儿童急性坏死性脑病的发病机制研究进展

Advances on the pathogenesis of acute necrotizing encephalopathy in children

急性坏死性脑病(ANE)在儿童中有极高的病死率,但由于其发病机制尚不明确,暂无较好的治疗方案。近年研究显示,细胞因子风暴、遗传易感性和线粒体功能障碍是导致ANE发病的关键环节。目前认为白细胞介素-6和肿瘤坏死因子-α介导的细胞因子风暴是儿童ANE发病的关键因素;基因突变导致的遗传易感性在其中起间接作用,有基因突变的儿童在接触流感病毒后更易患ANE;线粒体功能障碍与细胞因子、基因突变相互影响,共同促进ANE发病,但具体机制尚不清楚。望未来能更深入地了解儿童ANE的发病机制,开发出针对儿童ANE的治疗方案,限制ANE发生进展,降低患儿致残致死率。

Acute necrotizing encephalopathy(ANE)causes a very high mortality rate in children.However,the pathogenesis of ANE is still unclear,so no better treatment is available.Recent studies have shown that cytokine storms,genetic susceptibility,and mitochondrial dysfunction are key factors in the pathogenesis of ANE.It is now acknowledged that cytokine storms mediated by interleukin-6 and tumor necrosis factor-αare key factors in the pathogenesis of ANE in children.Genetic susceptibility due to genetic mutations plays an indirect role,and children with genetic mutations are more likely to develop ANE after exposure to influenza viruses.Mitochondrial dysfunction interacts with cytokines and gene mutations to jointly promote the development of ANE,but the exact mechanisms are unknown.We hope a deeper understanding of the pathogenesis of ANE will be achieved in the future.Therefore,the therapeutic regimens for ANE will be developed to limit the disease progression and to reduce the rate of disability and mortality in children.