CAJ | 학술논문

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50～75 ml/ (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6～24 h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.
목적 총결인동중증감호병방(PICU)수치환인중서혈세포종합정(hemophagocytic syndrome,HPS)적림상특정,탐토상방련속성혈액정화(continuous blood purification,CBP)재보조구치인동중증HPS환인구치중적작용.방법 2009년1월지2012년12월,상해교통대학부속인동의원PICU공수치HPS환인19례.기중12례재약물치료기출상진행CBP치료,모식위련속성정-정맥혈액려과투석(continuous vein-vein hemodialysis/filtration,CVVHDF)혹고용량혈액려과(high volume hemofiltration,HVHF),치환액제량위50～75 ml/ (kg·h).관찰치료전후주요기관공능、혈액전해질、혈액철단백등변화.결과 19례환인균급성기병,신속발전위다기관공능장애종합정(MODS).주요림상특정포괄불규칙발열혹고열、간비종대급명현간공능손해、신경계통공능장애화미만성혈관내응혈(DIC).사망8례,총병사솔42.1％,기중CBP치료조3례사망,미CBP치료조5례사망,사망균발생우3세이하환인.CBP치료6～24 h후,체온회복정상범위,혈액생화지표개선,혈청철단백、혈청곡병전안매(ALT)화유산탈경매(LDH)현저강저,혈기항(sCr)、뇨소담(BUN)등호전,기중4례합병호흡군박종합정(ARDS)환인폐부병변개선,호흡궤삼수하조.결론 HPS시인동MODS원인지일,3세이하예후차.CBP보조치료인동위중HPS합병MODS,구유단시간내강저체온,규정전해질문란,은정순배공능,개선폐、간、뇌공능등작용,가능시중증HPS유효적치료조시.
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/hemofiltration (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.