Skeletal muscle metabolism is impaired during exercise in glycogen storage disease type III
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Skeletal muscle metabolism is impaired during exercise in glycogen storage disease type III. / Preisler, Nicolai; Laforêt, Pascal; Madsen, Karen Lindhardt; Prahm, Kira Philipsen; Hedermann, Gitte; Vissing, Christoffer Rasmus; Galbo, Henrik; Vissing, John.
I: Neurology, Bind 84, Nr. 17, 28.04.2015, s. 1767-71.Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
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TY - JOUR
T1 - Skeletal muscle metabolism is impaired during exercise in glycogen storage disease type III
AU - Preisler, Nicolai
AU - Laforêt, Pascal
AU - Madsen, Karen Lindhardt
AU - Prahm, Kira Philipsen
AU - Hedermann, Gitte
AU - Vissing, Christoffer Rasmus
AU - Galbo, Henrik
AU - Vissing, John
N1 - © 2015 American Academy of Neurology.
PY - 2015/4/28
Y1 - 2015/4/28
N2 - 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.
AB - 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.
KW - Adolescent
KW - Adult
KW - Energy Metabolism
KW - Exercise
KW - Fructose
KW - Glycogen Storage Disease Type III
KW - Humans
KW - Muscle Weakness
KW - Muscle, Skeletal
KW - Young Adult
U2 - 10.1212/WNL.0000000000001518
DO - 10.1212/WNL.0000000000001518
M3 - Journal article
C2 - 25832663
VL - 84
SP - 1767
EP - 1771
JO - Neurology
JF - Neurology
SN - 0028-3878
IS - 17
ER -
ID: 162839547