| 刘满菊,李园园,王小稳,等.急性坏死性脑病儿童 25例临床特点及预后分析[J].安徽医药,2026,30(5):1033-1037. |
| 急性坏死性脑病儿童 25例临床特点及预后分析 |
| Clinical characteristics and prognosis in 25 children with acute necrotizing encephalopathy |
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| DOI：10.3969/j.issn.1009-6469.2026.05.037 |
| 中文关键词: 急性坏死性脑病 儿童 临床特点 格拉斯哥昏迷量表评分 意识障碍 |
| 英文关键词: Acute necrotizing encephalopathy Children Clinical characteristics Glasgow coma scale Altered consciousness |
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| 中文摘要: |
| 目的探讨急性坏死性脑病( ANE)儿童的临床特点及预后，提高临床工作者对该疾病的认识。方法回顾性分析 2022年 1月至 2023年 12月在郑州大学附属儿童医院确诊为 ANE的病儿的临床资料。结果 25例 ANE病儿中，男 18例，女 7例，其中 24例( 96%)出现高热及超高热。所有病儿格拉斯哥昏迷量表( GCS)评分 ≤8分，出现意识障碍或抽搐时的中位发热时间为 2d。22例( 88%)出现抽搐，其中 10例表现为惊厥持续状态。 22例( 88%)出现其他系统的伴随症状，以咳嗽、腹痛、呕吐、腹泻多见。实验室检查中，细胞因子升高 23例，以白细胞介素( IL)-6升高明显。白细胞升高 13例，血小板降低 3例， C反应蛋白( CRP)升高 19例，降钙素原( PCT)升高 23例，乳酸脱氢酶( LDH)升高 24例，肝功能异常 18例，以谷草转苷酶升高更为明显。 11例病儿有凝血功能异常。 13例病儿行腰穿检查，均有不同程度的蛋白量升高。 3例病儿因病情严重未行影像学检查，余 22例病儿影像学检查均显示对称丘脑受累，多累及基底节和脑干及侧脑室白质区域。 25例病儿中，支原体并发腺病毒感染 1例，人疱疹病毒感染 2例，流行性感冒 10例，新型冠状病毒感染 11例。病儿均采用了机械通气， 19例应用了丙种球蛋白， 21例应用了激素冲击治疗， 1例病儿行血浆置换 +血液净化， 1例病儿血浆置换 +连续肾脏替代疗法( CRRT)治疗，单纯血液净化 2例，行体外人工膜肺技术( ECMO)治疗有 1例。病儿均出现不同程度的器官功能障碍， 5(20%)例病儿存活。根据临床结局，分为生存组( 5例)与死亡组( 20例)两组间比较发现，死亡组 GCS评分较低、 IL-6水平、尿素、肌酐、乳酸较高以及凝血异常比例均高于存活组。结论 ANE可能与，病原体感染后免疫功能紊乱致细胞因子风暴有关。其临床表现缺乏特异性，往往以发热、意识障碍、抽搐为主。该病进展迅速，易合并多脏器功能损害，病死率高。目前尚无特效方法，大剂量激素冲击、丙种球蛋白应用、血浆置换、血液净化、 ECMO及对症治疗仍是目前应用较多的治疗方法，但疗效甚微。 GCS评分越低、 IL-6水平以及尿素、肌酐、乳酸以及凝血异常比例越高，死亡的风险就越大。 |
| 英文摘要: |
| Objective To summarized the clinical characteristics, treatment, and prognosis of children with acute necrotizing enceph.alopathy (ANE) through a retrospective analysis to increase the understanding of the clinical worker.Methods Clinical data of chil. dren diagnosed with ANE at Children's Hospital Affiliated to Zhengzhou University from January 2022 to December 2023 were select.ed.Results There were 25 cases, 18 males and 7 females. 24 cases(96%) experienced high or very high fever, and all children had aGlasgow coma scale (GCS) score of ≤8. The median time of altered consciousness or convulsions was 2 days. Seizures were observed in22 cases(88%) of patients, including 10 children with persistent state of convulsion. 22 cases(88%) of the children had other systemicsymptoms, including cough, abdominal pain, vomiting and diarrhea. In the laboratory tests, all 23 sick children had elevated cytokinesto varying degrees, with interleukin (IL)-6 showing a significant increase. There were 13 cases with elevated white blood cells, 3 caseswith decreased platelets, and 19 cases with elevated C-reactive protein (CRP). Procalcitonin (PCT) 23 cases and lactic dehydrogenase(LDH) 24 cases of almost all children were elevated to varying degrees. 18 children exhibited liver function abnormalities, with a morepronounced elevation in aspartate aminotransferase. Coagulation dysfunction was present in 11 children. Lumbar puncture was per.formed in 13 children, all showing elevated protein levels to varying extents. Three patients did not receive imaging examination due tosevere disease. Neuroimaging examination of the remaining 22 children showed symmetric thalamic involvement, usually involving thebasal ganglia, brainstem and lateral ventricle white matter area. Among 25 patients, 1 was infected with mycoplasma complicated withadenovirus, 2 with human herpesvirus, 10 with influenza, and 11 with COVID-19. Almost all of the children were treated with mechani. cal ventilation, 19 with propyl sphere, 21 with hormone shock therapy, 1 with plasma exchange and blood purification, 1 with plasmaexchange and continuous renal replacement therapy (CRRT), 2 with pure blood purification, and 1 with extracorporeal membrane oxy.genation (ECMO). Almost all patients had some degree of organ dysfunction, and 5 cases(20%) survived. According to the clinical out.come, the patients were divided into survival group (5 cases) and death group (20 cases). Compared with the death group , the survivalgroup had higher GCS score and lower IL-6 levels, urea, creatinine, lactic acid, and abnormal proportion of coagulation.Conclusions ANE may be associated with cytokine storm of immune dysfunction after infection. Its clinical manifestations are lack of specificity,usually fever, disturbance of consciousness and convulsions. The disease progresses rapidly. It is prone to multiple organ function dam.age, and has a high mortality rate. There is no specific method at present. High-dose hormone shock, propyl bulb application, plasmaexchange, blood purification,ECMO and symptomatic treatment are still the most widely used treatment methods.However,the efficacyis little and most of the children have poor prognosis. GCS scores, IL-6 levels,abnormalities in urea,creatinine,lactate,and coagulation may be important risk factors for death. |
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