慢性活动性 EBV 感染伴冠状动脉扩张的临床分析及配对病例对照研究。
Clinical analysis of chronic active EBV infection with coronary artery dilatation and a matched case-control study.
机构信息
Beijing Key Laboratory of Pediatric Hematology Oncology; National Key Discipline of Pediatrics (Capital Medical University); Key Laboratory of Major Diseases in Children, Ministry of Education; Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, People's Republic of China.
Hematology and Oncology Laboratory, Beijing Pediatric Research Institute, Beijing Children's Hospital Affiliated with Capital Medical University; National Center for Children's Health; Beijing Key Laboratory of Pediatric Hematology Oncology, Key Laboratory of Major Diseases in Children; Ministry of Education, National Key Discipline of Pediatrics, Beijing, 100045, People's Republic of China.
出版信息
Orphanet J Rare Dis. 2021 Jan 28;16(1):50. doi: 10.1186/s13023-021-01689-5.
OBJECTIVE
To investigate the clinical characteristics, treatment, prognosis and risk factors for chronic active Epstein-Barr Virus infection (CAEBV) associated with coronary artery dilatation (CAD) in children.
METHODS
Children with CAEBV associated with CAD hospitalized at Beijing Children's Hospital, Capital Medical University from March 2016 to December 2019 were analyzed. Children with CAEBV without CAD were selected as the control group and matched by sex, age, treatment and admission time. The clinical manifestations, laboratory and ultrasound examinations, treatment and prognosis of the children were collected in both groups.
RESULTS
There were 10 children with CAEBV combined with CAD, including 6 males and 4 females, accounting for 8.9% (10/112) of CAEBV patients in the same period, with an onset age of 6.05 (2.8-14.3) years. The median follow-up time was 20 (6-48) months. All the patients had high copies of EBV-DNA in whole blood [1.18 × 10 (1.90 × 10-3.96 × 10) copies/mL] and plasma [1.81 × 10 (1.54 × 10-1.76 × 10) copies/mL], and all biopsy samples (bone marrow, lymph nodes or liver) were all positive for Epstein-Barr virus-encoded small RNA. Among the 10 children, 8 had bilateral CAD, and 2 patients had unilateral CAD. After diagnosis, 7 children were treated with L-DEP chemotherapy in our hospital. After chemotherapy, four patients underwent allogeneic hematopoietic stem cell transplantation (HSCT). The others were waiting for HSCT. At the time of the last patients follow up record, the CAD had returned to normal in 3 patients, and the time from the diagnosis of CAD to recovery was 21 (18-68) days. LDH, serum ferritin, TNF-α and IL-10 levels were statistically significantly different between the two groups (P = 0.009, 0.008, 0.026 and 0.030). There were no significant differences in survival rate between the two groups (P = 0.416).
CONCLUSION
The incidence of CAEBV with CAD was low. CAEBV with CAD did not influence the prognosis. Patients who had high LDH, serum ferritin, TNF-α, and IL-10 levels early in their illness were more likely to develop CAD.
目的
探讨儿童慢性活动性 Epstein-Barr 病毒感染（CAEBV）并发冠状动脉扩张（CAD）的临床特征、治疗、预后及危险因素。
方法
分析 2016 年 3 月至 2019 年 12 月首都医科大学附属北京儿童医院收治的 CAEBV 并发 CAD 患儿。选择同期 CAEBV 无 CAD 患儿作为对照组，并按照性别、年龄、治疗和入院时间进行匹配。收集两组患儿的临床表现、实验室和超声检查、治疗和预后。
结果
10 例 CAEBV 合并 CAD 患儿，男 6 例，女 4 例，占同期 CAEBV 患儿的 8.9%（10/112），发病年龄 6.05（2.8-14.3）岁。中位随访时间 20（6-48）个月。所有患儿全血 EBV-DNA 拷贝数[1.18×10（1.90×10-3.96×10）拷贝/ml]和血浆 EBV-DNA 拷贝数[1.81×10（1.54×10-1.76×10）拷贝/ml]均较高，骨髓、淋巴结或肝脏活检均为 EBV 编码的小 RNA 阳性。10 例患儿中 8 例存在双侧 CAD，2 例存在单侧 CAD。诊断后，7 例患儿在我院接受 L-DEP 化疗，化疗后 4 例行异基因造血干细胞移植（HSCT）。其他患儿在等待 HSCT。末次随访时，3 例 CAD 恢复正常，从 CAD 诊断到恢复的时间为 21（18-68）d。两组 LDH、血清铁蛋白、TNF-α、IL-10 水平比较，差异均有统计学意义（P=0.009、0.008、0.026、0.030）。两组患儿的生存率比较，差异无统计学意义（P=0.416）。
结论
CAEBV 并发 CAD 的发生率较低，CAEBV 并发 CAD 不影响预后。起病时 LDH、血清铁蛋白、TNF-α、IL-10 水平较高的患者更易发生 CAD。
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