ISSN:
1432-1076
Keywords:
Key words: Hepatic failure – COX defect – Valproate – Child
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
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.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF01959226
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