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A multicenter prospective study on the management of hepatoblastoma in children:a report from the Chinese Children's Cancer Group

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World Journal of Pediatrics 2024年第6期20卷 590-601页

作者： Meng-Jie tang xiao-Li ma Xiang-Ling He Wei-hua Pan xiao-Hong Zhang Sha-Yi Jiang Ju gao Fu Li Wei Yao Song Gu Wei-Ling Zhang Qiang Zhao Shi-Hao huang Yong-Jun Fang Wei liu Hui-Zhong Niu chun-Mei Wang Li-Rong Sun Hui gao Yun-Peng Dai Shun-Gen huang Zhi-Yong Zhong Xi-Ge Wang Zhong-Rong Li Liang-chun Yang Ye-Ming Wu huan-Min Wang Xin Sun xiao-Jun yuan Department of Pediatric Hematology/Oncology Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineNo.1665Kongjiang RoadYangpu DistrictShanghai 200092China Medical Oncology Department Pediatric Oncology CenterBeijing Children's HospitalCapital Medical UniversityNational Center for Children's HealthBeijing 100045China Department of Hematology/Oncology Children's Medical CenterHunan Provincial People's HospitalThe First Affiliated Hospital of Hunan Normal UniversityChangsha 41005China Department of Pediatric Surgery Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200092China Department of Hematology/Oncology Guangzhou Women and Children's Medical CenterNational Children's Medical Center for South Central RegionGuangzhou 510623China Department of Hematology Shanghai Children's HospitalShanghai Jiao Tong UniversityShanghai 200062China Department of Pediatrics West China Second University HospitalSichuan UniversityChengdu 610041China Department of Hematology/Oncology Qilu Children's Hospital of Shandong UniversityJinan 250022China Department of Pediatric Surgery Children's Hospital of Fudan UniversityShanghai 201102China Department of General Surgery Shanghai Children's Medical Center(National Children's Medical Center-Shanghai)Shanghai Jiao Tong University School of MedicineShanghai 200127China Department of Pediatrics Beijing Tongren HospitalCapital Medical UniversityBeijing 100730China Department of Pediatric Oncology Tianjin Medical University Cancer Institute and HospitalTianjin 300060China Department of Hematology/Oncology Children's Hospital of Nanjing Medical UniversityNanjing 210008China Department of Hematology/Oncology Henan Children's HospitalZhengzhou 450018China Department of Pediatric General Surgery Hebei Children's Hospital of Hebei Medical UniversityShijiazhuang City 050031China Children's Hospital the First Affliated Hospital of Zhengzhou UniversityZhengzhou 450052China Department of Paediatric

Background This study aimed to identify survival risk factors in Chinese children with hepatoblastoma(HB)and assess the effectiveness of the new treatment protocol proposed by the Chinese Children's Cancer Group(CCCG)in *** A multicenter,prospective study that included 399 patients with HB from January 2015 to June 2020 was *** demographics,treatment protocols,and other related information were *** regression models and Kaplan-Meier curve methods were *** The 4-year event-free survival(EFS)and overall survival(OS)were 76.9 and 93.5%,*** 4-year EFS rates for the very-low-risk,low-risk,intermediate-risk,and high-risk groups were 100%,91.6%,81.7%,and 51.0%,*** 4-year 0S was 100%,97.3%,94.4%,and 86.8%,*** regression analysis found that age,tumor rupture(R+),and extrahepatic tumor extension(E+)were independent prognostic factors.A total of 299 patients had complete remission,and 19 *** with declining alpha-fetoprotein(AFP)>75%after the first two cycles of neoadjuvant chemotherapy had a better EFS and OS than those≤75%.Conclusions The survival outcome of HB children has dramatically improved since the implementation of CCCG-HB-2016 ***≥8 years,R+,and E+were independent risk factors for *** with a declining AFP>75%after the first two cycles of neoadjuvant chemotherapy had better EFS and OS.

关键词： Alpha-fetoprotein Hepatoblastoma Multicenter Prospective study Survival

The In-Flight Realtime Trigger and Localization Software of GECAM

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Research in Astronomy and Astrophysics 2024年第10期24卷 11-24页

作者： xiao-Yun Zhao Shao-Lin Xiong Xiang-Yang Wen Xin-Qiao Li Ce Cai Shuo xiao Qi Luo Wen-Xi Peng Dong-Ya Guo Zheng-hua An Ke Gong jin-yuan Liao Yan-Qiu Zhang Yue huang Lu Li Xing Wen Fei Zhang jing Duan Chen-Wei Wang Dong-Li Shi Peng Zhang Qi-bin Yi Chao-Yang Li Yan-bing Xu xiao-hua Liang Ya-Qing liu Da-Li Zhang Xi-lei Sun Fan Zhang Gang Chen huan-Yu Wang Sheng Yang xiao-jing liu Min gao mao-Shun Li jin-Zhou Wang Xing Zhou Yi Zhao Wang-Chen Xue Chao Zheng Jia-Cong liu Xing-Bo Han jin-Ling Qi Jia huang Ke-Ke Zhang Can Chen Xiong-Tao Yang Dong-Jie Hou Yu-Sa Wang Rui Qiao Xiang ma xiao-Bo Li Ping Wang Xin-Ying Song Li-Ming Song Shi-Jie Zheng bing Li Hong-Mei Zhang Yue Zhu Wei Chen Jian-Jian He Zhen Zhang jin Hou Hong-Jun Wang Yan-Chao Hao Xiang-Yu Wang Zong-yuan Yang Zhi-Long Wen Zhi Chang yuan-yuan Du Rui gao xiao-Fei Lan Yan-Guo Li Gang Li Xu-Fang Li Fang-Jun Lu Hong Lu bin Meng Feng Shi Hui Wang Hui-Zhen Wang Yu-Peng Xu Jia-Wei Yang Xue-Juan Yang Shuang-Nan Zhang Chao-Yue Zhang Cheng-Mo Zhang Zhi-Cheng tang Cheng Cheng Key Laboratory of Particle Astrophysics Institute of High Energy PhysicsChinese Academy of SciencesBeijing 100049China University of Chinese Academy of Sciences Chinese Academy of SciencesBeijing 100049China School of Information Science and Technology Southwest Jiaotong UniversityChengdu 611756China Key Laboratory of Stellar and Interstellar Physics and Department of Physics Xiangtan UniversityXiangtan 411105China Physics and Space Science College China West Normal UniversityNanchong 637002China Department of Astronomy Beijing Normal UniversityBeijing 100875China Innovation Academy for Microsatellites of CAS Shanghai 201203China Changchun University of Science and Technology Changchun 130022China Chinese Academy of Sciences South America Center for Astronomy National Astronomical ObservatoriesCASBeijing 100101China

Realtime trigger and localization of bursts are the key functions of GECAM,an all-sky gamma-ray monitor launched on 2020 December *** developed a multifunctional trigger and localization software operating in the CPU of the GECAM Electronic *** onboard software has the following features:high trigger efficiency for real celestial bursts with a suppression of false triggers caused by charged particle bursts and background fluctuation,dedicated localization algorithm optimized for both short and long bursts,and low time latency of the trigger information which is downlinked through the Global Short Message Communication service of the global BeiDou navigation *** paper provides a detailed description of the design and development of the trigger and localization software system for *** covers the general design,workflow,the main functions,and the algorithms used in the *** paper also includes on-ground trigger tests using simulated gamma-ray bursts generated by a dedicated X-ray tube,as well as an overview of the performance for real celestial bursts during its in-orbit operation.

关键词： telescopes instrumentation:detectors methods:observational

Understanding the metabolism of Mycoplasma mycoides subsp. capri in vitro by a transcriptomic analysis

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Journal of Integrative Agriculture 2018年第2期17卷 428-435页

作者： WANG xiao-hui WANG Yan-fang huaNG Hai-bi BAI Fan SHI xiao-na ma Chang-jiao gao yuan ZHANG Jian-hua ZHANG Wen-guang HAO Yong-qing Key Laboratory of Microbiology & Immunology College of Veterinary Medicine Inner Mongolia Agricultural University Foundation Medicine College Baotou Medical School Key Laboratory of Animal Genetics and Genomics Inner Mongolia Agricultural University

It is generally known that the culture for mycoplasma is time-consuming and a variety of nutrients are needed in the culture medium. This brings a lot of difficulties to mycoplasma research and application, including Mycoplasma mycoides subsp. capri（Mmc）. Furthermore, little research on the characteristics of Mmc metabolism has been reported. In this study, Mmc PG3 strain cultures were investigated for dynamic gene expression. Culture samples were harvested during logarithmic phase（PG3-1）, stationary phase（PG3-2）, decline phase（PG3-3） and late decline phase（PG3-4）. Twelve RNA samples（three replicates for each of the four growth stages considered） from these cultures were collected and sequenced. Paired comparison between consecutive growth phases in the four growth stages showed 45 significant differentially expressed genes（P〈0.01） were linked to PG3 metabolism. The enzymes these genes coded were mainly involved in ATP synthase, pyrimidine metabolism, nicotinate and nicotinamide metabolism, arginine and proline metabolism. Among these, cytidylate kinase, fructose 1,6-bisphosphate aldolases Class II, nicotinate-nucleotide adenylyltransferase and dihydrolipoamide dehydrogenase play a key role in Mmc metabolism. These results provide a baseline to build our understanding of the metabolic pathway of Mmc.

关键词： metabolism Mycoplasma mycoides subsp.capri transcriptomic

CEPC Technical Design Report

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Radiation Detection Technology and Methods 2024年第1期8卷 I0003-I0016,1-1091页

作者： Waleed Abdallah Tiago Carlos Adorno de Freitas Konstantin Afanaciev Shakeel Ahmad Ijaz Ahmed xiaocong Ai Abid Aleem Wolfgang Altmannshofer Fabio Alves Weiming An Rui An Daniele Paolo Anderle Stefan Antusch Yasuo Arai Andrej Arbuzov Abdesslam Arhrib Mustafa Ashry Sha Bai Yu Bai Yang Bai Vipul Bairathi Csaba Balazs Philip Bambade Yong Ban Tripamo Bandyopadhyay Shou-Shan Bao Desmond P.Barber Ayse Bat Varvara Batozskaya Subash Chandra Behera Alexander Belyaev Michele Bertucci xiao-Jun bi yuanjie bi Tianjian bian Fabrizio bianchi Thomas biekotter Michela biglietti Shalva bilanishvili Deng binglin Denis Bodrov Anton Bogomyagkov Serge Bondarenko Stewart Boogert maarten Boonekamp marcello Borri Angelo Bosotti Vincent Boudry Mohammed Boukidi Igor Boyko Ivanka Bozovic Giuseppe Bozzi Jean-Claude Brient Anastasiia Budzinskaya masroor Bukhari Vladimir Bytev Giacomo Cacciapaglia hua Cai Wenyong Cai Wujun Cai Yijian Cai Yizhou Cai Yuchen Cai Haiying Cai huacheng Cai Lorenzo Calibbi Junsong Cang Guofu Cao Jianshe Cao Antoine Chance Xuejun Chang Yue Chang Zhe Chang Xinyuan Chang Wei Chao Auttakit Chatrabhuti Yimin Che Yuzhi Che bin Chen Danping Chen Fuqing Chen Fusan Chen Gang Chen Guoming Chen hua-Xing Chen Huirun Chen jinhui Chen Ji-yuan Chen Kai Chen mali Chen Mingjun Chen Mingshui Chen Ning Chen Shanhong Chen Shanzhen Chen Shao-Long Chen Shaomin Chen Shiqiang Chen Tianlu Chen Wei Chen Xiang Chen xiaoyu Chen Xin Chen Xun Chen Xurong Chen Ye Chen Ying Chen Yukai Chen Zelin Chen Zilin Chen Gang Chen Boping Chen chunhui Chen Hok Chuen Cheng huajie Cheng Shan Cheng Tongguang Cheng Yunlong Chi Pietro Chimenti Wen Han Chiu Guk Cho Ming-chung Chu xiaotong Chu Ziliang Chu Guglielmo Coloretti Andreas Crivellin Hanhua Cui xiaohao Cui Zhaoyuan Cui Brunella D'Anzi Ling-Yun Dai Xinchen Dai Xuwen Dai Antonio De maria Nicola De Filippis Christophe De La Taille Francesca De Mori Chiara De Sio Elisa Del Core Shuangxue Deng Wei-Tian Deng Zhi Deng Ziyan Deng Bhupal Dev tang Dewen biagio Di Micco Ran Ding Siqin Dingl Yadong Ding Haiyi Dong Jianin AGH University of Science and Technology Faculty of Physics and Applied Computer ScienceKrakow Anhui University HefeiAnhui Applied Physics Department Federal Urdu University of ArtsScience and TechnologyIslamabad Banaras Hindu University Institute of ScienceVaranasi Bandirma Onyedi Eylul University Bandirma Beihang University Beijing Beijing Computational Science Research Center Beijing Beijing Conveyi LTD Beijing Beijing Glass Research Institute Co. LtdBeijing Beijing Institute of Technology Beijing Beijing Normal University Beijing Brown University ProvidenceRI Budker Institute of Nuclear Physics Novosibirsk Cadi Ayyad University Laboratory of Fundamental and Applied PhysicsPolydisciplinary FacultyMarrakech Cairo University Giza Center for Theory and Computation National Tsing Hua UniversityHsinchu Central China Normal University WuhanHubei Central South University ChangshaHunan Centro de Fisica Teorica de Particulas Universidade Tecnica de LisboaLisboa Chengdu KaitengSifang Digital Radio&TV Equipment Co. LtdChengduSichuan China academy of engineering physics MianyangSichuan China building materials academy Beijing China Center of Advanced Science and Technology Beijing China Institute of Atomic Energy Beijing China Jiliang University HangzhouZhejiang China Nuclear Instrument CO. LTDBeijing China University of Geosciences Beijing China University of Geosciences WuhanHubei China University of Mining and Technology XuzhouJiangsu China West Normal University NanchongSichuan Chongqing University Department of PhysicsChongqing Chulalongkorn University Bangkok City University of Hong Kong Department of PhysicsHong Kong Cockcroft Institute Daresbury Cornell University IthacaNY CPPM(AMU/IPhU&CNRS/INP) Marseille Dalian Minzu University DalianLiaoning Dalian University of Technology Department of PhysicsDalian Deutsches Elektronen-Synchrotron DESY Hamburg Dzhelepov Laboratory of Nuclear Problems of Joint Institute for Nuclear Research(DLNP jinR) Dubna East China University Of Science And Technol

The Circular Electron Positron Collider(CEPC)is a large scientific project initiated and hosted by China,fostered through extensive collaboration with international *** complex comprises four accelerators:a 30 GeV Linac,a 1.1 GeV Damping Ring,a Booster capable of achieving energies up to 180 GeV,and a Collider operating at varying energy modes(Z,W,H,and tt).The Linac and Damping Ring are situated on the surface,while the subterranean Booster and Collider are housed in a 100 km circumference underground tunnel,strategically accommodating future expansion with provisions for a potential Super Proton Proton Collider(SPPC).The CEPC primarily serves as a Higgs *** its baseline design with synchrotron radiation(SR)power of 30 MW per beam,it can achieve a luminosity of 5×10^(34)cm^(-2)s^(-1)per interaction point(IP),resulting in an integrated luminosity of 13 ab^(-1)for two IPs over a decade,producing 2.6 million Higgs *** the SR power to 50 MW per beam expands the CEPC's capability to generate 4.3 million Higgs bosons,facilitating precise measurements of Higgs coupling at sub-percent levels,exceeding the precision expected from the HL-LHC by an order of *** Technical Design Report(TDR)follows the Preliminary Conceptual Design Report(Pre-CDR,2015)and the Conceptual Design Report(CDR,2018),comprehensively detailing the machine's layout,performance metrics,physical design and analysis,technical systems design,R&D and prototyping efforts,and associated civil engineering ***,it includes a cost estimate and a preliminary construction timeline,establishing a framework for forthcoming engineering design phase and site selection *** is anticipated to begin around 2027-2028,pending government approval,with an estimated duration of 8 *** commencement of experiments and data collection could potentially be initiated in the mid-2030s.

关键词： initiated exceeding precise

AlGaN/GaN Metal-Insulator-Semiconductor High Electron-Mobility Transistor Using a NbAlO/Al_(2)O_(3) Laminated Dielectric by Atomic Laver Deposition

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Chinese Physics Letters 2012年第2期29卷 221-224页

作者： bi Zhi-Wei HAO Yue FENG Qian gao Zhi-yuan ZHANG jin-Cheng maO Wei ZHANG Kai ma xiao-hua liu Hong-Xia YANG Lin-An MEI Nan CHANG Yong-Ming Key Lab of Fundamental Science for National on Wide Band-Gap Semiconductor Technology School of MicroelectronicsXidian UniversityXi’an 710071 Lab of Photoelectronic Technology School of Electronic and Control EngineeringBeijing University of TechnologyBeijing 100124

We investigate the characteristics of AlGaN/GaN metal-insulator-semiconductor high-electron-mobility transistors (MIS-HEMTs) with a NbAlO/Al_(2)O_(3) lamination dielectric deposited by atomic layer deposition (ALD) as the gate insulator.A large gate voltage swing (GVS) of 3.96 V and a high breakdown voltage of-150 V for the MIS-HEMT were *** present the gate leakage current mechanisms and analyze the reason for the reduction of the leakage *** with traditional HEMTs,the maximum drain current is improved to 960ma/mm,indicating that NbAlO layers could reduce the surface-related depletion of the channel layer and increase the sheet carrier *** addition,the maximum oscillation frequency of 38.8 GHz shows that the NbAlO high-k dielectric can be considered as a potential gate oxide comparable with other dielectric insulators.

关键词： AlGaN/GaN HEMTs drain

Epidemiological Survey of Hemoglobinopathies Based on Next-Generation Sequencing Platform in Hunan Province,China

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biomedical and Environmental Sciences 2023年第2期36卷 127-134页

作者： XI Hui liu Qin XIE Dong hua ZHOU Xu tang Wang Lan tang De Guo ZENG chun Yan WANG Qiong NIE Xing Hui PENG jin Ping gao xiao Ya WU Hong Liang ZHANG Hao Qing QIU Li FENG Zong Hui WANG Shu yuan ZHOU Shu Xiang HE Jun ZHOU Shi Hao ZHOU Fa Qun ZHENG Jun Qing WANG Shun Yao CHEN Shi Ping ZHENG Zhi Fen ma xiao yuan FANG Jun Qun LIANG Chang biao WANG hua NHC Key Laboratory of birth Defect for Research and Prevention Hunan Provincial Maternal and Child Health Care HospitalChangsha 410028HunanChina Yongzhou Hospital for maternal and Child Health Care Yongzhou 425000HunanChina Changde Hospital for maternal and Child Health Care Changde 415099HunanChina Hengyang Hospital for maternal and Child Health Care Hengyang 421009HunanChina Shaoyang Hospital for maternal and Child Health Care Shaoyang 422001HunanChina Yiyang Hospital for maternal and Child Health Care Yiyang 413099HunanChina Yueyang Hospital for maternal and Child Health Care Yueyang 414021HunanChina Center of Prenatal Diagnosis Chenzhou No.1 People’s HospitalChenzhou 423099HunanChina Loudi Hospital for maternal and Child Health Care Loudi 417599HunanChina huaihua Hospital for maternal and Child Health Care Huaihua 418099HunanChina Xiangtan Hospital for maternal and Child Health Care Xiangtan 411104HunanChina Zhuzhou Hospital for maternal and Child Health Care Zhuzhou 412008HunanChina Changsha Hospital for maternal and Child Health Care Changsha 410007HunanChina Xiangxi Tujia and Miao Autonomous Prefecture Hospital for maternal and Child Health Care Jishou 416000HunanChina Zhang Jiajie Centre for maternal and child health family planning services Zhangjiajie 427299HunanChina BGI Genomics BGI-ShenzhenShenzhen 518083GuangdongChina Clinical Laboratory of BGI Health BGI-ShenzhenShenzhen 518083GuangdongChina BGI-Wuhan Clinical Laboratories BGI-ShenzhenWuhan 430074HubeiChina SEbiA Representative Office Shanghai 200001China

Objective This study was aimed at investigating the carrier rate of,and molecular variation in,α-andβ-globin gene mutations in Hunan *** We recruited 25,946 individuals attending premarital screening from 42 districts and counties in all 14 cities of Hunan *** screening was performed,and molecular parameters were *** The overall carrier rate of thalassemia was 7.1%,including 4.83%forα-thalassemia,2.15%forβ-thalassemia,and 0.12%for bothα-andβ-*** highest carrier rate of thalassemia was in Yongzhou(14.57%).The most abundant genotype ofα-thalassemia andβ-thalassemia was-α^(3.7)/αα(50.23%)andβ^(IVS-Ⅱ-654)/β^(N)(28.23%),***α-globin mutations[CD108(ACC>AAC),CAP+29(G>C),Hb Agrinio and Hb Cervantes]and sixβ-globin mutations[CAP+8(C>T),IVS-Ⅱ-848(C>T),-56(G>C),beta nt-77(G>C),codon 20/21(-TGGA)and Hb Knossos]had not previously been identified in ***,this study provides the first report of the carrier rates of abnormal hemoglobin variants andα-globin triplication in Hunan Province,which were 0.49%and 1.99%,*** Our study demonstrates the high complexity and diversity of thalassemia gene mutations in the Hunan *** results should facilitate genetic counselling and the prevention of severe thalassemia in this region.

关键词： Thalassemia Carrier rate Molecular spectrum Abnormal hemoglobin variants α-globin triplication

Self-powered solar-blind photodiodes based on EFG-grown(100)-dominant β-Ga_(2)O_(3) substrate

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Chinese Physics B 2021年第1期30卷 483-486页

作者： Xu-Long Chu Zeng liu Yu-Song Zhi yuan-yuan liu Shao-Hui Zhang Chao Wu Ang gao Pei-Gang Li Dao-You Guo Zhen-Ping Wu Wei-hua tang Laboratory of Information Functional materials and Devices School of Science&State Key Laboratory of Information Photonics and Optical CommunicationsBeijing University of Posts and TelecommunicationsBeijing 100876China Center of materials Science and Optoelectronics Engineering University of Chinese Academy of SciencesBeijing 100049China The Engineering Research Center for Semiconductor Integrated Technology Institute of SemiconductorsChinese Academy of SciencesBeijing 100083China China Aerospace System Simulation Technology Co. Ltd.(Beijing)Beijing 100195China Division of Interdisciplinary and Comprehensive Research&Platform for Characterization and Test Suzhou Institute of Nano-Tech and Nano-BionicsChinese Academy of SciencesSuzhou 215123China Center for Optoelectronics materials and Devices&Key Laboratory of Optical Field manipulation of Zhejiang Province Department of PhysicsZhejiang Sci-Tech UniversityHangzhou 310018China School of Nano-Tech and Nano-bionics University of Science and Technology of ChinaHefei 230026China College of Electronic and Optical Engineering&College of Microelectronics Nanjing University of Posts and TelecommunicationsNanjing 210046China National and Local Joint Engineering Laboratory for RF Integration and Micro-Packing Technologies Nanjing University of Posts and TelecommunicationsNanjing 210046China

We report the edge-defined-film-fed(EFG)-grown β-Ga_(2)O_(3)-based Schottky *** device has a reverse leakage current of ~nA and a rectified ratio of ~10^(4) at ±5 *** addition,the photodiode detector shows a dark current of 0.3 pA,a photo-responsivity(R) of 2.875 ma/W,a special detectivity(D*) of 10^(10) Jones,and an external quantum efficiency(EQE) of 1.4% at zero bias,illustrating a self-powered *** work may advance the development of the Ga_(2)O_(3)-based Schottky diode solar-blind photodetectors.

关键词： β-Ga_(2)O_(3)substrate Schottky photodiode solar-blind detection

Responsible genes in children with primary vesicoureteral reflux: findings from the Chinese Children Genetic Kidney Disease Database

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World Journal of Pediatrics 2021年第4期17卷 409-418页

作者： Jia-Lu liu Qian Shen Ming-Yan Wu Guang-hua Zhu Yu-Feng Li xiao-Wen Wang xiao-Shan tang Yun-Li bi Yi-Nv Gong jing Chen xiao-Yan Fang Yi-Hui Zhai bing-bing Wu Guo-Min Li Yu Bo Sun xiao-Jie gao Cui-hua liu xiao-Yun Jiang Sheng Hao Yu-Lin Kang Ying-Liang Gong Li-Ping Rong Di Li Si Wang Duan ma Jia Rao Hong Xu Chinese Children Genetic Kidney Disease Database(CCGKDD) “Internet Plus”Nephrology Alliance of the National Center for Children’s Care Department of Nephrology Children’s Hospital of Fudan UniversityNational Children’s Medical Center399 Wan Yuan RoadShanghai201102People’s Republic of China Shanghai Kidney Development and Pediatric Kidney Disease Research Center ShanghaiChina Department of Nephrology Shanghai Children’s HospitalShanghaiChina Department of Pediatric Nephrology Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghaiChina Wuhan Children’s Hospital Tongji Medical CollegeHuazhong University of Science and TechnologyWuhanChina Department of Urology Children’s Hospital of Fudan UniversityNational Children’s Medical CenterShanghaiChina Department of Rheumatology Children’s Hospital of Fudan UniversityNational Children’s Medical CenterShanghaiChina Clinical Genetic Center Children’s Hospital of Fudan UniversityNational Children’s Medical CenterShanghaiChina Department of Nephrology Shenzhen Children’s HospitalShenzhenGuangdongChina Department of Nephrology and Rheumatology Children’s Hospital Affiliated to Zhengzhou UniversityZhengzhou Key Laboratory of Pediatric Kidney Disease ResearchZhengzhouChina The First Affiliated Hospital of Zhongshan University GuangzhouChina Key Laboratory of Metabolism and Molecular Medicine Ministry of EducationDepartment of Biochemistry and Molecular BiologyInstitutes of Biomedical SciencesSchool of Basic Medical SciencesFudan UniversityShanghaiChina 不详

Background Primary vesicoureteral reflux(VUR)is a common congenital anomaly of the kidney and urinary tract(CAKUT)in *** present study identified the possible genetic contributions to primary VUR in *** Patients with primary VUR were enrolled and analysed based on a national multi-center registration network(Chinese Children Genetic Kidney Disease Database,CCGKDD)that covered 23 different provinces/regions in China from 2014 to *** causes were sought using whole-exome sequencing(WES)or targeted-exome *** A total of 379 unrelated patients(male:female 219:160)with primary VUR were ***-four(16.9%)children had extrarenal manifestations,and 165(43.5%)patients showed the coexistence of other CAKUT ***-eight patient(23.2%)exhibited impaired renal function at their last visit,and 18 of them(20.5%)developed ESRD at the median age of 7.0(IQR 0.9–11.4)years.A monogenic cause was identified in 28 patients(7.39%).These genes included PAX2(n=4),TNXB(n=3),GATA3(n=3),SLIT2(n=3),ROBO2(n=2),TBX18(n=2),and the other 11 genes(one gene for each patient).There was a significant difference in the rate of gene mutations between patients with or without extrarenal complications(14.1%vs.6%,P=0.035).The frequency of genetic abnormality was not statistically significant based on the coexistence of another CAKUT(9.6%vs.5.6%,P=0.139,Chi-square test)and the grade of reflux(9.4%vs.6.7%,P=0.429).Kaplan–Meier survival curve showed that the presence of genetic mutations did affect renal survival(Log-rank test,P=0.01).PAX2 mutation carriers(HR 5.1,95%CI 1.3–20.0;P=0.02)and TNXB mutation carriers(HR 20.3,95%CI 2.4–168.7;P=0.01)were associated with increased risk of progression to *** PAX2,TNXB,GATA3 and SLIT2 were the main underlying monogenic causes and accounted for up to 46.4%of monogenic *** complications and renal function were significantly related to the findings of genetic factors in children with p

关键词： Children Congenital anomalies of the kidney and urinary tract Gene mutation Vesicoureteral reflux Whole-exome sequencing

Catheter ablation versus medical therapy for atrial fibrillation with prior stroke history:a prospective propensity score-matched cohort study

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Journal of Geriatric Cardiology 2023年第10期20卷 707-715,I0006页

作者： Wen-Li DAI Zi-Xu ZHAO Chao JIANG liu HE Ke-Xin YAO Yu-Feng WANG Ming-Yang gao Yi-Wei LAI jing-Rui ZHANG Ming-xiao LI Song ZUO Xue-yuan GUO Ri-Bo tang Song-Nan LI Chen-Xi JIANG Nian liu De-Yong LONG Xin DU Cai-hua SANG Jian-Zeng DONG Chang-Sheng ma Department of Cardiology Beijing Anzhen HospitalCapital Medical UniversityNational Clinical Research Center for Cardiovascular DiseasesBeijingChina Heart Health Research Center BeijingChina Department of Cardiovascular Medicine the First Affiliated Hospital of Zhengzhou UniversityZhengzhouChina

BACKGROUND Patients with atrial fibrillation(AF)and prior stroke history have a high risk of cardiovascular events despite anticoagulation *** is unclear whether catheter ablation(CA)has further benefits in these *** AF patients with a previous history of stroke or systemic embolism(SE)from the prospective Chinese Atrial Fibrillation Registry study between August 2011 and December 2020 were included in the *** were matched in a 1:1 ratio to CA or medical treatment(MT)based on propensity *** primary outcome was a composite of all-cause death or ischemic stroke(IS)/*** During a total of 4.1±2.3 years of follow-up,the primary outcome occurred in 111 patients in the CA group(3.3 per 100 person-years)and in 229 patients in the MT group(5.7 per 100 person-years).The CA group had a lower risk of the primary outcome compared to the MT group[hazard ratio(HR)=0.59,95%CI:0.47–0.74,Pbined risk of all-cause death or IS/*** clinical trials are warranted to confirm the benefits of CA in these patients.

关键词： prospective fibrillation matched