Background

Acute necrotizing encephalopathy (ANE) is a fulminant disease with poor prognosis. Cytokine storm is the important phenomenon of ANE that affected the brain and multiple organs. The study aimed to identify whether hyperferritinemia was associated with poor prognosis in patients with ANE.

Methods

All patients with ANE had multiple symmetrical lesions located in the bilateral thalami and other regions such as brainstem tegmentum, cerebral white matter and cerebellum. Neurologic outcome at discharge was evaluated by pediatric neurologists using the system of Pediatric Cerebral Performance Category (PCPC) Scale. All risk factors associated with poor prognosis were further analyzed using receiver operating characteristic curve analysis.

Results

29 patients with ANE were enrolled in the current study. 9 (31%) patients achieved a favorable neurologic outcome and 20 (69%) patients had poor neurologic outcomes.

The group of poor neurologic outcome had significantly higher proportion of shock on admission and brainstem involvement. Based on multivariate logistic regression analysis, ferritin, AST, and ANE-severity score (ANE-SS) were the predictors associated with outcomes. The appropriate cutoff value for predicting neurologic outcomes in patients with ANE was 1823 ng/mL in ferritin, 78 U/L in AST and 4.5 in ANE-SS. Besides, comparison analyses showed that higher level of ferritin and ANE-SS were significantly correlated with brainstem involvement (p<0.05).

Conclusions

Ferritin may potentially be a prognostic factor in patients with ANE. Hyperferritinemia is associated with poor neurologic outcomes in patients with ANE and the ferritin levels more than 1823 ng/ml having about 8-fold increasing risk of poor neurologic outcome.

中文翻译：

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铁蛋白作为急性坏死性脑病儿童神经系统结果的有效预测因子

背景

急性坏死性脑病（ANE）是一种暴发性疾病，预后不良。细胞因子风暴是ANE影响大脑和多个器官的重要现象。该研究旨在确定高铁蛋白血症是否与 ANE 患者的不良预后相关。

方法

所有ANE患者均有多发对称性病变，位于双侧丘脑及脑干被盖、脑白质、小脑等其他区域。出院时的神经系统结果由儿科神经科医生使用儿科大脑表现类别（PCPC）量表系统进行评估。使用受试者工作特征曲线分析进一步分析与不良预后相关的所有危险因素。

结果

29 名 ANE 患者参加了本研究。9 名（31%）名患者取得了良好的神经系统结果，20 名（69%）名患者的神经系统结果较差。

神经系统结果较差的组入院时休克和脑干受累的比例显着较高。根据多变量逻辑回归分析，铁蛋白、AST 和 ANE 严重程度评分 (ANE-SS) 是与结果相关的预测因子。预测 ANE 患者神经系统结局的适当截止值为铁蛋白 1823 ng/mL、AST 78 U/L 和 ANE-SS 4.5。此外，比较分析显示，较高水平的铁蛋白和ANE-SS与脑干受累显着相关（p<0.05）。

结论

铁蛋白可能是 ANE 患者的一个潜在预后因素。高铁蛋白血症与 ANE 患者神经系统预后不良相关，铁蛋白水平超过 1823 ng/ml 时，神经系统预后不良的风险增加约 8 倍。