期刊
ACADEMIC RADIOLOGY
卷 26, 期 3, 页码 344-354出版社
ELSEVIER SCIENCE INC
DOI: 10.1016/j.acra.2018.04.024
关键词
Cystic fibrosis; Hyperpolarized Xe-129; MRI; Ventilation defect percent; Fractional ventilation
资金
- 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) [MOP 123431, PJT 153099]
- Cystic Fibrosis Canada Clinical Fellowship Award
- Research Training Competition (Restracomp) Fellowship from the Hospital for Sick Children
- Mitacs Elevate Postdoctoral Fellowship
Conventional pulmonary function tests appear normal in early cystic fibrosis (CF) lung disease. Therefore, new diagnostic approaches are required that can detect CF lung disease in children and monitor treatment response. Hyperpolarized (HP) gas (Xe-129 and He-3) magnetic resonance imaging (MRI) is a powerful, emergent tool for mapping regional lung function and may be well suited for studying pediatric CF. HP gas MRI is well tolerated, reproducible, and it can be performed longitudinally without the need for ionizing radiation. In particular, quantification of the distribution of ventilation, or ventilation defect percent (VDP), has been shown to be a sensitive indicator of CF lung disease and correlates well with pulmonary function tests. This article presents the current state of CF diagnosis and treatment and describes the potential role of HP gas MRI for detection of early CF lung disease and following the effects of interventions. The typical HP gas imaging workflow is described, along with a discussion of image analysis to calculate VDP, dosing considerations, and the reproducibility of VDP. The potential use of VDP as an outcome measure in CF is discussed, by considering the correlation with pulmonary function measures, preliminary interventional studies, and case studies involving longitudinal imaging and pulmonary exacerbations. Finally, emerging HP gas imaging techniques such as multiple breath washout imaging are introduced, followed by a discussion of future directions. Overall, HP gas MRI biomarkers are expected to provide sensitive outcome measures that can be used in disease surveillance as well as interventional studies involving novel CF therapies.
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