儿童急性坏死性脑病预后及影响因素分析

Analysis of prognosis and influencing factors of childhood acute necrotizing encephalopathy

目的分析儿童急性坏死性脑病(ANE)的预后及其影响因素。方法回顾性分析2012年3月至2019年2月首都医科大学附属北京儿童医院重症医学科确诊ANE患儿的临床资料。对存活患儿进行定期电话或门诊随访,应用儿童整体表现分类量表评估患儿生存质量。采用t检验或秩和检验进行组间比较,COX风险回归分析预后的影响因素。结果共纳入ANE患儿38例,男女比例为1.24∶1.00,中位发病年龄29.5(10.0~130.0)个月,中位随访时长27(15~96)个月。发病7 d、14 d、2个月总体生存率分别为57.9%、42.1%、34.2%;住院病死率为34.2%(13/38例),出院后1、3、12个月累计病死率均为68.4%(26/38例)。出院1年随访完全康复率为10.5%(4/38例)。单变量分析提示,入院前需要心肺复苏、入院格拉斯哥昏迷评分<5分、合并休克/脑疝/多脏器功能障碍并发症、肌酸激酶同工酶>100 U/L、乳酸脱氢酶>1000 U/L、低白蛋白血症、高血糖、高尿素氮血症、凝血酶原时间延长和国际标准化比值升高均与不良预后相关(β=3.519、6.967、6.803、3.000、6.389、3.471、2.252、1.616、2.377、3.092、2.713、4.510,均P<0.05)。大剂量甲泼尼龙[20~30 mg/(kg·d)]冲击治疗和丙种球蛋白(2 g/kg,分2~5 d静脉滴注)治疗是预后的保护性因素(β=0.625、0.405,均P<0.05)。COX多因素生存分析显示,大剂量甲泼尼龙[20~30 mg/(kg·d)]冲击治疗是ANE患儿预后的独立保护因素[95%CI:0.449(0.213~0.944),P=0.035]。结论早期应用大剂量甲泼尼龙冲击和丙种球蛋白治疗可能有助于改善ANE患儿临床结局。遗留神经系统后遗症的患儿应积极行康复治疗,生存质量可能逐步改善。

Objective To analyze the prognosis and influencing factors of acute necrotizing encephalopathy(ANE)in children.Methods Clinical data of ANE patients admitted to Pediatric Intensive Care Unit,Beijing Children′s Hospital,Capital Medical University from March 2012 to February 2019 were retrospectively analyzed.Survivors were followed up by telephone or outpatient department,and the quality of life was evaluated by pediatric overall performance category scale.The t-test or rank sum test was used for comparison between groups,and the COX risk regression was used to analyze the influencing factors of prognosis.Results A total of 38 patients were enrolled in this study with the male-to-female ratio of 1.24∶1.00,and median age of 29.5(10.0-130.0)months.They were followed up for the median of 27(15-96)months.The overall survival rate at 7 days,14 days and 2 months after disease onset were 57.9%,42.1%,and 34.2%,respectively.The mortality rate at discharge was 34.2%(13/38 cases),and the cumulative mortality rate at the 1st,3rd and 12th months after discharge was all 68.4%(26/38 cases).The complete reco-very rate was 10.5%(4/38 cases)after one-year follow-up.The univariate analysis indicated that cardiopulmonary resuscitation before admission,Glasgow coma score<5 at admission,complication with shock/cerebral hernia/multiple organ dysfunction syndrome,creatine kinase isoenzyme>100 U/L,lactate dehydrogenase>1000 U/L,hypoalbuminemia,hyperglycemia,hyperurea,prolonged prothrombin time and elevated international standardized ratio were risk factors for the prognosis of ANE in children(β=3.519,6.967,6.803,3.000,6.389,3.471,2.252,1.616,2.377,3.092,2.713,and 4.510,respectively,all P<0.05).Meanwhile,high-dose Methylprednisolone[20-30 mg/(kg·d)]and immunoglobulin(2 g/kg,divided into 2-5 days intravenous drip)treatment were protective factors(β=0.625,0.405,respectively,all P<0.05).The COX multivariate analysis showed that high-dose Methylprednisolone treatment[20-30 mg/(kg·d)]was an independent protective factor for the prognosis of children with ANE[95%CI:0.449(0.213-0.944),P=0.035].Conclusions Early application of high-dose Methylprednisolone and immunoglobulin may contribute to the good clinical outcome.Children with neurological sequelae should be actively treated with rehabilitation,and the quality of life may be gradually improved.