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February 18, 2014; 82 (7) Article

Bezafibrate in skeletal muscle fatty acid oxidation disorders

A randomized clinical trial

Mette Cathrine Ørngreen, Karen Lindhardt Madsen, Nicolai Preisler, Grete Andersen, John Vissing, Pascal Laforêt
First published January 22, 2014, DOI: https://doi.org/10.1212/WNL.0000000000000118
Mette Cathrine Ørngreen
From the Neuromuscular Clinic and Research Unit (M.C.Ø, K.L.M., N.P., G.A., J.V.), Department of Neurology, Rigshospitalet, University of Copenhagen, Denmark; and Centre de Référence de pathologie neuromusculaire Paris-Est (P.L.), Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, France.
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Karen Lindhardt Madsen
From the Neuromuscular Clinic and Research Unit (M.C.Ø, K.L.M., N.P., G.A., J.V.), Department of Neurology, Rigshospitalet, University of Copenhagen, Denmark; and Centre de Référence de pathologie neuromusculaire Paris-Est (P.L.), Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, France.
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Nicolai Preisler
From the Neuromuscular Clinic and Research Unit (M.C.Ø, K.L.M., N.P., G.A., J.V.), Department of Neurology, Rigshospitalet, University of Copenhagen, Denmark; and Centre de Référence de pathologie neuromusculaire Paris-Est (P.L.), Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, France.
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Grete Andersen
From the Neuromuscular Clinic and Research Unit (M.C.Ø, K.L.M., N.P., G.A., J.V.), Department of Neurology, Rigshospitalet, University of Copenhagen, Denmark; and Centre de Référence de pathologie neuromusculaire Paris-Est (P.L.), Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, France.
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John Vissing
From the Neuromuscular Clinic and Research Unit (M.C.Ø, K.L.M., N.P., G.A., J.V.), Department of Neurology, Rigshospitalet, University of Copenhagen, Denmark; and Centre de Référence de pathologie neuromusculaire Paris-Est (P.L.), Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, France.
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Pascal Laforêt
From the Neuromuscular Clinic and Research Unit (M.C.Ø, K.L.M., N.P., G.A., J.V.), Department of Neurology, Rigshospitalet, University of Copenhagen, Denmark; and Centre de Référence de pathologie neuromusculaire Paris-Est (P.L.), Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, France.
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Citation
Bezafibrate in skeletal muscle fatty acid oxidation disorders
A randomized clinical trial
Mette Cathrine Ørngreen, Karen Lindhardt Madsen, Nicolai Preisler, Grete Andersen, John Vissing, Pascal Laforêt
Neurology Feb 2014, 82 (7) 607-613; DOI: 10.1212/WNL.0000000000000118

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Abstract

Objective: To assess whether bezafibrate increases fatty acid oxidation (FAO) and lowers heart rate (HR) during exercise in patients with carnitine palmitoyltransferase (CPT) II and very long-chain acyl-CoA dehydrogenase (VLCAD) deficiencies.

Methods: This was a 3-month, randomized, double-blind, crossover study of bezafibrate in patients with CPT II (n = 5) and VLCAD (n = 5) deficiencies. Primary outcome measures were changes in FAO, measured with stable-isotope methodology and indirect calorimetry, and changes in HR during exercise.

Results: Bezafibrate lowered low-density lipoprotein, triglyceride, and free fatty acid concentrations; however, there were no changes in palmitate oxidation, FAO, or HR during exercise.

Conclusion: Bezafibrate does not improve clinical symptoms or FAO during exercise in patients with CPT II and VLCAD deficiencies. These findings indicate that previous in vitro studies suggesting a therapeutic potential for fibrates in disorders of FAO do not translate into clinically meaningful effects in vivo.

Classification of evidence: This study provides Class I evidence that bezafibrate 200 mg 3 times daily is ineffective in improving changes in FAO and HR during exercise in adults with CPT II and VLCAD deficiencies.

GLOSSARY

CI=
confidence interval;
CPT=
carnitine palmitoyltransferase;
FAO=
fatty acid oxidation;
FFA=
free fatty acid;
HR=
heart rate;
LDL=
low-density lipoprotein;
MET=
metabolic equivalent;
PPAR=
peroxisome proliferator-activated receptor;
VLCAD=
very long-chain acyl-CoA dehydrogenase;
V̇o2max=
maximal oxygen uptake capacity

Footnotes

  • Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • Supplemental data at www.neurology.org

  • Received April 17, 2013.
  • Accepted in final form November 12, 2013.
  • © 2014 American Academy of Neurology
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