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Refractory very early-onset inflammatory bowel disease associated with cytosolic isoleucyl-tRNA synthetase deficiency:A case report

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World Journal of Gastroenterology 2020年第15期26卷 1841-1846页

作者： Andrew Fagbemi William G Newman Stuart G tangye Stephen m Hughes Edmund Cheesman Peter D Arkwright Department of Paediatric Gastroenterology Royal Manchester Children’s HospitalManchester M199WLUnited Kingdom Department of medical Genetics Manchester University NHS Foundation TrustManchester M139WLUnited Kingdom Evolution and Genomic Sciences University of ManchesterManchester M139WLUnited Kingdom Garvan Institute of medical Research DarlinghurstNSW 2010Australia St Vincent’s Clinical School Faculty of MedicineUNSW AustraliaSydneyNSW 2010Australia Department of Paediatric Allergy and Immunology Royal Manchester Children’s HospitalManchester M139WLUnited Kingdom Department of Paediatric Histopathology St Mary’s HospitalManchester M139WLUnited Kingdom Lydia Becker Institute of Immunology and Inflammation University of ManchesterManchester M139WLUnited Kingdom

BACKGROUND Aminoacyl tRNA synthetases/ligases(ARSs)are highly conserved enzymes involved in attaching amino acids to tRNA promoting protein *** deficiencies of ARSs localized to the mitochondria classically present with neuropathology,the clinical features of cytosolic ARS deficiencies are more *** have previously been associated with neonatal hepatitis,but never with early-onset inflammatory bowel *** SUmmARY A nine-year-old Bangladeshi boy presented with neonatal liver failure and deranged clotting,transaminitis and *** parents were first *** older brothers and a sister were *** patient suffered from loose stools from early infancy which became more troublesome and persistent from five years old with ten bloody motions a *** endoscopies showed persistent pancolitis,which was refractory to mesalazine,corticosteroids,azathioprine,sirolimus and anti-tNF(adalimumab)therapy,but has improved recently with subcutaneous *** Genome Sequencing revealed a novel pathogenic missense variant(c.290A>G)in the cytosolic isoleucyl-tRNA synthetase gene,leading to an amino acid substitution(***97Gly).Pathogenic variants in other genes associated with inflammatory bowel disease(IBD)(ADAm17,EGFR,FOXP3,IL10ra,IL10RB,IL21R,NCF4,StAt3)were *** assays demonstrated markedly elevated IL-2,IL-5,IL-13,IL-9 and IL-10 by the patient’s CD4+t-cells,while IL-17A,IL-17F,IFNβwere lower,and tNFαnot significantly different when compared to healthy *** this case report provides evidence that recessive mutations in cytosolic isoleucyltRNA synthetase are a novel monogenic cause of IBD,which should be considered,particularly in infants and children with a history of neonatal hepatitis and very early-onset IBD poorly responsive to treatment.

关键词： Inflammatory bowel disease Hepatitis Gene Cytosolic isoleucine tRNA synthase

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Enhancement of Corrosion Resistance of NiCrFeBSi Coatings Obtained by Flame thermal Spray Process Adding an Electroless Nickel Coating Ni-P

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Journal of Surface Engineered materials and Advanced technology 2017年第4期7卷 86-97页

作者： Arturo Barba-Pingarrón raúl Valdez-Navarro Félix Sánchez-De Jesus Ana maría Bolarín-miró rafael González-Parra Alba Covelo-Villar miguel ángel Hernández-Gallegos Carlos Domínguez-Ríos Centro de Ingeniería de Superficies y Acabados (CENISA) Facultad de Ingeniería Universidad Nacional Autónoma de México 2 Nivel Edificio “T” (Bernardo Quintana) Circuito Exterior Ciudad Universitaria Ciudad de México México área Académica de Ciencias de la tierra y materiales Universidad Autónoma del Estado de Hidalgo Pachuca México Centro de Investigación en materiales Avanzados Chihuahua México

Flame thermal Spray (FtS) coatings frequently show some porosity and reduced adherence to substrate, which affect its properties, especially its corrosion resistance. In this work, the corrosion resistance of AISI 1018 carbon steel coated by different methods is compared: electroless nickel (EN) coating, NiCrFeBSi obtained by FtS, duplex coatings of an EN deposit on a layer of NiCrFeBSi obtained by FtS and a layer of NiCrFeBSi on an EN deposit. the coatings were characterized using optical microscopy and scanning electron microscopy techniques, EDS microprobe microanalysis, roughness as well as electrochemical polarization tests to obtain the corrosion rate. the results show the enhancement in the corrosion resistance in saline medium of the duplex coatings, especially of the EN coating on FtS layer.

关键词： Flame thermal Spray Coatings Electroless Nickel Plating Duplex Coatings Corrosion Resistance

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the role of whole brain radiation therapy in the management of newly diagnosed brain metastases： a systematic review and evidence-based clinical practice guideline

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中国神经肿瘤杂志 2010年第1期8卷 57-57页

作者： Gaspar LE mehta mP Patchell ra Burri SH Robinson PD morris RE Ammirati m Andrews DW Asher AL Cobbs CS Kondziolka D Linskey mE Loeffler JS mcDermott m mikkelsen t Olson JJ Paleologos NA Ryken tC Kalkanis SN. Department of radiation Oncology University of Colorado-Denver Denver CO USA.

QUEStION: Should patients with newly-diagnosed metastatic brain tumors undergo open surgical resection versus whole brain ra- diation therapy (W-BRt) and/or other treatment modalities such as radiosurgery, and in what clinical settings? tARGEt POPULA- tION: these recommendations apply to adults with a newly diagnosed single brain metastasis amenable to surgical ***- mENDAtIONS: Surgical resection plus WBRt versus surgical resection alone Level 1 Surgical resection followed by WBRt represents a superior treatment modality, in terms of improving tumor control at the original site of the metastasis and in the brain overall,

关键词：放射治疗临床实践大脑诊断手术切除指南基础证据

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住院医师采用体检与手提式超声检查估计右房压的对比

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世界核心医学期刊文摘(心脏病学分册) 2007年第9期 27-28页

作者： Brennan J.m. Blair J.E. Goonewardena S. K.t. Spencer 杜媛 the University of Chicago Chicago IL United States

医师于床旁根据颈静脉波形准确估计右房(ra)压力的能力较弱,尤其是培训医师。用传统超声测定下腔静脉(IVC)可以准确预测ra压力,但是由于费用高、不便携以及需要专业训练以便获取并解释检测结果等特点,使得这种方法不适于常规临床应用。... 详细信息

医师于床旁根据颈静脉波形准确估计右房(ra)压力的能力较弱,尤其是培训医师。用传统超声测定下腔静脉(IVC)可以准确预测ra压力,但是由于费用高、不便携以及需要专业训练以便获取并解释检测结果等特点,使得这种方法不适于常规临床应用。本研究旨在比较利用体格检查与手提式超声(HCU)检查ra压力升

关键词：内科住院医师患者 IVC 超声 ra

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卒中介入治疗培训指南：国际多学会共识文件

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国际脑血管病杂志 2017年第5期25卷 396-399页

作者： Lavine SD Cockroft K Hoh B Bambakidis N Khalessi AA Woo H Riina H Siddiqui A Hirsch JA Chong W Rice H Wenderoth J mitchell P Coulthard A Signh tJ Phatorous C Khangure m Klurfan P ter Brugge K Iancu D Gunnarsson t Pongpech S Rodesch G Soderman m taylor A Krings t Orbach D Picard L Suh DC Zheng HQ Jansen O muto m Szikora I Pierot L Brouwer P Gralla J Renowden S Andersson t Fiehler J turjman F White P Januel AC Spelle L Kulcsar Z Chapot R Biondi A Dima S taschner C Szajner m Krajina A Sakai N matsumaru Y Yoshknura S Ezura m Fujinaka t Iihara K Ishii A Higashi t Hirohata m Hyodo A Ito Y Kawanishi m Kiyosue H Kobayashi E Kobayashi S Kuwayama N matsumoto Y miyachi S murayama Y Nagata I Nakahara I Nemoto S Niimi Y Oishi H Satomi J Satow t Sugiu K tanaka m terada t Yamagami H Diaz O Lylyk P Jayaraman mV Patsalides A Gandhi CD Lee SK Abruzzo t Albani B Ansari SA Arthur AS Baxter BW Bulsara KR Chen m Almandoz JE Fraser JF Heck DV Hetts SW Hussain mS Klucznik RP Leslie-mawzi tm mack WJ mctaggart ra meyers Pm mocco J Prestigiacomo CA Pride GL rasmussen PA Starke Rm Sunenshine PJ tarr RW Frei DF Pabo m Nogueira RG Zaidat OO Jovin t Linfante I Yavagal D Liebeskind D Novakovic R Pongpech S 许岩孙瑞郭芮兵 From the American Association of Neurological Surgeons/Congress of Neurological Surgeons (AANS/CNS) Rolling Meadows IL USA American Society of Neuroradiology (ASNR) Oak Brook IL USA Asian Australasian Federation of lnterventional and therapeutic Neuroradiology (AAFItN) Bali Indonesia Australian and New Zealand Society of Neuroradiobgy-Conjomt Committee for Recognition of training in Interventional Neuroradiology (CCINR) representing the raNZCR (ANZSNR) ANZAN and NSA Sydney Australia Canadian Interventional Neuro Group (CING) Edmonton AB Canada World Federation of Interventional and therapeutic Neuroradiology (WFItN) Gold Coast Australia European Society of Neuroradiology (ESNR) Milan Italy European Society of minimally Invasive Neurologic therapy (ESmINt) Nice France Japanese Society for Neuroendovascular therapy (JSNEt) Okayama Japan Sociedad Ibero Latino Americana de Neuroradiologica (SILAN) Cartagena Colombia Society of Neurolnterventional Surgery (SNIS) Washington DC USA Society of Vascular and Interventional Neurology (SVIN) Brooklyn NY USA 南京大学医学院附属金陵医院(南京军区南京总医院)神经内科 210002 解放军第一0一医院(无锡太湖医院)神经内科无锡214004

1背景缺血性卒中是全球人口死亡和残疾的首要原因。很多急性大血管闭塞（emergent large vesselocclusion，ELVO）患者都会遗留长期残疾。事实上，这些颅内大动脉闭塞经常会导致大面积脑损伤，进而造成患者死亡或严重致残。

关键词：缺血性卒中培训指南介入治疗文件学会国际血管闭塞动脉闭塞

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±800kV云广线换流站母线电晕特性试验研究

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高电压技术 2008年第9期34卷 1788-1791页

作者：万保权张小武张广洲邬雄饶宏倪小林罗宾王琦李妮国网电力科学研究院武汉430074 中国南方电网公司技术研究中心广州510623

为实现±800kV直流换流站母线电晕特性影响换流站管母线的优化设计和经济运行,对不同对地高度的φ250和φ300管母线的起晕电压、无线电干扰、可听噪声和地面合成场强进行了实验室试验。试验表明,φ250、300管母线施加±800kV直流电压时... 详细信息

为实现±800kV直流换流站母线电晕特性影响换流站管母线的优化设计和经济运行,对不同对地高度的φ250和φ300管母线的起晕电压、无线电干扰、可听噪声和地面合成场强进行了实验室试验。试验表明,φ250、300管母线施加±800kV直流电压时,管母线在高度10m和12m时起晕电压>±1200kV;管母线下地面合成场强m,可听噪声m。

关键词： ±800 kV 换流站管母线电晕特性试验

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SARS-CoV-2 interaction with Siglec-1 mediates trans-infection by dendritic cells

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Cellular & molecular Immunology 2021年第12期18卷 2676-2678页

作者： Daniel Perez-Zsolt Jordana muñoz-Basagoiti Jordi Rodon marc Elosua-Bayes Dàlia raïch-Regué Cristina Risco martin Sachse maria Pino Sanjeev Gumber mirko Paiardini Jakub Chojnacki Itziar Erkizia Xabier muñiz-trabudua Ester Ballana Eva Riveira-muñoz marc Noguera-Julian Roger Paredes Benjamin trinité Ferran tarrés-Freixas Ignacio Blanco Victor Guallar Jorge Carrillo JuliàBlanco Amalio telenti Holger Heyn Joaquim Segalés Bonaventura Clotet Javier martinez-Picado Júlia Vergara-Alert Nuria Izquierdo-Useros IrsiCaixa AIDS Research Institute 089BadalonaSpain IRtA Centre de Recerca en Sanitat Animal(CReSAIRTA-UAB)Campus de la UAB08193Bellaterra(Cerdanyola del Vallès)Spain CNAG-CRG Centre for Genomic Regulation(CRGBarcelona Institute of Science and Technology(BIST)08028BarcelonaSpain Centro Nacional de Biotecnología CSIC28049MadridSpain Division of microbiology and Immunology Yerkes National Primate Research CenterEmory UniversityAtlantaGAUSA Department of Pathology and Laboratory medicine School of MedicineEmory UniversityAtlantaGeorgiaUSA Division of Pathology Yerkes National Primate Research CenterEmory UniversityAtlantaGeorgiaUSA Germans trias i Pujol Research Institute(IGtP) Can Ruti Campus089BadalonaSpain Barcelona Supercomputing Center(BSC) 08034BarcelonaSpain Catalan Institution for Research and Advanced Studies(ICREA) 08010BarcelonaSpain University of Vic-Central University of Catalonia(UVic-UCC) Vic08500Spain Department of Integrative Structural and Computational Biology Scripps ResearchLa JollaCA92037USA Universitat Pompeu Fabra(UPF) BarcelonaSpain UAB CReSA(IRTA-UAB)Campus de la UAB08193Bellaterra(Cerdanyola del Vallès)Spain Departament de Sanitat i Anatomia Animals Facultat de VeterinàriaUAB08193Bellaterra(Cerdanyola del Vallès)Spain

Antigen-presenting cells(APCs)may be resistant to SARS-CoV-2 infection but still contribute to viral *** such as sialic acid-binding Ig-like lectin 1(Siglec-1/CD169)mediate the attachment of viruses to ***,we show that APCs effectively capture SARS-CoV-2 within compartments via recognition of *** receptor interacts with sialylated gangliosides on membranes of SARS-CoV-2 variants.

关键词： infection Antigen media

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Enhanced CD19 activity in B cells contributes to immunodeficiency in mice deficient in the ICF syndrome gene Zbtb24

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Cellular & molecular Immunology 2023年第12期20卷 1487-1498页

作者： Zhengzhou Ying Swanand Hardikar Joshua B.Plummer tewfik Hamidi Bin Liu Yueping Chen Jianjun Shen Yunxiang mu Kevin m.mcBride taiping Chen Department of Epigenetics and molecular Carcinogenesis The University of Texas MD Anderson Cancer CenterHoustonTX77030USA Program in Genetics and Epigenetics The University of Texas MD Anderson Cancer Center UTHealth Graduate School of Biomedical SciencesHoustonTX77030USA

Immunodeficiency,centromeric instability,and facial anomalies(ICF)syndrome is a rare autosomal recessive disorder characterized by DNA hypomethylation and antibody *** is caused by mutations in DNmt3B,ZBtB24,CDCA7,or *** progress has been made in elucidating the roles of these genes in regulating DNA methylation,little is known about the pathogenesis of the life-threatening hypogammaglobulinemia ***,we show that mice deficient in Zbtb24 in the hematopoietic lineage recapitulate the major clinical features of patients with ICF ***,Vav-Cre-mediated ablation of Zbtb24 does not affect lymphocyte development but results in reduced plasma cells and low levels of Igm,IgG1,and ***24-deficient mice are hyper and hypo-responsive to t-dependent and t-independent type 2 antigens,respectively,and marginal zone B-cell activation is ***,Zbtb24-deficient B cells show severe loss of DNA methylation in the promoter region of Il5ra(interleukin-5 receptor subunit alpha),and Il5ra derepression leads to elevated CD19 *** disruption of Cd19 can revert the hypogammaglobulinemia phenotype of Zbtb24-deficient *** results suggest the potential role of enhanced CD19 activity in immunodeficiency in ICF syndrome.

关键词： ICF syndrome ZBtB24 CD19 IL-5ra hypogammaglobulinemia

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温度对堆肥孔隙比渗透性以及可压缩性的影响

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农业环境科学学报 2004年第4期23卷 791-795页

作者：常志州 A.H.m.Veeken 江苏省农业科学院农业资源与环境研究中心江苏南京210014 Sub-Department Environmental technology Wa-geningen University the Netherlands

以猪粪及庭园垃圾为材料,在一种可测定堆肥物理结构参数的装置上,研究了温度对堆肥物料可压缩比、孔隙比以及渗透性的影响。结果表明,温度由20℃升到60℃,堆肥物料弹性模数(β)由3.08×10-4上升到8.01×10-4m2·N-1,因而影响堆体的可压... 详细信息

以猪粪及庭园垃圾为材料,在一种可测定堆肥物理结构参数的装置上,研究了温度对堆肥物料可压缩比、孔隙比以及渗透性的影响。结果表明,温度由20℃升到60℃,堆肥物料弹性模数(β)由3.08×10-4上升到8.01×10-4m2·N-1,因而影响堆体的可压缩比、孔隙比以及渗透性等物理结构参数,这种影响随堆肥物料的不同而异,因此,在进行堆肥高度设计时,应充分考虑温度可能对堆体物理结构参数的影响,使堆肥设计更科学合理。

关键词：堆肥温度可压缩比孔隙比渗透性

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铀矿尾矿区的氢气浓度、地表氡流量和其他放射性变化的研究

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世界核地质科学 1985年第1期 52-60页

作者： J.Bigu m.Greniev N.K.Dave t.P.Lim J.L.Chakravatti 李珍福

在铀矿尾矿地区进行了氡气浓度、地表氡流量和其他放射性变化的研究。对气象数据,如大气压力、空气温度和降雨量以及包含有温度,孔隙度、226ra含量的土壤剖面数据进行了测量。其目的是为了研究这些数据的变化与放射性资料之间的关系。... 详细信息

在铀矿尾矿地区进行了氡气浓度、地表氡流量和其他放射性变化的研究。对气象数据,如大气压力、空气温度和降雨量以及包含有温度,孔隙度、226ra含量的土壤剖面数据进行了测量。其目的是为了研究这些数据的变化与放射性资料之间的关系。在土壤的不同深度和土壤/空气的分界面进行了氡气测量。结果表明:氡气浓度随土壤深度的增加而增大;而释放到空气中的氡受土壤/空气的边界层的制约,在此边界层的孔隙中的氡浓度受到大气压力与其他气象条件变化的重大影响。

关键词：尾矿坝氧流量水力冲填坝地表地球表面氧气浓度大气压力空气压力气体压力聚氯乙烯管射气放射性热电偶热敏元件子体铀矿

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