Acute Necrotizing Encephalopathy

急性坏死性脑病
  • 文章类型: Journal Article
    急性坏死性脑病(ANE)是一种破坏性的神经系统疾病,可在各种全身性感染后出现,包括流感和SARS-Cov-2。基于多病例数据的儿科患者中COVID-19相关ANE的临床特征尚未得到描述,仍然不清楚。我们回顾了12例儿科患者,以更好地描述患有COVID-19的ANE的临床特征。
    我们回顾性收集并总结了COVID-19患儿ANE的临床特征。收集了12名儿童的临床数据,包括他们的一般地位,临床症状,实验室测试,和神经影像学特征。
    在受试者中,5岁以上10人,占83.33%。受影响的人中有很大一部分是女性(66.67%)。主要表现包括发热(100%),意识受损(100%),和抽搐(75%)。我们确定,在这项研究中,白细胞介素(IL)-6和IL-10以及肿瘤坏死因子-α和干扰素-γ的增加不能预测COVID-19儿童的严重ANE和死亡率。所有患儿均表现为神经影像学异常,有多个对称分布的病变,涉及丘脑,基底神经节,小脑,和大脑半球。12名儿童中有8人死亡,导致死亡率为66.67%,这些孩子中有75%是女性。重要的是,我们发现,急性发作惊厥或意识障碍后及时服用甘露醇可能会降低ANE患儿COVID-19引起的高死亡率.
    COVID-19与儿童ANE相关的特征是症状突然发作,快速的疾病进展,和高死亡率。
    UNASSIGNED: Acute necrotizing encephalopathy (ANE) is a devastating neurologic condition that can arise following a variety of systemic infections, including influenza and SARS-Cov-2. The clinical features of COVID-19-associated ANE in pediatric patients based on multi-case data have not yet been described and remain obscure. We reviewed 12 pediatric patients to better describe the clinical features of ANE with COVID-19.
    UNASSIGNED: We retrospectively collected and summarized the clinical features of ANE in children with COVID-19. Clinical data were collected from 12 children, including their general status, clinical symptoms, laboratory tests, and neuroimaging features.
    UNASSIGNED: Among the subjects, 10 were over 5 years old and they accounted for 83.33%. A large percentage of those affected (66.67%) were females. The major manifestations included fever (100%), impaired consciousness (100%), and convulsions (75%). We determined that increased interleukin (IL)-6 and IL-10, and tumor necrosis factor-α and interferon gamma were not predictive of severe ANE and mortality in children with COVID-19 in this study. All children presented with abnormal neuroimaging with multiple and symmetrically distributed lesions, involving the thalamus, basal ganglia, cerebellum, and brain hemispheres. Eight of the 12 children died, resulting in a mortality rate of 66.67%, and 75% of these children were females. Importantly, we found the timely administration of mannitol after an acute onset of convulsions or disturbance of consciousness may be decreased the high mortality induced by ANE children with COVID-19.
    UNASSIGNED: COVID-19 associated with ANE in children is characterized by sudden symptom onset, rapid disease progression, and high mortality.
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  • 文章类型: Journal Article
    BACKGROUND: Acute necrotizing encephalopathy (ANE) of childhood is a rare but critical disease with global distribution. Few studies have focused on investigating the relationship between O blood type and the prognosis of ANE.
    METHODS: We analyzed retrospectively the data of patients with ANE admitted to the Beijing Children\'s Hospital from March 2012 to February 2019. Baseline data, clinical characteristics, examination, treatment, and prognosis of O blood group were compared with those of the non-O blood group. Cox regression was used to observe the independent prognostic factors in ANE.
    RESULTS: Thirty-one ANE patients were recruited, and 8 (25.8%) of these patients were in the O blood group. The rest (n = 23; 74.2%) were in the non-O blood group. No significant differences were found in the demographic characteristics, clinical features, examinations, and treatments between the two groups (p > 0.05). At 28 days after discharge, the overall survival rate of the O blood group was significantly higher than that of the non-O blood group (χ2 = 5.630, p = 0.018). At 1 year after discharge, the survival quality of the O blood group was higher than that of the non-O blood group (p = 0.006). After adjusting for the confounding factors, Cox regression analysis showed that O blood type might be a protective factor in ANE [hazard ratio = 0.283, 95% CI = 0.081-0.988; p = 0.048].
    CONCLUSIONS: O blood type may be a protective factor for patients with ANE.
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  • 文章类型: Journal Article
    Acute necrotizing encephalopathy (ANE) is a rare disease in childhood. We reviewed the 10-year data from a local pediatric department, reported the clinical characteristics, laboratory tests, neuroimaging findings, and outcome of the acute necrotizing encephalopathy cases and identified the potential factors affecting the outcome. Eight episodes of acute necrotizing encephalopathy among 7 patients were recorded, in which all of them had an initial presentation of fever and seizure. We identified that acute necrotizing encephalopathy patients with a severe score of Glasgow Coma Scale (GCS) on presentation, brainstem involvement in magnetic resonance imaging (MRI) of the brain, and higher MR imaging scores were associated with worse outcome. Association of outcome with acute necrotizing encephalopathy severity score, platelet count, and serum alanine aminotransferase level did not reach a statistically significant level. These results highlight the importance of combined clinical, laboratory, and neuroimaging findings in determining the prognostic outcome of acute necrotizing encephalopathy patients.
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