Valproate-induced hepatic failure in a case of cytochrome c oxidase deficiency.
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.
Chabrol
B
B
Service de Neuropédiatrie, Hôpital d'Enfants, CHU de la Timone, Marseille, France.
Mancini
J
J
Chretien
D
D
Rustin
P
P
Munnich
A
A
Pinsard
N
N
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*
D000596:Amino Acids; D007773:Lactates; D011773:Pyruvates; D014635:Valproic Acid