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水蒸气在褐煤孔隙中的吸附行为:试验研究与分子动力学模拟
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作者 王成勇 邢耀文 +3 位作者 王市委 陈鹏 李吉辉 桂夏辉 《洁净煤技术》 CAS CSCD 北大核心 2024年第5期162-171,共10页
深入理解水在褐煤上的吸附是褐煤干燥提质技术的理论基础之一。采用傅里叶变换红外光谱(FTIR)、扫描电子显微镜(SEM)和低温氮吸附/脱附试验分析了褐煤的含氧官能团和孔隙结构,利用水蒸气吸附/脱附试验和分子动力学(MD)模拟探究了水蒸气... 深入理解水在褐煤上的吸附是褐煤干燥提质技术的理论基础之一。采用傅里叶变换红外光谱(FTIR)、扫描电子显微镜(SEM)和低温氮吸附/脱附试验分析了褐煤的含氧官能团和孔隙结构,利用水蒸气吸附/脱附试验和分子动力学(MD)模拟探究了水蒸气在褐煤孔隙中的吸附行为。研究结果表明:褐煤样品丰富的含氧官能团和较多的微孔、中孔(孔径约2 nm),为水蒸气提供了吸附位点和吸附场所。水蒸气在煤样上的吸附过程可分为3个阶段。第1阶段,相对蒸气压(P/P_(0))<0.21,水分子直接吸附于含氧官能团上,此时的吸附速度最大;第2阶段(P/P_(0)=0.21~<0.71),水分子与已吸附的水分子相互作用,促使水团簇逐渐生长;第3阶段(P/P_(0)≥0.71),水团簇填充孔隙,并发生毛细凝聚,此时的吸附速度略大于第2阶段。根据Dent模型对水蒸气吸附等温线的拟合结果,吸附类型属于多级吸附,包括初级吸附(第1阶段)和次级吸附(第2、3阶段)。初级吸附能(-48.77 kJ/mol)显著大于水的液化热(E L=-43.99 kJ/mol),而次级吸附能(-42.28 kJ/mol)略小于E L,表明吸附于褐煤的水呈液态。水蒸气脱附过程存在明显的脱附迟滞现象,表明水蒸气吸附稳定,较难去除,其中高压迟滞发生在P/P_(0)≈0.4~0.9,主要由毛细凝聚和“墨水瓶”效应引起;而低压迟滞发生在P/P_(0)<0.4,由水分子与含氧官能团间强相互作用引起。MD模拟结果与水蒸气吸附/脱附等温线分析结果一致,水分子优先吸附在孔隙中,与孔隙壁的含氧官能团形成氢键,且其扩散系数(2.98×10^(-5)cm^(2)/s)与液态水的分子扩散系数相近。 展开更多
关键词 褐煤 孔隙结构 含氧官能团 水蒸气吸附 Dent模型 分子动力学模拟
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以范可尼综合征和早发肾功能异常起病的Dent病1家系报道并文献复习
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作者 王利霞 马丽娟 张宏文 《临床肾脏病杂志》 2024年第2期171-174,共4页
Dent病(Dent disease)是一种罕见的X连锁隐性遗传性的近端肾小管疾病,因CLCN5基因(Dent病1型)和OCRL基因(Dent病2型)突变所致[1-2],临床主要特征为小分子蛋白尿(low-molecular weight proteinuria,LMWP)、高钙尿症(hypercalciuria)和肾... Dent病(Dent disease)是一种罕见的X连锁隐性遗传性的近端肾小管疾病,因CLCN5基因(Dent病1型)和OCRL基因(Dent病2型)突变所致[1-2],临床主要特征为小分子蛋白尿(low-molecular weight proteinuria,LMWP)、高钙尿症(hypercalciuria)和肾钙质沉着症(nephrocalcinosis),随病程进展部分患者可出现肾功能异常或肾衰竭[3-5]。此外,Dent病临床还可以有其他多种表现,包括泛氨基酸尿、磷酸盐尿、糖尿症、血尿、尿酸化功能障碍、佝偻病或骨软化症等[6-7]。 展开更多
关键词 Dent病 范可尼综合征 肾功能异常
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THREE KINDS OF DENTABILITIES IN BANACH SPACES AND THEIR APPLICATIONS
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作者 张子厚 周晶 《Acta Mathematica Scientia》 SCIE CSCD 2024年第2期445-454,共10页
In this paper,we study some dentabilities in Banach spaces which are closely related to the famous Radon-Nikodym property.We introduce the concepts of the weak^(*)-weak denting point and the weak^(*)-weak^(*)denting p... In this paper,we study some dentabilities in Banach spaces which are closely related to the famous Radon-Nikodym property.We introduce the concepts of the weak^(*)-weak denting point and the weak^(*)-weak^(*)denting point of a set.These are the generalizations of the weak^(*)denting point of a set in a dual Banach space.By use of the weak^(*)-weak denting point,we characterize the very smooth space,the point of weak^(*)-weak continuity,and the extreme point of a unit ball in a dual Banach space.Meanwhile,we also characterize an approximatively weak compact Chebyshev set in dual Banach spaces.Moreover,we define the nearly weak dentability in Banach spaces,which is a generalization of near dentability.We prove the necessary and sufficient conditions of the reflexivity by nearly weak dentability.We also obtain that nearly weak dentability is equivalent to both the approximatively weak compactness of Banach spaces and the w-strong proximinality of every closed convex subset of Banach spaces. 展开更多
关键词 weak^(*)-weak denting point nearly weak dentability very smooth space point of weak^(*)-weak continuity extreme point approximatively weak compactness w-strong proximinality REFLEXIVITY
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表现为大量蛋白尿的儿童Dent病一例
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作者 李华荣 陈朝英 +1 位作者 涂娟 万灵 《罕见病研究》 2024年第1期114-117,共4页
本文报道1例表现为大量蛋白尿的Dent病患儿诊疗过程。患儿男性,3岁1个月,常规体检时发现有大量蛋白尿,无低白蛋白血症,尿蛋白电泳提示以小分子量蛋白为主,同时伴高钙尿症,有阴离子间隙正常的代谢性酸中毒,无糖尿,无氨基酸尿,肾脏超声未... 本文报道1例表现为大量蛋白尿的Dent病患儿诊疗过程。患儿男性,3岁1个月,常规体检时发现有大量蛋白尿,无低白蛋白血症,尿蛋白电泳提示以小分子量蛋白为主,同时伴高钙尿症,有阴离子间隙正常的代谢性酸中毒,无糖尿,无氨基酸尿,肾脏超声未见肾脏钙质沉着。患儿无生长发育落后及肾脏疾病家族史。完善全外显子组基因检测到OCRL1基因第15号外显子存在1处错义变异c.1477C>T(p.Arg493Trp)。诊断Dent病明确后,限制高钙、高钠及高草酸盐饮食摄入,同时加用枸橼酸钾颗粒及氢氯噻嗪口服。目前随访2个月,尿钙水平下降,肾功能稳定。本文通过典型病例报道及既往文献回顾,以期提高临床医师对该疾病的认识,并为该疾病的诊治提供借鉴。 展开更多
关键词 低分子量蛋白尿 高钙尿症 Dent病 基因 罕见病
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Bending Failure Mode and Prediction Method of the Compressive Strain Capacity of A Submarine Pipeline with Dent Defects
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作者 HOU Fu-heng JIA Lu-sheng +3 位作者 CHEN Yan-fei ZHANG Qi ZHONG Rong-feng WANG Chun-sha 《China Ocean Engineering》 SCIE EI CSCD 2024年第4期636-647,共12页
A dent is a common type of defects for submarine pipeline.For submarine pipelines,high hydrostatic pressure and internal pressure are the main loads.Once pipelines bend due to complex subsea conditions,the compression... A dent is a common type of defects for submarine pipeline.For submarine pipelines,high hydrostatic pressure and internal pressure are the main loads.Once pipelines bend due to complex subsea conditions,the compression strain capacity may be exceeded.Research into the local buckling failure and accurate prediction of the compressive strain capacity are important.A finite element model of a pipeline with a dent is established.Local buckling failure under a bending moment is investigated,and the compressive strain capacity is calculated.The effects of different parameters on pipeline local buckling are analyzed.The results show that the dent depth,external pressure and internal pressure lead to different local buckling failure modes of the pipeline.A higher internal pressure indicates a larger compressive strain capacity,and the opposite is true for external pressure.When the ratio of external pressure to collapse pressure of intact pipeline is greater than 0.1,the deeper the dent,the greater the compressive strain capacity of the pipeline.And as the ratio is less than 0.1,the opposite is true.On the basis of these results,a regression equation for predicting the compressive strain capacity of a dented submarine pipeline is proposed,which can be referred to during the integrity assessment of a submarine pipeline. 展开更多
关键词 submarine pipeline dent defect bending load local buckling compressive strain capacity
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1例儿童1型Dent病伴Bartter综合征病例分析并文献复习
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作者 杨鸿源 吴雅莹 《儿科药学杂志》 2024年第1期37-41,共5页
目的:分析1例1型Dent病伴Bartter综合征样患儿的临床症状及诊疗过程,以提高临床对该疾病的认识。方法:采用回顾性分析方法,收集我院经临床特征和基因检测确诊的1例1型Dent病伴Bartter综合征患儿的临床资料,分析其临床特征及诊疗过程并... 目的:分析1例1型Dent病伴Bartter综合征样患儿的临床症状及诊疗过程,以提高临床对该疾病的认识。方法:采用回顾性分析方法,收集我院经临床特征和基因检测确诊的1例1型Dent病伴Bartter综合征患儿的临床资料,分析其临床特征及诊疗过程并复习文献。结果:本例患儿以Bartter综合征样表现(顽固性低钾血症,血浆肾素、醛固酮水平升高,血压正常)为初始表现,并有尿蛋白定量明显升高,以低分子量蛋白(LMWP)尿为主,α1、β2微球蛋白(MG)水平明显升高,基因分析提示CLCN5基因c.1265-c.1266 insA(半合子突变)。但本例患儿初始肾功能正常,泌尿系统超声检查结果未见异常。初期采用饮食调控(如低草酸盐、低钠、低钙等),口服补钾及盐酸贝那普利0.25 mg/(kg·d)治疗1个月后,发生急性肾损伤,给予甲泼尼龙及血液净化治疗后肾功能好转。结论:Dent病临床表型复杂,需及时完善基因检测协助诊断,具有Bartter综合征临床症状的Dent病患者易发展为肾功能衰竭,应及时评估肾功能及长期规律随访。 展开更多
关键词 1型Dent病 BARTTER综合征 CLCN5基因突变 肾功能衰竭 基因检测
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控屏时代
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作者 孙启禄 《疯狂英语(新悦读)》 2024年第7期22-26,75,76,共7页
Australian teenagers will receive les-sons on how to manage their screen time at school.A pilot project involving 3000 stu-dents will begin and is expected to expand to include 30,000 Year 7 and 8 children start-ing f... Australian teenagers will receive les-sons on how to manage their screen time at school.A pilot project involving 3000 stu-dents will begin and is expected to expand to include 30,000 Year 7 and 8 children start-ing from 2025.The program,which is the first of its kind in the world,will be delivered by the mental health organization the Black Dog Institute and the Bupa Foundation.According to a survey by News Corp,50%of Aus-tralians feel depressed after spending time on social media.In response to young peoples requests for help in managing their online lives,the Black Dog Institute decided to launch this program. 展开更多
关键词 dents MANAGING LIVES
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Denting Nanospheres with a Short Peptide 被引量:1
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作者 Sha Lin Hui Sun +4 位作者 Erik Jan Cornel Jin-Hui Jiang Yun-Qing Zhu Zhen Fan Jian-Zhong Du 《Chinese Journal of Polymer Science》 SCIE CAS CSCD 2021年第12期1538-1549,共12页
Dented n anospheres show promisi ng potential in drug delivery,nano motors,etc.However,it is still challe nging to prepare them by homopolymer self-assembly because of the strict structural requirements of the homopol... Dented n anospheres show promisi ng potential in drug delivery,nano motors,etc.However,it is still challe nging to prepare them by homopolymer self-assembly because of the strict structural requirements of the homopolymer.Herein,we propose a strategy for preparing dented nano spheres from homopolymers by co-assembly with a short peptide.They were co-assembled from poly(2-hydroxy-3-((4-(ethoxycarbonyl)phenyl)amino)propyl methacrylate)(PHBzoMA59)and(S)-2-((S)-2-((((9H-fluoren-9-yl)methoxy)carbonyl)amino)-3-phenylpro-panamido)-3-phenyIpropanoic acid(Fmoc-FF-OH).PHBzoMA homopolymers can only self-assemble into nanospheres without dent,and the addition of a short peptide in troduced hydroge n bondi ng and complementary π-π stacki ng interactions led to the final dented nano sphere morphology.The weight fracti ons of the short peptide can be adjusted to regulate the final morphology.It was con firmed that the radius of curvature of the dent on the surface was related to the organic bubble inside the protospheres prepared at critical aggregation concentration(CAC).The orga nic bubble can be adjusted by altering the kind of orga nic solve nt and solution pH,which allowed control over the dented nano sphere dimension.The use of differe nt orga nic solve nts with various polarities allows adjustment of the interfacial tension,and hence the denting degree.This degree can also be controlled by manipulating the solution pH to(de)protonate the short peptide and homopolymer.Furthermore,the versatility of this method was highlighted by using a differe nt homopolymer and the applicability of the resulting den ted nanospheres was demonstrated by decoration with gold nanoparticles.Overall,this study provided important insights and a new simple strategy to prepare den ted n anospheres in a con trolled fashion. 展开更多
关键词 Dented nanospheres HOMOPOLYMER Short peptide Hydrogen bonding π-πStacking
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Cooperative Channel and Optimized Route Selection in Adhoc Network 被引量:1
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作者 D.Manohari M.S.Kavitha +1 位作者 K.Periyakaruppan B.Chellapraba 《Intelligent Automation & Soft Computing》 SCIE 2023年第5期1547-1560,共14页
Over the last decade,mobile Adhoc networks have expanded dramati-cally in popularity,and their impact on the communication sector on a variety of levels is enormous.Its uses have expanded in lockstep with its growth.D... Over the last decade,mobile Adhoc networks have expanded dramati-cally in popularity,and their impact on the communication sector on a variety of levels is enormous.Its uses have expanded in lockstep with its growth.Due to its instability in usage and the fact that numerous nodes communicate data concur-rently,adequate channel and forwarder selection is essential.In this proposed design for a Cognitive Radio Cognitive Network(CRCN),we gain the confidence of each forwarding node by contacting one-hop and second level nodes,obtaining reports from them,and selecting the forwarder appropriately with the use of an optimization technique.At that point,we concentrate our efforts on their channel,selection,and lastly,the transmission of data packets via the designated forwarder.The simulation work is validated in this section using the MATLAB program.Additionally,steps show how the node acts as a confident forwarder and shares the channel in a compatible method to communicate,allowing for more packet bits to be transmitted by conveniently picking the channel between them.We cal-culate the confidence of the node at the start of the network by combining the reliability report for thefirst hop and the reliability report for the secondary hop.We then refer to the same node as the confident node in order to operate as a forwarder.As a result,we witness an increase in the leftover energy in the output.The percentage of data packets delivered has also increased. 展开更多
关键词 Adhoc Network confident FORWARDER one-hop optimized route selection secondary report channel selection
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Dent病研究进展
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作者 杨玉娇 王墨 《临床医学进展》 2023年第5期8181-8188,共8页
Dent病(Dent disease)是一种罕见的X连锁隐性遗传肾小管疾病。已被证实CLCN5及OCRL1为其致病基因,另有部分病例致病基因不明。随着Dent病分子遗传学研究的不断深入,关于Dent病的研究有了新的进展,现就Dent病的致病基因及其致病机制、临... Dent病(Dent disease)是一种罕见的X连锁隐性遗传肾小管疾病。已被证实CLCN5及OCRL1为其致病基因,另有部分病例致病基因不明。随着Dent病分子遗传学研究的不断深入,关于Dent病的研究有了新的进展,现就Dent病的致病基因及其致病机制、临床表型及治疗进展等方面进行综述。 展开更多
关键词 Dent病 致病机制 治疗
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A Summit of Little Substance
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作者 CHARLES ONUNAIJU 《ChinAfrica》 2023年第2期16-17,共2页
The US-Africa Leaders Summit was held in Washington D.C.from 13 to 15 December 2022.As expected,it drew considerable media attention.Coming eight years after the first such summit held in 2014,it did not have much to ... The US-Africa Leaders Summit was held in Washington D.C.from 13 to 15 December 2022.As expected,it drew considerable media attention.Coming eight years after the first such summit held in 2014,it did not have much to look back in terms of antecedents and continuity. 展开更多
关键词 dents SUMMIT AFRICA
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Dent病6例临床诊治分析 被引量:12
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作者 张宏文 张琰琴 +2 位作者 刘晓宇 肖慧捷 姚勇 《临床儿科杂志》 CAS CSCD 北大核心 2016年第6期418-420,共3页
目的 探讨Dent 病的诊断及治疗.方法 回顾性分析2014 年1 月至2015 年8 月收治的曾被外院误诊为肾病综合征的6 例Dent 病患儿的临床特点、诊治过程以及相关致病基因突变分析.结果 6例患儿均为男性,年龄4.5 - 9.8 岁,临床主要表现为达肾... 目的 探讨Dent 病的诊断及治疗.方法 回顾性分析2014 年1 月至2015 年8 月收治的曾被外院误诊为肾病综合征的6 例Dent 病患儿的临床特点、诊治过程以及相关致病基因突变分析.结果 6例患儿均为男性,年龄4.5 - 9.8 岁,临床主要表现为达肾病水平的蛋白尿,有-过性低白蛋白血症(26 - 30 g/L),均无明显水肿、高胆固醇血症.其中4 例患儿行肾活检,2 例为轻度系膜增生性肾小球肾炎、2 例为局灶节段性肾小球硬化.6 例患儿在对足量激素耐药后均联用至少一种或多种免疫抑制剂,蛋白尿均未见明显变化.入院后检查示低分子蛋白尿,肾早期损伤指标α1 微球蛋白(α1-MG)与微量白蛋白(MA)的比值均〉 1,伴高钙尿症、不伴肾功能异常,其中2 例B超可见肾脏钙化影像,符合Dent 病临床诊断.进一步的基因分析证实6 例患儿均存在CLCN5 基因致病突变.结论 作为一种罕见的遗传性肾小管疾病,Dent 病在临床上易被误诊误治,应引起临床儿科医师的重视.肾早期损伤指标α1-MG/MA 〉 1,可作为肾小管性蛋白尿的诊断标准之一. 展开更多
关键词 Dent病 肾病综合征 CLCN5基因
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Dent病合并肾衰竭2例报告并文献复习 被引量:4
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作者 张宏文 苏白鸽 +3 位作者 王芳 刘晓宇 肖慧捷 姚勇 《临床儿科杂志》 CAS CSCD 北大核心 2018年第6期416-419,共4页
目的探讨儿童Dent病合并肾衰竭的病因及预后。方法分析2014年1月至2016年12月收治的2例Dent病合并肾衰竭患儿的临床资料,并结合文献进行复习。结果 2例患儿均为男性,年龄分别为8、10岁;既往肾功能均正常,无肾脏钙化;肾衰竭发病前1周内... 目的探讨儿童Dent病合并肾衰竭的病因及预后。方法分析2014年1月至2016年12月收治的2例Dent病合并肾衰竭患儿的临床资料,并结合文献进行复习。结果 2例患儿均为男性,年龄分别为8、10岁;既往肾功能均正常,无肾脏钙化;肾衰竭发病前1周内均有上呼吸道病毒感染史。例1疾病急性期(10天)肾活检提示合并急性小管间质性肾炎,经激素治疗后肾功能完全恢复;例2病程6个月时肾活检提示合并亚急性小管间质性肾炎,经激素治疗后肾功能稍有改善但未恢复正常。结论对于Dent病患儿发生急性肾衰竭,特别是有上呼吸道病毒感染等诱因者,应及早行肾活检,以除外合并急性间质性肾炎的可能,从而及时治疗、改善预后。 展开更多
关键词 Dent病 肾衰竭 急性小管间质性肾炎 儿童
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儿童2型Dent病1例并文献复习 被引量:6
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作者 李国民 方晓燕 +6 位作者 徐虹 沈茜 孙利 翟亦晖 郭慕依 安宇 吴冰冰 《中国循证儿科杂志》 CSCD 2014年第6期456-459,共4页
目的总结1例2型Dent病患儿临床资料,提高对该病的认识。方法报道1例2型Dent病患儿的临床发现、相关实验室检查指标和肾活检病理改变。对该家系相关成员行CLCN5和OCRL基因外显子及附近调控区域直接测序,分析突变位点,并文献复习。结果患... 目的总结1例2型Dent病患儿临床资料,提高对该病的认识。方法报道1例2型Dent病患儿的临床发现、相关实验室检查指标和肾活检病理改变。对该家系相关成员行CLCN5和OCRL基因外显子及附近调控区域直接测序,分析突变位点,并文献复习。结果患儿,男,6岁起病,首发症状为单纯性蛋白尿,未见先天性白内障、智力低下、认知障碍及发育迟缓。实验室检查提示低分子蛋白尿,高钙尿症,镜下血尿,血清乳酸脱氢酶和磷酸肌酸激酶增高,肾活检病理提示轻微病变。患儿CLCN5和OCRL基因测序分析发现,CLCN5基因未见致病性突变,ORCL基因c.260del A(p.Q87fs105X)纯合突变,确诊为2型Dent病。家系OCRL基因突变分析显示,患儿父亲c.260del A未检出突变,母亲携带c.260del A杂合突变。c.260del A突变为移码突变,可导致OCRL基因编码蛋白截短。结论 2型Dent病以低分子蛋白尿、高钙尿症和镜下血尿为特征,血清乳酸脱氢酶、磷酸肌酸激酶增高和OCRL基因纯合移码突变支持2型Dent病诊断。 展开更多
关键词 2型Dent病 CLCN5基因 OCRL基因
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Influence of dent on residual ultimate strength of 2024-T3 aluminum alloy plate under axial compression 被引量:2
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作者 李志刚 张明义 +5 位作者 刘富 马春生 张金换 胡忠民 张嘉振 赵亚男 《Transactions of Nonferrous Metals Society of China》 SCIE EI CAS CSCD 2014年第10期3084-3094,共11页
Drop-weight impact tests were conducted on 2024-T3 aluminum plates with five types of impactors, and then the effects of the dent on the residual ultimate strength of the 2024-T3 specimens were investigated through ax... Drop-weight impact tests were conducted on 2024-T3 aluminum plates with five types of impactors, and then the effects of the dent on the residual ultimate strength of the 2024-T3 specimens were investigated through axial compression tests. Results indicate that with increase in dent depth, the five types of dents affect the ultimate strength of the plate in different trends. Nevertheless, other than the plate global deflection caused by impacting, the dent itself has unremarkable effect on the ultimate strength. The mathematical expressions are derived regarding the relationship between impact energy factor and the dent depth factor as well as the compressive ultimate strength reduction rate and the dent depth factor. 展开更多
关键词 aluminum alloy plate IMPACTOR DENT residual ultimate strength compression
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合并肾囊性病变儿童1型Dent病1例报告 被引量:2
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作者 张宏文 苏白鸽 +4 位作者 徐可 王芳 刘晓宇 肖慧捷 姚勇 《临床儿科杂志》 CAS CSCD 北大核心 2019年第2期141-143,共3页
目的探讨儿童Dent病1型合并肾囊性病变的可能发病原因。方法回顾1例16岁男性肾囊性病变患儿的临床资料,并复习相关文献。结果患儿发现蛋白尿、双肾多发囊肿4年,肾功能异常3年,临床主要表现为肾病水平的小分子蛋白尿、高钙尿症、肾功能... 目的探讨儿童Dent病1型合并肾囊性病变的可能发病原因。方法回顾1例16岁男性肾囊性病变患儿的临床资料,并复习相关文献。结果患儿发现蛋白尿、双肾多发囊肿4年,肾功能异常3年,临床主要表现为肾病水平的小分子蛋白尿、高钙尿症、肾功能异常并进行性加重、双肾多发囊肿、低钾血症。肾脏病理为局灶节段性肾小球硬化伴肾小管间质损伤。CLCN 5基因突变分析示c. 1975 delC(p.R 659 Gfs*7),NPHP相关基因未见突变。诊断为Dent病1型合并肾囊性病变、慢性肾脏病。结论 Dent病1型合并肾囊性病变较为罕见,其确切机制尚不清楚。 展开更多
关键词 Dent病 肾囊性病变 低钾血症 儿童
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小儿Dent病临床特征和基因突变分析 被引量:10
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作者 李鹏 黄建萍 《临床儿科杂志》 CAS CSCD 北大核心 2008年第4期298-301,共4页
目的探讨小儿Dent病的临床特征和分子遗传学特点。方法以2例拟诊的Dent病患儿及其家系中的女性为研究对象。盐析法从外周血白细胞提取基因组DNA,PCR扩增CLCN5基因所有的编码序列,PCR产物直接测序。结果2例患儿均有小分子量蛋白尿和高钙... 目的探讨小儿Dent病的临床特征和分子遗传学特点。方法以2例拟诊的Dent病患儿及其家系中的女性为研究对象。盐析法从外周血白细胞提取基因组DNA,PCR扩增CLCN5基因所有的编码序列,PCR产物直接测序。结果2例患儿均有小分子量蛋白尿和高钙尿症,其中1例有肾脏钙化,2例肾功能均正常。在这2个家系发现了2个CLCN5基因的突变,分别为L594fsX595和R637X,其中L594fsX595为新发现的突变。结论持续性小分子量蛋白尿和高钙尿症是国内小儿Dent病的主要临床特征,并证实了我国Dent病患儿存在CLCN5基因的突变。 展开更多
关键词 Dent病 CLCN5 小分子量蛋白尿 高钙尿症
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儿童Dent病9例临床特征及基因分析 被引量:2
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作者 李华荣 陈朝英 孙金山 《中国医刊》 CAS 2018年第10期1139-1144,共6页
目的总结儿童Dent病的临床特点并分析其基因突变类型。方法收集2013年1月至2017年10月于本院住院的9例临床诊断为Dent病患儿的临床资料,并进行CLCN5和OCRL1基因检测。结果 9例患儿均表现为不同程度的小分子量蛋白尿,8例蛋白尿达肾病水... 目的总结儿童Dent病的临床特点并分析其基因突变类型。方法收集2013年1月至2017年10月于本院住院的9例临床诊断为Dent病患儿的临床资料,并进行CLCN5和OCRL1基因检测。结果 9例患儿均表现为不同程度的小分子量蛋白尿,8例蛋白尿达肾病水平,8例有高钙尿症,2例有佝偻病,1例有血尿,1例有糖尿,均无肾功能不全,2例肾脏超声于肾盏中可见钙化影,1例伴有白内障及轻度智力发育落后。其中5例患儿检测到CLCN5基因突变,包括1例无义突变c.1249C>T(p.R417X),1例错义突变c.1484A>G(p.495,Y>C),3例框移突变IVS6,+2T>C、c.1187_1189delGCA(p.G396_I397delinsV)、c.1066:delT(p.L356Wfs461)。1例患儿检测到OCRL1基因为错义突变c.812T>C(p.271, I>T)。结论通过CLCN5和OCRL1基因分析,共检测到5种CLCN5基因突变和1种OCRL1基因突变,均为非热点突变,提示中国儿童Dent病基因突变可能有其自身特点。部分Dent病可表现为肾病水平的蛋白尿合并白蛋白尿,应进一步完善尿蛋白电泳及基因检测明确诊断。 展开更多
关键词 低分子量蛋白尿 高钙尿症 Dent病 基因
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加拿大沥青改进测力延度试验(DENT)介绍 被引量:9
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作者 聂忆华 胡静轩 《中外公路》 北大核心 2018年第3期242-247,共6页
路面沥青低温开裂和耐疲劳开裂现象非常普遍,中国采用延度试验进行控制,效果并不理想。该文介绍加拿大安大略省成功研究和推广应用的DENT试验方法,该法为延度、测力延度试验的改进方法,提出了3个双边缺口韧带宽度同时拉伸测量,用基本断... 路面沥青低温开裂和耐疲劳开裂现象非常普遍,中国采用延度试验进行控制,效果并不理想。该文介绍加拿大安大略省成功研究和推广应用的DENT试验方法,该法为延度、测力延度试验的改进方法,提出了3个双边缺口韧带宽度同时拉伸测量,用基本断裂功、塑性断裂功、临界开裂位移CTOD等指标来评价路面沥青的低温性能及耐疲劳性能。重点介绍了此法与延度、测力延度方法的差异、试验理论、试验数据采集分析方法、研究推广应用情况等,为中国沥青路面低温性能和耐疲劳开裂性能的提升提供重要参考。 展开更多
关键词 路面沥青 低温性能 疲劳性能 DENT试验
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Dynamic response analysis of the submarine suspended pipeline impacted by dropped objects based on LS-DYNA 被引量:4
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作者 娄敏 明海芹 《Marine Science Bulletin》 CAS 2015年第2期39-55,共17页
Based on ANSYS/LS-DYNA, the dynamics analysis software, and using nonlinear dynamic finite element method, the process of a submarine pipeline impacted by dropped objects is simulated, Some conclusions are drawn: Fir... Based on ANSYS/LS-DYNA, the dynamics analysis software, and using nonlinear dynamic finite element method, the process of a submarine pipeline impacted by dropped objects is simulated, Some conclusions are drawn: First, the damage depth of suspended pipe is smaller than that of the bare pipe in case of the same fall energy, and with the increase of fall energy, the difference grows; Second, with the falling object's speed and mass rising, the deformation of pipeline intensifies at the impact site and the maximum vibration amplitude of submarine pipeline increases; Third, when the fall energy is equal, the smaller the contact area of falling objects and pipeline is, the greater the damage depth of pipeline becomes; Fourth, changes of seabed soil parameters (shear elastic modulus, internal friction angle, density) have less influence on the suspended pipeline's dent depths and maximum vibration amplitude, 展开更多
关键词 suspended pipeline IMPACT dent depths dynamic response
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