摘要
目的:分析1例预后良好的急性坏死性脑病(ANE)患儿的临床特点,提高临床医生对该病的认识。方法:收集1例ANE患者的临床资料,结合相关文献,回顾性分析ANE患者的临床特征和诊治经验。结果:3岁男孩,既往体健,因肺炎入院,抗感染治疗后好转。第4天开始患儿再次出现反复高热,第10天突发癫痫样抽搐,随即昏迷。头部核磁共振(MRI)显示双侧丘脑和脑干等部位出现多发对称性坏死,临床诊断为ANE和全身炎症反应综合征(SIRS),并给予抗感染、甲泼尼龙冲击、人免疫球蛋白静点及对症支持治疗。3个月内定期行门诊随访,现患儿发育商及社会生活能力评估为边缘水平,有望达到生活自理,仍在康复治疗中。结论:ANE患者早期临床表现缺乏特异性,常伴高热,疾病进展快,病情危重。头部MRI是诊断ANE的主要依据。早期、及时的免疫调节治疗有效,可改善ANE患儿预后。
Objective:To analyze the clinical characteristics of one child with acute necrotizing encephalopathy(ANE)with goodPrognosis,and to improve the clinicians’understandings of the disease.Methods:The clinical materials of aPatient with ANE were collected,and the clinical features and diagnosis and treatment experience were retrospectively analyzed combined with the relative literatures.Results:ThePreviously healthy boy,aged 3 years old,was admitted to the hospital due toPneumonia and his condition was improved after anti-infection treatment.On the 4th day,thePatient had a high fever again;on the 10th day,thePatient suddenly became generalized convulsive seizures,then thePatient was in coma.The brain magnetic resonance imaging(MRI)revealed the multiple and symmetric necrosis in the bilateral thalami,brainstem and other areas.ThePatient was diagnosed as ANE and systemic inflammatory response syndrome(SIRS)and was treated with anti-infection,intravenous high-dose methylprednisolone,intravenous immunoglobulin and symptomatic treatment.After regular outpatient follow-up of 3 months,both Gesell Development and Development of Social Skills assessment of thePatient improved to the marginal defect level;thePatient was expected to achieve self-care and was still in rehabilitation treatment.Conclusion:The early clinical manifestations of ANEPatients are lack of specificity,often accompaning high fever,rapid diseaseProgression and critical condition;the diagnosis of ANE is mainly based on brain MRI findings;early and timely immunomodulatory therapy is effective and can improve the thePrognosis of thePatients with ANE.
作者
杨宝霞
黎萍
王聪
张鹏
郑百红
许忠
YANG Baoxia;LI Ping;WANG Cong;ZHANG Peng;ZHENG Baihong;XU Zhong(Department of Pediatrics,Second Hospital,Jilin University,Changchun 130041,China;Department of DevelopmentalPediatrics,Second Hospital,Jilin University,Changchun 130041,China;Department of Radiology,Second Hospital,Jilin University,Changchun 130041,China)
出处
《吉林大学学报（医学版）》
CAS
CSCD
北大核心
2019年第6期1449-1453,共5页
Journal of Jilin University：Medicine Edition
基金
吉林省科技厅自然科学基金资助课题(20160101046JC)
关键词
急性坏死性脑病
双侧丘脑坏死
免疫调节治疗
病例报告
acute necrotizing encephalopathy
bilateral thalamus necrosis
immunomodulatory therapy
case report
