导师风采
张蕊
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个人信息

Personal Information

  • 教授
  • 导师类别:硕士,博士生导师
  • 性别: 女
  • 学历:博士研究生
  • 学位:博士

联系方式

Contact Information

  • 所属院系:附属北京儿童医院
  • 所属专业: 儿内科学
  • 邮箱 : ruizh1973@126.com
  • 工作电话 : 010-59617623

个人简介

Personal Profile

      长期致力于儿童血液病临床与科研工作,主要研究方向为儿童噬血细胞综合征、朗格罕细胞组织细胞增生症等组织细胞疾病的发病机制以及难治复发病例的诊疗。目前担任全国儿童EBV感染协作组副组长,中华医学会儿科学分会血液学组秘书,国家卫生健康委儿童血液病专病委员会秘书,中国医师协会血液科医师分会组织细胞疾病专业委员会委员等。《中华儿科学杂志》第16届通迅编委,《中华医学杂志》英文版第9届通迅编委。

近5年主持国自然面上项目、北京市科技新星计划、北京市科技计划首都特色项目、首都卫生发展科研专项、北京市医院管理中心儿科协同重点项目子课题、北京航空航天大学北京高精尖创新中心任务等国家及省部级课题8项,近5年以第一或通迅作者在国际血液学权威杂志BLOOD(2篇)、Haematologica(2篇)、Journal of Hematology & Oncology(1篇)等发表SCI及核心期刊论文40余篇,作为骨干成员获批国家发明专利1项。


  • 研究方向Research Directions
儿童血液系统疾病精准治疗,儿童组织细胞疾病发病机制及治疗,慢性活动性EB病毒感染发病机制及治疗
2. 机电结构优化与控制 研究内容:在对机电结构进行分析和优化的基础上,运用控制理论进行结构参数的调整,使结构性能满足设计要求。1. 仿生结构材料拓扑优化设计, 仿生机械设计 研究内容:以仿生结构为研究对象,运用连续体结构拓扑优化设计理论和方法,对多相仿生结构(机构)材料进行2. 机电结构优化与控制 研究内容:在对机电结构进行分析和优化的基础上,运用控制理论进行结构参数的调整,使结构性能满足设计要求。1. 仿生结构材料拓扑优化设计, 仿生机械设计 研究内容:以仿生结构为研究对象,运用连续体结构拓扑优化设计理论和方法,对多相仿生结构(机构)材料进行整体布局设计。 整体布局设计。
团队展示

      北京儿童医院组织细胞病专业团队:

      临床医生9人,主任医师4人,副主任医师1人,主治医师1人,住院医师3人。博士学历4人,硕士学历4人。      

      研究人员7人,研究员2人、副研究员2人、副主任技师2人和助理研究员1人,博士学历6人,硕士学历1人。

      团队成员曾获得北京市科学技术奖三等奖1项。


项目情况

目前主持在研课题:

1.   国家自然科学基金面上项目:CASP8AP2基因移码突变在儿童朗格罕细胞组织细胞增生症发病机制中作用的研究 

2.   首都卫生发展科研专项自主创新项目:儿童朗格罕细胞组织细胞增生症的精准分层治疗研究

3.   北京市医院管理局儿科学科协同发展中心儿科专项重点项目子课题:儿童血液/肿瘤规范化诊治体系的建立与应用-儿童噬血细胞性淋巴组织细胞增生症精准分层诊治与预后评估研究


研究成果

发表文章(第一作者或通信作者):

1.    Xiaoman Bi, Qing Zhang, Lei Chen, Dan Liu, Yueying Li,Xiaoxi Zhao, Ya Zhang, Liping Zhang, Jingkun Liu, Chaoyi Wu, Zhigang Li, YunzeZhao, Honghao Ma, Gang Huang, Xin Liu, Qian-Fei Wang, Rui Zhang.NBAS,a gene involved in cytotoxic degranulation, is recurrently mutated in pediatrichemophagocytic lymphohistiocytosis. Journal of hematology & oncology. 2022 Jul 28; 15 (1) :101. 

2.    Qing Zhang , * Yun-Ze Zhao,,* Hong-Hao Ma, Dong Wang, LeiCui, Wei-Jing Li, Ang Wei, Chan-Juan Wang, Tian-You Wang, Zhi-Gang Li, and RuiZhang. A study of ruxolitinib response–basedstratifiedtreatment for pediatric hemophagocyticlymphohistiocytosis. BLOOD. 2022;16(139):3493-504.

3.    Ang Wei, Xia Lu, HonghaoMa, Hongyun Lian, Xu Yang, Liping Zhang, Dong Wang, Sitong Chen, Qing Zhang,Zhigang Li, Rui Zhang, Jigang Yang, Tianyou Wang.  18F-FDG PET/CT for Identifying thePotential Primary Diseases and Predicting Prognosis of Secondary HemophagocyticLymphohistiocytosis in Children.  Contrast media & molecularimaging. 2022; 2022 :4849081.

4.    Zhuo Li, Ning Sun, QingZhang, Hong-Hao Ma, Dong Wang, Yun-Ze Zhao, Chang-Qing Yang, Rui Zhang, Li-BoZhao. Development and application of an LC-MS/MS method for pharmacokineticstudy of ruxolitinib in children with hemophagocytic lymphohistiocytosis. Analyticalmethods : advancing methods and applications 2022 Jun 16;14(23):2293-2303

5.    Wenxin Ou, Honghao Ma, AngWei, Yunze Zhao, Liping Zhang, Hongyun Lian, Qing Zhang, Sitong Chen, DongWang, Zhigang Li, Rui Zhang, Tianyou Wang. Clinical significance of cerebrospinal fluid soluble CD25in pediatric hemophagocytic lymphohistiocytosis with central nervous systeminvolvement. Pediatric blood & cancer. 2022 Apr20; e29712. 

6.    Dong Wang, Xi-Hua Chen,Ang Wei, Chun-Ju Zhou, Xue Zhang, Hong-Hao Ma, Hong-Yun Lian, Li Zhang, QingZhang, Xiao-Tong Huang, Chan-Juan Wang, Ying Yang, Wei Liu, Tian-You Wang*,Zhi-Gang Li*, Lei Cui*, Rui Zhang*. Clinical features and treatment outcomes ofpediatric Langerhans cell histiocytosis with macrophage activationsyndrome-hemophagocytic lymphohistiocytosis. Orphanet journal of rarediseases 2022 Apr 04;17(1):151 

7.    Jia-Feng Yao, Dong Wang,Hong-Hao Ma, Hong-Yun Lian, Li Zhang, Tian-You Wang, Zhi-Gang Li, Jin Jiang*,Lei Cui*, Rui Zhang*. Characteristics and Treatment Outcomes of PediatricLangerhans Cell Histiocytosis with Thymic Involvement. The Journal ofpediatrics 2022 05;244:194-202 

8.    Hongyun Lian, Lei Cui,Ying Yang, Ang Wei, Hua Cheng, Na Li, Li Zhang, Honghao Ma, Xiaoxi Zhao, TianyouWang*, Zhigang Li*, Rui Zhang*. Second-line regimen for CNS-involved pediatricLangerhans cell histiocytosis.   Pituitary 2022Feb;25(1):108-115

9.    Xia Lu#, Ang Wei#, XuYang, Jun Liu, Siqi Li, Ying Kan, Wei Wang, Tianyou Wang, Rui Zhang*, JigangYang*  . The Role of Pre-therapeutic 18F-FDG PET/CT in PediatricHemophagocytic Lymphohistiocytosis With Epstein-Barr Virus Infection. Frontiersin medicine 2021;8:836438 

10. Hongyun Lian, Ang Wei,Lejian He, Ying Yang, Honghao Ma, Liping Zhang, Yitong Guan, Qing Zhang, DongWang, Zhigang Li*, Rui Zhang*, Tianyou Wang*. Clinical Analysis of Pediatric Systemic JuvenileXanthogranulomas: A Retrospective Single-Center Study.   Frontiers inpediatrics. 2021; 9 :672547. 

11. Chan-Juan Wang, Lei Cui,Hong-Hao Ma, Dong Wang, Li Zhang, Hong-Yun Lian, Wei-Jing Li, Qing Zhang,Tian-You Wang*, Zhi-Gang Li*, Rui Zhang*. BRAF V600E Mutation in Cell-Free DNA,Rather than in Lesion Tissues, at Diagnosis Is An Independent Prognostic Factorin Children with Langerhans Cell Histiocytosis. Molecular cancertherapeutics 2021 07;20(7):1316-1323 

12. Honghao Ma#, LipingZhang#, Ang Wei, Jun Yang, Dong Wang, Qing Zhang, Yunze Zhao, Sitong Chen,Hongyun Lian, Li Zhang, Chunju Zhou, Maoquan Qin, Zhigang Li*, Tianyou Wang*,Rui Zhang*.   Outcome of L-DEP regimen for treatment of pediatricchronic active Epstein-Barr virus infection. Orphanet journal of rare diseases2021 06 10;16(1):269

13. Ang Wei#, Honghao Ma#, LipingZhang, Zhigang Li, Yitong Guan, Qing Zhang, Dong Wang, Hongyun Lian, RuiZhang*, Tianyou Wang*. Clinical analysis of chronic active EBV infection with coronary artery dilatation anda matched case-control study.  Orphanet journal of rarediseases. 2021 0128; 16 (1) :50. 

14. Yang Y#, Wang C#, Wang D#, Cui L, Li N, Lian H, MaH, Zhao Y, Zhang L, Liu W, Wang Y, Wu W, Zhang R*, Li Z*, Wang T*. Clinical study ofMAP2K1-mutated Langerhans cell histiocytosis in children. J Cancer Res ClinOncol 2021;148:2517–2527

15. Zhao XX, Lian HY, Zhang L,Ma HH, Wang D, Zhao YZ, Liu W, Chen XH, Wang TY, Li ZG, Zhang R.Significance of serum Th1/Th2 cytokine levels in underlying diseaseclassification of childhood HLH. Cytokine.  2021; 149 :155729.

16. Shi H, He H, Cui L,Kvedaraite E, Bian Z, Huang T, Lee C, Li ZG, He J, Gong Y, Li Z, Zhou J, ZengY, Li X, Ni Y, Bai Z, Liu C, Li N, Ma H, Wang D, Lan Y, Ginhoux F, ZhangR, Liu B. Transcriptomic Landscape of Circulating Mononuclear Phagocytes inLangerhans Cell Histiocytosis at Single-cell Level. Blood. 2021 Blood. 202110 07; 138 (14) :1237-1248.

17. Qing Zhang, Yun-Ze Zhao,Hong-Hao Ma, Dong Wang, Nan Zhang, Zhi-Gang Li, Rui Zhang. Successful rescue ofa lethal Griscelli syndrome type 2 presenting with neurological involvement andhemophagocytic lymphohistiocytosis: a case report. BMC pediatrics 2021 0531;21(1):253 

18. Qing Zhang, Ang Wei,Hong-Hao Ma, Li Zhang, Hong-Yun Lian, Dong Wang, Yun-Ze Zhao, Lei Cui, Wei-JingLi, Ying Yang, Tian-You Wang, Zhi-Gang Li, and Rui Zhang. A pilot study ofruxolitinib as a front-line therapy for 12 children with secondaryhemophagocytic lymphohistiocytosis. Haematologica 2021,106(7):1892-1901

19. Ying Yang, Dong Wang, LeiCui, Hong-Hao Ma, Li Zhang, Hong-Yun Lian, Qing Zhang, Xiao-Xi Zhao, Li-PingZhang, Yun-Ze Zhao, Na Li, Tian-You Wang, Zhi-Gang Li, Rui Zhang. Effectivenessand Safety of Dabrafenib in the Treatment of 20 Chinese Children withBRAFV600E-Mutated LCH. Cancer research and treatment. 2021 Jan;53(1):261-269;IF(2020):4.679

20.  Ying Yang, Dong Wang, NaLi, Honghao Ma, Hongyun Lian, Lei Cui, Qing Zhang, Xiaoxi Zhao, Liping Zhang,Yunze Zhao, Chanjuan Wang, Li Zhang, Tianyou Wang, Zhigang Li, Rui Zhang.Improvement in Pituitary Imaging After Targeted Therapy in Three Children withBRAF-Mutated Langerhans Cell Histiocytosis with Pituitary Involvement. OncoTargets Ther 2020;13:12357-63  IF(2020):4.147

21.  Lei Cui, Li Zhang,Hong-Hao Ma, Chan-Juan Wang, Dong Wang, Hong-Yun Lian, Wei-Jing Li, Qing Zhang,Na Li, Tian-You Wang*, Zhi-Gang Li*, Rui Zhang*. Circulating cell-freeBRAFV600E during chemotherapy is associated with prognosis of children withLangerhans cell histiocytosis. Haematologica. 2020 Sep 1;105(9):e444-447.

22.  Yun-Ze Zhao, Zhi-Gang Li,Li Zhang, Hong-Yun Lian, Hong-Hao Ma, Dong Wang, Xiao-Xi Zhao, Qing Zhang,Tian-You Wang, Rui Zhang. L‐DEP regimen salvagetherapy for paediatric patients with refractory Epstein‐Barr virus‐associated haemophagocyticlymphohistiocytosis. British Journal of Haematology 2020 11; 191 (3) :453-459.

23.  Guang Hua Zhu#, Li PingZhang#, Zhi Gang Li, Ang Wei, Ying Yang, Yu Tian, Hong Hao Ma, Dong Wang, Xiao XiZhao, Yun Ze Zhao, Na Li, Wei Liu, Tian You Wang*, Rui Zhang*. Associationsbetween PRF1 Ala91Val polymorphism and risk of hemophagocytic lymphohistiocytosis:a meta-analysis based on 1366 subjects. World J Pediatr WJP 2020Dec;16(6):598-606 

24.  Ang Wei#, Honghao Ma#,Zhigang Li, Liping Zhang, Qing Zhang, Dong Wang, Hongyun Lian, Rui Zhang*, TianyouWang*. Short-term effectiveness of ruxolitinib in the treatment of recurrent orrefractory hemophagocytic lymphohistiocytosis in children. InternationalJournal of Hematology 2020 Oct; 112 (4) :568-576. 

25.  Na Li, Lei Cui, HonghaoMa, Zhihua Gong, Hongyun Lian, Chanjuan Wang, Qing Zhang, Xiaoxi Zhao, XihuaChen, Yu Tian, Ying Yang, Ang Wei, Rui Zhang*, Zhigang Li*, Tianyou Wang*  Osteopontin is highly secreted in the cerebrospinal fluid ofpatient with posterior pituitary involvement in Langerhans cellhistiocytosis.International journal of laboratory hematology 2020Dec;42(6):788-795 

26.  Honghao Ma#, Rui Zhang#,Liping Zhang, Ang Wei, Xiaoxi Zhao, Ying Yang, Wei Liu, Zhigang L*i, MaoquanQin*, Tianyou Wang* Treatment of pediatric primary hemophagocyticlymphohistiocytosis with the HLH-94/2004 regimens and hematopoietic stem celltransplantation in China. Annals of hematology 2020Oct;99(10):2255-2263 

27.   Zhao Y, Li Z, Zhang L, LianH, Ma H, Wang D, Zhao X, Zhang Q, Wang T, ZhangR*. Clinical Features and Outcomes of Patients with HemophagocyticLymphohistiocytosis at Onset of Systemic Autoinflammatory Disorder and Comparewith Epstein-Barr virus (EBV)-related Hemophagocytic Lymphohistiocytosis.  Medicine. 2020, 99(1):e18503 

28.  Wei A, Ma H, LiZ, Zhang L, Zhang R*, Wang T*. Type-IV Glycogen Storage DiseaseAssociated with Hemophagocytic lymphositiocytosis: a case report. Journal ofPediatric Hematology and Oncology 2020;42(5):368-369 

29.  Wei A, Ma H, Zhang L, LiZ, Zhang Q, Wang D, Zhang L, Lian H, Zhang R, Wang T. Hemophagocyticlymphohistiocytosis resulting from a cytokine storm triggered by septicemia ina child with chronic granuloma disease: a case report and literature review.BMC Pediatr. 2020 Mar 3;20(1):100.

30.  Wei A, Ma H, LiZ, Zhang L, Zhang R*, Wang T*. Successful treatment of a childwith idiopathic multicentric Castleman disease associated with hemophagocyticlymphohistiocytosis using tocilizumab. Pediatr Blood Cancer. 2019Jul;66(7):e27759. 

31.  Zhang L, LiZ, Liu W, Ma H, Wang T*, Zhang R*. Genetic characterizationof pediatric primary hemophagocytic lymphohistiocytosis in China: asingle-center study. Ann Hematol. 2019 Oct;98(10):2303-2310 

32.  Zhao XX, Lian HY, Zhang L,Ma HH, Wang D, Zhao YZ, Liu ZW, Wang TY, Zhang R, Li ZG. Significance of serumferritin level in hemophagocytic lymphohistiocytosis diagnosis. Int J LabHematol. 2019, 41(4):503-508 

33.  Zhang Q, Ma H, Ma J, WangD, Zhao Y, Wang T, Li Z, Wu R, Zhang R. Clinical and genetic analysis ofimmunodeficiency-related diseases associated with PIK3CD mutations.Pediatr Investig. 2019 Jan 8;2(4):257-262.

34.  Yun‐Ze Zhao, Hua Cheng, Chang‐Hong Ding, Hong‐Hao Ma, Tong‐Li Han, Jiu‐Wei Li, Dong Wang, Zhi‐Gang Li, Tian‐You Wang, Rui Zhang. Neurological Abnormality Could be the First andOnly Symptom of Familial Hemophagocytic Lymphohistiocytosis, Report of TwoFamilies. Chinese Medical Journal. 2018,131(24): 3004-3006

35.  Dong Wang, Lei Cui,Zhi-Gang Li, Li Zhang, Hong-Yun Lian, Hong-Hao Ma, Yun-Ze Zhao, Xiao-Xi Zhao,Tian-You Wang, Rui Zhang. Clinical Research of Pulmonary Langerhans CellHistiocytosis in Children.  ChineseMedical Journal. 2018,131(15):1794-1798

36.  Yun-Ze Zhao, Lei Cui,Zhi-Gang Li, Li Zhang, Hong-Yun Lian, Hong-Hao Ma, Dong Wang, Xiao-Xi Zhao,Tian-You Wang, Rui Zhang. Central Nervous System Involvement in 179 ChineseChildren with Hemophagocytic Lymphohistiocytosis.  Chinese Medical Journal. 2018,131(15):1786-1792

37. 田宇#、张蕊#、张利平、杨颖、魏昂、彭晓霞、李志刚、王天有*.常用一线单药治疗儿童新诊断ITP Meta分析. 中国小儿血液与肿瘤杂志,2021,26(2):21-28

38. 廉红云#、李志刚、崔蕾、魏昂、杨颖、程华、何乐健、张莉、赵晓曦、王天有、张蕊*.系统性幼年黄色肉芽肿4例临床分析. 中国小儿血液与肿瘤杂志, 2021,26 (2):84-88.

39. 廉红云#、赵晓曦、张莉、崔蕾、马宏浩、王冬、赵云泽、李志刚、王天有、张蕊*.儿童皮肤单器官受累的朗格罕细胞组织细胞增生症的临床分析. 中华实用儿科临床杂志,2021,36(07):520-524

40.   张晴崔蕾, 马宏浩,  王冬, 赵云泽, 王天有,李志刚*, 张蕊*. 血浆及全血EB病毒DNA对EB病毒相关噬血细胞性淋巴组织细胞增生症患儿预后的影响. 中华实用儿科临床杂志 2020;35:1139-43.

41.    魏昂、马宏浩、张利平、吴润晖、张蕊*,王天有. 以噬血细胞综合症为首发表现的肝炎相关再生障碍性贫血5例临床分析. 中华儿科杂志2020,58(3):213-217

42.  张蕊,王天有*. 噬血细胞性淋巴组织细胞增生症——罕见病,大问题. 中华儿科杂志2019,57(10):740-742

43.  廉红云,张莉,李志刚,马宏浩,王冬,赵云泽,赵晓曦,王天有,张蕊*. 儿童颅面骨受累的朗格罕细胞组织细胞增生症临床分析. 中华实用儿科临床杂志2019, 34(15):1151-1155

44. 马宏浩, 王天有, 张莉, 廉红云, 王冬, 赵云泽, 赵晓曦, 张蕊*. 儿童EB病毒相关噬血细胞性淋巴组织细胞增生症的预后相关危险因素分析. 北京医学, 2018, 40(7):622-628


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