ISSN:
1432-1076
Keywords:
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 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.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF01959226
Permalink