Diagnosis of argininosuccinic aciduria after valproic acid‐induced hyperammonemia

Morgan, Harley B.; Swaiman, Kenneth F.; Johnson, Barry D.

doi:10.1212/wnl.37.5.886

Cited by 5 publications

(1 citation statement)

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“…31 Similarly, hyperammonemia was precipitated by VPA therapy in a patient with arginosuccinic aciduria. 32 The toxic effect of VPA in children with urea cycle disorders may relate to inhibition of carbamyl phosphate synthesis by VPA, possibly due to the formation of CoA esters of VPA resulting in a decrease of available free CoA. 33.34 This report describes two unrelated families with probable inborn errors of metabolism where a child died of liver failure following the use of VPA.…”

Section: Discussionmentioning

confidence: 99%

The High Incidence of Valproate Hepatotoxicity in Infants May Relate to Familial Metabolic Defects

Appleton¹,

Farrell²,

Applegarth³

et al. 1990

Can. j. neurol. sci.

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ABSTRACT:The incidence of fatal hepatic failure associated with valproic acid (VPA) therapy is highest in children under the age of three years, particularly in those with developmental delay. The pathogenesis of VPA hepatotoxicity is unclear but may relate to the accumulation of a toxic metabolite of VPA which impairs fatty-acid oxidation. We describe two unrelated infants with developmental delay who developed hepatic failure while receiving VPA. Siblings of both children subsequently developed hepatic steatosis and intractable seizures without being exposed to VPA. This suggests that that the two children who developed liver failure when receiving VPA may have had a familial metabolic disorder. Familial metabolic disorders may account partly for the higher incidence of fatal hepatotoxicity described in infants receiving VPA.

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Section: Discussionmentioning

confidence: 99%