title: Increased inspiratory and expiratory muscle strength following respiratory muscle strength training (RMST) in two patients with late-onset Pompe disease.
abstract: Respiratory muscle strength training (RMST) is an exercise-based intervention which targets respiratory muscle weakness. We implemented RMST in two patients with late-onset Pompe disease (LOPD), both who had received long-term enzyme replacement therapy and had severe respiratory weakness. Over 16-32 weeks, inspiratory muscle strength increased by 73-74%. Expiratory muscle strength increased 31-48% over 12-22 weeks. These findings suggest that RMST may increase respiratory muscle strength, even in the setting of LOPD and severe baseline weakness.
authors.affiliation: Department of Surgery, Division of Speech Pathology & Audiology, Duke University, Durham, NC, USA. harrison.jones@duke.edu
authors.forename: Harrison N
authors.initials: HN
authors.lastname: Jones
authors.forename: Tronda
authors.initials: T
authors.lastname: Moss
authors.forename: Laurie
authors.initials: L
authors.lastname: Edwards
authors.forename: Priya S
authors.initials: PS
authors.lastname: Kishnani
mesh_terms: D045853:Exhalation / Q000502:physiology; D005260:Female; D006009:Glycogen Storage Disease Type II / Q000188:drug therapy / Q000534:rehabilitation*; D006801:Humans; D001239:Inhalation / Q000502:physiology; D008297:Male; D008875:Middle Aged; D053580:Muscle Strength / Q000502:physiology*; D055070:Resistance Training / Q000379:methods*; D012132:Respiratory Muscles / Q000502:physiology*; D000520:alpha-Glucosidases / Q000627:therapeutic use
chemical_list: C509951:GAA protein, human; D000520:alpha-Glucosidases