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No effect of triheptanoin on exercise performance in McArdle disease

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Madsen, KL, Laforêt, P, Buch, AE, Stemmerik, MG, Ottolenghi, C, Hatem, SN, Raaschou-Pedersen, DT, Poulsen, NS, Atencio, M, Luton, M-P, Ceccaldi, A, Haller, RG, Quinlivan, R, Mochel, F & Vissing, J 2019, 'No effect of triheptanoin on exercise performance in McArdle disease' Annals of Clinical and Translational Neurology, bind 6, nr. 10, s. 1949-1960. https://doi.org/10.1002/acn3.50863

APA

CBE

Madsen KL, Laforêt P, Buch AE, Stemmerik MG, Ottolenghi C, Hatem SN, Raaschou-Pedersen DT, Poulsen NS, Atencio M, Luton M-P, Ceccaldi A, Haller RG, Quinlivan R, Mochel F, Vissing J. 2019. No effect of triheptanoin on exercise performance in McArdle disease. Annals of Clinical and Translational Neurology. 6(10):1949-1960. https://doi.org/10.1002/acn3.50863

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Author

Madsen, Karen L ; Laforêt, Pascal ; Buch, Astrid E ; Stemmerik, Mads G ; Ottolenghi, Chris ; Hatem, Stéphane N ; Raaschou-Pedersen, Daniel T ; Poulsen, Nanna S ; Atencio, Maria ; Luton, Marie-Pierre ; Ceccaldi, Alexandre ; Haller, Ronald G ; Quinlivan, Ros ; Mochel, Fanny ; Vissing, John. / No effect of triheptanoin on exercise performance in McArdle disease. I: Annals of Clinical and Translational Neurology. 2019 ; Bind 6, Nr. 10. s. 1949-1960.

Bibtex

@article{fe8bb25d60d148948dae0bd6c689a18e,
title = "No effect of triheptanoin on exercise performance in McArdle disease",
abstract = "Objective: To study if treatment with triheptanoin, a 7-carbon triglyceride, improves exercise tolerance in patients with McArdle disease. McArdle patients have a complete block in glycogenolysis and glycogen-dependent expansion of tricarboxylic acid cycle (TCA), which may restrict fat oxidation. We hypothesized that triheptanoin metabolism generates substrates for the TCA, which potentially boosts fat oxidation and improves exercise tolerance in McArdle disease. Methods: Double-blind, placebo-controlled, crossover study in patients with McArdle disease completing two treatment periods of 14 days each with a triheptanoin or placebo diet (1 g/kg/day). Primary outcome was change in mean heart rate during 20 min submaximal exercise on a cycle ergometer. Secondary outcomes were change in peak workload and oxygen uptake along with changes in blood metabolites and respiratory quotients. Results: Nineteen of 22 patients completed the trial. Malate levels rose on triheptanoin treatment versus placebo (8.0 ± SD2.3 vs. 5.5 ± SD1.8 µmol/L, P < 0.001), but dropped from rest to exercise (P < 0.001). There was no difference in exercise heart rates between triheptanoin (120 ± SD16 bpm) and placebo (121 ± SD16 bpm) treatments. Compared with placebo, triheptanoin did not change the submaximal respiratory quotient (0.82 ± SD0.05 vs. 0.84 ± SD0.03), peak workload (105 ± SD38 vs. 102 ± SD31 Watts), or peak oxygen uptake (1938 ± SD499 vs. 1977 ± SD380 mL/min). Interpretation: Despite increased resting plasma malate with triheptanoin, the increase was insufficient to generate a normal TCA turnover during exercise and the treatment has no effect on exercise capacity or oxidative metabolism in patients with McArdle disease.",
author = "Madsen, {Karen L} and Pascal Lafor{\^e}t and Buch, {Astrid E} and Stemmerik, {Mads G} and Chris Ottolenghi and Hatem, {St{\'e}phane N} and Raaschou-Pedersen, {Daniel T} and Poulsen, {Nanna S} and Maria Atencio and Marie-Pierre Luton and Alexandre Ceccaldi and Haller, {Ronald G} and Ros Quinlivan and Fanny Mochel and John Vissing",
note = "{\circledC} 2019 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals, Inc on behalf of American Neurological Association.",
year = "2019",
month = "10",
doi = "10.1002/acn3.50863",
language = "English",
volume = "6",
pages = "1949--1960",
journal = "Annals of Clinical and Translational Neurology",
issn = "2328-9503",
publisher = "John Wiley and Sons Ltd",
number = "10",

}

RIS

TY - JOUR

T1 - No effect of triheptanoin on exercise performance in McArdle disease

AU - Madsen, Karen L

AU - Laforêt, Pascal

AU - Buch, Astrid E

AU - Stemmerik, Mads G

AU - Ottolenghi, Chris

AU - Hatem, Stéphane N

AU - Raaschou-Pedersen, Daniel T

AU - Poulsen, Nanna S

AU - Atencio, Maria

AU - Luton, Marie-Pierre

AU - Ceccaldi, Alexandre

AU - Haller, Ronald G

AU - Quinlivan, Ros

AU - Mochel, Fanny

AU - Vissing, John

N1 - © 2019 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals, Inc on behalf of American Neurological Association.

PY - 2019/10

Y1 - 2019/10

N2 - Objective: To study if treatment with triheptanoin, a 7-carbon triglyceride, improves exercise tolerance in patients with McArdle disease. McArdle patients have a complete block in glycogenolysis and glycogen-dependent expansion of tricarboxylic acid cycle (TCA), which may restrict fat oxidation. We hypothesized that triheptanoin metabolism generates substrates for the TCA, which potentially boosts fat oxidation and improves exercise tolerance in McArdle disease. Methods: Double-blind, placebo-controlled, crossover study in patients with McArdle disease completing two treatment periods of 14 days each with a triheptanoin or placebo diet (1 g/kg/day). Primary outcome was change in mean heart rate during 20 min submaximal exercise on a cycle ergometer. Secondary outcomes were change in peak workload and oxygen uptake along with changes in blood metabolites and respiratory quotients. Results: Nineteen of 22 patients completed the trial. Malate levels rose on triheptanoin treatment versus placebo (8.0 ± SD2.3 vs. 5.5 ± SD1.8 µmol/L, P < 0.001), but dropped from rest to exercise (P < 0.001). There was no difference in exercise heart rates between triheptanoin (120 ± SD16 bpm) and placebo (121 ± SD16 bpm) treatments. Compared with placebo, triheptanoin did not change the submaximal respiratory quotient (0.82 ± SD0.05 vs. 0.84 ± SD0.03), peak workload (105 ± SD38 vs. 102 ± SD31 Watts), or peak oxygen uptake (1938 ± SD499 vs. 1977 ± SD380 mL/min). Interpretation: Despite increased resting plasma malate with triheptanoin, the increase was insufficient to generate a normal TCA turnover during exercise and the treatment has no effect on exercise capacity or oxidative metabolism in patients with McArdle disease.

AB - Objective: To study if treatment with triheptanoin, a 7-carbon triglyceride, improves exercise tolerance in patients with McArdle disease. McArdle patients have a complete block in glycogenolysis and glycogen-dependent expansion of tricarboxylic acid cycle (TCA), which may restrict fat oxidation. We hypothesized that triheptanoin metabolism generates substrates for the TCA, which potentially boosts fat oxidation and improves exercise tolerance in McArdle disease. Methods: Double-blind, placebo-controlled, crossover study in patients with McArdle disease completing two treatment periods of 14 days each with a triheptanoin or placebo diet (1 g/kg/day). Primary outcome was change in mean heart rate during 20 min submaximal exercise on a cycle ergometer. Secondary outcomes were change in peak workload and oxygen uptake along with changes in blood metabolites and respiratory quotients. Results: Nineteen of 22 patients completed the trial. Malate levels rose on triheptanoin treatment versus placebo (8.0 ± SD2.3 vs. 5.5 ± SD1.8 µmol/L, P < 0.001), but dropped from rest to exercise (P < 0.001). There was no difference in exercise heart rates between triheptanoin (120 ± SD16 bpm) and placebo (121 ± SD16 bpm) treatments. Compared with placebo, triheptanoin did not change the submaximal respiratory quotient (0.82 ± SD0.05 vs. 0.84 ± SD0.03), peak workload (105 ± SD38 vs. 102 ± SD31 Watts), or peak oxygen uptake (1938 ± SD499 vs. 1977 ± SD380 mL/min). Interpretation: Despite increased resting plasma malate with triheptanoin, the increase was insufficient to generate a normal TCA turnover during exercise and the treatment has no effect on exercise capacity or oxidative metabolism in patients with McArdle disease.

UR - http://www.scopus.com/inward/record.url?scp=85073624286&partnerID=8YFLogxK

U2 - 10.1002/acn3.50863

DO - 10.1002/acn3.50863

M3 - Journal article

VL - 6

SP - 1949

EP - 1960

JO - Annals of Clinical and Translational Neurology

JF - Annals of Clinical and Translational Neurology

SN - 2328-9503

IS - 10

ER -

ID: 58348261