Valproate-induced hepatic failure in a case of cytochromec oxidase deficiency
- 1 February 1994
- journal article
- case report
- Published by Springer Nature in European Journal of Pediatrics
- Vol. 153 (2) , 133-135
- https://doi.org/10.1007/bf01959226
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.Keywords
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