摘要
目的总结儿童重症监护病房（PICU）收治患儿中噬血细胞综合征（hemophagocytic syndrome，HPS）的临床特征，探讨床旁连续性血液净化（continuous blood purification，CBP）在辅助救治儿童重症HPS患儿救治中的作用。方法2009年1月至2012年12月，上海交通大学附属儿童医院PICU共收治HPS患儿19例。其中12例在药物治疗基础上进行CBP治疗，模式为连续性静-静脉血液滤过透析（continuous vein—vein hemodialysis／filtration，CVVHDF）或高容量血液滤过（high volume hemofiltration，HVHF），置换液剂量为50—75ml／（kg·h）。观察治疗前后主要器官功能、血液电解质、血液铁蛋白等变化。结果19例患儿均急性起病，迅速发展为多器官功能障碍综合征（MODS）。主要临床特征包括不规则发热或高热、肝脾肿大及明显肝功能损害、神经系统功能障碍和弥漫性血管内凝血（DIC）。死亡8例，总病死率42．1％，其中CBP治疗组3例死亡，未CBP治疗组5例死亡，死亡均发生于3岁以下患儿。CBP治疗6—24h后，体温恢复正常范围，血液生化指标改善，血清铁蛋白、血清谷丙转氨酶（ALT）和乳酸脱氢酶（LDH）显著降低，血肌酐（sCr）、尿素氮（BUN）等好转，其中4例合并呼吸窘迫综合征（ARDS）患儿肺部病变改善，呼吸机参数下调。结论HPS是儿童MODS原因之一，3岁以下预后差。CBP辅助治疗儿童危重HPS合并MODS，具有短时间内降低体温，纠正电解质紊乱，稳定循环功能，改善肺、肝、脑功能等作用，可能是重症HPS有效的治疗措施。
Objective To study critical hemophagocytic syndrome （HPS） or macrophage activation syndrome （MAS） presented with multiple organ dysfunction syndrome （MODS） in pediatric intensive care unit （PICU）, including clinical features and outcomes In order to explore the effect of bedside continuous hemodialysis/hemofihration （CBP） as adjuvant treatment for severe HPS/MAS. Methods A total of 19 children with HPS/MAS were hospitalized met the diagnostic criteria for HPS from January, 2009 to December, 2012. Twelve cases were treated with CBP by continuous venin-venin hemodialysis/hemofiltration （CVVHDF） or high-volume hemofiltration （HVHF） following conventional anti- inflammatory therapy. The replacement liquid dose was 50-75 ml/ （kg ~ h）. The organs function were evaluated and laboratory biomarkers including blood electrolytes, ferritin changes were measured before and after CBP treatment. Results Ninteen cases of HPS were acute onset and developed to MODS rapidiy after admission to PICU. The main clinical features were the irregular fever or high fever, hepatosplenomegaly and significant liver damage, nervous system dysfunction and disseminated intravascular coagulation （DIC）. Eight cases were death and mortality rate was 42. 1%, and all death occurred in those aged less than 3 years old. The mortality rate were 25% （3/12） and 71.4% （5/7） in CBP group and non-CBP group respectively. After CBP for 6-24 hours, the fever returned to normal range and blood electrolytes improved. The serum ferritin, serum alanine aminotransferase （ALT） and lactate dehydrogenase （ LDH ） reduced significantly. Serum creatinine （sCr）, blood urea nitrogen （BUN） level improved. Four cases with acute respiratory distress syndrome （ARDS） improved and the ventilator parameters were downregulated. Conclusions Our findings indicate that HPS/MAS complicated with MODS is life threatening with high mortality rate. CBP therapy can lower the fever within a short time, correct electrolyte imbalance, stable circulatory function, improve the lung, liver, and brain function. It is suggested that CBP may be the potential effective therapy in severe HPS/MAS with MODS in children.
出处
《中华急诊医学杂志》
CAS
CSCD
北大核心
2013年第7期749-754,共6页
Chinese Journal of Emergency Medicine
基金
国家十二五重大科技支撑计划（国科2012BA104801-05）
上海市科委科技攻关项目（12411952404）
