儿童噬血细胞综合征中枢神经系统受累及其与临床相关性分析  

Central nervous system involvement in children with hemophagocytic syndrome and its clinical correlation

作　　者：李纯莎 黄娇娇 罗南都 何志旭 黄佩 陈艳 Li Chunsha;Huang Jiaojiao;Luo Nandu;He Zhixu;Huang Pei;Chen Yan(Department of Pediatrics,the First Affiliated Hospital of Zunyi Medical University,Guizhou Children’s Hospital,Zunyi Guizhou 563099,China;Collaborative Innovation Center for Tissue Injury Repair and Regenerative,Zunyi Medical University,Zunyi Guizhou 563099,China)

机构地区：[1]遵义医科大学附属医院,贵州省儿童医院小儿内科,贵州遵义563099 [2]遵义医科大学组织损伤修复与再生医学省部共建协同创新中心,贵州遵义563099

出　　处：《遵义医科大学学报》2023年第5期498-504,共7页Journal of Zunyi Medical University

基　　金：贵州省科技计划项目[NO:黔科合平台人才-CXTD(2021)010];遵义市科技计划项目[NO:遵市科合HZ字(2021)10];贵州省研究生科研基金资助项目[NO:黔教合YJSKYJJ(2021)178]。

摘　　要：目的分析儿童噬血细胞综合征(HLH)中发生中枢神经系统受累情况,并分析其与预后、临床特征和实验室指标的相关性。方法收集2015年1月—2022年4月于我院诊治的共119例HLH患儿的临床资料,将患儿按照是否有中枢神经系统受累情况分为中枢神经系统受累组、非中枢神经系统受累组,并分析其与临床特征及实验室相关指标的相关性。结果中枢神经系统受累组30例(25.21%),其中死亡23例(76.67%),非中枢神经系统受累组89例(74.79%),其中死亡13例(14.61%),中枢神经系统受累组死亡率明显高于非中枢神经系统受累组(P<0.01),且生存时间更短(P<0.01)。中枢神经系统受累组肾脏受累、呼吸系统受累、脾大、DIC、合并肺出血、合并消化道出血发生率更高(P<0.05),淋巴结肿大发生率更低(P<0.05)。经Logistics危险因素回归分析,结果显示呼吸系统受累、心血管系统受累、合并消化道出血、肌酸激酶是影响患儿中枢神经系统受累的危险因素(P<0.05)。Spearman相关性分析显示:肾脏受累、呼吸系统受累、心血管系统受累、DIC、脾大、合并肺出血、合并消化道出血与中枢神经系统受累呈正相关(P<0.05);淋巴结肿大与中枢神经系统受累呈负相关(P<0.05)。两组间中性粒细胞、AST、尿素、尿酸、肌酸激酶、CK-MB、凝血酶原时间、活化部分凝血活酶时间、红细胞、白蛋白水平具有显著差异,Spearman相关性分析显示:中性粒细胞、AST、尿素、尿酸、肌酸激酶、CK-MB、凝血酶原时间、活化部分凝血酶时间与中枢神经系统受累呈正相关(P<0.05);红细胞、白蛋白与有中枢神经受累呈负相关(P<0.05)。结论中枢神经系统受累组HLH患儿死亡率更高,与部分临床特征及实验室相关指标具有相关性,因此发现其相关指标的变化后需警惕中枢神经系统受累,提前预防。Objective To analyze the incidence of central nervous system involvement in children with Hemophagocytic syndrome(HLH)and its correlation with clinical features and laboratory parameters.Methods The clinical data of 119 children with HLH treated in our hospital from January 2015 to April 2022 were collected,and the children were divided into the central nervous system involvement group and the non-central nervous system involvement group according to whether they had central nerve involvement,and the correlation between the clinical characteristics and laboratory related indicators was analyzed.Results There were 30 cases(25.21%)in the central nervous system involved group,of which 23 cases(76.67%)died,and 89 cases(74.79%)in the non-central nervous system involved group,of which 13 cases(14.61%)died.The mortality rate of the central nervous system involvement group was significantly higher than that of the non-central nerve system involvement group(P<0.01),and the survival rate was lower(P<0.01).The incidence of renal involvement,respiratory system involvement,splenomegaly,DIC,pulmonary hemorrhage and gastrointestinal hemorrhage was higher in central nervous system involvement group(P<0.05),and the incidence of lymphadenectasis was lower(P<0.05).Risk factor analysis:Logistic regression analysis showed that respiratory system involvement,cardiovascular system involvement,gastrointestinal bleeding and creatine kinase were risk factors for central nervous system involvement(P<0.05).The spearman correlation coefficient analysis showed that renal involvement,respiratory involvement,cardiovascular involvement,DIC,splenomegaly,pulmonary hemorrhage and gastrointestinal hemorrhage were positively correlated with central nervous system involvement(P<0.05).There was a negative correlation between lymphadenectasis and central nervous system involvement(P<0.05).There were significant differences in neutrophils,AST,urea,uric acid,creatine kinase,CK-MB,prothrombin time,activated partial thromboplastin time,red blood cells and a

关 键 词：噬血细胞综合征 中枢神经系统受累 相关性分析 儿童 

分 类 号：R748[医药卫生—神经病学与精神病学]