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Aims
Pompe disease is an autosomal recessive lysosomal storage disorder resulting from deficiency of acid α‐glucosidase (GAA) enzyme. Histopathological hallmarks in skeletal muscle tissue are fibre vacuolization and autophagy. Since 2006, enzyme replacement therapy (ERT) is the only approved treatment with human recombinant GAA alglucosidase alfa. We designed a study to examine ERT‐related skeletal...
Electrocardiogram (ECG) represents, with the electroencephalogram (EEG), the principal bio-vital signals, providing information on the human physical condition. Although surface electromyogram (sEMG) is not classified as bio-vital, it is frequently used to integrate and increase information obtained from ECG and EEG, making these three signals the main electric-biological signals. The sEMG is widely...
Gait analysis is widely used in clinics to study walking abnormalities for surgery planning, definition of rehabilitation protocols, and objective evaluation of clinical outcomes. Surface electromyography allows the study of muscle activity non-invasively and the evaluation of the timing of muscle activation during movement. The aim of this study was to present a normative dataset of muscle activation...
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