Xiong Hui, Liu Chen-tao, Zhang Yue-hua, Bao Xin-hua, Jiang Yu-wu, Zhao Hong, Wu Xiao-ping, Qin Jiong
Department of Pediatrics, Peking University First Hospital, Beijing 100034, China.
Zhonghua Er Ke Za Zhi. 2012 Dec;50(12):890-4.
Children with refractory epilepsy who suffered from severe liver function impairment during valproic acid (VPA) treatment at routine dosage were studied. The clinical manifestations and therapeutic approaches were investigated in order to improve its diagnosis and management.
Clinical information as well as features and management of 4 inpatients who were suffered from intractable epilepsy with severe liver function impairment induced by VPA since 2006 were collected and analyzed, including age of onset of epilepsy, VPA using age and the time when liver injury occurred, clinical manifestations, auxiliary examinations and management.
Among the 4 cases, three were male and one was female. The admitted age ranged from 1 - 9 years and 1 month. The course of disease was 25 d - 6 months. They manifested as refractory epilepsy of epilepsia partialis continua which was difficult to control. After using VPA for 62 d (50 - 76 d), all developed severe impairment of liver synthetic function which was not related to the concentration of VPA. One was diagnosed with Alpers syndrome, two were suspicious of Alpers syndrome, and the other was diagnosed gliocytoma after brain biopsy. VPA was stopped immediately and symptomatic therapies were used. Other than that, intravenous injection of L-carnitine in 3 cases recovered the liver function.
VPA-associated severe hepatotoxicity can manifest first as impaired liver synthetic function. Besides alanin transaminase and aspartate transaminase, the liver synthetic function test is more important than monitoring of liver enzymatic functions in monitoring for the hepatotoxicity. Intravenous injection of L-carnitine in early stage showed good treatment effect.
研究难治性癫痫患儿在常规剂量丙戊酸(VPA)治疗期间出现严重肝功能损害的情况。探讨其临床表现及治疗方法,以提高诊断和治疗水平。
收集并分析自2006年以来4例因VPA导致严重肝功能损害的难治性癫痫住院患者的临床资料、特点及治疗情况,包括癫痫起病年龄、VPA用药年龄及肝损伤发生时间、临床表现、辅助检查及治疗措施。
4例患者中,男性3例,女性1例。入院年龄为1岁至9岁1个月。病程为25天至6个月。均表现为难治性持续性部分性癫痫,难以控制。使用VPA 62天(50 - 76天)后,均出现严重的肝脏合成功能损害,且与VPA浓度无关。1例诊断为Alpers综合征,2例疑似Alpers综合征,另1例经脑活检诊断为胶质细胞瘤。立即停用VPA并采取对症治疗。此外,3例静脉注射左卡尼汀后肝功能恢复。
VPA相关的严重肝毒性可首先表现为肝脏合成功能受损。在监测肝毒性时,除谷丙转氨酶和谷草转氨酶外,肝脏合成功能检测比监测肝酶功能更重要。早期静脉注射左卡尼汀显示出良好的治疗效果。