儿童重症监护病房中噬血细胞综合征患儿临床特征及预后因素分析  

Analysis of clinical characteristics and prognostic factors of hemophagocytic lymphohistiocytosis in pediatric intensive care unit

作　　者：李婷 张晨美[1] 叶盛[1] Li Ting;Zhang Chenmei;Ye Sheng(Department of Pediatric Intensive Care Unit,the Children′s Hospital,Zhejiang University School of Medicine,National Clinical Research Center for Child Health,Hangzhou 310052,China)

机构地区：[1]浙江大学医学院附属儿童医院儿童重症监护病房,国家儿童健康与疾病临床医学研究中心,杭州310052

出　　处：《中华实用儿科临床杂志》2022年第9期676-681,共6页Chinese Journal of Applied Clinical Pediatrics

摘　　要：目的探讨儿童重症监护病房(PICU)收治的噬血细胞综合征(HLH)患儿的临床特点及死亡相关危险因素。方法回顾性分析2014年1月至2020年12月浙江大学医学院附属儿童医院PICU收治的68例HLH患儿资料。根据入PICU 24 h内最低小儿危重病例评分(PCIS)分值, 分为非危重组、危重组及极危重组, 按照临床预后分为死亡组和存活组。比较各组患儿临床指标差异, 并采用多因素Logistic回归分析死亡相关危险因素。结果 68例HLH患儿收住PICU, 中位年龄26个月, 男29例, 女39例, 总病死率45.59%(31/68例)。EB病毒感染为主要病因。非危重组、危重组和极危重组病死率分别为21.05%(8/38例)、57.14%(8/14例)、93.75%(15/16例), 3组病死率比较差异有统计学意义(P<0.05)。存活组与死亡组比较, PCIS、儿童器官功能障碍评分2(PELOD-2)、发热持续时间、6 h内机械通气、血管活性药物应用、消化道出血、肺出血、弥散性血管内凝血(DIC)、肝胆系统功能障碍(HBD)、急性肾损伤(AKI)、酸中毒[pH、碱剩余(BE)]、血红蛋白(Hb)水平、凝血活酶时间(PT)、活化部分凝血活酶时间(APTT)、血清肌酐(Scr)、白细胞介素6(IL-6)的差异均有统计学意义(均P<0.05)。多因素Logistic回归分析显示下列因素与死亡相关:PCIS评分(OR=0.800, 95%CI:0.707~0.905, P<0.001), Hb水平(OR=0.929, 95%CI:0.871~0.991, P=0.027), APTT(OR=0.954, 95%CI:0.910~0.990, P=0.047), AKI(OR=29.064, 95%CI:3.072~274.957, P=0.003)。结论 HLH是一种高致死风险疾病, 低PCIS评分、贫血、APTT延长及并发AKI为PICU中HLH患儿死亡的危险因素。Objective To explore the clinical features and the mortality risk factors of hemophagocytic lymphohistiocytosis(HLH)in pediatric intensive care unit(PICU).Methods Clinical data of 68 children diagnosed as HLH and treated in PICU,the Children′s Hospital,Zhejiang University School of Medicine between January 2014 and December 2020 were retrospectively analyzed.According to the lowest pediatric critical illness score(PCIS)within 24 h after admission to PICU,they were divided into non-critical group,critical group and extremely critical group.Moreover,they were further divided into survivors and non-survivors based on the prognosis.Clinical characteristics in each group were analyzed and compared.Logistic regression analysis was performed to obtain the estimates of odds ratio(OR)and corresponding 95%confidence interval(CI)of possible predictive factors for death.Results The median age of recruited 68 children with HLH and treated in PICU was 26 months,involving 39 female and 29 male patients.The overall mortality rate was 45.59%(31/68 cases).Epstein-Barr virus(EBV)infection was the major cause of HLH.The mortality of non-critical group,critical group and extremely critical group were 21.05%(8/38 cases),57.14%(8/14 cases)and 93.75%(15/16 cases),the difference was statistically significant(P<0.05).The PCIS,the pediatric logistic organ dysfunction score 2(PELOD-2),duration of fever,mechanical ventilation within 6 h,vasoactive use,gastrointestinal and pulmonary hemorrhage,disseminated intravascular coagulation(DIC),hepatobiliary dysfunction(HBD)and acute kidney injury(AKI),acidosis[pH and base excess(BE)],hemoglobin(Hb),prothrombin time(PT),and activated partial thromboplastin time(APTT),serum creatinine(Scr),interleukin-6(IL-6)were significantly different between survivors and non-survivors(all P<0.05).The Logistic regression analysis showed that PCIS(OR=0.800,95%CI:0.707-0.905,P<0.001),Hb(OR=0.929,95%CI:0.871-0.991,P=0.027),APTT(OR=0.954,95%CI:0.910-0.990,P=0.047),and AKI(OR=29.064,95%CI:3.072-274.957,P=0.003)were ri

关 键 词：噬血细胞综合征 儿童 儿童重症监护病房 

分 类 号：R725.5[医药卫生—儿科]