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。

abstractsObjective: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 risk factors for the death of HLH. Conclusions:HLH has a very high mortality and requires critical care in PICU, low PCIS, anemia, prolonged APTT and AKI are independent risk factors for the death of HLH.