Neuropediatrics Original Paper

European Journal of Pediatrics

, Volume 153, Issue 2, pp 133-135

First online:

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

  • B. ChabrolAffiliated withService de Neuropédiatrie, Hôpital d'Enfants, CHU de la Timone
  • , J. ManciniAffiliated withService de Neuropédiatrie, Hôpital d'Enfants, CHU de la Timone
  • , D. ChretienAffiliated withUnité de Recherche sur les Handicaps Génétiques de l'Enfant INSERM, Hôpital des Enfants-Malades
  • , P. RustinAffiliated withUnité de Recherche sur les Handicaps Génétiques de l'Enfant INSERM, Hôpital des Enfants-Malades
  • , A. MunnichAffiliated withUnité de Recherche sur les Handicaps Génétiques de l'Enfant INSERM, Hôpital des Enfants-Malades
  • , N. PinsardAffiliated withService de Neuropédiatrie, Hôpital d'Enfants, CHU de la Timone

Rent the article at a discount

Rent now

* Final gross prices may vary according to local VAT.

Get Access

Abstract

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