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Abstract #0832

LONGITUDINAL MONITORING OF DISEASE PROGRESSION IN CHILDREN WITH MILD CYSTIC FIBROSIS USING HYPERPOLARISED GAS MRI AND LUNG CLEARANCE INDEX

Laurie Smith1,2, Paul J.C. Hughes1, Felix Horn1, Helen Marshall1, Graham Norquay1, Guilhem Collier1, David Hughes2, Chris Taylor2, Noreen West2, Ina Aldag2, Alex Horsley3, and Jim Wild1

1University of Sheffield, Sheffield, United Kingdom, 2Sheffield Children's Hospital, Sheffield, United Kingdom, 3Manchester Adult CF Centre, Manchester, United Kingdom

Hyperpolarised gas ventilation MRI is a sensitive method for evaluating disease progression in subjects with cystic fibrosis and normal spirometry. Ventilation defect % (VD%) increased in 10/11 subjects studied with a mean change of 201%. The MRI coefficience of variance (CV) of signal intensity was similarly sensitive to change. 10/11 subjects had increased lung clearance index (LCI) at 2-years but no subject had abnormal spirometry at either visit. The % change in LCI demonstrated strong correlations with the % change in CV outcomes. VD% and CV reflect different but complimentary aspects of lung disease that appear to track disease progression.

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