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Hyperpolarized 129Xe MRI Picks Up Early Disease Markers of Lymphangioleiomyomatosis

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A small study including 22 patients with lymphangioleiomyomatosis (LAM), a rare disease associated with cystic destruction of the pulmonary parenchyma and chronic respiratory failure, indicates 129Xe MRI can detect early ventilation abnormalities and airspace dilation in patients with mild disease, which could make this an appealing tool to screen and manage patients and assess treatment efficacy. In the study, ventilation deficits were observed in all patients with LAM, including those with normal pulmonary function and few cysts. 129Xe VDP was strongly correlated with the ratio of forced expiratory volume in 1 second (FEV1) over forced vital capacity (FVC) and the diffusing capacity of the lungs for carbon monoxide (DLCO). Additionally, the apparent diffusion coefficient (ADC) was found to be elevated relative to values previously reported in adults, and it correlated with several pulmonary-function test (PFT) parameters.

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Walkup LL, Roach DJ, Hall CS, et al. Cyst ventilation heterogeneity and alveolar-airspace dilation as early disease markers in lymphangioleiomyomatosis. Ann Am Thorac Soc. 2019 Apr 30. doi: 10.1513/AnnalsATS.201812-880OC.

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