Murakami K, Sugimoto T, Woo M, Nishida N, Muro H
Nakano Children's Hospital, Osaka, Japan.
Epilepsia. 1996 Jul;37(7):687-9. doi: 10.1111/j.1528-1157.1996.tb00634.x.
We analyzed urinary valproate (VPA) metabolites and carnitine concentrations in a child who accidentally ingested 400 mg/kg VPA. The concentration of 4-en VPA, the presumed major factor in VPA-induced hepatotoxicity, was markedly increased, without liver dysfunction or hyperammonemia. The other major abnormality was decreased beta-oxidation and markedly increased omega-oxidation. After L-carnitine supplementation, VPA metabolism returned to normal. The level of valproylcarnitine was not increased and therefore was not affected by L-carnitine. L-Carnitine may be useful in treating patients with coma after VPA overdose.
我们分析了一名意外摄入400mg/kg丙戊酸（VPA）儿童的尿中丙戊酸代谢物及肉碱浓度。4-烯丙戊酸是丙戊酸诱导肝毒性的推测主要因素，其浓度显著升高，但未出现肝功能障碍或高氨血症。另一个主要异常是β-氧化减少和ω-氧化显著增加。补充左旋肉碱后，丙戊酸代谢恢复正常。丙戊酰肉碱水平未升高，因此不受左旋肉碱影响。左旋肉碱可能对治疗丙戊酸过量后昏迷的患者有用。
