Unusual cause of hyperammonemia in two cases with short-term and long-term valproate therapy successfully treated by single dose carglumic acid.
Valproic acid (VPA) is an antiepileptic drug which is used in the treatment of various seizure disorders including tonic-clonic, myoclonic, absence, partial seizures and psychiatric disorders. VPA is usually well tolerated, but severe adverse effects may occur. Hyperammonaemic encephalopathy (HE) is a rare and potentially fatal complication of VPA treatment. The mechanism by which valproate induces hyperammonemia remains incompletely understood but is likely to relate to the urea cycle. Herein we present two cases with valproate-induced hyperammonemia at therapeutic valproate levels without signs of liver failure and were successfully treated by a single dose of carglumic acid.
Department of Pediatric Metabolism and Nutrition, Diyarbakır Children's Hospital, Diyarbakır, Turkey.
Ciğdem Seher
CS
Kasapkara
Department of Pediatrics, Diyarbakır Children's Hospital, Diyarbakır, Turkey.
Murat
M
Kanğın
Department of Pediatrics, Diyarbakır Children's Hospital, Diyarbakır, Turkey.
Funda Feryal
FF
Taş
Department of Pediatric Neurology, Diyarbakır Children's Hospital, Diyarbakır, Turkey.
Yasemin
Y
Topçu
Department of Pediatrics, Diyarbakır Children's Hospital, Diyarbakır, Turkey.
Remezan
R
Demir
Department of Pediatric Endocrinology, Diyarbakır Children's Hospital, Diyarbakır, Turkey.
Mehmet Nuri
MN
Ozbek
Carglumic acid; valproate therapy; valproate-induced hyperammonemia