Enhanced urinary spontaneous luminescence in duchenne muscular dystrophy
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Genetic deficiency of dystrophin leads to disability and premature death in Duchenne muscular dystrophy, affecting the heart as well as skeletal muscle. Here we report that cardiosphere-derived cells (CDCs), which are being tested clinically for the treatment of Duchenne cardiomyopathy, improve cardiac and skeletal myopathy in the mdx mouse model of DMD and in human Duchenne cardiomyocytes. Injection of CDCs into the hearts of mdx mice augments cardiac function, ambulatory capacity and survival. Exosomes secreted by human CDCs reproduce the benefits of CDCs in mdx mice and in human Duchenne cardiomyocytes. The findings further motivate the testing of CDCs in Duchenne patients, while identifying exosomes as next-generation therapeutic candidates.
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This study describes the amount of physical activity and perception of physical activity in boys with Duchenne muscular dystrophy (DMD) compared to healthy boys. A questionnaire described 6 domains of physical activity. Four Duchenne muscular dystrophy subgroups were made: early and late ambulatory, nonambulatory with relative good, or limited arm function. Eighty-four boys with Duchenne muscular dystrophy (15.0 ± 6.4 years) and 198 healthy boys (14.0 ± 4.3 years) participated. Daily activities were more passive for boys with Duchenne muscular dystrophy. Physical activity was less and low demanding compared to healthy boys. It decreased with disease severity ( P < .05), whereas screen time increased ( P < .05). Benefits of physical activity in boys with Duchenne muscular dystrophy were having fun and making friends. Barriers were lack of sport facilities and insufficient health. This study helps to quantify poor engagement in physical activity by boys with Duchenne muscular dystrophy, and demonstrates factors that contribute to it. Suggestions to stimulate physical activity are made.
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Polyvinyl alcohol – CdS compounds doped with different concentrations of Cu, Al and Mn were produced. It is expected that these compounds have one-dimensional (polymeric) structure. Their luminescence spectra were investigated with the aim of use of luminescence for the structure characterization through comparison of luminescent properties of compounds and other CdS based materials. It was found that addition of copper leads to luminescence quenching while intensity of luminescence of compound doped with manganese reaches maximum at some concentration of additive and aluminum does not influence noticeably on the luminescence intensity of the compound. Luminescence spectra were not significantly changed at the doping. They are different from spectrum of crystalline CdS and similar to luminescence of CdS dissolved in glass. It was also found that luminescence of compounds is highly polarized, luminescence of glass is less polarized and luminescence of crystal CdS is not polarized.
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