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Intravenous L-carnitine and acetyl-L-carnitine in medium-chain acyl-coenzyme A dehydrogenase deficiency and isovaleric acidemia.

Publication ,  Journal Article
Van Hove, JL; Kahler, SG; Millington, DS; Roe, DS; Chace, DH; Heales, SJ; Roe, CR
Published in: Pediatr Res
January 1994

The purpose of this study was to determine whether treatment with L-carnitine or acetyl-L-carnitine enhances the turnover of lipid or branched-chain amino acid oxidation in patients with inborn errors of metabolism. Increasing i.v. doses of L-carnitine and acetyl-L-carnitine were given to one patient with medium-chain acyl-CoA dehydrogenase deficiency and to another with isovaleric acidemia. Both patients were in stable condition and receiving oral L-carnitine supplements. The excretion of carnitine and disease-specific metabolites was measured. The incorporation of L-carnitine in the intracellular pool was demonstrated using stable isotopes and mass spectrometry. Increasing doses of either i.v. L-carnitine or acetyl-L-carnitine did not stimulate the excretion of octanoylcarnitine in the patient with medium-chain acyl-CoA dehydrogenase deficiency, nor did it raise the plasma levels of either cis-4-decenoate or octanoylcarnitine. Similarly, increasing doses of either i.v. L-carnitine or acetyl-L-carnitine did not enhance the excretion of isovalerylcarnitine in a patient with isovaleric acidemia. The excretion of isovalerylglycine actually decreased. We conclude that there was no evidence of enhanced fatty acid beta-oxidation or enhanced branched-chain amino acid oxidation in vivo by the administration of high doses of L-carnitine or acetyl-L-carnitine in these two patients. Because only one individual with each disorder was studied, the data are only indicative and may not necessarily be representative of all individuals with these disorders. Definite settlement of this issue will require further studies in additional subjects.

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Published In

Pediatr Res

DOI

ISSN

0031-3998

Publication Date

January 1994

Volume

35

Issue

1

Start / End Page

96 / 101

Location

United States

Related Subject Headings

  • Safety
  • Pentanoic Acids
  • Pediatrics
  • Lipid Metabolism, Inborn Errors
  • Injections, Intravenous
  • Humans
  • Hemiterpenes
  • Female
  • Child, Preschool
  • Carnitine
 

Citation

APA
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MLA
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Van Hove, J. L., Kahler, S. G., Millington, D. S., Roe, D. S., Chace, D. H., Heales, S. J., & Roe, C. R. (1994). Intravenous L-carnitine and acetyl-L-carnitine in medium-chain acyl-coenzyme A dehydrogenase deficiency and isovaleric acidemia. Pediatr Res, 35(1), 96–101. https://doi.org/10.1203/00006450-199401000-00020
Van Hove, J. L., S. G. Kahler, D. S. Millington, D. S. Roe, D. H. Chace, S. J. Heales, and C. R. Roe. “Intravenous L-carnitine and acetyl-L-carnitine in medium-chain acyl-coenzyme A dehydrogenase deficiency and isovaleric acidemia.Pediatr Res 35, no. 1 (January 1994): 96–101. https://doi.org/10.1203/00006450-199401000-00020.
Van Hove JL, Kahler SG, Millington DS, Roe DS, Chace DH, Heales SJ, et al. Intravenous L-carnitine and acetyl-L-carnitine in medium-chain acyl-coenzyme A dehydrogenase deficiency and isovaleric acidemia. Pediatr Res. 1994 Jan;35(1):96–101.
Van Hove, J. L., et al. “Intravenous L-carnitine and acetyl-L-carnitine in medium-chain acyl-coenzyme A dehydrogenase deficiency and isovaleric acidemia.Pediatr Res, vol. 35, no. 1, Jan. 1994, pp. 96–101. Pubmed, doi:10.1203/00006450-199401000-00020.
Van Hove JL, Kahler SG, Millington DS, Roe DS, Chace DH, Heales SJ, Roe CR. Intravenous L-carnitine and acetyl-L-carnitine in medium-chain acyl-coenzyme A dehydrogenase deficiency and isovaleric acidemia. Pediatr Res. 1994 Jan;35(1):96–101.

Published In

Pediatr Res

DOI

ISSN

0031-3998

Publication Date

January 1994

Volume

35

Issue

1

Start / End Page

96 / 101

Location

United States

Related Subject Headings

  • Safety
  • Pentanoic Acids
  • Pediatrics
  • Lipid Metabolism, Inborn Errors
  • Injections, Intravenous
  • Humans
  • Hemiterpenes
  • Female
  • Child, Preschool
  • Carnitine