Decreased fasting free fatty acids with L-carnitine in children with carnitine deficiency

W. F. Schwenk, D. E. Hale, M. W. Haymond

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

At the time of acute presentation, children with carnitine deficiency may have increased free fatty acid concentrations and hypoglycemia. However, whether carnitine replacement affects the plasma concentration of these substrates remains to be determined. Therefore, to evaluate the effect of carnitine replacement on plasma substrate and hormone concentrations, five children with carnitine deficiency (two idiopathic, two secondary to long-chain acyl coenzyme A dehydrogenase deficiency, one secondary to isovaleric acidemia) were fasted overnight before and after treatment with oral carnitine (80 ± 7 mg · kg-1 · day-1). During carnitine supplementation, plasma total carnitine (19 ± 4 versus 45 ± 6 nmol/ml, pretreatment versus treatment, respectively) and free carnitine (11 ± 3 versus 31 ± 6 nmol/ml), as well as red blood cell total carnitine (0.057 ± 0.019 versus 0.130 ± 0.019 nmol/mg of hemoglobin) increased (p < 0.05). Fasting plasma glucose (83 ± 4 versus 85 ± 3 mg/dl) and ketone body (0.54 ± 0.18 and 0.56 ± 0.20 mM) concentrations did not change with carnitine supplementation, but plasma free fatty acids (1.28 ± 0.32 versus 0.77 ± 0.07 mM) decreased (p < 0.05). No differences in fasting insulin, growth hormone, or cortisol concentrations were observed. Urinary excretion of free carnitine (0.1 ± 0.0 versus 2.4 ± 0.7 μmol/mg creatinine), total carnitine (0.3 ± 0.1 versus 3.4 ± 0.9 μmol/mg creatinine) and acyl carnitine (0.2 ± 0.1 versus 0.9 ± 0.3 μmol/mg creatinine) increased (p < 0.05) with carnitine supplementation. The decreased plasma free fatty acid concentrations with carnitine supplementation may be due to more efficient fatty acid oxidation and/or increased urinary excretion of fatty acids as acylcarnitines.

Original languageEnglish (US)
Pages (from-to)491-494
Number of pages4
JournalPediatric Research
Volume23
Issue number5
StatePublished - 1988
Externally publishedYes

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Carnitine
Nonesterified Fatty Acids
Fasting
Creatinine
Long-Chain Acyl-CoA Dehydrogenase
Fatty Acids
Ketone Bodies
Hypoglycemia
Growth Hormone
Hydrocortisone

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Schwenk, W. F., Hale, D. E., & Haymond, M. W. (1988). Decreased fasting free fatty acids with L-carnitine in children with carnitine deficiency. Pediatric Research, 23(5), 491-494.

Decreased fasting free fatty acids with L-carnitine in children with carnitine deficiency. / Schwenk, W. F.; Hale, D. E.; Haymond, M. W.

In: Pediatric Research, Vol. 23, No. 5, 1988, p. 491-494.

Research output: Contribution to journalArticle

Schwenk, WF, Hale, DE & Haymond, MW 1988, 'Decreased fasting free fatty acids with L-carnitine in children with carnitine deficiency', Pediatric Research, vol. 23, no. 5, pp. 491-494.
Schwenk, W. F. ; Hale, D. E. ; Haymond, M. W. / Decreased fasting free fatty acids with L-carnitine in children with carnitine deficiency. In: Pediatric Research. 1988 ; Vol. 23, No. 5. pp. 491-494.
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