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Genes & Diseases 2023年第6期10卷 2233-2236页

作者： Xiangjun Liu Jingang Gui Xiaolin Wang Laboratory of Tumor Immunology Beijing Pediatric Research InstituteBeijing Children's HospitalCapital Medical UniversityNational Center for Children's Health(NCCH)Beijing 100045China

severe acute respiratory syndrome coronavirus-2(sARsCoV-2),which causes coronavirus disease 2019(COVID-19)and initially infects epithelial cells of the upper respiratory tract,has recently caused a global health emergency and is still a potential threat to humans.1 Multiple studies have suggested alterations in the immune system,including excessive neutrophil activation and lymphopenia,and excessive inflammatory responses as the key mechanisms for severesymptoms.2However,which cell types contribute to excessive inflammation remains to be *** analysis of human blood immune cells provides insights into the coordinated response to sARsCoV-2 infections and is a significant task for restraining the virus and controlling disease progression.

关键词： neutrophil respiratory alterations

Loop CD20/CD19 CAR-T cells eradicate B-cell malignancies efficiently

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science China(Life sciences) 2023年第4期66卷 754-770页

作者： Zhaoqi Chen Yan Liu Nianci Chen Haiyan Xing Zheng Tian Kejing Tang Qing Rao Yingxi Xu Ying Wang Min Wang Jianxiang Wang state Key Laboratory of Experimental Hematology Institute of Hematology&Blood Diseases HospitalChinese Academy of Medical Sciences&Peking Union Medical CollegeTianjin 300020China national Clinical Research center for blood Diseases Institute of Hematology and Blood Diseases HospitalChinese Academy of Medical Sciences&Peking Union Medical CollegeTianjin 300020China Haihe Laboratory of Cell Ecosystem Institute of Hematology&Blood Diseases HospitalChinese Academy of Medical Sciences&Peking Union Medical CollegeTianjin 300020China Tianjin Key Laboratory of Cell Therapy for blood Diseases Institute of Hematology and Blood Diseases HospitalChinese Academy of Medical Sciences&Peking Union Medical CollegeTianjin 300020China

CD19 chimeric antigen receptor(CAR)T cells have shown robust efficacy in relapsed and refractory acute lymphoblastic leukemia(R/R ALL),but compromising result in chronic lymphoblastic leukemia(CLL)and non-Hodgkin’s lymphoma(NHL).CD19-relapse and the lack of CAR-T cell persistence which result in treatment failure are considerable obstacles to ***-T targeting CD20 is an option for salvaging CD19 CAR-T *** studies have established variant structures of bispecific CAR-T which could avoid antigen-loss and immune ***,we constructed tandem and loop CAR structures targeting both CD19 and CD20 *** CAR-T cells could eliminate either CD19 or CD20 negative lymphoma cells,suggesting they exhibited dual antigen targeting of CD19 and *** comparing the efficiency of four bispecific CAR modified T cells,it was found that loop2019 CAR was the best structure among them to eradicate lymphoma cell lines and patients’primary lymphoma or CLL cells in a very low dose in vitro and prolong the survival time dramatically in lymphoma xenograft mice *** data highlighted the potential of loop2019 CAR-T in clinical treatment.

关键词： chimeric antigen receptor CD19 CD20 bispecific targeting CLL lymphoma

Immunoglobulin G4-related disease involving multiple systems:A case report

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World Journal of Clinical Cases 2022年第8期10卷 2543-2549页

作者： Yu-Qiong An Ning Ma Yong Liu Heart center Beijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijing 100045China Department of Ultrasound Beijing-Shijitan HospitalCapital Medical UniversityBeijing 100038China

BACKGROUND Ig G4-related disease(Ig G4-RD),an immune-mediated chronic progressive fibroinflammatory disease,can affect the functions of several *** common characteristics can be observed in different Ig G4-RDs,such as higher prevalence in middle-aged and elderly male patients,raised serum Ig G4 levels,abundant infiltration of Ig G4-positive cells and fibrosis,diffuse or localized swelling of the affected organs,and good response to glucocorticoids *** sUMMARY A 72-year-old man complained of left upper abdominal pain 3 mo ago,and he was diagnosed with acute onset of chronic cholecystitis and acute pancreatitis in the local *** improved after relevant *** days ago,his abdominal pain worsened,and he was admitted to our hospital for further *** ultrasound showed that the pancreas presented with sausagelike swelling and the parenchymal echo was diffusely *** volume was increased,while the wall was rough and thickened with bilateral ***,the left submandibular gland was enlarged,accompanied with significantly increased blood flow ***,we found that the adventitia of the abdominal aorta and right iliac artery was thickened *** Ig G4 was elevated to 12600 mg/***,the patient was diagnosed with Ig *** treatment with methylprednisolone,he had an uneventful course and was discharged in good *** Ig G4-RD can involve almost any *** has a significant role in timely and accurately diagnosis.

关键词： Immunoglobulin G4 Autoimmune pancreatitis Cholecystitis Arteritis Ultrasound Case report

Tacrolimus for children with refractory nephrotic syndrome:a one-year prospective,multicenter,and open-label study of Tacrobell®,a generic formula

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World Journal of Pediatrics 2016年第1期12卷 60-65页

作者： Eun Mi Yang sang Taek Lee Hyun Jin Choi Hee Yeon Cho Joo Hoon Lee Hee Gyung Kang Young seo Park Hae Il Cheong Il-soo Ha Department of Pediatrics Chonnam National University HospitalGawngjuKorea Department of Pediatrics Seoul National University Children's HospitalSeoulKorea Department of Pediatrics Samsung Medical CenterSungkynkwan University School of MedicineSeoulKorea Department of Pediatrics Asan Medical Center Children's HospitalUniversity of Ulsan College of MedicineSeoulKorea Research Coordination center for Rare Diseases Seoul National University HospitalSeoulKorea Kidney Research Institute Medical Research CenterSeoul National University College of MedicineSeoulKorea

Background:Cyclosporine A and tacrolimus(TAC)are often used as a second-line treatment for children with refractory nephrotic syndrome(Ns).This study was undertaken to investigate the efficacy and safety of Tacrobell®,a locally produced generic form of ***:This study was a one-year prospective,open-label,single-arm,multicenter ***-four children with steroid-dependent Ns(sDNs)and 33 children with steroid-resistant Ns(sRNs)were *** primary endpoints were defined as the remission rates,whereas the secondary endpoints were recognized as the duration of remission and adverse effects of ***:After one-year treatment,34(77.3%)of the 44 patients with sDNs were in complete remission,and 6(13.6%)were in partial ***(43.2%)patients did not relapse during the study;for those who did relapse,the mean duration of remission was 4.6±2.9 *** number of relapse episodes during the study period(0.90 per patient-year)was significantly lower than that in the preceding year(2.8 per patientyear).After treatment for 3 and 6 months,12(36.4%)of the 33 patients with sRNs were in remission,and after treatment for 12 months,the number of patients had increased to 13(39.4%).The mean time to achieve remission was 4.0±3.2 *** remission(duration,3.7±2.7 months),12(54.5%)of 22 patients *** fasting blood glucose and blood pressure levels during the therapy were similar to those at the time of study ***:Treatment with Tacrobell®was effective and safe for children with refractory *** efficacy of this generic form of TAC was better than that of the original TAC formula.

关键词： generic drugs nephrotic syndrome tacrolimus

The role of bone marrow-derived cells in the origin of liver cancer revealed by single-cell sequencing

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Cancer Biology & Medicine 2020年第1期17卷 142-153页

作者： Lu Chen Xianfu Yi Piao Guo Hua Guo Ziye Chen Chunyu Hou Lisha Qi Yongrong Wang Chengwen Li Peng Liu Yucun Liu Yuanfu Xu Ning Zhang The concrete information of affiliations should be presented for instanceDepartment of Tianjin Medical University Cancer Institute and HospitalNational Clinical Research Center for CancerKey Laboratory of Cancer Prevention and TherapyTianjin's Clinical Research Center for CancerTianjin 300070China school of Biomedical Engineering and Technology Tianjin Medical UniversityTianjin 300070China The center for Translational Cancer Research Peking University First HospitalBeijing 100034China state Key Laboratory of Experimental Hematology National Clinical Research Center for Blood DiseasesInstitute of Hematology&Blood Diseases HospitalChinese Academy of Medical Sciences&Peking Union Medical CollegeTianjin 300020China CAMs Key Laboratory for Prevention and Control of Hematological Disease Treatment Related Infection Tianjin 300020China Cytogenetics Laboratory Institute of Hematology and Blood Diseases HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeTianjin 300020China

Objective:Epithelial cancers often originate from progenitor cells,while the origin of hepatocellular carcinoma(HCC)is still ***,one of the deadliest cancers,is closely linked with liver injuries and chronic inflammation,which trigger massive infiltration of bone marrow-derived cells(BMDCs)during liver ***:To address the possible roles of BMDCs in HCC origination,we established a diethylnitrosamine(DEN)-induced HCC model in bone marrow transplanted *** and frozen tissue immunofluorescence were used to verify DENinduced HCC in the pathology of the *** cellular origin of DEN-induced HCC was further studied by single cell sequencing,single-cell nested PCR,and immunofluorescence-fluorescence in situ ***:studies by using single cell sequencing and biochemical analysis revealed that HCC cells in these mice were coming from donor mice BMDCs,and not from recipient ***,the copy numbers of mouse orthologs of several HCC-related genes previously reported in human HCC were also altered in our mouse ***-induced HCCs exhibited a similar histological phenotype and genomic profile as human ***:These results suggested that BMDCs are an important origin of HCC,which provide important clues to HCC prevention,detection,and treatments.

关键词： Hepatocellular carcinoma bone marrow-derived cells(BMDCs) origination genome sequencing copy number alteration

Ten cases of intradiaphragmatic extralobar pulmonary sequestration:a single-center experience

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World Journal of Pediatric surgery 2022年第2期5卷 5-10页

作者： Yue Gao Xu Han Jie Jin Zheng Tan Department of Paediatric Thoracic surgery The Children's HospitalZhejang University School of MedicineNational Clinical Research Center for Child HealthNational Regional Medical Center for ChildrenHangzhouZhejiangChina Clinical laboratory The Children's HospitalZhejiang University School of MedicineNational Clinical Research Center for Child HealthNational Regional Medical Center for ChildrenHangzhouZhejiangChina

Background Intradiaphragmatic extralobar pulmonary sequestration(IDEPs)is a rare type of pulmonary sequestration(Ps).The purpose of this study is to assess diagnosis and operative treatment of *** Patients with Ps who were diagnosed and treated in our center from January 2015 to December 2020 were analyzed retrospectively to identify patients with *** Totally,215 patients with Ps were treated surgically,including 10 cases with *** ultrasounds and postnatal-enhanced CT showed the presence of IDEPs in four cases and in seven cases,*** three-dimensional(3D)reconstruction software was performed perfectly to identify the location of the lesions in 10 *** surgeries were performed smoothly by laparoscopic surgery in one case,video-assisted thoracic surgery(VATs)in five cases and Da Vinci robot-assisted thoracoscopic surgery(DVRATs)in four *** the VATs group,the average operative duration,intraoperative blood loss volume,length of stay after operation,and postoperative thoracic catheter indwelling duration were 48 min,3.8 mL,6.4 days and 2.2 days,*** of the DVRATs group were 80 min,3.5 mL,4.3 days and 1.5 days,*** side effects had *** The 3D reconstruction software was proven to be capable in assisting the assessment of *** suggested early surgery to treat IDEPs,and the best path was accessing the mass from the *** DVRATs and VATs for the treatment of an IDEPs are safe,feasible,and ***,DVRATs provides a 3D magnified view,more flexibility and precision.

关键词： operative diagnosis treatment

ETV2 mediates endothelial transdifferentiation of glioblastoma

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signal Transduction and Targeted Therapy 2018年第1期3卷 291-301页

作者： Chengjian Zhao Gustavo AGomez Yuwei Zhao Yu Yang Dan Cao Jing Lu Hanshuo Yang shuo Lin Department of Molecular Cell and Developmental BiologyUniversity of California-Los AngelesLos AngelesCA 90095USA blood Research Laboratory Chengdu Blood CenterSichuanChina state Key Laboratory of Biotherapy and Cancer center West China HospitalSichuan Universityand Collaborative Innovation Center for BiotherapySichuanChina

Glioblastoma multiforme(GBM)is characterized by extensive endothelial *** studies suggest that a subpopulation of endothelial cells originates via vasculogenesis by the transdifferentiation of GBM tumor cells into endothelial cells(endotransdifferentiation).The molecular mechanism underlying this process remains poorly ***,we show that the expression of ETs variant 2(ETV2),a master regulator of endothelial cell development,is highly correlated with *** studies demonstrate that ETV2 is sufficient and necessary for the transdifferentiation of a subpopulation of CD133+/Nestin+GBM/neural stem cells to an endothelial *** studies of ChIP-seq with gain-of-function RNA-seq data sets suggest that ETV2,in addition to activating vascular genes,represses proneural genes to direct *** endotransdifferentiation by ETV2 is VEGF-A independent,it likely accounts for the observed resistance of GBM tumor cells to antiangiogenesis *** characterization of the regulatory networks mediated by ETV2 in endo-transdifferentiation of GBM tumor cells should lead to the identification of more effective therapeutic targets for GBM.

关键词： CD133 trans ETV

Probing the fate of transplanted hematopoietic stem cells: is the combinational approach “FIT” for purpose?

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science China(Life sciences) 2020年第11期63卷 1755-1758页

作者： Fang Dong Hui Cheng Hideo Ema Tao Cheng state Key Laboratory of Experimental Hematology National Clinical Research Center for Blood DiseasesInstitute of Hematology&Blood Diseases HospitalChinese Academy of Medical Sciences&Peking Union Medical CollegeTianjin 300020China center for stem Cell Medicine Chinese Academy of Medical SciencesTianjin 300020China Department of stem Cell&Regenerative Medicine Peking Union Medical CollegeTianjin 300020China

Hematopoietic stem cells (HsCs)—with their self-renewal and multi-lineage differentiation potential—are top of the hematopoietic cell hierarchy. stress conditions activate the quiescent HsCs, which in turn enter cel... 详细信息

关键词： hematopoietic al. stem

Fludarabine and intravenous busulfan conditioning with post-transplantation cyclophosphamide for allogeneic peripheral stem cell transplantation for adult patients with lymphoid malignancies:a prospective single-arm phase Ⅱ study

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Frontiers of Medicine 2021年第1期15卷 108-115页

作者： Ling Wang Lining Wang Xing Fan Wei Tang Jiong Hu Department of Hematology Blood&Marrow Transplantation CenterCollaborative Innovation Center of HematologyRuijin Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200025China

Post-transplantation cyclophosphamide(PT-Cy)alone or in combination with other immunosuppressive drugs has emerged as a promising strategy in the setting of allogeneic hematopoietic stem cell *** survival rate was reported in lymphoid malignancies following PT-Cy strategy compared with myeloid disease in non-myeloablative bone marrow transplant ***,we aimed to evaluate the safety and efficacy of PT-Cy combined with cyclosporine as graft-versus-host disease(GVHD)prophylaxis after myeloablative conditioning and T cell-replete peripheral stem cell transplantation in lymphoid *** single-arm phase Ⅱ clinical trial(NCT01435447)involving 31 adult patients was conducted from January 2013 to June *** donor-type neutrophil engraftment rate was 100%,and the overall incidence of grade Ⅱ to Ⅳ and grade Ⅲ to Ⅳ acute GVHD was 39%and 24%,*** cumulative incidence rates of chronic GVHD(35%),including moderate to severe forms(10%),were reduced compared with those of the historical group(P=0.03 and P=0.04,respectively).With a median follow-up of 18 months,the estimated 2-year overall and event-free survival was 64.8%(95%confidence interval:47.8%–86.7%)and 58.4%(95%CI:41.9%–81.7%),*** 2-year cumulative incidence rate of relapse was 19.5%(95%CI:9.0%–35.8%),whereas the non-relapse mortality rate was 21.8%(95%CI:11.3%–38.1%).These results demonstrated the feasibility of PT-Cy as GVHD prophylaxis in this clinical *** strategy could significantly reduce the incidence of chronic GVHD and its moderate to severe forms but not of acute GVHD and results in similar survival outcomes compared with the historical group.A prospective study with additional patients is warranted to confirm the role of PT-Cy in lymphoid malignancy.

关键词： post-transplantation cyclophosphamide allogeneic hematopoietic stem cell transplantation lymphoid malignancies

Pediatric reference intervals in China(PRINCE):design and rationale for a large,multicenter collaborative cross-sectional study

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science Bulletin 2018年第24期63卷 1626-1634页

作者： Xin Ni Wenqi song Xiaoxia Peng Ying shen Yaguang Peng Qiliang Li Yan Wang Lixin Hu Yanying Cai Hong shang Min Zhao Hong Jiang Yaoguo Huang Runqing Mu Wenxiang Chen Mingting Peng Chuanbao Zhang Jie Zeng Chenbin Li Hongling Yang Yongmei Jiang Jin Xu Guixia Li Hongbing Chen Yun Xiang sancheng Cao Zhenxin Guo Dapeng Chen Beijing Children's Hospital Capital Medical UniversityNational Center for Children's HealthBeijing 100045China Xi'an Children's Hospital Xi'an 710003China Henan Children's Hospital Zhengzhou Children's Hospital Zhengzhou 450053China Children's Hospital of Chongqing Medical University Chongqing 400014China The First Hospital of China Medical University Shenyang 110001China national center for Clinical Laboratories(NCCL) Beijing 100730China Guangzhou Women and Children's Medical center Guangzhou 510623China West China second University Hospital sichuan University Chengdu 610041China Children's Hospital of Fudan University Shanghai 210002China Children's Hospital of Hebei Province Shijiazhuang 050031China Children's Hospital of Nanjing Medical University Nanjing 210008China Wuhan Women and Children Medzcal Care Centes Wuhan 430016China

There is a lack of accurate pediatric reference intervals(RIs) in China, with most commonly used RIs established without consideration of the effect of age and gender. The Pediatric Reference Intervals in China(PRINCE) project aims to establish and verify pediatric RIs for 31 common laboratory *** project will be a large, multicenter cross-sectional study:14,490 healthy children and adolescents aged up to 19 years will be surveyed by 10 children's hospitals and one pediatric department of a university hospital. To evaluate the feasibility and efficiency of the study methods, 602 children were surveyed in the pilot phase of the PRINCE study in April 2017: it found that some measurands were distinctly age dependent and that there were differences between values for males and females. The results of the pilot study affirmed the necessity of the PRINCE project for Chinese pediatrics. The pilot also indicated potential difficulties in the full survey, e.g., difficulties in recruiting children aged under 3 years and insufficient collection of blood samples from infants. The operation of the PRINCE project has been modified based on the findings in the pilot study toward improving the validity of the PRINCE project and promoting its openness and transparency.

关键词： Reference interval Pediatric China Cross-sectional study