摘要
目的探讨儿童噬血细胞综合征(hemophagocytic syndrome,HPS)的临床特征与预后的相关因素。方法回顾性分析42例儿童HPS患者的临床资料。根据确诊后28 d内患儿存活情况,分为存活组(24例)和病死组(18例),分别使用单因素分析、多因素logistic回归和受试者工作特征(ROC)曲线进行分析。结果42例儿童HPS患者发病性别比例为男:女=1∶1.21,存活率为57.14%。病因方面:原发性噬血19例,病毒感染16例,疱疹病毒(EBV)感染15例。相较于存活组,病死组患儿瘀斑发生率高,脾脏肿大发生率低,差异有统计学意义(P<0.05)。与存活组相比,病死组淋巴细胞(LYM)计数、红细胞(RBC)计数及血小板(PLT)计数降低,同时凝血酶原时间(PT)延长,血清乳酸脱氢酶(LDH)水平增高,差异均有统计学意义(P<0.05)。多因素logistic回归显示,RBC降低和LDH升高是儿童HPS患者28 d死亡的危险因素(优势比分别为0.277,1.001,P<0.05)。ROC曲线分析显示,当RBC最优截断值为3.595×10^(12) L^(-1)时,敏感度为83.3%,特异度为54.2%;当LDH最优截断值为978.5 U·L^(-1)时,敏感度为61.1%,特异度为75.0%。结论儿童HPS确诊时RBC计数<3.595×10^(12) L^(-1)或LDH>978.5 U·L^(-1),提示28 d内病死率高。
Objective To investigate the clinical features and prognostic factors of hemophagocytic syndrome(HPS)in children.Methods Clinical data of 42 pediatric HPS patients were retrospectively analyzed.According to the survival of patients within 28 days after diagnosis,the children were divided into survival group(24 cases)and death group(18 cases).Analyses were performed using univariate analysis,multivariate logistic regression and receiver operating characteristic(ROC)curves,respectively.Results The sex ratio of 42 children with HPS was male:female=1∶1.21 and the survival rate was 57.14%.In terms of etiology,19 cases were primary haemophagy,16 cases were infected with virus,and 15 cases were infected with Epstein-Barr virus.Compared with the survival group,the incidence of ecchymosis was higher in the death group,and the incidence of splenomegaly was lower,with statistical significance(P<0.05).Compared with the survival group,lymphocyte count,red blood cell count and platelet count in the death group were decreased,while PT and LDH were significantly increased,with statistical significance(P<0.05).Multivariate logistic regression showed that decreased red blood cell count(RBC)and elevated LDH were risk factors for 28d death in children with HPS(odds ratios were 0.277,1.001,P<0.05).ROC curve analysis showed that when the optimal cut-off value of red blood cell count(RBC)was 3.595×10^(12) L^(-1),the sensitivity and specificity were 83.3%and 54.2%respectively,and when the optimal cut-off value of LDH was 978.5 U·L^(-1),the sensitivity and specificity were 61.1%and 75%respectively.Conclusion RBC<3.595×10^(12) L^(-1) or LDH>978.5 U·L^(-1) at diagnosis of HPS in children may suggest a high mortality within 28 days.
作者
魏琛
程志伟
牛铭
陶琳琳
郝长付
WEI Chen;CHENG Zhiwei;NIU Ming;TAO Linlin;HAO Changfu(Department of Pediatrics,The Third Affiliated Hospital of Zhengzhou University,Zhengzhou Henan 450052,China;Department of Medical Record Management,The Third Affiliated Hospital of Zhengzhou University,Zhengzhou Henan 450052,China;Department of Child and Adolecence,Public Health College of Zhengzhou University,Zhengzhou Henan 450001,China)
出处
《河南医学高等专科学校学报》
2022年第2期129-134,共6页
Journal of Henan Medical College
基金
国家自然科学基金(82173491)。
关键词
噬血细胞综合征
儿童
生存分析
临床特征
预后
hemophagocytic syndrome
children
survival analysis
clinical characteristics
prognosis
