Response to the comments on ‘Acute hepatic injury in a child with Dravet syndrome: No protective effect of stiripentol’

Goorhuis et al. add some very important information about the problem of medication-induced hepatotoxicity in children with Dravet syndrome. The girl they describe was treated with valproic acid, stiripentol and acetaminophen. Stiripentol is thought to prevent liver damage in acetaminophen intoxication. Stiripentol inhibits the synthesis of N-acetyl-pbenzoquinone-imine, a highly reactive acetaminophen-metabolite. In rats, stiripentol is able to prevent hepatic necrosis after acetaminophen overdose and in 24 children treated with the combination of stiripentol, VPA, and TPM, no liver problems have been reported. The four children we have described and the girl described by Goorhuis et al. have in common that they all used valproic acid and acetaminophen, which strongly favours the hypothesis that this combination is causing hepatotoxicity. Recently, at the 7th EPNS congress in Kusadasi, Turkey, a poster was presented of a 4-year-old boy with Dravet syndrome suffering from severe hepatic damage and hyperammonemia after treatment with valproic acid and phenobarbitone. Also in this case, liver problems developed after he had been given