Neuromuscul Disord 2022 04 17;32(4):284-294. Epub 2022 Feb 17.
Department of Pediatrics, Neurology and Pathology, Division of Genetics and Genomic Medicine, University of California, Irvine School of Medicine, 101 The City Drive South, ZC4482, Orange, CA, USA; Department of Neurology, University of California, Irvine School of Medicine, Orange, CA, USA; Department of Pathology & Laboratory Medicine, University of California, Irvine School of Medicine, Orange, CA, USA. Electronic address:
Pompe disease is a progressive myopathy resulting from deficiency in lysosomal enzyme acid α-glucosidase (GAA), which leads to glycogen accumulation in lysosomes primarily in skeletal and cardiac muscle. Enzyme replacement therapy (ERT) with recombinant human (rh) GAA works well in alleviating the cardiomyopathy; however, many patients continue to have progressive muscle weakness. The purpose of this study was to evaluate the effectiveness of a respiratory training combined with 24-week supervised resistance training program on muscle strength (measured by Biodex)), and respiratory function including maximum inspiratory pressure (MIP), maximum expiratory pressure (MEP) in subjects with late onset Pompe disease receiving ERT. Read More