检索结果-南通市图书馆

World Journal of Pediatrics 2020年第5期16卷 514-519页

作者： Wei Wang Yu Zhou lin-qing zhong Zhuo Li Shan Jian Xiao-Yan Tang Hong-Mei Song Department of Pediatrics Peking Union Medical College HospitalChinese Academy of Medical SciencesBeijingChina School of Medicine Tsinghua UniversityBeijingChina

Background The nucleotide-binding oligomerization domain-like receptor protein 12(NLRP12)-autoinflammatory disorder(NLRP12-AD)is a rare autoinflammatory disease characterized by recurrent fever,rash as well as musculoskeletal symptoms,which is rarely reported in Asian *** Three cases of NLRP12-AD presented to our hospital were studied after parental consents were *** presentations were recorded on a standardized case report *** of NLRP12 were detected by primary immunodeficiency disease panels and further examined by Sanger *** literature search for relevant studies of systemic autoinflammatory disorders,especially NLRP12-AD between January,2000 and January,2019 was carried and the clinical data were *** were made between groups in terms of onset age and of *** All our patients presented with fever,rash and arthritis/arthralgia,and sensorineural as well as sensorineural deafness(1/3),uveitis(1/3),abdominal pain(1/3),and myalgia(1/3).Two novel mutation variations,p.W581X and p.L558R,are reported *** addition,we also found that two patients inherited the mutated alleles from their healthy parents,and this may be evidence of *** Although the genotypes are similar,the clinical manifestations between Chinese patients and Western patients vary thus highlighting the possible influence of ethnic and environmental *** the other hand,some genetic mutations may lead to specific phenotype,as we have found a high prevalence of sensorineural hearing loss among p.R284X patients.

关键词： Autoinflammatory disease NLRP12-AD Recurrent fever

Characterization of microbiota in systemic-onset juvenile idiopathic arthritis with different disease severities

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

World Journal of Clinical Cases 2019年第18期7卷 2734-2745页

作者： Yan-qing Dong Wei Wang Ji Li Ming-Sheng Ma lin-qing zhong Qi-Jiao Wei Hong-Mei Song Department of Pediatrics Peking Union Medical College Hospital Chinese Academy of Medical Sciences and Peking Union Medical College

BACKGROUND Systemic-onset juvenile idiopathic arthritis (SoJIA) is one of most serious subtypes of juvenile idiopathic arthritis. Although the pathogenesis of SoJIA remains unclear, several studies have suggested a correlation between gut dysbiosis and JIA. Further understanding of the intestinal microbiome may help to establish alternative ways to treat, or even prevent, the disease. AIM To explore alterations in fecal microbiota profiles in SoJIA patients and to evaluate the correlations between microbiota and clinical parameters. METHODS We conducted an observational single-center study at the Pediatric Department of Peking Union Medical College Hospital. Children who were diagnosed with SoJIA at our institution and followed for a minimum period of six months after diagnosis were recruited for the study. Healthy children were recruited as a control group (HS group) during the same period. Clinical data and stool samples were collected from SoJIA patients when they visited the hospital. RESULTS The SoJIA group included 17 active and 15 inactive consecutively recruited children;the control group consisted of 32 children. Firmicutes and Bacteroidetes were the two most abundant phyla among the total sample of SoJIA children and controls. There was a significant difference among the three groups in observed species, which was the highest in the Active-SoJIA group, followed by the Inactive-SoJIA group and then HS group (Active-SoJIA vs HS: P = 0.000;and Inactive-SoJIA vs HS: P = 0.005). We observed a lower Firmicutes/Bacteroidetes ratio in SoJIA patients (3.28 ± 4.47 in Active-SoJIA, 5.36 ± 8.39 in Inactive-SoJIA,and 5.67 ± 3.92 in HS). We also observed decreased abundances of Ruminococcaceae (14.9% in Active-SoJIA, 17.3% in Inactive-SoJIA, and 22.8% in HS;Active-SoJIA vs HS: P = 0.005) and Faecalibacterium (5.1% in Active-SoJIA, 9.9% in Inactive-SoJIA, and 13.0% in HS;Active-SoJIA vs HS: P = 0.000) in SoJIA compared with HS. By contrast, the abundance of Bacteroidacea

关键词： Microbiota Systemic-onset juvenile idiopathic arthritis Disease activity Dysbiosis

Microstructure and properties of coarse-grained WC-10Co cemented carbides with different carbon contents during heat treatments

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Rare Metals 2024年第4期43卷 1786-1792页

作者： Yuan-Feng Xie Xing-Cheng Xie zhong-Wu Li Rui-Jun Cao zhong-Kun lin qing Li Chen-Guang lin Powder Metallurgy and Special Materials Research Department General Research Institute for Nonferrous MetalsBeijing100088China

The effects of cryogenic treatment(CT)and tempering-cryogenic treatment(TCT)on the microstructure and properties of coarse-grained WC-10Co cemented carbides with different carbon contents were *** binder phase,WC mean grain sizes,W solubility in the binder,relative magnetic s aturation,densities,hardness,wear resistance and second phase precipitation of cemented carbides with different heat treatments were *** results show that there are significant changes of microstructure and properties in the samples with CT and TCT,especially due to the precipitation of metastable nanoparticles W_(x)Co_(y)C_(z) in the binder during the heat treatments of CT and *** the simultaneous combination of microstructure and nanoparticle-reinforced binder,a dramatically improved combination of hardness and wear resistance of the samples after TCT has been achieved.

关键词： Cryogenic treatment Tempering Coarsegrained cemented carbide Carbon content Nanoparticlereinforced binder

维普期刊数据库

Generic Modeling and Control Framework for Power Systems Dominated by Power Converters Connected Through a Passive Transmission and Distribution Grid

在线全文

学校读者我要写书评

暂无评论

CSEE Journal of Power and Energy Systems 2024年第1期10卷 292-301页

作者： qing-Chang zhong Marcio Stefanello Department of Electrical and Computer Engineering Illinois Institute of TechnologyChicagoIL 60616USA Syndem LLC USA.M.Stefanello is with the Fundacao Universidade Federal do Pampa at AlegreteRS97546-550Brazil

In this paper,a compact mathematical model having an elegant structure,together with a generic control framework,are proposed for generic power systems dominated by power converters that are interconnected through a passive transmission and distribution(T&D)grid,by adopting the port-Hamiltonian(pH)systems theory and the fundamental circuit *** models of generic T&D lines are developed and then the model of a generic T&D grid is *** the proposed control framework,the controlled converters are proven to be passive and Input-to-State Stable(ISS).The compact mathematical model is scalable and can be applied to power systems with multiple power electronic converters with generic passive controllers,passive local loads,and different types of passive T&D lines connected in a meshed configuration without self-loops,so it is very ***,the resulting power system is proven to be ISS as *** analysis is carried out without assumptions on constant frequency/voltage,constant loads,and/or lossless networks,except the need of passivity for all parts involved,and without using the Clarke/Park transformations or the graph *** simplify the presentation,three-phase balanced systems are adopted but the results can be easily adapted for single-phase or unbalanced three-phase systems.

关键词： Input-to-State Stable(ISS) passivity,portHamiltonian(pH)systems Power electronic converters dominated power systems Synchronized and Democratized(SYNDEM)grid architecture

Market Equilibrium Based on Cloud-edge Collaboration

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

CSEE Journal of Power and Energy Systems 2024年第1期10卷 96-104页

作者： Tong Cheng Haiwang zhong qing Xia the State Key Laboratory of Power Systems the Department of Electrical EngineeringTsinghua UniversityBeijing 100084China

Market participants can only bid with lagged information disclosure under the existing market mechanism,which can lead to information asymmetry and irrational market behavior,thus influencing market *** promote rational bidding behavior of market participants and improve market efficiency,a novel electricity market mechanism based on cloudedge collaboration is proposed in this *** market information,called residual demand curve,is published to market participants in real-time on the cloud side,while participants on the edge side are allowed to adjust their bids according to the information disclosure prior to closure *** proposed mechanism can encourage rational bids in an incentive-compatible way through the process of dynamic equilibrium while protecting participants’*** paper further formulates the mathematical model of market equilibrium to simulate the process of each market participant’s strategic bidding behavior towards equilibrium.A case study based on the IEEE 30-bus system shows the proposed market mechanism can effectively guide bidding behavior of market participants,while condensing exchanged information and protecting privacy of participants.

关键词： Cloud-edge collaboration market mechanism residual demand curve strategic bidding

Unique mutation spectrum of progressive pseudorheumatoid dysplasia in the Chinese population:a retrospective genotype-phenotype analysis of 105 patients

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

World Journal of Pediatrics 2023年第7期19卷 674-686页

作者： Wei Wang Si-Hao Gao Min Weil lin-qing zhong Wei Liu Shan Jian Juan Xiao Cai-Hui Zhang Jian-Guo Zhang Xiao-Feng Zeng Wei-Bo Xia Zheng-qing Qiu Hong-Mei Song Department of Pediatrics Peking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeNo.1 ShuaifuyuanDongchengBeijing 100730China Department of Radiology Peking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100730China Department of Orthopedic Surgery Peking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100730China Department of Rheumatology and Clinical Immunology Chinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100730China National Clinical Research Center for Dermatologic and Immunologic Diseases(NCRC-DID) Ministry of Science and TechnologyBeijing 100730China State Key Laboratory of Complex Severe and Rare Diseases Peking Union Medical College Hospital(PUMCH)Beijing 100730China Key Laboratory of Rheumatology and Clinical Immunology Ministry of EducationBeijing 100730China Department of Endocrinology National Health Commission Key Laboratory of EndocrinologyPeking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100730China

Background Progressive pseudorheumatoid dysplasia(PPRD)is a rare genetic disease with autosomal recessive *** was a lack of genotype-phenotype correlation data from the Chinese *** study aimed to identify the genotype and phenotype characteristics of Chinese PPRD patients and to conduct a genotype-phenotype analysis of Chinese PPRD *** Genetic analysis was performed for suspected PPRD patients from Peking Union Medical College ***-cal records were collected from the electronic medical record system and patient-held portable health *** Chinese PPRD cases were gathered from both international and Chinese local *** collected demographic infor-mation,genetic variants,clinical manifestations,and imaging characteristics for further *** We included 105 Chinese PPRD patients in the current ***-three variants,including nine novels and five hotspot variants,were identified,with 26/33(79%)variants exclusively seen in the Chinese *** PPRD patients share a phenotype similar to that in international *** involvement may progress with age(R2=0.2541).Long bone shortening and severe deformities occur in three patients with biallelic null variants,of which at least one vari-ant is located in exon *** hotspot variants,c.624dupA(p.C209Mfs*21)were associated with later onset and more involved *** joints were more likely to be affected in patients carrying c.624dupA(p.C209Mfs*21)and c.866dupA(p.S209Efs*13).Shoulder joints are more likely to be involved in patients with biallelic null variants(P=0.027).Conclusions Chinese PPRD patients share a unique mutation *** the five hotspot variants,c.624dupA is associated with later onset of disease,more extensive joint involvement,and a tendency to affect elbow *** null variants with at least one variant in exon 2 could be a likely cause of long bone shortening and severe deformities.

关键词： CCN6 Genetics Mutations Progressive pseudorheumatoid dysplasia WISP3

Sand control mechanism of radial well filled with phase change material in hydrate reservoir

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Petroleum Science 2024年第4期21卷 2571-2582页

作者： Xiao-Qiang Liu zhong-Xi Han Zhi-lin Luo Hai-Long Lu Ying Sun qing You Tian-Kui Guo Zhan-qing Qu School of Energy Resources China University of Geosciences (Beijing) Beijing Key Laboratory of Unconventional Natural Gas Geological Evaluation and Development Engineering China University of Geosciences (Beijing) China Research Institute of Petroleum Exploration and Development Engineering Technology Research Institute of Southwest Oil & Gas Field Company PetroChina Beijing International Center for Gas Hydrate School of Earth and Space Sciences Peking University School of Advanced Material Peking University Shenzhen Graduate School School of Petroleum Engineering China University of Petroleum (East China)

Radial well filled with phase change material has been proposed as a novel sand control method for hydrate exploitation. In order to reveal the sand control mechanism, CFD-DEM coupling method is applied to simulate the migration, settlement, and blockage processes of sand particles in the radial *** obtained results indicate that three scenarios have been recognized for sand particles passing through sand control medium, based on the diameter ratio of sand control medium to sand particle（Dd）:fully passing（Dd= 8.75-22.5）, partially passing and partially blocked（Dd= 3.18-5.63）, and completely blocked（Dd= 2.18-3.21）. After being captured by the sand control medium, sand particles can block pores, which increases fluid flow resistance and causes a certain pressure difference in the radial *** pressure in the radial well should be lower than the hydrate phase equilibrium pressure during sand control design, for the purpose of promoting hydrate decomposition, and sand capture. The length of the radial well should be optimized based on the reservoir pore pressure, production pressure difference,bottom hole pressure, and the pressure gradient in the radial well. It should be noticed that the sand control medium leads to a decrease in permeability after sand particles captured. Even the permeability is reduced to several hundred millidarcy, it is still sufficient to ensure the effective flow of gas and water after hydrate decomposition. Increasing fluid velocity reduces the blocking capacity of the sand control medium, mainly because of deterioration in bridging between sand particles.

关键词： Hydrate reservoir Sand control Radial well CFD-DEM

在线全文

同方期刊数据库

学校读者我要写书评

暂无评论

海珠区极端短时强降水的特征分析

广东气象 2024年第3期46卷 41-44页

作者：王禹张荣艳陈炜杰林中庆黄启锋朱洁斐广州市海珠区气象局广东广州510220 吉安市泰和县气象局江西吉安343700

选取2017—2022年广州市海珠区内23个区域气象站的逐小时降水观测资料,统计分析了海珠区内极端短时强降水的特征,结果表明:海珠区极端短时强降水有“一年多两年少”的年变化特征,年内分布主要集中在4—9月(主汛期),且在5、8月呈“双峰... 详细信息

选取2017—2022年广州市海珠区内23个区域气象站的逐小时降水观测资料,统计分析了海珠区内极端短时强降水的特征,结果表明:海珠区极端短时强降水有“一年多两年少”的年变化特征,年内分布主要集中在4—9月(主汛期),且在5、8月呈“双峰”特征,前汛期的峰值高于后汛期的峰值;凌晨、傍晚到上半夜是极端短时强降水的易发时段,恰好与下班高峰期重合,给城市交通增加了巨大的压力。小时雨量在50~59.9 mm范围内出现的次数最多,占比超过50%;极端短时强降水年极值在8月出现次数最多,2019年7月22日16:00出现了期间极端最强短时强降水,达80.9 mm。极端短时强降水年平均次数在海珠区中部、东北角、西北角最多,尤其中部是极端短时强降水的易发区域,说明不同高低的城市建筑物会产生不同的地表粗糙程度,会使降水系统的移动速度减慢,导致雨强增大。

关键词：气候学极端短时强降水区域自动站海珠区

维普期刊数据库

Recent progress in self-propelled particles

在线全文

学校读者我要写书评

暂无评论

Journal of Hydrodynamics 2024年第1期36卷 61-77页

作者： Zhen-yu Ouyang Jian-zhong lin Laboratory of Impact and Safety Engineering Ministry of EducationNingbo UniversityNingbo315201China Department of Mechanics State Key Laboratory of Fluid Power and Mechatronic SystemsZhejiang UniversityHangzhou310027China

Self-propelled particles are commonly found in a large number of planktonic organisms such as bacteria,fungi,and algae in nature,and researchers have taken a long interest in exploring their swimming mechanisms for more than a *** in the past 20 years,with the development of computational fluid dynamics and flow display technology,as well as the need for the design of synthetic self-propelled particles and micro-swimming devices,self-propelled particles have become the forefront and hotspot of current research in the field of fluid *** paper first introduces the swimming characteristics of common self-propelled particles,leading to a classic“squirmer”type self-propelled particle *** this basis,a systematic introduction and summary of the theoretical and numerical simulation research of“squirmer”will be ***,the main challenges and opportunities faced by the current research will be summarized.

关键词： Self-propelled particles multiphase flow hydrodynamic interaction

Selective photosynthesis of imines from biomass-derived aldehydes over Ni/TiO_(2)

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Rare Metals 2024年第3期43卷 1116-1124页

作者： Wu-lin Li qing-qing Zhang lin Dong Wen-Jun Zhang Li-Li lin Zu-Peng Chen Jiangsu Co-Innovation Center of Efficient Processing and Utilization of Forest Resources International Innovation Center for Forest Chemicals and MaterialsCollege of Chemical EngineeringNanjing Forestry UniversityNanjing210037China Institute of Industrial Catalysis State Key Laboratory of Green Chemistry Synthesis TechnologyCollege of Chemical EngineeringZhejiang University of TechnologyHangzhou310014China

Photocatalytic reductive amination of biomass-derived aldehydes is a desirable way to selectively upgrade biomass into value-added nitrogen-containing chemicals under mild ***,it is challenging to produce imines in high selectivity because of the undesirable side reactions caused by the activity of functional ***,we demonstrate the highly reactive and selective production of imines from biomass derived aldehydes via the photocatalytic reductive amination,using a defective TiO_(2)supported nickel *** employment of methanol as the hydrogen donor and ammonia solution as the nitrogen source avoids the use of high-pressure H_(2)and expensive amines,rendering the current catalytic process safe,economical,and environmentally *** depth investigations attribute the improved separation and transfer of photogenerated charge carriers to the presence of oxygen vacancies and decorated Ni nanoparticles,thereby accelerating the production of imines from benzaldehyde amination(conversion,95.8%;seleetivity,95.2%).Furthermore,the developed system could be easily translated to the photocatalytic conversions of various biomass derived aldehydes,which provided an example of a cost-effective and sustainable approach for the valorization of biomass derived feedstocks.

关键词： Photocatalysis Biomass derivatives Imines Reduction amination Heterogeneous catalyst

维普期刊数据库