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Abnormal ventilation during exercise in McArdle's syndrome: modulation by substrate availability.

R G Haller, S F Lewis

    Neurology
    |May 1, 1986
    PubMed
    Summary
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    Patients with McArdle disease exhibit higher ventilation during exercise compared to healthy individuals. This abnormal breathing pattern improves with glucose or fasting, suggesting a link between energy metabolism and exercise tolerance.

    Area of Science:

    • Exercise Physiology
    • Metabolic Myopathies

    Background:

    • McArdle disease (glycogen storage disease type V) results from myophosphorylase deficiency.
    • Impaired glycogenolysis affects energy production during exercise, potentially impacting cardiorespiratory responses.

    Purpose of the Study:

    • To investigate the ventilatory response to exercise in individuals with McArdle disease.
    • To explore the role of substrate availability (glucose and fatty acids) in modulating exercise ventilation in this population.

    Main Methods:

    • Evaluated ventilation during cycle ergometry in four men with McArdle disease and healthy controls.
    • Manipulated substrate availability through glucose infusion and fasting.
    • Administered nicotinic acid to inhibit lipolysis.

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    Main Results:

    • McArdle patients showed significantly higher ventilation relative to oxygen uptake during submaximal exercise.
    • Glucose infusion or fasting normalized the exercise ventilatory response.
    • Inhibition of lipolysis exacerbated excessive ventilation.

    Conclusions:

    • McArdle disease is associated with abnormal exercise ventilation, characterized by increased ventilation relative to oxygen uptake.
    • Altered substrate utilization, particularly reduced reliance on glycolysis, contributes to this phenomenon.
    • Excessive ventilation and subsequent respiratory alkalosis may be key factors in exercise intolerance in McArdle disease.