以IgA肾病起病的儿童慢性活动性EB病毒感染1例  

Chronic active EB virus infection in a child with the onset of IgA nephropathy

作　　者：范树颖[1] 王欣[1] 魏宁 周秋梅 王文红[1] Fan Shuying;Wang Xin;Wei Ning;Zhou Qiumei;Wang Wenhong(Department of Nephrology,Tianjin Children's Hospital,Children's Hospital,Tianjin University,Tianjin Institute of Pediatrics,Tianjin Key Laboratory of Birth Defects for Prevention and Treatment,Tianjin 300134,China)

机构地区：[1]天津市儿童医院,天津大学儿童医院肾脏内科,天津市儿科研究所,天津市儿童出生缺陷防治重点实验室,天津300134

出　　处：《中华肾脏病杂志》2024年第2期137-140,共4页Chinese Journal of Nephrology

基　　金：天津市医学重点学科(专科)建设项目(TJYXZDXK-040A)。

摘　　要：慢性活动性EB病毒(chronic active Epstein-Barr virus,CAEBV)感染使肾脏受累的病例较为罕见。该文报道1例以IgA肾病(IgA nephropathy,IgAN)起病的儿童多系统损害CAEBV感染病例。患儿以间断性肉眼血尿起病,肾活检示局灶增生性IgAN,予甲泼尼龙冲击继口服泼尼松治疗。治疗随诊过程中出现间断血EB病毒(Epstein-Barr virus,EBV)载量升高和EBV抗体异常,EBV及金黄色葡萄球菌混合感染所致肺炎、阑尾周围脓肿和全血细胞减少,考虑CAEBV感染。进一步检查,超声心动图示肺动脉高压,头颅CT示双侧基底节区多发钙化,骨髓活检示骨髓EBV-DNA 6.5×10^(3)拷贝/L。肾组织免疫组化示CD8(散在+),约50个/高倍镜视野(HPF);CD4(灶性+),局部约40个/HPF;CD68(-);EBV潜伏膜蛋白1(-);EBV编码的小RNA(散在+),约25个/HPF。感染EBV的淋巴细胞亚群检测示CD4^(+)T细胞EBV-DNA 3.4×10^(4)拷贝/100万个细胞,CD8^(+)T细胞EBV-DNA 3.3×10^(5)拷贝/100万个细胞,B细胞EBV-DNA 1.25×10^(4)拷贝/100万个细胞,NK细胞/NK T细胞EBV-DNA 2.3×10^(4)拷贝/100万个细胞。临床诊断为IgAN(EBV相关性)和CAEBV感染。目前予泼尼松口服治疗,建议造血干细胞移植,治疗随诊中。Chronic active Epstein-Barr virus(CAEBV)infection with renal involvement is not common.The paper reported a child of multisystem-compromised CAEBV infection with the onset of IgA nephropathy(IgAN).The child presented with intermittent gross hematuria,and renal biopsy showed focal proliferative IgAN,administered methylprednisolone pulse followed by oral prednisolone treatment.Intermittent increase of blood Epstein-Barr virus(EBV)load and abnormal EBV antibody,pneumonia caused by EBV and Staphylococcus aureus-mixed infection,periappendiceal abscess,and pancytopenia occurred during treatment follow-up.The CAEBV infection was considered.Echocardiography suggested pulmonary hypertension.Head CT presented multiple calcifications in the bilateral basal ganglia.Bone marrow biopsy showed bone marrow EBV-DNA 6.5×10^(3) copies per liter.Immunohistochemistry of renal biopsy showed about 50 CD8^(+)(scattered+)cells per high power field(HPF),about 40 CD4^(+)(focal+)cells per HPF(local),CD68^(+)(-),latent membrane protein 1(-),EBV-encoded small RNA(scattered+)approximately 25 cells per HPF.The lymphocyte subsets infected with EBV showed CD4^(+)T cells EBV-DNA 3.4×10^(4) copies per 1 million cells,CD8^(+)T cells EBV-DNA 3.3×10^(5) copies per 1 million cells,B cells EBV-DNA 1.25×10^(4) copies per 1 million cells,NK cells/NK T cells EBV-DNA 2.3×10^(4) copies per 1 million cells.The clinical diagnosis was CAEBV infection and EBV-associated IgAN.The patient currently receives oral prednisone treatment,and it is recommended to undergo hematopoietic stem cell transplantation and treatment is under follow up.

关 键 词：疱疹病毒4型 人 肾小球肾炎 IgA 儿童 

分 类 号：R725.1[医药卫生—儿科] R726.9[医药卫生—临床医学]