儿童急性坏死性脑病治疗研究进展

Research Progress on the Treatment of Acute Necrotizing Encephalopathy in Children

儿童急性坏死性脑病(acute necrotizing encephalopathy of children, ANEC)多见于病毒感染诱发,临床以意识水平迅速发生障碍、惊厥发作为主要表现,目前发病机制暂不清楚,目前普遍认为与细胞因子风暴有关,基因易感性也在发病中起到一定作用。急性坏死性脑病早期缺乏特异性临床表现,严重威胁儿童生命,目前暂无特殊治疗方式,除对症支持治疗外,控制细胞因子风暴同样重要,主要包括丙种球蛋白、糖皮质激素、IL-6阻滞剂(托珠单抗)等被用于治疗该病,早期及时控制细胞因子风暴有助于改善患儿预后,但仍遗留较高的致残率,需进一步研究对于急性坏死性脑病的治疗方式,从而提高生存率和降低致残率。

Acute necrotizing encephalopathy in children (ANEC) is often induced by viral infection. The main clinical manifestations are rapid disturbance of consciousness and convulsion. At present, the pathogenesis is not clear. It is generally believed that it is related to cytokine storm, and genetic susceptibility also plays a certain role in the pathogenesis. Acute necrotizing encephalopathy is the lack of specific clinical manifestations in the early stage, which seriously threatens children’s life. At present, there is no special treatment. In addition to symptomatic support treatment, it is also im-portant to control cytokine storm, mainly including gamma globulin, glucocorticoid, IL-6 blocker (trozumab). Early and timely control of cytokine storm is helpful to improve the prognosis of chil-dren, but it still leaves a high disability rate. It is necessary to further study the treatment of acute necrotizing encephalopathy so as to improve the survival rate and reduce the rate of disability.