title: Valproate-induced hepatic failure in a case of cytochrome c oxidase deficiency.
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 cytochrome c 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.
authors.affiliation: Service de Neuropédiatrie, Hôpital d'Enfants, CHU de la Timone, Marseille, France.
authors.forename: B
authors.initials: B
authors.lastname: Chabrol
authors.forename: J
authors.initials: J
authors.lastname: Mancini
authors.forename: D
authors.initials: D
authors.lastname: Chretien
authors.forename: P
authors.initials: P
authors.lastname: Rustin
authors.forename: A
authors.initials: A
authors.lastname: Munnich
authors.forename: N
authors.initials: N
authors.lastname: Pinsard
mesh_terms: D000596:Amino Acids / Q000097:blood; D002675:Child, Preschool; D002849:Chromatography, Gas; D030401:Cytochrome-c Oxidase Deficiency; D004831:Epilepsies, Myoclonic / Q000188:drug therapy; D017809:Fatal Outcome; D005260:Female; D006501:Hepatic Encephalopathy / Q000097:blood / Q000139:chemically induced*; D006801:Humans; D007773:Lactates / Q000097:blood; D011773:Pyruvates / Q000097:blood; D014635:Valproic Acid / Q000009:adverse effects*
chemical_list: D000596:Amino Acids; D007773:Lactates; D011773:Pyruvates; D014635:Valproic Acid