Use of the muscle volume analyzer to evaluate enzyme replacement therapy in late-onset Pompe disease.
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.
Sugai
Fuminobu
F
Kokunai
Yosuke
Y
Yamamoto
Yoichi
Y
Hashida
Goichi
G
Shimazu
Kengo
K
Mihara
Masahito
M
Inoue
Satoru
S
Sakoda
Saburo
S
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
D011994:Recombinant Proteins; D000520:alpha-Glucosidases