title: Exercise capacity in a child with McArdle disease.
abstract: We report the exercise capacity of an 8-year-old boy with clinical, histological, biochemical, and genetic evidence of McArdle disease. The patient presented with severe myalgia, proteinuria, hematuria, pyrexia, and elevated creatine kinase after swimming. After pre-exercise ingestion of sucrose, he performed treadmill exercise to symptom limitation. His peak oxygen uptake (18.8 mL/kg/min) and ventilatory threshold (16.0 mL/kg/min) were reduced by 40% and 20% compared with healthy age-matched and gender-matched controls. The results suggest that exercise capacity is reduced early in life in patients with McArdle disease and suggest the need for prophylactic exercise training (following pre-exercise feeding to prevent rhabdomyolysis) to minimize deconditioning.
authors: Universidad Europea de Madrid, 28670 Madrid, Spain.
authors: Margarita
authors: M
authors: Pérez
authors: José L
authors: JL
authors: Maté-Muñoz
authors: Carl
authors: C
authors: Foster
authors: Juan C
authors: JC
authors: Rubio
authors: Antoni L
authors: AL
authors: Andreu
authors: Miguel A
authors: MA
authors: Martín
authors: Joaquín
authors: J
authors: Arenas
authors: Alejandro
authors: A
authors: Lucia
mesh_terms: D016022:Case-Control Studies; D002648:Child; D003402:Creatine Kinase / Q000378:metabolism; D005080:Exercise Test; D005081:Exercise Therapy; D017079:Exercise Tolerance / Q000502:physiology*; D006012:Glycogen Storage Disease Type V / Q000201:enzymology / Q000503:physiopathology* / Q000628:therapy; D006801:Humans; D008297:Male; D016555:Matched-Pair Analysis; D010101:Oxygen Consumption / Q000502:physiology*; D012016:Reference Values
chemical_list: D003402:Creatine Kinase