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Exercise capacity in a child with McArdle disease
Margarita Pérez1, José L Maté-Muñoz, Carl Foster
1Universidad Europea de Madrid, 28670 Madrid, Spain.
Journal of Child Neurology
|August 24, 2007
Summary
Children with McArdle disease have significantly reduced exercise capacity early in life. Prophylactic exercise training and pre-exercise feeding may help manage symptoms and prevent deconditioning in these patients.
Area of Science:
- Exercise physiology
- Rare genetic disorders
- Metabolic myopathies
Background:
- McArdle disease is a glycogen storage disease affecting muscle energy metabolism.
- Impaired glycogenolysis leads to exercise intolerance and potential rhabdomyolysis.
- Understanding early-life exercise capacity is crucial for disease management.
Observation:
- An 8-year-old boy with confirmed McArdle disease exhibited severe symptoms after exercise.
- Symptoms included myalgia, elevated creatine kinase, proteinuria, hematuria, and pyrexia.
- Peak oxygen uptake and ventilatory threshold were substantially lower than in healthy peers.
Findings:
- The patient's peak oxygen uptake was reduced by 40% compared to controls.
- Ventilatory threshold was reduced by 20% compared to controls.
- These findings indicate significantly impaired exercise capacity from a young age.
Implications:
- Exercise capacity is compromised early in the course of McArdle disease.
- Pre-exercise carbohydrate intake is vital to prevent exercise-induced rhabdomyolysis.
- Early, supervised exercise training may be beneficial to mitigate deconditioning.
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