VALPROATE-INDUCED HEPATIC-FAILURE IN A CASE OF CYTOCHROME-C-OXIDASE DEFICIENCY

被引：34

作者：

CHABROL, B ^{[1
]}

MANCINI, J ^{[1
]}

CHRETIEN, D ^{[1
]}

RUSTIN, P ^{[1
]}

MUNNICH, A ^{[1
]}

PINSARD, N ^{[1
]}

机构：

[1] HOP ENFANTS MALAD,INSERM,UNITE RECH HANDICAPS GENET,F-75743 PARIS 15,FRANCE

来源：

EUROPEAN JOURNAL OF PEDIATRICS | 1994年 / 153卷 / 02期

关键词：

HEPATIC FAILURE; COX DEFECT; VALPROATE; CHILD;

D O I：

10.1007/BF01959226

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

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.

引用

页码：133 / 135

页数：3

共 16 条

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