic fibrosis. http://ow.ly/E6Gc30nFMlb