Quantitative hyperpolarized 129Xe ventilation MRI was compared to FEV1, the spriometric gold standard for assessing lung function, and lung clearance index (LCI), an emerging pulmonary function test to assess global ventilation heterogeneity, in 12 pediatric cystic fibrosis subjects. A range of severity and spatial distributions of 129Xe ventilation deficits were observed, with a 129Xe ventilation defect percentage (VDP) of 18.0% ± 8.1%. VDP did not correlate with FEV1 (p=0.45) but correlated very strongly with LCI (p=0.0001), suggesting that the spatial distribution of defects in the 129Xe images represents obstructed areas of the lung that give rise to elevated LCI.
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