title: Use of the muscle volume analyzer to evaluate enzyme replacement therapy in late-onset Pompe disease.
abstract: The muscle volume analyzer (MVA) can predict limb muscle weight based on bioelectric impedance analysis, whereas the conventional handheld dynamometer (HHD) measures muscle strength. In this study, a 26-year-old female on invasive ventilation due to late-onset Pompe disease was treated with enzyme replacement therapy (ERT) for 12 months. MVA measurements demonstrated time-dependent improvement from the baseline compared to HHD measurements, showing remarkably fluctuating muscle strength. Thus, the MVA can be used as an alternative, particularly for patients suffering from severe limb muscle weakness.
authors.forename: Fuminobu
authors.initials: F
authors.lastname: Sugai
authors.forename: Yosuke
authors.initials: Y
authors.lastname: Kokunai
authors.forename: Yoichi
authors.initials: Y
authors.lastname: Yamamoto
authors.forename: Goichi
authors.initials: G
authors.lastname: Hashida
authors.forename: Kengo
authors.initials: K
authors.lastname: Shimazu
authors.forename: Masahito
authors.initials: M
authors.lastname: Mihara
authors.forename: Satoru
authors.initials: S
authors.lastname: Inoue
authors.forename: Saburo
authors.initials: S
authors.lastname: Sakoda
mesh_terms: D000328:Adult; D017668:Age of Onset; D017097:Electric Impedance; D004568:Electrodiagnosis / Q000295:instrumentation / Q000379:methods*; D056947:Enzyme Replacement Therapy / Q000379:methods*; D005260:Female; D006009:Glycogen Storage Disease Type II / Q000175:diagnosis* / Q000188:drug therapy* / Q000503:physiopathology; D006801:Humans; D007866:Leg / Q000473:pathology / Q000503:physiopathology; D018908:Muscle Weakness / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D018482:Muscle, Skeletal / Q000187:drug effects* / Q000201:enzymology / Q000503:physiopathology; D009133:Muscular Atrophy / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D017063:Outcome Assessment, Health Care / Q000379:methods*; D011994:Recombinant Proteins / Q000494:pharmacology / Q000627:therapeutic use; D020127:Recovery of Function / Q000187:drug effects / Q000502:physiology; D012121:Respiration, Artificial; D012133:Respiratory Paralysis / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D016896:Treatment Outcome; D000520:alpha-Glucosidases / Q000172:deficiency / Q000494:pharmacology / Q000627:therapeutic use
chemical_list: D011994:Recombinant Proteins; D000520:alpha-Glucosidases