摘要
目的 探讨儿童急性坏死性脑病（acute necrotizing encephalopathy,ANE）的临床特点及诊疗体会.方法 选取2014年1月至2018年4月本院儿科临床诊断ANE 10例,回顾性分析其临床表现、实验室检查及影像学资料.结果 共纳入10例患儿.发热和抽搐发作是最常见的临床症状,出现率100％,早期无特异性表现,表现为急性呼吸道感染或者胃肠炎症状;病情加重时可出现精神萎靡不振、嗜睡或烦躁不安、频繁抽搐等.9例患儿出现了不同程度的意识障碍.患儿可并发多脏器功能不全、噬血细胞综合征等.共8例患儿进行了MRI检查,急性期双侧多部位长T1、长T2信号影,弥散加权（DWI）及FLAIR呈高信号影.6例患儿进行了头部CT检查,4例患儿发现脑白质密度弥漫性减低.10例患儿均进行了脑脊液检测,脑脊液中蛋白均有不同程度增高,白细胞则无明显增高.10例患儿中,3例死亡,1例正常出院,1例合并噬血细胞综合征,转入小儿血液内科化疗,5例转入康复科治疗.结论儿童ANE病情凶险,进展迅速,病死率高,存活者多有严重神经系统后遗症,值得临床医生关注和警惕.
Objective To summary the clinical characteristics of acute necrotizing encephalopathy in childhood so as to estimate the prognosis and guide the therapy. Methods We collected and analyzed retro-spectively the data of 10 acute necrotizing encephalopathy cases hospitalized in the pediatric department of our hospital from January 2014 to April 2018. The clinical manifestations,laboratory examinations and imaging features were retrospectively analyzed. Results A total of 10 cases all had fever and convulsions （100%）. There was no specific clinical manifestation in the early stage,always showing the symptoms of acute respira-tory infection or acute gastroenteritis. Mental malaise,lethargy,restlessness and frequent convulsions occured when the conditions aggravated. Nine patients had different degrees of consciousness disorder. It could be complicated by multiple organ dysfunction syndrome,hemophagocytic syndrome and so on. Eight patients un-derwent MRI. In acute phase, homogeneously prolonged T1 and T2 relaxation time of the brain lesions on MRI were found in most patients,and diffusion-weighted imaging （ DWI） and FLAIR showed high signal in-tensity. Six cases underwent head CT examination, hypodensities were seen on 4 cases. Cerebrospinal fluid （CSF） was detected in all patients,and the protein levels in CSF increased to varying degrees,while white blood cells did not increase. Of the 10 patients,3 died,1 was discharged normally,1 had hemophagocytic syn-drome,transferred to pediatric hematology department,and 5 patients were transferred to rehabilitation depart-ment. Conclusion Acute necrotizing encephalopathy in children always make progress rapidly, has high mortality,many survivors have severe neurological sequelae,which is worthy of being attention.
作者
王玉静
裴亮
许巍
王丽杰
杨妮
刘春峰
Wang Yujing;Pei Liang;Xu Wei;Wang Lijie;Yang Ni;Liu Chunfeng(Department of Pediatric Intensive Care Unit,Shengjing Hospital of China Medical University,Shenyang 110004,China)
出处
《中国小儿急救医学》
CAS
2018年第8期590-596,共7页
Chinese Pediatric Emergency Medicine
基金
辽宁省中央引导地方科技发展专项（2018108001）
辽宁省重点研发计划指导计划项目（2017225003）
关键词
急性坏死性脑病
儿童
临床特点
Acute necrotizing encephalopathy
Children
Clinical characteristics
