报道1例丙戊酸(VPA)诱发婴儿肝衰竭病例。患儿,女,8个月16天,自生后1月余即被诊断为婴儿痉挛,以较大剂量VPA及氯硝西泮(CNP)治疗,近日合并肺炎伴癫癎发作加重,且颜面、颈胸部出现较多针尖大小出血点,伴牙龈出血,四肢尤双下肢明显凹陷性水肿。实验室检查见血象三系降低,严重低蛋白血症,但肝酶谱ALT正常,VPA及CNP血药浓度均高于参考范围,考虑为VPA诱发肝衰竭。结合该病例的特殊临床表现和近年文献复习,对VPA诱发肝衰竭的高危因素、发生机制、早期临床判断、以及防治对策进行分析讨论。

A case of valproic acid (VPA)-induced hepatic failure was reported. An 8.5-month-old female developed infantile spasm at 1 month of life. She was then treated with high-dose VPA and clonazepam (CNP). She manifested with frequent seizures in the presence of pneumonia. Gingival bleeding, as well as multiple punctuated hemorrhagic spots were observed on her face and cervico-thoracic areas. There was severe edema on her extremities, especially the lower extremities. A significant reduction of full blood count and severe hypoproteinemia were noted although transaminase ALT was still within normal range. Both serum VPA and CNP levels were beyond the reference range. Diagnosis of VPA-induced hepatic failure was based on the clinical and laboratory findings. This study reviewed the high risk factors, the pathogenesis, early diagnosis, prevention and treatment of VPA-induced hepatic failure based on the case report and published literatures.