Exercise capacity in a child with McArdle disease.
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.
Pérez
Margarita
M
Universidad Europea de Madrid, 28670 Madrid, Spain.
Maté-Muñoz
José L
JL
Foster
Carl
C
Rubio
Juan C
JC
Andreu
Antoni L
AL
Martín
Miguel A
MA
Arenas
Joaquín
J
Lucia
Alejandro
A
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
D003402:Creatine Kinase