Enhanced lipid utilization in infants receiving oral l-carnitine during long-term parenteral nutrition

  • Richard A. Helms
    Correspondence
    Reprint requests: Richard A. Helms, Pharm.D., Associate Professor, Department of Clinical Pharmacy, 26 South Dunlap, Memphis, TN 38163.
    Affiliations
    Department of Clinical Pharmacy, University of Tennessee, Memphis, USA

    Department of Pediatrics, University of Tennessee, Memphis, USA

    Department of Human Nutrition, University of Tennessee, Memphis, USA

    The Center for Pediatric Pharmacokinetics and Therapeutics, University of Tennessee, Memphis, USA

    University of Florida, Gainesville USA
    Search for articles by this author
  • Peter F. Whitington
    Affiliations
    Department of Clinical Pharmacy, University of Tennessee, Memphis, USA

    Department of Pediatrics, University of Tennessee, Memphis, USA

    Department of Human Nutrition, University of Tennessee, Memphis, USA

    The Center for Pediatric Pharmacokinetics and Therapeutics, University of Tennessee, Memphis, USA

    University of Florida, Gainesville USA
    Search for articles by this author
  • Elizabeth C. Mauer
    Affiliations
    Department of Clinical Pharmacy, University of Tennessee, Memphis, USA

    Department of Pediatrics, University of Tennessee, Memphis, USA

    Department of Human Nutrition, University of Tennessee, Memphis, USA

    The Center for Pediatric Pharmacokinetics and Therapeutics, University of Tennessee, Memphis, USA

    University of Florida, Gainesville USA
    Search for articles by this author
  • Elena F. Catarau
    Affiliations
    Department of Clinical Pharmacy, University of Tennessee, Memphis, USA

    Department of Pediatrics, University of Tennessee, Memphis, USA

    Department of Human Nutrition, University of Tennessee, Memphis, USA

    The Center for Pediatric Pharmacokinetics and Therapeutics, University of Tennessee, Memphis, USA

    University of Florida, Gainesville USA
    Search for articles by this author
  • Michael L. Christensen
    Affiliations
    Department of Clinical Pharmacy, University of Tennessee, Memphis, USA

    Department of Pediatrics, University of Tennessee, Memphis, USA

    Department of Human Nutrition, University of Tennessee, Memphis, USA

    The Center for Pediatric Pharmacokinetics and Therapeutics, University of Tennessee, Memphis, USA

    University of Florida, Gainesville USA
    Search for articles by this author
  • Peggy R. Borum
    Affiliations
    Department of Clinical Pharmacy, University of Tennessee, Memphis, USA

    Department of Pediatrics, University of Tennessee, Memphis, USA

    Department of Human Nutrition, University of Tennessee, Memphis, USA

    The Center for Pediatric Pharmacokinetics and Therapeutics, University of Tennessee, Memphis, USA

    University of Florida, Gainesville USA
    Search for articles by this author
      This paper is only available as a PDF. To read, Please Download here.
      Fourteen infants requiring long-term total parenteral nutrition but able to tolerate small quantities of enteral feedings were randomized into carnitine treatment and placebo control groups. All infants had received nutritional support devoid of carnitine. Plasma carnitine levels and observed plasma lipid indices were not different before supplementation. Under standardized, steady-state conditions, 0.5 g/kg fat emuision (intralipid) was administered intravenously over 2 hours both before and after infants received 7 days of continuous nasogastric or gastric tube l-carnitine (50 μmol/kg/day) or placebo. Plasma triglyceride, free fatty acid, acetoacetate, β-hydroxybutyrate, and carnitine concentrations were observed at 0 (start of lipid infusion), 2, and 4 hours for pre- and post-treatment periods, and in addition at 6 and 8 hours after carnitine supplementation. Infants receiving carnitine had significantly greater β-hydroxybutyrate plasma concentrations (P<0.05) and carnitine (P<0.001) at 0, 2, 4, 6, and 8 hours, and greater plasma acetoacetate concentrations (P<0.05) at 2, 4, 6, and 8 hours, compared with controls. Twenty-four-hour urinary carnitine excretion was very low for both groups before supplementation; after supplementation, excretion was higher (P<0.05) in the carnitine group. No significant differences were found between groups for plasma triglyceride or free fatty acid concentrations at any observation period. This study demonstrated enhanced fatty acid oxidation, as evidenced by increased ketogenesis, with l-carnitine supplementation in infants receiving long-term total parenteral nutrition.
      To read this article in full you will need to make a payment

      References

        • Bremer J
        Carnitine metabolism and functions.
        Physiol Rev. 1983; 63: 1420-1480
        • Borum PR
        Carnitine.
        Ann Rev Nutr. 1983; 3: 233-259
        • Schmidt-Sommerfeld E
        • Penn D
        • Wolf H
        Carnitine blood concentrations and fat utilization in parenterally alimented premature newborn infants.
        J Pediatr. 1982; 100: 260-264
        • Schiff D
        • Chen F
        • Seccombe D
        • et al.
        Plasma carnitine levels during intravenous feeding of the neonate.
        J Pediatr. 1979; 95: 1043-1046
        • Yeh Y
        • Cooke RJ
        • Zee P
        Impairment of lipid emulsion metabolism associated with carnitine insufficiency in premature infants.
        J Pediatr Gastroenterol Nutr. 1985; 4: 795-798
        • Takahashi M
        • Sawaguchi S
        Lipid metabolism in parenterally alimented neonates: carnitine blood concentrations and fat utilization.
        Indian J Pediatr. 1983; 50: 161-168
        • Schmidt-Sommerfeld E
        • Penn D
        • Sodha RJ
        • Progler M
        • Novak M
        • Schneider H
        Transfer and metabolism of carnitine and carnitine esters in the in vitro perfused human placenta.
        Pediatr Res. 1985; 19: 700-706
        • Novak M
        • Monkus EF
        • Chung D
        • et al.
        Carnitine in the perinatal metabolism of lipids. I. Relationship between maternal and fetal plasma levels of carnitine and acylcarnitines.
        Pediatrics. 1981; 67: 95-100
        • Penn D
        Decreased tissue carnitine concentrations in newborn infants receiving total parenteral nutrition.
        J Pediatr. 1981; 98: 976-978
        • Borum PR
        • York CM
        • Broquist HP
        Carnitine content of liquid formulas and special diets.
        Am J Clin Nutr. 1979; 32: 2272-2276
        • Kreutz FH
        Enzymatische Glyzerinbestimmung.
        Klin Wochenschr. 1962; 40: 362
        • Miles J
        A microfluorometric method for determination of free fatty acids in plasma.
        J Lipid Res. 1983; 24: 96
        • Persson B
        Determination of plasma acetoacetate and β-hydroxybutarate in newborn infants by an enzymatic fluorometric micro-method.
        Scand J Clin Lab Invest. 1969; 25: 918
        • Cederblad G
        • Lindstedt S
        A method for the determination of carnitine in the picomole range.
        Clin Chim Acta. 1972; 37: 235-243
        • Schmidt-Sommerfield E
        • Penn D
        • Wolf H
        Carnitine deficiency in premature infants receiving total parenteral nutrition: effect of l-carnitine supplementation.
        J Pediatr. 1983; 102: 931-935
        • Orzali A
        • Donzelli F
        • Enzi E
        • et al.
        Effect of carnitine on lipid metabolism in the newborn. I. Carnitine supplementation during total parenteral nutrition in the first 48 hours of life.
        Biol Neonate. 1983; 43: 186-190
        • Orzali A
        • Maetzke E
        • Donzelli F
        • et al.
        Effect of carnitine on lipid metabolism in the neonate. II. Carnitine addition to lipid infusion during prolonged total parenteral nutrition.
        J Pediatr. 1984; 104: 436-440
        • Curran JS
        • Williams PR
        • Kanarek KS
        • et al.
        An evaluation of orally supplemented l-carnitine in premature infants receiving Intralipid 20%.
        Acta Chir Schand. 1983; 517 ([Suppl]): 157-164
        • Warshaw JB
        • Curry E
        Comparison of serum carnitine and ketone body concentrations in breast- and in formula-fed newborn infants.
        J Pediatr. 1980; 97: 122-125
        • Novak M
        • Monkus EF
        • Burch M
        • et al.
        The effects of a l-carnitine supplemented soybean formula on the plasma lipids of infants.
        Acta Chir Scand. 1983; 517 ([Suppl]): 149-155
        • Duran M
        • deKlerk JBC
        • Wadman SK
        • et al.
        Systemic carnitine deficiency: benefit of oral carnitine supplements vs. persisting biochemical abnormalities.
        Eur J Pediatr. 1984; 142: 224-228
        • Chapoy PR
        • Angelini C
        • Brown WJ
        • et al.
        Systemic carnitine deficiency: a treatable inherited lipid-storage disease presenting as Reye's syndrome.
        N Engl J Med. 1980; 303: 1389-1394
        • Whitington PF
        Cholestasis associated with total parenteral nutrition in infants.
        Hepatology. 1985; 5: 693-696