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Impaired fatty acid oxidation in children on valproic acid and the effect of L-carnitine

Departments of Neurology (Drs Kossak and Tonsgard), Pediatrics (Drs Schmidt-Sommerfeld, Penn, and Tonsgard), and Medicinci (Dr Schoeller), and the Joseph Kennedy Mental Retardation Center, The University of Chicago. Chicago, IL; and the Department of Genetics (Dr. Rinaldo), Yale University, New Haven, CT

Fatty acid oxidation was studied in 12 patients (aged 3 to 19 years) receiving valproic acid (VPA), predominantly as monotherapy, before and after 1 month of L-carnitine supplementation (50 mg/kg/day po) in order to determine whether L-carnitine plays a role in preventing the hepatotoxic effects of this drug. Five of these patients were also studied prior to VPA treatment. Only one patient taking VPA had an abnormally low plasma free carnitine. Acyl-/free carnitine ratios were elevated in five patients on VPA and normalized after L-carnithe supplementation. Mean plasma concentrations of free fatty acids, ß-OH-butyrate, and cumulative excretion of ¹³CO₂ after administration of 1-¹³C-octanoic acid were not changed by VPA or L-carnitine treatment. Urinary dicarboxylic acids, acylglycines, and octanoylcarnitine were elevated during VPA therapy and unaltered by L-carnitine. These results suggest that, in patients at low risk for VPA-induced hepatotoxicity (patients aged >2 years and taking VPA as monotherapy), VPA causes metabolic abnormalities resembling those found in inborn errors of mitochondria1 ß-oxidation which are not corrected by L-carnitine.

Address correspondence and reprint requests to Dr. Eberhard Schmidt-Sommerfeld, Department of Prdiatrics. Louisiana State University, 1542 Tulane Avenue, New Orleans, LA 70112–2822

Supported by the Children's Research Foundation of the University of Chicago and N1H grant DK 26678 Received November 16, 1992 Accepted for publication in final form March 31, 1993

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