European Journal of Pediatrics

, Volume 153, Issue 2, pp 133–135 | Cite as

Valproate-induced hepatic failure in a case of cytochromec oxidase deficiency

  • B. Chabrol
  • J. Mancini
  • D. Chretien
  • P. Rustin
  • A. Munnich
  • N. Pinsard
Neuropediatrics Original Paper


We report a 3 year-old girl with a myoclonic epilepsy. A fatal hepatic failure occurred after 3 months of valproate (VPA) therapy. In this patient, a defect of cytochromec oxidase (COX) was demonstrated in her circulating lymphocytes. The enzyme was also found to be deficient in post-mortem liver and in cultured skin fibroblasts. However, a fully functional respiratory chain was found in muscle. VPA administration apparently triggered the hepatic failure, given this patient's background of partial COX deficiency.

Key words

Hepatic failure COX defect Valproate Child 



cytochromec oxidase




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Copyright information

© Springer-Verlag 1994

Authors and Affiliations

  • B. Chabrol
    • 1
  • J. Mancini
    • 1
  • D. Chretien
    • 2
  • P. Rustin
    • 2
  • A. Munnich
    • 2
  • N. Pinsard
    • 1
  1. 1.Service de Neuropédiatrie, Hôpital d'EnfantsCHU de la TimoneMarseille Cedex 5France
  2. 2.Unité de Recherche sur les Handicaps Génétiques de l'Enfant INSERMHôpital des Enfants-MaladesParis Cedex 15France

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