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• W2975792375 abstract "There is currently no cure for the fatal X-linked disorder Duchenne muscular dystrophy (DMD) and clinically-applicable translational trials continue to rely heavily on use of animal models. The Royal Veterinary College has a unique colony of dystrophin deficient dogs: the deltaE50-MD dog model. Magnetic Resonance Imaging (MRI) is a valuable non-invasive technique for monitoring the progression of the disease in human DMD patients. The aim was to determine if the deltaE50-MD dog has a skeletal muscle MRI phenotype that resembles that of DMD boys and to determine MRI differences of skeletal muscles in dystrophic and wildtype dogs. MRI was conducted in 15 deltaE50-MD dogs and 10 age-matched littermate wildtype male dogs under general anaesthetic, every 12 weeks from 3 months to 18 months of age. Up to 7 pelvic limb muscles and 4 lumbar muscles were assessed bilaterally in each dog. Four MRI biomarkers assessed were muscle volume normalised to femur length, T2 map signal intensity (SI), T1w to post gadolinium T1w SI ratio and muscle heterogeneity. The mean was calculated for each pair of muscles for all MRI biomarkers. Mixed model analyses (SPSS) were used to examine differences between groups at different ages. Muscle volumes were significantly smaller (p<0.001) in affected dogs when compared to wildtype dogs in all examined muscles, apart from the cranial sartorius. T2 map SI (p<0.001) and muscle heterogeneity (p<0.05) were significantly higher in affected dogs when compared to wildtype dogs. Affected dogs had significantly increased post gadolinium T1w SI in all muscles (p<0.05), apart from the cranial sartorius muscle, when compared to wildtype dogs. The most useful MRI biomarkers found in this study were muscle volume normalised to femur length and T2 map SI. Both of these biomarkers were able to show differences between groups and track changes longitudinally. These MRI biomarkers will be useful to determine efficacy of therapeutic clinical trials in the colony." @default.
• W2975792375 created "2019-10-03" @default.
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• W2975792375 date "2019-10-01" @default.
• W2975792375 modified "2023-09-27" @default.
• W2975792375 title "P.318MRI evaluation of skeletal muscle in the deltaE50-MD dog model of Duchenne muscular dystrophy" @default.
• W2975792375 doi "https://doi.org/10.1016/j.nmd.2019.06.432" @default.
• W2975792375 hasPublicationYear "2019" @default.
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