Abstract
A 3-year-old girl developed acute liver disease after treatment with valproate for 6 months. She developed the typical features of Reye syndrome. Serum free carnitine was decreased as well as 3-keto-valproic acid, the main metabolite of β-oxidation of valproate. The serum valproate concentration was at the upper limit of the therapeutic range. The possible importance of carnitine in the pathogenesis of valproate induced liver disease is discussed.
Similar content being viewed by others
References
Chapoy RR, Angelini C, Brown WJ, Stiff JE, Shug AL, Cederbaum SD (1980) Systemic carnitine deficiency — A treatable inherited lipid storage disease presenting as Reye's syndrome. New Engl J Med 303:1389–1394
Fukami MH, Williamson JR (1971) On the mechanism of inhibition of fatty acid oxidation by 4-pentenoic acid in rat liver mitochondria. J Biol Chem 246:1206–1212
Gerber N, Dickinson RG, Harland RC, Lynn R Houghton D (1979) Reye-like syndrome associated with valproic acid therapy. J Pediatr 95:142–144
Glasgow AM, Chase HP (1975) Production of the features of Reye's syndrome in rats with 4-pentenoic acid. Pediatr Res 9: 133–138
Glasgow A, Eng G, Engel A (1980) Systemic carnitine deficiency simulating recurrent Reye syndrome. J Pediatr 96:889–891
Le Bihan (1980) Fatal hepatic failure and sodium valproate. The Lancet ii: 1298
Löscher W (1981) Concentration of metabolites of valproic acid in plasma of epileptic patient. Epilepsia 22:169–178
Marquis NR, Fritz IB (1964) Enzymological determination of free carnitine concentration in rat tissues. J Lipid Res 5: 184–187
Parker PH, Helinek GL, Ghishan FK, Greene HL (1981) Recurrent pancreatitis induced by valproic acid. Gastroenterology 80:826–828
Romshe CA, Hilty MD, McClung HJ, Kerzner B, Reiner Ch B (1981) Amino acid pattern in Reye syndrome: comparison with clinically similar entities. J Pediatr 98:788–790
Schäfer H, Lührs R (1978) Metabolite pattern of valproic acid. Arzneimittel Forsch 28:657–662
Suchy FJ, Balistreri WF, Buchino JJ, Sondheimer JM (1979) Acute hepatic failure associated with the use of sodium valproate. New Engl J Med 300:962–966
Ware S, Millward-Sadler GH (1980) Acute liver disease associated with sodium valproate. The Lancet ii:1110–1113
Young RSK (1980) Fatal Reye-like syndrome associated with valproic acid. Ann Neurol 7:389–392
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Böhles, H., Richter, K., Wagner-Thiessen, E. et al. Decreased serum carnitine in valproate induced Reye syndrome. Eur J Pediatr 139, 185–186 (1982). https://doi.org/10.1007/BF01377353
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01377353