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Clinical and genetic characteristics of mucopolysaccharidosis type Ⅵ according to the Russian registry
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作者 Anastasia O Vechkasova Ekaterina Yu Zakharova +5 位作者 Natalia V Buchinskaya Nato D Vashakmadze Leyla S Namazova-Baranova Dmitry O Ivanov Sergei I Kutsev Mikhail M Kostik 《World Journal of Clinical Pediatrics》 2025年第4期277-287,共11页
BACKGROUND Mucopolysaccharidosis typeⅥ(MPSⅥ)is a chronic,progressive,inherited disease with multiorgan involvement and a restricted life expectancy.AIM To investigate the epidemiological,clinical,and genetic charact... BACKGROUND Mucopolysaccharidosis typeⅥ(MPSⅥ)is a chronic,progressive,inherited disease with multiorgan involvement and a restricted life expectancy.AIM To investigate the epidemiological,clinical,and genetic characteristics of patients with mucopolysaccharidosis type 6 and their outcomes using the Russian Federation's national registry,as per the Russian registry,and compare them with previously published data.METHODS In a retrospective cohort study,clinical,laboratory data,molecular genetic analysis results,and enzyme replacement therapy(ERT)data were extracted and analyzed from the Russian MPSⅥregistry for 53 patients,comprising 26 males(49.1%)and 27 females(50.9%).RESULTS The median age of first symptoms was 2 years,ranging from the first months of life to 20 years.A positive family history of MPSⅥwas reported in 19/53(35.8%)patients,a negative family history in 24(45.3%),and missing information in 10(18.9%).The main features of the disease were hepatomegaly(n=23;60.5%),splenomegaly(n=15,39.5%),involvement of otolaryngological organs(n=24/33;72.7%),umbilical and inguinal hernia(n=19/36;52.8%),heart involvement(n=26/32;81.3%)with valve involvement(n=25/26;96.2%)and linear growth delay(n=30/39,76.9%).Two patients(3.8%)died.The most common variants identified in the ARSB gene were c.454C>T and c.194C>T.At the time of data collection,ERT had ever received 48/53(90.5%)patients.CONCLUSION No correlation was observed between the age of onset of the first symptoms,the severity of clinical manifestations,enzyme activity,or nucleotide variants in the ARSB gene. 展开更多
关键词 Mucopolysaccharidosis typeⅥ ARSB Enzyme replacement therapy Genotype-phenotype correlation registER Russian Federation
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The Study of Legal Property of Household Registration System in Our Country
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作者 Linfeng Zheng Shiyou Wang 《International Journal of Technology Management》 2013年第7期103-105,共3页
From "the case that three girls have some fate but have different value" to "the case that Hai Cheng took a police substation to court with three times", currently the household registration system of our country ... From "the case that three girls have some fate but have different value" to "the case that Hai Cheng took a police substation to court with three times", currently the household registration system of our country not only plays the role of account registration and account management, but also undertakes the limitation of migration movement, and for the additional functions of the lowest life guarantee, social insurance, compulsory education, employment and other social security benefits, it also makes a difference between local registered permanent residence habitant and nonlocal registered permanent residence habitant. Just because of these additional functions, the household registration system has become complicated, which originally has clear nature. Therefore, only it is necessary to define the legal nature of our country's current household registration system, can executive power be supervised and the rights of citizens get relief. 展开更多
关键词 Household registration Examination and approval of registered permanent residence migration Legal nature Reform of household registration system.
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基于分布式应用的系统注册服务Register的实现 被引量:2
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作者 童晓阳 高培文 《小型微型计算机系统》 EI CSCD 北大核心 2000年第9期925-927,共3页
本文研究的是分布式集成体系结构中的基本系统服务之一注册服务 Register的实现 .介绍了注册服务 Register在分布式集成体系结构中的任务和作用 ,对其内部工作机制和工作流程进行剖析 ,说明了 Register所实现的功能 ,最后对提高 Registe... 本文研究的是分布式集成体系结构中的基本系统服务之一注册服务 Register的实现 .介绍了注册服务 Register在分布式集成体系结构中的任务和作用 ,对其内部工作机制和工作流程进行剖析 ,说明了 Register所实现的功能 ,最后对提高 Register性能的几个问题进行了分析 . 展开更多
关键词 系统服务 注册服务 registER 分布式操作系统
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Analysis of the Nationally Registered Rice Varieties in the Last Decade
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作者 张现伟 李经勇 +4 位作者 官治文 姚雄 肖人鹏 刘强明 唐永群 《Agricultural Science & Technology》 CAS 2017年第3期487-489,共3页
The nationally registered rice varieties during 2006-2015 were analyzed and there were 49.4 varieties on average annually registered in the last decade. In terms of the structure of varieties, there was a decreasing t... The nationally registered rice varieties during 2006-2015 were analyzed and there were 49.4 varieties on average annually registered in the last decade. In terms of the structure of varieties, there was a decreasing trend for the proportion of three-line hybrid rice varieties but a rapid increase for that of two-line hybrid rice varieties. The percentage of rice varieties developed by scientific research institu- tions was decreasing but that developed by seed companies kept a rapid increase, indicating the commercial breeding conducted by seed companies has become a principal model in rice breeding. With regard to grain quality of varieties, the con- ventional rice varieties showed an increasing trend while the hybrid rice varieties had a decreasing trend in the proportion of high quality varieties, indicating hybrid rice needs improving more in grain quality in breeding programs. 展开更多
关键词 Rice NationaUy registered Variety Breeding body Varietal type High-quality rate
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Gender Differences in Non-English Major Graduates' English Compositions——A Study Based on the Use of Oral/Written Register Features
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作者 武蕊 《Sino-US English Teaching》 2007年第1期24-29,共6页
This article applies the theories of gender differences in language use, makes a survey of non-English major graduates' English writing, and analyzes the gender differences in EFL writing proficiency and particularly... This article applies the theories of gender differences in language use, makes a survey of non-English major graduates' English writing, and analyzes the gender differences in EFL writing proficiency and particularly in the use of oral/written register features, aiming to provide suggestions for gendered teaching in EFL writing. 展开更多
关键词 GENDER DIFFERENCE EFL writing oral and written register feature
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An Analysis of the Variation of Euphemism Under Register Theory
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作者 徐静 《海外英语》 2011年第2X期247-247,249,共2页
The euphemism is a phenomenon of language variation with different language varieties. And this phenomenon is closely related with social context. Register, as a concrete representation of context, especially its teno... The euphemism is a phenomenon of language variation with different language varieties. And this phenomenon is closely related with social context. Register, as a concrete representation of context, especially its tenor of discourse, makes great influence on what kind of euphemism and what level of euphemism people will use to achieve effective communication. Therefore, euphemism as language variation will be analyzed from the perspective of tenor of discourse in Halliday's Register Theory in order to enhance learners' pragmatic awareness and pragmatic competence when learning English euphemism or in daily communication. 展开更多
关键词 EUPHEMISM LANGUAGE VARIATION registER TENOR of DISCOURSE
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Registered nurse-administered sedation for gastrointestinal endoscopic procedure 被引量:2
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作者 Somchai Amornyotin 《World Journal of Gastrointestinal Endoscopy》 CAS 2015年第8期769-776,共8页
The rising use of nonanesthesiologist-administered sedation for gastrointestinal endoscopy has clinical significances. Most endoscopic patients require some forms of sedation and/or anesthesia. The goals of this sedat... The rising use of nonanesthesiologist-administered sedation for gastrointestinal endoscopy has clinical significances. Most endoscopic patients require some forms of sedation and/or anesthesia. The goals of this sedation are to guard the patient's safety, minimize physical discomfort, to control behavior and to diminish psychological responses. Generally, moderate sedation for these procedures has been offered by the non-anesthesiologist by using benzodiazepines and/or opioids. Anesthesiologists and non-anesthesiologist personnel will need to work together for these challenges and for safety of the patients. The sedation training courses including clinical skills and knowledge are necessary for the registered nurses to facilitate the patient safety and the successful procedure. However, appropriate patient selection and preparation, adequate monitoring and regular training will ensure that the use of nurse-administered sedation is a feasible and safe technique for gastrointestinal endoscopic procedures. 展开更多
关键词 registered NURSE SEDATION Gastrointestinalendoscopy SAFETY COMPLICATION
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Anomaly Detection of Store Cash Register Data Based on Improved LOF Algorithm 被引量:3
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作者 Ke Long Yuhang Wu Yufeng Gui 《Applied Mathematics》 2018年第6期719-729,共11页
As the cash register system gradually prevailed in shopping malls, detecting the abnormal status of the cash register system has gradually become a hotspot issue. This paper analyzes the transaction data of a shopping... As the cash register system gradually prevailed in shopping malls, detecting the abnormal status of the cash register system has gradually become a hotspot issue. This paper analyzes the transaction data of a shopping mall. When calculating the degree of data difference, the coefficient of variation is used as the attribute weight;the weighted Euclidean distance is used to calculate the degree of difference;and k-means clustering is used to classify different time periods. It applies the LOF algorithm to detect the outlier degree of transaction data at each time period, sets the initial threshold to detect outliers, deletes the outliers, and then performs SAX detection on the data set. If it does not pass the test, then it will gradually expand the outlying domain and repeat the above process to optimize the outlier threshold to improve the sensitivity of detection algorithm and reduce false positives. 展开更多
关键词 CASH registER Data ANOMALY Detection K-Means Clustering Optimized LOF ALGORITHM SAX Test
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Use of Service Middleware Based on ECHO with CSW for Discovery and Registry of MODIS Data 被引量:2
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作者 CHEN Zeqiang CHEN Nengcheng 《Geo-Spatial Information Science》 2010年第3期191-200,共10页
Nowadays, NASA is producing several terabytes Moderate Resolution Imaging Spectroradiometer (MODIS) data everyday; how to find the data with criteria, such as specific times, locations, and scales using an internati... Nowadays, NASA is producing several terabytes Moderate Resolution Imaging Spectroradiometer (MODIS) data everyday; how to find the data with criteria, such as specific times, locations, and scales using an international standard becomes more and more important. In this paper, a service-oriented architecture for use of the integration Earth Observation System Clearing- HOuse (ECHO) with the Open Geospatial Consortium (OGC) Catalogue Service--Web profile (CSW) is put forward. The architecture consists of three roles: a service requester (the user), a service provider (the ECHO metadata server), and a service broker (the GeoNetwork CSW and MODIS registry service middleware). The core component-MODIS registry service middleware includes three components: metadata fetcher, metadata transformer, and metadata register. The metadata fetcher is used to fetch metadata from ECHO metadata server; the metadata transformer is responsible for transform metadata from one form to another; the metadata register is in charge of registering ISO19139-based metadata to CSW. A prototype system is designed and implemented by using the service middleware technology and a standard interface and protocol. The feasibility and the response time of registry and retrieval of MODIS data are evaluated by means of a realistic LPDAAC_ECS MODIS data center. The implementation of this prototype system and the experiment show that the architecture and method is feasible and effective. 展开更多
关键词 ECHO GeoNetwork ISO19139 model mapping metadata transform metadata register
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A distributed cross-domain register filefor reconfigurable cryptographic processor 被引量:1
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作者 Zhang Baoning Ge Wei Wang Zhen 《Journal of Southeast University(English Edition)》 EI CAS 2017年第3期260-265,共6页
Due to the fact that the register files seriously affect the performance and area of coarse-grained reconfigurable cryptographic processors, an efficient structure of the distributed cross-domain register file is prop... Due to the fact that the register files seriously affect the performance and area of coarse-grained reconfigurable cryptographic processors, an efficient structure of the distributed cross-domain register file is proposed to realize a cryptographic processor with a high performance and a lowarea cost. In order to meet the demands of high performance and high flexibility at a lowarea cost, a union structure with the multi-ports access structure, i, e., a distributed crossdomain register file, is designed by analyzing the algorithm features of different ciphers. Considering different algorithm requirements of the global register files and local register files,the circuit design is realized by adopting different design parameters under TSMC( Taiwan Semiconductor Manufacturing Company) 40 nm CMOS( complementary metal oxide semiconductor) technology and compared with other similar works. The experimental results showthat the proposed distributed cross-domain register structure can effectively improve the performance of the unit area, of which the total performance of block per cycle is improved by17. 79% and performance of block per cycle per area is improved by 117%. 展开更多
关键词 RECONFIGURABLE PROCESSOR BLOCK CIPHER parallelimplementation registER FILE
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Impact of Enterprise Ownership and Size on Registered Hematotoxicity in Benzene-exposed Workers 被引量:3
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作者 WANG Bo Shen HAN Lei +7 位作者 YANG Dan Dan ZHANG Juan YIN Li Hong ZHANG Meng Ying XU Kai XING Cai Hong ZHU Bao Li PU Yue Pu 《Biomedical and Environmental Sciences》 SCIE CAS CSCD 2018年第12期917-921,共5页
Benzene is a classified as ahuman carcinogen that can cause myelodysplastic syndrome and acute myelogenousleukemia;.This compound is also a potent toxin suppressing the bone marrow.Accordingly,benzene causes serious h... Benzene is a classified as ahuman carcinogen that can cause myelodysplastic syndrome and acute myelogenousleukemia;.This compound is also a potent toxin suppressing the bone marrow.Accordingly,benzene causes serious hematological adverse effects,such as pancytopenia and aplastic 展开更多
关键词 SOE Impact of Enterprise Ownership and Size on registered Hematotoxicity in Benzene-exposed Workers TWA
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Paleoproterozoic Multistage Metamorphic Ages Registered in the Precambrian Basement Rocks at the Southeastern Margin of the North China Craton and Their Geological Implications 被引量:7
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作者 LIU Yican ZHANG Pingang +1 位作者 WANG Chengcheng NIE Jiazhen 《Acta Geologica Sinica(English Edition)》 SCIE CAS CSCD 2016年第6期2265-2266,共2页
The Precambrian basement rocks in the Bengbu and neighboring areas, located at the southeastern margin of the North China Craton, occur as granulite terrains and xenoliths in the Mesozoic dioritic porphyry.
关键词 Pb Paleoproterozoic Multistage Metamorphic Ages registered in the Precambrian Basement Rocks at the Southeastern Margin of the North China Craton and Their Geological Implications
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Improving on Linear Scan Register Allocation 被引量:1
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作者 Shahrzad Kananizadeh Kirill Kononenko 《International Journal of Automation and computing》 EI CSCD 2018年第2期228-238,共11页
Register allocation is a major step for all compilers. Various register allocation algorithms have been developed over the dec- ades. This work describes a new class of rapid register allocation algorithms and present... Register allocation is a major step for all compilers. Various register allocation algorithms have been developed over the dec- ades. This work describes a new class of rapid register allocation algorithms and presents experimental data on their behavior. Our re- search encourages the avoidance of graphing and graph-coloring based on the fact that precise graph-coloring is nondeterministic poly- nomial time-complete (NP-complete), which is not suitable for real-time tasks. In addition, practical graph-coloring algorithms tend to use polynomial-time heuristics. In dynamic compilation environments, their super linear complexity makes them unsuitable for register allocation and code generation. Existing tools for code generation and register allocation do not completely fulfill the requirements of fast compilation. Existing approaches either do not allow for the optimization of register allocation to be achieved comprehensively with a sufficient degree of performance or they require an unjustifiable amount of time and/or resources. Therefore, we propose a new class of register allocation and code generation algorithms that can be performed in linear time. These algorithms are based on the mathematic- al foundations of abstract interpretation and the computation of the level of abstraction. They have been implemented in a specialized library for just-in-time compilation. The specialization of this library involves the execution of common intermediate language (CIL) and low level virtual machine (LLVM) with a focus on embedded systems. 展开更多
关键词 register allocation just-in-time compilation code generation static analysis dynamic analysis.
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Stability of Nonlinear Feedback Shift Registers with Periodic Input 被引量:2
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作者 Bo Gao Xuan Liu +5 位作者 Xiaobo Wu Shudong Li Zhongzhou Lan Hui Lu Boyan Liu 《Computers, Materials & Continua》 SCIE EI 2020年第2期833-847,共15页
The stability of Non-Linear Feedback Shift Registers(NFSRs)plays an important role in the cryptographic security.Due to the complexity of nonlinear systems and the lack of efficient algebraic tools,the theorems relate... The stability of Non-Linear Feedback Shift Registers(NFSRs)plays an important role in the cryptographic security.Due to the complexity of nonlinear systems and the lack of efficient algebraic tools,the theorems related to the stability of NFSRs are still not well-developed.In this paper,we view the NFSR with periodic inputs as a Boolean control network.Based on the mathematical tool of semi-tensor product(STP),the Boolean network can be mapped into an algebraic form.Through these basic theories,we analyze the state space of non-autonomous NFSRs,and discuss the stability of an NFSR with periodic inputs of limited length or unlimited length.The simulation results are provided to prove the efficiency of the model.Based on these works,we can provide a method to analyze the stability of the NFSR with periodic input,including limited length and unlimited length.By this,we can efficiently reduce the computational complexity,and its efficiency is demonstrated by applying the theorem in simulations dealing with the stability of a non-autonomous NFSR. 展开更多
关键词 Non-Linear Feedback Shift register(NFSR) Boolean Network(BN) Semi-Tensor Product(STP) transition matrix STABILITY periodic input
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Relationship of academic courses and clinical internships to performance on the National Qualified Examination for Registered Professional Nurses (NQEX-RPN) 被引量:1
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作者 Huimin Xiao Weiwei Wu +1 位作者 Yan Lin Xuan Zhang 《International Journal of Nursing Sciences》 2014年第4期400-404,共5页
Background:Success in the National Qualified Examination for Registered Professional Nurses(NQEX-RPN)is a critical issue for each nursing student and every nursing school.However,to date,little research has been condu... Background:Success in the National Qualified Examination for Registered Professional Nurses(NQEX-RPN)is a critical issue for each nursing student and every nursing school.However,to date,little research has been conducted regarding the degree of risk for licensure failure associated with specific academic courses and clinical internships in China's Mainland.Objective:This study aimed to explore the relationship of academic courses and clinical internships to performance on the NQEX-RPN in China's Mainland.Methods:This was a retrospective descriptive study.Two hundred twenty-eight new graduates were recruited froma four-year baccalaureate nursing programin China's Mainland.The scores of academic courses and clinical internships were recorded from the Teaching and Learning administration of the university.The NQEX-RPN performance data was collected from the Provincial Health Department.Personal information was collected from the participants.Multiple stepwise regression was used to identify predictors of the NQEX-RPN.Results:Twenty-three courses and four clinical internships were positively associated with NQEX-RPN performance.Surgical nursing,Medical nursing,Computer basics and Community health care explained the 36.2%of the total variance of Theoretical Knowledge(F=31.313,p=0.000);clinical internships of obstetrics and gynecology nursing,pediatrics and surgical nursing explained the 28.1%of the total variance of Application Skills(F=28.852,p=0.000).Conclusion:Surgical-medicine nursing,Community health care,Computer basics and clinical internships are beneficial in assisting students and educators to identify the risk of students'performance in the NQEX-RPN. 展开更多
关键词 Nursing baccalaureate Education National Qualified Examination for registered Professional Nurses Regress analysis
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Cochrane方法学数据库 (Cochrane Methodology Register)正式收录《中国循证医学杂志》 被引量:1
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《中国循证医学杂志》 CSCD 2004年第12期833-833,891,共2页
关键词 中国循证医学杂志 文章 CMR COCHRANE Cochrane Methodology register 数据库 数据库系统
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Register Reallocation for Soft Error Reduction 被引量:1
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作者 WEN Peng YAN Guochang +1 位作者 LI Xuhui YING Shi 《Wuhan University Journal of Natural Sciences》 CAS 2014年第6期519-525,共7页
Subsequently to the problem of performance and energy overhead, the reliability problem of the system caused by soft error has become a growing concern. Since register file(RF) is the hottest component in processor,... Subsequently to the problem of performance and energy overhead, the reliability problem of the system caused by soft error has become a growing concern. Since register file(RF) is the hottest component in processor, if not well protected, soft errors occurring in it will do harm to the system reliability greatly. In order to reduce soft error occurrence rate of register file, this paper presents a method to reallocate the register based on the fact that different live variables have different contribution to the register file vulnerability(RFV). Our experimental results on benchmarks from MiBench suite indicate that our method can significantly enhance the reliability. 展开更多
关键词 register allocation soft error reliability
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Register varieties and college English writing teaching
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作者 张昀 《Sino-US English Teaching》 2008年第7期29-31,共3页
Register, or the functional variety of language, is presented in situational contexts. The situational context is composed of field, tenor and mode of discourse, which manifests the register's features. An author is ... Register, or the functional variety of language, is presented in situational contexts. The situational context is composed of field, tenor and mode of discourse, which manifests the register's features. An author is to make different choices to express meanings, i.e., to choose expressions among potential meanings that are appropriate to contexts. The paper intends to present the instructive and practical values of register in English writing teaching. 展开更多
关键词 registER VARIETY situational context CHOICE
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The global registry of COVID-19 clinical trials: indicating the design of traditional Chinese medicine clinical trials 被引量:1
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作者 Xuxu Wei Mengzhu Zhao +6 位作者 Chen Zhao Xiaoyu Zhang Ruijin Qiu Yiyi Lin Yang Sun Manke Guan Hongcai Shang 《TMR Modern Herbal Medicine》 2020年第3期140-146,共7页
Objective:To analyze the registration information of Corona Virus Disease 2019(COVID-19)related clinical trials from all the clinical trial registry accepted by the International Committee of Medical Journal Editors(I... Objective:To analyze the registration information of Corona Virus Disease 2019(COVID-19)related clinical trials from all the clinical trial registry accepted by the International Committee of Medical Journal Editors(ICMJE).Methods:All the database of ICMJE-accepted clinical trial registry platform were searched for COVID-19 related clinical trials that registered from December 8,2019 to February 19,2020.Results:All the database totally contained data of 209 COVID-19 related clinical trials,including 66(31.6%)traditional Chinese medicine(TCM)related studies and 143(68.4%)non-TCM related studies.176(84.2%)clinical trials were registered in ChiCTR,as 33(15.8%)were registered in ClinicalTrials.gov.There were 152(72.7%)intervention trials,including 9(4.3%in total)preventive trials,136(65.1%in total)therapeutic trials and 7(3.3%in total)convalescent trials.Conclusion:At the present stage,clinical studies are still aiming at exploring the effective treatment of COVID-19,and the existing treatments need more high-quality clinical evidence supporting.Traditional Chinese medicine may have played an important role in all stages of the epidemic preventing and control,and the enthusiasm of carrying out scientific research has also been greatly improved.The relevant policies of clinical trial registration should be popularized among researchers,including traditional Chinese medicine researchers.And the methodological quality of clinical trials’design urges improving. 展开更多
关键词 COVID-19 CLINICAL TRIAL registER Data analysis
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