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Abstract #1185

Ventilation heterogeneity of pediatric cystic fibrosis accessed via lung clearance index and hyperpolarized 129Xe MRI

Laura L Walkup1, Robert P Thomen1, Emily Bell2, Beth Decker2, Zackary I Cleveland1, John Paul Clancy2, and Jason C Woods1

1Center for Pulmonary Imaging Research, Division of Pulmonary Medicine and Department of Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States, 2Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States

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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