Effect of Bezafibrate on Muscle Metabolism in Patients With Fatty Acid Oxidation Defects
Status:
Completed
Trial end date:
2011-09-01
Target enrollment:
Participant gender:
Summary
The investigators propose to evaluate the effect of bezafibrate on metabolism during exercise
in 22 adult patients affected with carnitine palmitoyltransferase II (CPTII) or very-long
chain acyl-CoA-dehydrogenase (VLCAD) deficiencies. This study will be an 9-month, randomized,
double-blind, placebo-controlled crossover trial. The trial will be conducted in two centers:
Institut de Myologie, Pitié-Salpêtrière Hospital in France, and Rigshospitalet, University of
Copenhagen, in Denmark. The main criteria for assessing the potential effect of this drug
will be the fat oxidation rate studied during a moderate workload on cycle ergometer, after
infusion of stable isotopes (palmitate and glucose tracers).