目的 探讨儿童丙戊酸钠（VPA）相关高血氨脑病（VHE）的临床特点. 方法 检索山西医科大学第一医院病案数字化综合管理系统,收集2006年7月至2015年7月诊断为VHE、年龄≤16岁所有住院患儿的病历资料进行回顾性分析.主要分析指标为VHE临床表现,治疗措施,治疗前后VPA血药浓度、血氨水平,心电图、视频脑电图和头部CT检查结果,以及临床转归. 结果 纳入分析的VHE患儿共12例,男性7例,女性5例;年龄10~15岁;原发疾病为癫痫者5例、双相情感障碍4例、胶质瘤2例、病毒性脑炎1例;VPA用药剂量为22~28 mg/kg;7例首次使用VPA;4例单用VPA,8例联用其他抗癫痫药;误诊为脑出血4例,脑水肿2例.12例患儿均表现为意识障碍、嗜睡,有7例发展至昏迷;6例在出现神经精神系统症状前出现恶心、呕吐等消化道症状,4例出现扑翼样震颤,3例出现烦躁、激越,2例出现共济失调,1例癫痫发作频繁;实验室检查示12例患儿血氨水平均升高,为85~219 μmol/L,平均（143±47）μmol/L;7例VPA血药浓度高于参考值上限,为102~125 mg/L.视频脑电图检查结果示12例均为δ波、θ波为主的弥漫性慢波.出现VHE症状后,7例停用VAP,5例VAP剂量减半,并给予降氨、降颅压等对症治疗.所有患儿治疗1~5 d临床症状完全缓解,血氨水平降至26~66 μmol/L;7 d后VPA血药浓度为30~63 mg/L,视频脑电图节律恢复为 α波. 结论 儿童VHE易误诊,主要临床表现为意识模糊、嗜睡、昏迷、共济失调、扑翼样震颤和癫痫发作频率增加,部分患儿出现神经精神系统症状前有消化道症状,血氨水平升高不明显.停药、减量、降氨、降颅压及对症处理后预后良好.
Objective To explore the clinical characteristics of valproic acid-induced hyperammone-mic encephalopathy （VHE） in children.Methods The digital administration system of medical record in the First Hospital of Shanxi Medical University was searched.The clinical data of children who with VHE, age 〈16 years and hospitalized from July 2006 to July 2015 were collected and analyzed retrospectively.The main indicators of analysis included children＇ clinical manifestations, treatments, blood concentration of valproate （VPA） and level of blood ammonia before and after treatment, results of electrocardiogram （ECG）, video electroencephalogram （EEG）, cranial computed tomography and clinical outcome.ResultsA total of 12 children were enrolled in the analysis comprising 7 boys and 5 girls with age from 10 to 15 years.The number of children with primary diseases of epilepsy, bipolar affective disorder, glioma and viral encephalitis were 5, 4, 2 and 1, respectively.The doses of VPA was 22-28 mg/kg for all cases.Seven cases were treated with VPA first time.Four cases were treated with VPA only.Eight cases received combined medications with other anti-epileptic drugs.There were 4 and 2 cases who were misdiagnosed as cerebral hemorrhage and brain edema, respectively.The 12 patients developed disorder of consciousness, drowsiness, and 7 cases developed to coma.Six cases had digestive symptoms which including nausea and vomiting before the appearance of nervous system symptoms.In addition, the number of cases with asterixis, irritability and agitation, ataxia, and frequent seizures were 4, 3, 2, and 1, respectively.The results of laboratory tests showed the children＇ blood ammonia level were increased to 85-219 μmol/L, the average level was （143±47）μmol/L.Seven cases＇ VPA blood concentration was 102-125 mg/L, which was the upper limit reference.The results of 12 patients＇ vedio-EEG were diffused inactive echoes which mainly δ and θ wave.After the appearance of symptom of VHE, seven cases were stoppe
Adverse Drug Reactions Journal