Skeletal muscle metabolism is impaired during exercise in glycogen storage disease type III
| Autor: | Gitte Hedermann, Kira Philipsen Prahm, Pascal Laforêt, Christoffer Rasmus Vissing, John Vissing, Nicolai Preisler, Henrik Galbo, Karen Lindhardt Madsen |
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| Rok vydání: | 2015 |
| Předmět: |
Adult
medicine.medical_specialty Glycogenolysis Adolescent Fructose Exercise intolerance Glycogen storage disease type III Glycogen Storage Disease Type III Young Adult chemistry.chemical_compound Internal medicine medicine Humans Ingestion Glycogen storage disease Muscle Skeletal Exercise Muscle Weakness business.industry Skeletal muscle Muscle weakness medicine.disease Endocrinology medicine.anatomical_structure chemistry Neurology (clinical) medicine.symptom Energy Metabolism business |
| Zdroj: | Neurology. 84:1767-1771 |
| ISSN: | 1526-632X 0028-3878 |
| DOI: | 10.1212/wnl.0000000000001518 |
| Popis: | Objective: Glycogen storage disease type IIIa (GSDIIIa) is classically regarded as a glycogenosis with fixed weakness, but we hypothesized that exercise intolerance in GSDIIIa is related to muscle energy failure and that oral fructose ingestion could improve exercise tolerance in this metabolic myopathy. Methods: We challenged metabolism with cycle-ergometer exercise and measured substrate turnover and oxidation rates using stable isotope methodology and indirect calorimetry in 3 patients and 6 age-matched controls on 1 day, and examined the effect of fructose ingestion on exercise tolerance in the patients on another day. Results: Total fatty acid oxidation rates during exercise were higher in patients than controls, 32.1 (SE 1.2) vs 20.7 (SE 0.5; range 15.8–29.3) μmol/kg/min ( p = 0.048), and oxidation of carbohydrates was lower in patients, 1.0 (SE 5.4) vs 38.4 (SE 8.0; range 23.0–77.1) μmol/kg/min ( p = 0.024). Fructose ingestion improved exercise tolerance in the patients. Conclusion: Similar to patients with McArdle disease, in whom muscle glycogenolysis is also impaired, GSDIIIa is associated with a reduced skeletal muscle oxidation of carbohydrates and a compensatory increase in fatty acid oxidation, and fructose ingestion improves exercise tolerance. Our results indicate that GSDIIIa should not only be viewed as a glycogenosis with fixed skeletal muscle weakness, but should also be considered among the glycogenoses presenting with exercise-related dynamic symptoms caused by muscular energy deficiency. Classification of evidence: This study provides Class IV evidence that ingestion of fructose improves exercise tolerance in patients with GSDIIIa. |
| Databáze: | OpenAIRE |
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