Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2006 Sep-Oct;89(1-2):58-63.
doi: 10.1016/j.ymgme.2006.06.004. Epub 2006 Jul 27.

Metabolic control during exercise with and without medium-chain triglycerides (MCT) in children with long-chain 3-hydroxy acyl-CoA dehydrogenase (LCHAD) or trifunctional protein (TFP) deficiency

Melanie B Gillingham  1 , Bradley Scott, Diane Elliott, Cary O Harding
Affiliations

Metabolic control during exercise with and without medium-chain triglycerides (MCT) in children with long-chain 3-hydroxy acyl-CoA dehydrogenase (LCHAD) or trifunctional protein (TFP) deficiency

Melanie B Gillingham et al. Mol Genet Metab. 2006 Sep-Oct.

Abstract

Exercise induced rhabdomyolysis is a complication of long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) and mitochondrial trifunctional protein (TFP) deficiency that frequently leads to exercise avoidance. Dietary therapy for most subjects includes medium-chain triglyceride (MCT) supplementation but analysis of diet records indicates that the majority of patients consume oral MCT only with breakfast and at bedtime. We hypothesized that MCT immediately prior to exercise would provide an alternative fuel source during that bout of exercise and improve exercise tolerance in children with LCHAD deficiency. Nine subjects completed two 45 min moderate intensity (60-70% predicted maximum heart rate (HR)) treadmill exercise tests. Subjects were given 4 oz of orange juice alone or orange juice and 0.5 g MCT per kg lean body mass, 20 min prior to exercise in a randomized cross-over design. ECG and respiratory gas exchange including respiratory quotient (RQ) were monitored. Blood levels of acylcarnitines, creatine kinase, lactate, and beta-hydroxybutyrate were measured prior to and immediately after exercise, and again following 20 min rest. Creatine kinase and lactate levels did not change with exercise. There was no significant difference in RQ between the two exercise tests but there was a decrease in steady-state HR following MCT supplementation. Cumulative long-chain 3-hydroxyacylcarnitines were 30% lower and beta-hydroxybutyrate was three-fold higher after the MCT-pretreated exercise test compared to the test with orange juice alone. Coordinating MCT supplementation with periods of increased activity may improve the metabolic control of children with LCHAD and TFP deficiency following exercise.

PubMed Disclaimer

Figures

Fig. 1
Fig. 1
Change in steady-state HR between two moderate intensity exercise tests in children with LCHAD or TFP deficiency. Mean HR during 35–40min of the exercise test pretreated with MCT (124 ± 14) was significantly lower than the mean HR of the exercise test pretreated with orange juice alone (132 ± 16) at P ≤ 0.05.
Fig. 2
Fig. 2
Mean (±standard deviation) plasma sum of long-chain hydroxyacylcarnitines prior to, and following moderate intensity exercise and after 20 min of recovery in nine children with LCHAD or TFP deficiency. ■represent levels during the exercise test pretreated with orange juice alone. formula image represent levels during the exercise test pretreated with orange juice and MCT. There was no significant difference between tests pre- or post-exercise. There was significantly lower plasma hydroxyacylcarnitines after 20 min of recovery when subjects were pretreated with orange juice and MCT. * indicates a signifiant difference between the two tests at P < 0.05.
Fig. 3
Fig. 3
Mean (+standard deviation) plasma β-hydroxybutyrate prior to moderate intensity exercise and after 20 min of recovery in nine children with LCHAD or TFP deficiency.■ represent levels during the exercise test pretreated with orange juice alone. formula image represent levels during the exercise test pretreated with orange juice and MCT. * indicates a significant difference between the two tests at P ≤ 0.05.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Jeukendrup AE, Saris WH, Wagenmakers AJ. Fat metabolism during exercise: a review–part II: regulation of metabolism and the effects of training. Int. J. Sports Med. 1998;19:293–302. - PubMed
    1. Odle J. New insights into the utilization of medium-chain triglycerides by the neonate: observations from a piglet model. J. Nutr. 1997;127:1061–1067. - PubMed
    1. Odle J, Benevenga NJ, Crenshaw TD. Utilization of medium-chain triglycerides by neonatal piglets: II. Effects of even- and odd-chain triglyceride consumption over the Wrst 2 days of life on blood metabolites and urinary nitrogen excretion. J. Anim. Sci. 1989;67:3340–3351. - PubMed
    1. Gillingham MB, Weleber RG, Neuringer M, Connor WE, Mills M, van Calcar S, Ver Hoeve J, Wolff J, Harding CO. EVect of optimal dietary therapy upon visual function in children with long-chain 3-hydroxyacyl CoA dehydrogenase and trifunctional protein deficiency. Mol. Genet. Metab. 2005;86:124–133. - PMC - PubMed
    1. Ibdah JA, Bennett MJ, Rinaldo P, Zhao Y, Gibson B, Sims HF, Strauss AW. A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women. N. Engl. J. Med. 1999;340:1723–1731. - PubMed

Publication types