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Toward a better understanding of typeⅠinterferonopathies:a brief summary,update and beyond

World Journal of Pediatrics 2020年第1期16卷 44-51页

作者： zhong-xun Yu Hong-Mei Song Department of Pediatrics Peking Union Medical College HospitalChinese Academy of Medical SciencesBeijingChina

Backgrounds TypeⅠinterferonopathy is a group of autoinflammatory disorders associated with prominent enhanced typeⅠinterferon *** mechanisms are complex,and the clinical phenotypes are *** review briefly summarized the recent progresses of typeⅠinterferonopathy focusing on the clinical and molecular features,pathogeneses,diagnoses and potential *** sources Original research articles and literature reviews published in PubMed-indexed *** TypeⅠinterferonopathies include Aicardi-Goutières syndrome,spondyloenchondro-dysplasia with immune dysregulation,stimulator of interferon genes-associated vasculopathy with onset in infancy,X-linked reticulate pigmentary disorder,ubiquitin-specific peptidase 18 deficiency,chronic atypical neutrophilic dermatitis with lipodystrophy,and Singleton-Merten syndrome *** disorders including interferon-stimulated gene 15 deficiency and DNAseⅡdeficiency are believed to be interferonopathies as *** calcification,skin vasculopathy,interstitial lung disease,failure to thrive,skeletal development problems and autoimmune features are *** responses to nucleic acid stimuli and defective regulation of protein degradation are main mechanisms in disease *** generation Janus kinase inhibitors including baricitinib,tofacitinib and ruxolitinib are useful for disease *** transcriptase inhibitors seem to be another option for Aicardi-Goutières *** Tremendous progress has been made for the discovery of typeⅠinterferonopathies and responsible *** kinase inhibitors and other agents have potential therapeutic *** basic,translational and clinical studies towards disease monitoring and powerful therapies are warranted.

关键词： Hereditary autoinflammatory diseases Interferon typeⅠ Janus kinase inhibitors

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Effect of die structure parameters on deformation behavior of bent tube in free bending process

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Journal of Iron and Steel Research International 2023年第3期30卷 487-503页

作者： Wei Wang Cheng Cheng Li Wan Jie Tao xun-zhong Guo Chun-mei Liu College of Material Science and Technology Nanjing University of Aeronautics and AstronauticsNanjing 211100JiangsuChina State Key Laboratory of Digital Manufacturing Equipment and Technology Huazhong University of Science and TechnologyWuhan 430074HubeiChina Capital Aerospace Machinery Corporation Beijing 100076China

The bending section of tube between the bending die and the guider is in a less constrained state during the free bending *** free bending dies have the important impact on the plastic deformation behavior and the forming quality of *** study the evolution law of the deformation behavior of tube with the die structure parameters and optimize the free bending die parameters,the free bending experiments and the corresponding numerical simulations were carried *** design principle of free bending die was *** free bending experiment was conducted to verify the reliability of the numerical simulation *** on the numerical simulation results,the influence of the distance between the center point of bending die and the front end of guider on the forming quality of bent tube is more obvious than that of the fillet of ***,the fillet of bending die hardly affects the stress and strain distribution and the evolution of the wall ***,the free bending experiments with the newly determined free bending dies were *** ultimate bending radius of bent tube is reduced and the forming quality is improved.

关键词： Free bending Die structure parameter Forming quality Numerical simulation Tube

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Endoscopic retrograde cholangiopancreatography management for choledochocele in a young female

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Hepatobiliary & Pancreatic Diseases International 2023年第6期22卷 658-660页

作者： Lin-Ping Cao xun zhong Kang-Jie Chen Jun Yu Department of Hepatobiliary and Pancreatic Surgery the First Affiliated HospitalZhejiang University School of MedicineHangzhou 310003China

To the Editor:Choledochocele,also known as type III choledochal cysts in the classification by Todani et al.[1],is a congenital abnormality of the biliary *** is characterized by a cystic dilation of intramural segment of the distal common bile duct(CBD)protruding into the descending *** makes up about 0.5%−4%of choledochal cysts[1,2].Compared with other subtypes,the incidence of choledochocele is extremely low and it frequently presents in adults at a relatively older age,with an average age of 51 years[3].The diagnosis and treatment are ***,we present a case of a young female patient with this rare disease entity,who recovered after effective endoscopic retrograde cholangiopancreatography(ERCP)management.

关键词： female retrograde

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NEW WAVE-DEPENDENT FORMULAE FOR SEA SPRAY FLUX AT AIR-SEA INTERFACE

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Journal of Hydrodynamics 2009年第4期21卷 573-581页

作者： SHI Jian ZHAO Dong-liang LI xun-qiang zhong zhong Key Laboratory of Coastal Disaster and Defense Ministry of Education Hohai University Nanjing 210098 China Institute of Meteorology PLA University of Science and Technology Nanjing 211101 China College of Physical and Environment Oceanography Ocean University of China Qingdao 266100 China

A bubble-derived Sea Spray Generation Function （SSGF） was deduced, which was integrated into the new wave-dependent SSGF. The effect of sea spray feedback was parameterized by the feedback coefficients A and B. The relationship of A（B） and the wind-sea Reynolds number was derived, which shows that the feedback effect of sea spray is decreasing with the wave developing. New sea spray flux formulae, including the new SSGF and new feedback coefficients, were proposed, which can reveal clearly the influence of wave state on sea spray fluxes. Spray fluxes in the Humidity Exchange Over the Sea （HEXOS） experiment were simulated again, and results show that spray momentum flux is small in this experiment, but spray heat flux is large. Comparisons of the sea spray fluxes calculated by two kinds of formulae in different wave states show that previous wave-independent spray flux formulae are the special cases of the presented new wave dependent formulae for sea spray flnx.

关键词： sea sray flux HEXOS wave state Sea Spray Generation Function (SSGF)

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An Improved Parameter Dimensionality Reduction Approach Based on a Fast Marching Method for Automatic History Matching

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Fluid Dynamics & Materials Processing 2022年第3期18卷 609-628页

作者： Hairong Zhang Yongde Gao Wei Li Deng Liu Jing Cao Luoyi Huang xun zhong Southern Marine Science and Engineering Guangdong Laboratory Zhanjiang Bay LaboratoryZhanjiang524000China Zhanjiang Branch of China National Offshore Oil Corporation Zhanjiang524000China College of Petroleum Engineering Yangtze UniversityWuhan430100China

History matching is a critical step in reservoir numerical simulation *** is typically hindered by difficulties associated with the high-dimensionality of the problem and the gradient calculation ***,a multi-step solving method is proposed by which,first,a Fast marching method(FMM)is used to calculate the pressure propagation time and determine the single-well sensitive ***,a mathematical model for history matching is implemented using a Bayesian ***,an effective decomposition strategy is adopted for parameter dimensionality ***,a localization matrix is constructed based on the single-well sensitive area data to modify the gradient of the objective *** method has been verified through a water drive conceptual example and a real field *** results have shown that the proposed method can generate more accurate gradient information and predictions compared to the traditional analytical gradient methods and other gradient-free algorithms.

关键词： History matching parameter dimensionality reduction sensitive area gradient correction

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A Model for the Connectivity of Horizontal Wells in Water-Flooding Oil Reservoirs

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Fluid Dynamics & Materials Processing 2022年第5期18卷 1441-1468页

作者： Chenyang Shi Fankun Meng Hongyou Zhang HuiJiang Chang xun zhong Jie Gong and Fengling Li Cooperative Innovation Center of Unconventional Oil and Gas(Ministry of Education&Hubei Province) Yangtze UniversityWuhan430100China School of Petroleum Engineering Yangtze UniversityWuhan430100China Key Laboratory of Drilling and Production Engineering for Oil and Gas Wuhan430100China Tianjin Branch of CNOOC Ltd.Tianjin300459China Oilfield Technology Service Company Xinjiang Oilfield CompanyPetroChinaKaramay834000China

As current calculation models for inter-well connectivity in oilfields can only account for vertical wells,an updated model is elaborated here that can predict the future production performance and evaluate the connectivity of horizontal wells(or horizontal and vertical wells).In this model,the injection-production system of the considered reservoir is simplified and represented with many connected ***,the horizontal well is modeled with multiple connected wells without considering the pressure loss in the horizontal *** this approach,the production performance for both injection and production wells can be obtained by calculating the bottom-hole flowing pressure and oil/water saturation according to the material balance equation and a saturation front-tracking *** effort is also provided to optimize(to fit known historical production performances)the two characteristic problem parameters,namely,the interwell conductivity and connected volume by means of a SPSA gradient-free *** order to verify the validity of the model,considering a heterogenous reservoir,three conceptual examples are constructed,where the number ratio between injection and production wells are 1/4,4/1 and 4/5,*** is shown that there is a high consistency between simulation results and field data.

关键词： Connectivity model horizontal well production performance interwell connectivity connected volume

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Syntheses,Crystal Structures,Luminescent and Magnetic Properties of Three Ni(Ⅱ),Zn(Ⅱ) and Cd(Ⅱ) Coordination Polymers Based on an Ether-bridged Tetracarboxylic Acid

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Chinese Journal of Structural Chemistry 2020年第5期39卷 967-977页

作者： LI Yu CHEN Yong-Xuan ZHAO Zhen-Yu ZOU xun-zhong FENG An-Sheng Guangdong Research Center for Special Building Materials and Its Green Preparation Technology/Foshan Research Center for Special Functional Building Materials and Its Green Preparation Technology Guangdong Industry PolytechnicGuangzhou 510300China School of Intelligent Manufacturing and Equipment Shenzhen Institute of Information TechnologyShenzhen 518172China

Two 1D and one 3D coordination polymers,namely[Ni2(μ3-deta)(H2 biim)3(H2O)2]n(1),{[Zn2(μ4-deta)(Phen)2(H2O)]·H2O}n(2)and[Cd2(μ6-deta)(Phen)2(H2O)]n(3),have been constructed hydrothermally using H4deta(H4deta=2,3,3’,4’-diphenyl ether tetracarboxylic acid),H2 biim(H2 biim=2,2’-biimidazole),phen(phen=1,10-phenanthroline),and nickel,zinc or cadmium chlorides at 160℃.The products were isolated as stable crystalline solids and characterized by IR spectra,elemental analyses,thermogravimetric analyses(TGA)and single-crystal X-ray diffraction ***-crystal X-ray diffraction analyses revealed that three compounds crystallize in the triclinic system,space group■.Compounds 1 and 2 disclose a 1D chain *** 3 features a 3D *** structural diversity of compounds 1~3 is driven by the metal(Ⅱ)node and the type of supporting *** studies for compound 1 demonstrate an antiferromagnetic coupling between the adjacent Ni(Ⅱ)*** luminescence behavior of compounds 2 and 3 was also investigated.

关键词： coordination polymer tetracarboxylic acid magnetic properties luminescent properties

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Syntheses, Crystal Structures, Magnetic and Luminescent Properties of Ni(Ⅱ), Cd(Ⅱ) and Pb(Ⅱ) Coordination Compounds Constructed from 5-Fluoronicotinic Acid

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Chinese Journal of Structural Chemistry 2019年第6期38卷 999-1011页

作者： LI Yu ZOU xun-zhong QIU Wen-Da FENG An-Sheng CHEN Xiao-Ling School of Eco-Environmental Engineering/Foshan Research Center for Special Functional Building Materials and Its Green Preparation Technology Guangdong Industry PolytechnicGuangzhou 510300China School of Chemical Engineering and Light Industry Guangdong University of TechnologyGuangzhou 510006China

Five coordination compounds, namely [Ni2(5-Fnic)2(H2 O)4](1), [Ni(5-Fnic)(μ-5-Fnic)(H2 biim)]n(2), {[Cd((μ-5-Fnic)2(H2 O)2]×4,4’-bipy}n(3), [Pb2(μ-5-Fnic)2(μ3-5-Fnic)2(H2 O)2]n(4), and [Pb(5-Fnic)2(4,4’-bipy)(H2 O)](5), have been constructed hydrothermally using 5-FnicH(5-FnicH = 5-fluoronicotinic acid), H2 biim(H2 biim = 2,2’-biimidazole), 4,4’-bipy(4,4’-bipy = 4,4’-bipyridine), NiCl2×6 H2 O, CdCl2×H2 O and PbCl2. The products were isolated as stable crystalline solids and were characterized by IR spectra, elemental analyses, thermogravimetric analyses(TGA), and single-crystal X-ray diffraction analyses. Structures of 1~5 range from discrete 0 D monomers(1 and 5) to 1 D coordination polymers(2 and 4) and 2 D metal-organic network(3). A broad structural diversity of 1~5 is guided by the type of the metal(Ⅱ) node and the introduction of auxiliary ligands. The magnetic(for 2) and luminescent(for 3~5) properties were also investigated and discussed.

关键词： coordination compound 5-fluoronicotinic acid magnetic properties,luminescent properties

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Australian Rotarian Mr. Kollberg's Inspection Tour in Binxian

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International Understanding 2006年第4期 61-63页

作者： zhong xun

From November 3 to 7, Mr. Kollberg, one of the CAFIU＇s friends from Australian Rotary made inspection tour in Binxian County, Shaanxi Province. Since 1996, donated more than $6 130 projects including Australian Rotary have million to support about construction of ＂Hoping schools＂, provision of medical equipments, poor student sponsorships, women employment and equipment for the handicapped and solving drinking water problems for the local villagers. Another purpose of his tour was to discuss on the next year＇s plan with the local departments concerned.

关键词：澳大利亚扶轮社中国国际关系

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Analysis of clinical characteristics of children with Aicardi-Goutieres syndrome in China

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World Journal of Pediatrics 2022年第7期18卷 490-497页

作者： Wei Wang Ting-Yan He Li-Ping Zou Wen-Dao Li zhong-xun Yu Ming-Sheng Ma Jun Yang Hong-Mei Song Peking Union Medical College Hospital.Chinese Academy of Medical Sciences Peking Union Medical CollegeNo.1 Shuaifuyuan WangfujingDongcheng DistrictBeijing 100730China Shenzhen Children's Hospital No.7019 Yitian RoadFutian DistrictShenzhen 518048China The General Hospital of the People's Liberation Army No.28 Fuxing RoadHaidian DistrictBeijing 10000China

Background Aicardi-Goutieres syndrome(AGS)is an inflammatory disorder belonging to the type I interferonopathy *** clinical diagnosis of AGS is difficult,which can lead to a high mortality ***,there is a lack of large-sample research data on AGS in *** aim to summarize the clinical characteristics of Chinese patients with AGS and provide clues for clinical *** The genetic and clinical features of Chinese patients with AGS were ***-time polymerase chain reac-tion was used to detect expression of interferon-stimulated genes(ISGs).Results A total of 23 cases were included,consisting of 7 cases of AGS1 with three prime repair exonuclease 1 mutations,3 of AGS2 with ribonuclease H2 subunit B(RNASEH2B)mutations,3 of ASG3 with RNASEH2C,1 of AGS4 with RNASEH2A mutations,2 of AGS6 with adenosine deaminase acting on RNA 1 mutations,and 7 of AGS7 with interferon induced with helicase C domain 1 *** before the age of 3 years occurred in 82.6%.Neurologic involvement was most common(100%),including signs of intracranial calcification which mainly distributed in the bilateral basal ganglia,leukodystrophy,dystonia,epilepsy,brain atrophy and *** disability,language disability and motor skill impairment were also *** manifestations(60.87%)were dominated by a chilblain-like *** such as microcephaly(47.62%),short stature(52.38%),liver dysfunction(42.11%),thyroid dysfunction(46.15%),positive autoimmune antibod-ies(66.67%),and elevated erythrocyte sedimentation rate(53.85%)were also *** phenotypes of 2 cases fulfilled the diagnostic criteria for systemic lupus erythaematosus(SLE).One death was *** expression were *** AGS is a systemic disease that causes sequelae and mortality.A diagnosis of AGS should be considered for patients who have an early onset of chilblain-like rash,intracranial calcification,leukodystrophy,dystonia,developmental delay,positive autoimm

关键词： Aicardi-Goutieres syndrome Chinese Diagnosis Manifestations

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