目的探讨血清中腺苷脱氨酶(ADA)水平在儿童EB病毒感染相关噬血细胞综合征(EBV-HLH)中的诊断及预后判断价值。方法选取2015年1月~2017年12月期间浙江大学医学院附属儿童医院收治的初诊EBV-HLH患者72例和慢性活动性EB病毒(CAEBV)感染患儿8...目的探讨血清中腺苷脱氨酶(ADA)水平在儿童EB病毒感染相关噬血细胞综合征(EBV-HLH)中的诊断及预后判断价值。方法选取2015年1月~2017年12月期间浙江大学医学院附属儿童医院收治的初诊EBV-HLH患者72例和慢性活动性EB病毒(CAEBV)感染患儿80例,HLH患者均符合HLH-2004诊断标准,同期50例健康体检儿童作为健康对照组。应用全自动生化分析仪测定各组患者血清ADA水平,采用受试者工作特征曲线(ROC)分析ADA临床诊断价值,Youden指数法确定最佳工作点。结果EBV-HLH组、CAEBV组及健康对照组儿童血清ADA水平分别为102.4±64.9U/L、42.8±22.1U/L和13.3±3.2U/L。EBV-HLH组ADA水平明显高于CAEBV组,差异有统计学意义(Z=4.709,P=0.000);CAEBV组高于健康对照组,差异有统计学意义(Z=5.008,P=0.000)。以慢性活动性EBV感染组为对照组,利用ROC曲线分析ADA在EBV-HLH中的诊断价值,曲线下面积最高为0.893(P=0.000),以57.5U/L为阈值时,敏感度为74.7%,特异性为89.1%,阳性预测值和阴性预测值分别为90.3%、72.1%。在EBV-HLH组中,ADA与EBV拷贝数呈正相关(r=0.608,P=0.000)。10例EBV-HLH死亡患儿血清ADA水平明显高于存活患儿(154.2±47.3U/L vs 92.5±63.4U/L,P=0.002)。结论血清ADA水平可作为EBV-HLH诊断的常规辅助项目,且在与慢性活动性EBV感染鉴别诊断中具有重要意义。展开更多
目的:总结1例慢性活动性EB病毒感染相关性噬血细胞综合征患儿的临床特点及诊疗经过,提高对该疾病的认识。方法:回顾性分析2022年10月山东大学附属威海市立医院儿科收治的1例慢性活动性EB病毒感染相关性噬血细胞综合征患儿的临床特点及...目的:总结1例慢性活动性EB病毒感染相关性噬血细胞综合征患儿的临床特点及诊疗经过,提高对该疾病的认识。方法:回顾性分析2022年10月山东大学附属威海市立医院儿科收治的1例慢性活动性EB病毒感染相关性噬血细胞综合征患儿的临床特点及诊疗经过,并进行相关文献复习。结果:患儿以发热、反复肝损害、全血细胞减少、凝血功能紊乱为突出表现,病情发展迅速,短时间内死亡,结合相关检查,诊断为慢性活动性EB病毒感染相关性噬血细胞综合征。结论:对于慢性活动性EB病毒感染患儿,在明确诊断后应积极采取有效治疗措施,一旦引起相关噬血细胞综合征,死亡率极高。Objective: To summarize the clinical characteristics and diagnosis and treatment process of a child with chronic active EB virus infection associated hemophagocytic syndrome, and improve the understanding of this disease. Methods: Retrospective analysis of the clinical characteristics and diagnosis and treatment process of a child with chronic active EB virus infection associated hemophagocytic syndrome admitted to the pediatrics department of Weihai Municipal Hospital affiliated with Shandong University in October 2022, and relevant literature review. Results: The child presented with prominent symptoms such as fever, repeated liver damage, decreased whole blood cells, and coagulation dysfunction. The condition developed rapidly and died within a short period of time. Combined with relevant examinations, the diagnosis was chronic active EB virus infection associated hemophagocytic syndrome. Conclusions: For children with chronic active EB virus infection, effective treatment measures should be actively taken after a clear diagnosis. Once it causes related hemophagocytic syndrome, the mortality rate is extremely high.展开更多
目的:分析EB病毒相关性噬血细胞综合征(EB virus-associated hemophagocytic syndrome,EBVAHS)患儿EB病毒潜伏膜蛋白1(latent membrane protein 1,LMP1)、辅助性T细胞1(helper T cell1,Th1)细胞因子的表达水平及其与疗效的关系。方法:...目的:分析EB病毒相关性噬血细胞综合征(EB virus-associated hemophagocytic syndrome,EBVAHS)患儿EB病毒潜伏膜蛋白1(latent membrane protein 1,LMP1)、辅助性T细胞1(helper T cell1,Th1)细胞因子的表达水平及其与疗效的关系。方法:抽取郑州大学附属儿童医院儿科2014年2月至2018年1月收治的116例EB病毒感染患儿为研究对象,分为单核细胞增多症(infect iousmononucleosis,IM)组与EBV-AHS组,其中IM 66例,EBV-AHS 50例。EBV-AHS组按照国际组织细胞协会推荐的HLH-2004方案给予治疗,于治疗4周后评定近期疗效。另选取同期体检的健康儿童30例作为对照组。对照组于体检时、IM组于确诊时、EBV-AHS组于治疗前、治疗后采血,检测血清LMP1抗体表达及Th1细胞因子水平。结果:与对照组相比,IM组和EBV-AHS组患儿确诊时血清LMP1抗体、白介素(IL)-2、干扰素(IFN)-γ及肿瘤坏死因子(TNF)-α水平显著增高,差异有统计学意义(P<0.05)。IM组和EBV-AHS组患儿血清LMP1抗体,IL-2,IFN-γ,TNF-α水平差异亦有统计学意义(P<0.05)。治疗后EBV-AHS组50例患儿中,疾病缓解者17例(34.00%),有效者22例(44.00%),余下11例(22.00%)疾病活动。疾病缓解者、有效者血清LMP1抗体,IL-2,IFN-γ,TNF-α水平明显较治疗前降低(P<0.05),而疾病活动者血清LMP1抗体,IL-2,IFN-γ,TNF-α水平较治疗前无明显改善(P>0.05);且疾病缓解者、有效者、疾病活动者血清LMP1抗体,IL-2,IFN-γ,TNF-α水平均依次升高,差异有统计学意义(P<0.05)。结论:EBV-AHS患儿血清LMP1抗体表达及Th1细胞因子水平较IM患儿及正常儿童显著升高,且其与疗效密切相关。展开更多
文摘目的探讨血清中腺苷脱氨酶(ADA)水平在儿童EB病毒感染相关噬血细胞综合征(EBV-HLH)中的诊断及预后判断价值。方法选取2015年1月~2017年12月期间浙江大学医学院附属儿童医院收治的初诊EBV-HLH患者72例和慢性活动性EB病毒(CAEBV)感染患儿80例,HLH患者均符合HLH-2004诊断标准,同期50例健康体检儿童作为健康对照组。应用全自动生化分析仪测定各组患者血清ADA水平,采用受试者工作特征曲线(ROC)分析ADA临床诊断价值,Youden指数法确定最佳工作点。结果EBV-HLH组、CAEBV组及健康对照组儿童血清ADA水平分别为102.4±64.9U/L、42.8±22.1U/L和13.3±3.2U/L。EBV-HLH组ADA水平明显高于CAEBV组,差异有统计学意义(Z=4.709,P=0.000);CAEBV组高于健康对照组,差异有统计学意义(Z=5.008,P=0.000)。以慢性活动性EBV感染组为对照组,利用ROC曲线分析ADA在EBV-HLH中的诊断价值,曲线下面积最高为0.893(P=0.000),以57.5U/L为阈值时,敏感度为74.7%,特异性为89.1%,阳性预测值和阴性预测值分别为90.3%、72.1%。在EBV-HLH组中,ADA与EBV拷贝数呈正相关(r=0.608,P=0.000)。10例EBV-HLH死亡患儿血清ADA水平明显高于存活患儿(154.2±47.3U/L vs 92.5±63.4U/L,P=0.002)。结论血清ADA水平可作为EBV-HLH诊断的常规辅助项目,且在与慢性活动性EBV感染鉴别诊断中具有重要意义。
文摘目的:总结1例慢性活动性EB病毒感染相关性噬血细胞综合征患儿的临床特点及诊疗经过,提高对该疾病的认识。方法:回顾性分析2022年10月山东大学附属威海市立医院儿科收治的1例慢性活动性EB病毒感染相关性噬血细胞综合征患儿的临床特点及诊疗经过,并进行相关文献复习。结果:患儿以发热、反复肝损害、全血细胞减少、凝血功能紊乱为突出表现,病情发展迅速,短时间内死亡,结合相关检查,诊断为慢性活动性EB病毒感染相关性噬血细胞综合征。结论:对于慢性活动性EB病毒感染患儿,在明确诊断后应积极采取有效治疗措施,一旦引起相关噬血细胞综合征,死亡率极高。Objective: To summarize the clinical characteristics and diagnosis and treatment process of a child with chronic active EB virus infection associated hemophagocytic syndrome, and improve the understanding of this disease. Methods: Retrospective analysis of the clinical characteristics and diagnosis and treatment process of a child with chronic active EB virus infection associated hemophagocytic syndrome admitted to the pediatrics department of Weihai Municipal Hospital affiliated with Shandong University in October 2022, and relevant literature review. Results: The child presented with prominent symptoms such as fever, repeated liver damage, decreased whole blood cells, and coagulation dysfunction. The condition developed rapidly and died within a short period of time. Combined with relevant examinations, the diagnosis was chronic active EB virus infection associated hemophagocytic syndrome. Conclusions: For children with chronic active EB virus infection, effective treatment measures should be actively taken after a clear diagnosis. Once it causes related hemophagocytic syndrome, the mortality rate is extremely high.