目的 探讨儿童重症监护病房（PICU）收治首次发作难治性癫痫持续状态（RSE）临床特征及预后。方法 回顾性收集2017年1月—2021年12月PICU收入的RSE患儿的临床资料，依据生存状态分存活组和死亡组。总结性别、年龄、体重指数、病因、入院时格拉斯哥昏迷评分（GCS）、头部影像学、脑电图、抗癫痫药物、出院时改良Rankin量表（mRS）等。分析RSE患儿的临床特点、治疗及预后。结果 合计收治RSE患儿80例，男性39例（48.8%），发病年龄中位数38（12~80.8）月，其中超难治性癫痫持续状态（SRSE）12例（15%）。RSE原因为重症脑炎50例，癫痫综合征9例、颅内肿瘤/出血7例、免疫性脑炎4例、遗传代谢脑病4例、脓毒症脑病3例、化脓性脑膜炎3例。需机械通气53例（66.3%），机械通气时间中位数96（54~239）h。77例头颅磁共振成像检查，其中异常61例（79.2%）。惊厥发作持续时间中位数19（2~46）h，住PICU时间中位数8.5（5~17.8）d，死亡19例（住院病死率23.8%）。61例患者存活出院时神经功能恢复良好者（mRS<3）23例（37.7%）。比较存活组和死亡组，入院GCS评分、影像学异常比例有显著差异（均P<0.05）。结论 本组儿童新发RSE的病因主要为重症脑炎，入院时GCS评分、头颅影像结果对预后判断具有参考价值。

Objective To investigate the clinical characteristics and prognosis of refractory status epilepticus (RSE) patients admitted to the pediatric intensive care unit (PICU). Methods Clinical data of children with RSE admitted to the PICU from January 2017 to December 2021 were retrospectively collected, and they were divided into survival and death groups based on survival status. Gender, age, body mass index, etiology, Glasgow Coma Score (GCS) at admission, head imaging, electroencephalogram, antiepileptic drugs, and modified Rankin Scale (mRS) at discharge were summarized. The clinical characteristics, treatment and prognosis of children with RSE were analyzed. Results A total of 80 children with RSE were admitted, 39 (48.8%) were male, median age at onset was 38 (12-80.8) months, including 12 (15%) cases of super-refractory status epilepticus sustained (SRSE).The causes of RSE were severe encephalitis in 50 cases, epileptic syndromes in 9 cases, intracranial tumors/haemorrhages in 7 cases, immune encephalitis in 4 cases, hereditary-metabolic encephalopathy in 4 cases, septic encephalopathy in 3 cases, and septic meningitis in 3 cases. Mechanical ventilation was required in 53 cases (66.3%), and the median duration of mechanical ventilation was 96 (54-239) h. Seventy-seven cases were examined by cranial magnetic resonance imaging, of which 61 cases (79.2%) were abnormal. The median duration of convulsive seizures was 19 (2~46) h, the median length of stay in the PICU was 8.5 (5-17.8) d, and there were 19 deaths (hospitalization morbidity and mortality rate of 23.8%). 61 patients survived to be discharged with a good recovery of neurological function (mRS < 3) in 23 cases (37.7%). Comparing the survival and death groups, there was a significant difference in the proportion of admission GCS scores and imaging abnormalities (both P<0.05). Conclusion The etiology of new-onset RSE in this group of children was mainly severe encephalitis, and the GCS scores and cranial imaging results on admission were of reference value for prognostic judgment.