Abstract: Objective    To analyze the clinical characteristics and clinical outcomes of the children with FIRES at acute stage， aiming to find the risk factors related to prognosis and provide reference for clinical decision of FIRES. Methods This is a retrospective single-center cohort study， in which the clinical data of all FIRES children hospitalized in Children’s Hospital of Chongqing Medical University from June 2015 to June 2021 were analyzed. These children were followed up and their prognoses were evaluated by modified Rankin score， and then they were divided into groups for summary and analysis.  Results    A total of 54 children were included in this study， including 30 males and 24 females.The median onset age was 5.9 years. There were 11 toddlers（20.4%），18 preschool children（33.3%），and 25 school-age children（46.3%）.During the follow-up，3 children died and 2 were lost to the follow-up.The median follow-up period was 44 months for the other children. On discharge，31 cases（57.4%）were classified as good prognosis while the other 23（42.6%）as poor prognosis. There were 44 cases（84.6%） with good prognosis and 8 cases（15.4%）with poor prognosis at 1 year after discharge.With the extension of survival time，the mRS score was apparently improved， and the prognosis tended to improve.Overall，44 children（89.8%）developed secondary epilepsy at the chronic stage，of which 61.4% （27 cases ） were drug-resistant epilepsy. 41 children（83.7%，41/49）had neurological sequelae， inclu-ding motor impairment（n=7），mental retardation（n=30）and neurobehav-ioral abnormalities（n=10）.The types of anesthetics used in combination（OR=11.67，95% CI 2.09-65.18，P=0.005）in acute stage were independent risk factors for the poor prognosis on discharge. Conclusion    Most FIRES children have good prognosis after treatment. During one-year follow-up period，most of the children present with epilepsy at chronic stage， and may have different degrees of neurological sequelae， but their neurological function gradually improves over time.The types of anesthetics used in combination in the acute phase are the independent risk factors of poor prognosis of FIRES on discharge.

Key words: child, fever, epilepsy syndrome, prognostic factors, status epilepticus

摘要： 目的    分析儿童发热感染相关性癫痫综合征（febrile infection-related epilepsy syndrome，FIRES）急性期临床特点及临床转归，寻找与预后相关的危险因素，为FIRES的临床决策提供参考。方法    该研究为单中心回顾性队列研究，分析2015年6月至2021 年6月在重庆医科大学附属儿童医院住院的所有FIRES患儿临床资料，并对纳入研究者进行随访，以改良Rankin量表（modified Rankin score，mRS）评估预后及分组，归纳分析。结果    最终纳入54例FIRES患儿，包括男30例、女24例。中位发病年龄5.9岁，婴幼儿11例（20.4%）、学龄前期18例（33.3%）、学龄期25例（46.3%）；随访期间，3例死亡、2例失访，中位随访时间为44个月。出院时预后良好31例（57.4%）、预后不良23例（42.6%），出院1年时预后良好44例（84.6%）、预后不良8例（15.4%），随着生存时间的延长，mRS评分有明显改善，预后有改善趋势。44例（89.8%）慢性期表现为癫痫，其中61.4%（27例）为药物难治性癫痫。41例（83.7%，41/49）遗留有不同程度神经系统后遗症，包括运动障碍7例、智力障碍30例和心理行为异常10例。急性期联合使用麻醉药物种类（OR=11.67，95% CI 2.09～65.18，P=0.005）是出院时预后不良的独立危险因素。结论    FIRES经过治疗绝大多数患儿出院预后良好，随访1年其慢性期表现主要为癫痫，可有不同程度的神经系统后遗症，但患儿神经功能随时间延长逐渐好转。急性期联合使用麻醉药物种类是FIRES出院时不良预后的独立危险因素。

关键词: 儿童, 发热, 癫痫综合征, 预后因素, 癫痫持续状态