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MYH11延长突变导致巨膀胱-小结肠-肠蠕动不良综合征1例报告及文献复习

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临床儿科杂志 2024年第9期42卷 798-804页

作者：周洁刘克强王金玲王莹上海交通大学医学院附属新华医院儿消化营养科上海市儿科医学研究所上海市小儿消化与营养重点实验室上海200092

目的报告1例MYH11基因杂合新发突变导致巨膀胱-小结肠-肠蠕动不良综合征(MMIHS)患儿的临床表现与遗传信息,并通过文献回顾性分析进行基因型-表型关联研究。方法分析2023年10月因“反复腹胀、呕吐3年余”来院就诊的MMIHS患儿临床资料,采... 详细信息

目的报告1例MYH11基因杂合新发突变导致巨膀胱-小结肠-肠蠕动不良综合征(MMIHS)患儿的临床表现与遗传信息,并通过文献回顾性分析进行基因型-表型关联研究。方法分析2023年10月因“反复腹胀、呕吐3年余”来院就诊的MMIHS患儿临床资料,采集患儿及父母与姐姐的外周血样,通过Trio-WES及Sanger测序筛查致病突变。并对相关文献进行总结分析。结果患儿,男,15岁,主要临床表现为频繁发作的腹胀、呕吐和腹痛,腹部立位片及上消化道造影提示肠梗阻,给予灌肠、抗感染和静脉营养支持等治疗后病情稳定。基因检测结果显示,患儿携带MYH11基因杂合新发变异c.5819delC(***1940Hisfs*91),该变异导致肌球蛋白重链C端延长,父母及姐姐均未检测出该变异。文献检索共纳入7篇与MYH11基因变异有关文献,变异类型包括错义突变、移码突变和染色体微缺失。共20例,2例因提前终止妊娠性别不明,其余患者男女比2∶1。基因型-表型关联分析发现,15例携带MYH11基因延长突变显性遗传患者的发病年龄9(0~28)岁,尚未报道死亡案例。5例携带双等位基因丧失功能突变的隐性遗传患者发病年龄均小于1岁,死亡4例(80%)。结论本例MMIHS患儿携带MYH11基因c.5819delC(***1940Hisfs*91)变异。与携带MYH11基因变异隐性遗传的患者相比,MYH11延长突变显性遗传患者大部分发病较晚,临床表现较轻,生存率较高。

关键词：巨膀胱-小结肠-肠蠕动不良综合征 MYH11基因儿童

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蒸压加气混凝土砌块的性能与质量控制

中国厨卫 2024年第8期23卷 69-71页

作者：王金玲辽宁省检验检测认证中心沈阳110032

蒸压加气混凝土砌块因其轻质、高强、保温性好、吸声性好、耐火性强、无有害气体散发等优点,在建筑领域得到了广泛应用。为确保其性能和质量,需要进行有效的质量控制。文章从蒸压加气混凝土砌块的性能和质量控制两方面进行了综述,包括... 详细信息

蒸压加气混凝土砌块因其轻质、高强、保温性好、吸声性好、耐火性强、无有害气体散发等优点,在建筑领域得到了广泛应用。为确保其性能和质量,需要进行有效的质量控制。文章从蒸压加气混凝土砌块的性能和质量控制两方面进行了综述,包括砂、粉煤灰、石灰和水泥等关键原材料的质量要求。通过对这些方面的控制,可以保证蒸压加气混凝土砌块具有良好的性能和稳定的质量。

关键词：蒸压加气混凝土砌块性能质量控制

Integrity monitoring scheme for single-epoch GNSS PPP-RTK positioning

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Satellite Navigation 2023年第1期4卷 13-27,I0002页

作者： Wenhao Zhang jinling wang University of New South Wales(UNSW) SydneyAustralia

Integrity monitoring for precise point positioning is critical for safety-related *** the increasing demands of high-accuracy autonomous navigation for unmanned ground and aerial vehicles,the integrity monitoring method of high-precision positioning has become an essential *** high precision Global Navigation Satellite Systems(GNSS)positioning is widely used in such applications,there are still many difculties in the integrity monitoring method for the multi-frequency multi-GNSS undiferenced and uncombined Precise Point Positioning(PPP).The main difculties are caused by using the measurements of multiple epochs in *** on the baseline Multiple Hypothesis Solution Separation(MHSS)Advanced Receiver Autonomous Integrity Monitoring(ARAIM)algorithm,this paper discusses the feasibility of the pseudorange-based baseline ARAIM method on the single-epoch PPP based on Real-Time Kinematic(RTK)networks(PPP-RTK)framework to overcome these *** addition,a new scheme is proposed to transfer the conventional PPP process into the single-epoch PPP-RTK *** simulation results using the proposed model are analyzed in this *** Protection Levels(PLs)estimated by PPP Wide-lane Ambiguity Resolution(PPP-WAR)model with regional corrections can reach the meter level and the PLs estimated by PPP Ambiguity Resolution(PPP-AR)and PPP-RTK models are usually the sub-meter *** a horizontal Alert Limit(AL)of 1.5 m,the global coverage of availability above 99.9%for PPP-WAR,PPP-AR,and PPP-RTK can reach 92.6%,99.4%,and 99.7%*** results using real kinematic data also show that tight PLs can be achieved when the observation conditions are good.

关键词： Integrity monitoring Precise point positioning Multi-GNSS Ambiguity resolution Single-epoch positioning

新疆多线缨小蜂属多利多线缨小蜂亚属记述(膜翅目:缨小蜂科)

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西北农业学报 2024年第8期33卷 1567-1579页

作者：沙仁高娃朱丽得孜·艾山王金玲新疆大学生命科学与技术学院新疆生物资源基因工程重点实验室乌鲁木齐830017

采用形态鉴定的方法鉴定了采自新疆的多线缨小蜂属(Polynema Haliday)的多利多线缨小蜂亚属(Doriclytus Foerster)4个种,其中1新种新疆多线缨小蜂[Polynema(Doriclytu)xinjiangense Sharengaowa&Aishan ***.],以及中国新记录种3种,分别为褐柄多线缨小蜂[P.(Doriclytus)atratum Haliday,1833]、长管多线缨小蜂[P.(Doriclytus)euchariforme Haliday,1833]、林木多线缨小蜂[P.(Doriclytus)woodi Hinks,1950],对种征进行了详细描述,并附形态特征图,提供了中国多利多线缨小蜂亚属[P.(Doriclytus)Foerster]已知种检索表。

关键词：小蜂总科多线缨小蜂新疆分类

Homo erectus pekinensis's age and environment——with discussion on the relationship among Homo erectus from Beijing,Nanjing and Chenjiawo

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Science China Earth Sciences 2004年第1期47卷 68-77页

作者： LIU jinling wang Weiming Nanjing Institute of Geology and Palaeontology Chinese Academy of Sciences Nanjing 210008 China Nanjing Institute of Geology and Palaeontology Chinese Academy of Sciences Nanjing 210008 China

This paper inspects the relationship between Homo erectus from Beijing, Nanjing and Chenjiawo on the viewpoint of environmental variations. Pieces of evidence show that Nanjing and Chenjiawo Homo erectus lived in glacial age at the Marine Oxygen Isotope Stage (MIS) 16, while Homo erectus pekinensis in major interglacial age at MlSs 15-13. It is also recovered that the cave deposits in Tangshan, Nanjing, and Zhoukoudian, Beijing, as well as the deposits at the Homo erectus site in Chenjiawo, have undergone some similar development process. All of them originated from early-middle Middle Pleistocene, and have gone through the test of the major glacial age at MIS 16 and the major interglacial period at the paleosol stage S5. At the same time, they were also closely connected with some environment events, such as the uplift of the Qinghai-Xizang Plateau, the formation of glaciations and the loess accumulations.

关键词： Homo erectus from Beijing, Nanjing and Chenjiawo, climate and environment, comprehensive comparisons.

A review of measurement methods for peracetic acid (PAA)

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Frontiers of Environmental Science & Engineering 2020年第5期14卷 127-136页

作者： Chen Cheng Haodong Li jinling wang Hualin wang Xuejing Yang National Engineering Laboratory for Industrial Wastewater Treatment East China University of Science and Technology(ECUST)Shanghai 200237China State Key Laboratory of Chemical Engineering ECUSTShanghai 200237China

Peroxyacetic acid has been widely used in food,medical,and synthetic chemical fields for the past several ***,peroxyacetic acid has gradually become an effective alternative disinfectant in wastewater disinfection and has strong redox capacity for removing micro-pollutants from drinking ***,commercial peroxyacetic acid solutions are primarily multi-component mixtures of peroxyacetic acid,acetic acid,hydrogen peroxide,and *** the process of water treatment,peroxyacetic acid and hydrogen peroxide(H2O2)often coexist,which limits further investigation on the properties ofperoxyacetic ***,analytical methods need to achieve a certain level of selectivity,particularly when peroxyacetic acid and hydrogen peroxide *** review summarizes the measurement and detection methods of peroxyacetic acid,comparing the principle,adaptability,and relative merits of these methods.

关键词： Peroxyacetic acid Measurement methods Titration Colorimetric Chromatography NMR

A review of pulse electrolysis for efficient energy conversion and chemical production

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Journal of Energy Chemistry 2021年第8期30卷 69-82,I0003页

作者： Tao Liu jinling wang Xuejing Yang Ming Gong Department of Chemistry and Shanghai Key Laboratory of Molecular Catalysis and Innovative Materials Fudan University200438 ShanghaiChina National Engineering Laboratory for Industrial Wastewater Treatment East China University of Science and Technology200237 ShanghaiChina

Electrochemical transformation emerges as an important solution to sustainable energy conversion and chemical *** electrolytic systems usually operate under galvanostatic or potentiostatic conditions that sometimes result in unsatisfactory efficiencies or *** electrolysis by pulsating and programming the potentials/currents can feature unique tunability to the electrodeelectrolyte interface properties that can give rise to drastically different electrochemical behaviors compared to the steady-state *** invented almost 100 years ago,pulse electrolysis has received little attention over the period,but has recently attracted a revived focus toward the energyefficient *** review will summarize the history and recent efforts of pulse electrolysis in three categories:water electrolysis,CO_(2)electrolysis and other *** each section,the advantage of pulse electrolysis over steady-state electrolysis will be discussed in detail,giving a comprehensive overview of the pulse effect on the electrolytic ***,we will provide our vision of future directions in pulse electrolysis based on previous works.

关键词： Pulse electrolysis Energy conversion Chemical production Water splitting CO_(2) reduction Energy electrocatalysis

新型闪速浮选机回收分级沉砂中石墨矿的试验研究

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矿冶工程 2024年第4期44卷 181-184页

作者：张福亚樊学赛张跃军王金玲北矿机电科技有限责任公司北京100160 矿冶科技集团有限公司北京100160

将闪速浮选工艺应用于晶质石墨浮选中,研究了闪速浮选机叶轮转速、表观充气速率和泡沫层厚度对石墨矿分选指标的影响。结果表明,在叶轮转速750 r/min、表观充气速率0.3 m^(3)/(m^(2)·min)、泡沫层厚度20 mm条件下,石墨矿分选指标较好... 详细信息

将闪速浮选工艺应用于晶质石墨浮选中,研究了闪速浮选机叶轮转速、表观充气速率和泡沫层厚度对石墨矿分选指标的影响。结果表明,在叶轮转速750 r/min、表观充气速率0.3 m^(3)/(m^(2)·min)、泡沫层厚度20 mm条件下,石墨矿分选指标较好。精矿粒级分析和显微观测结果表明,闪速浮选对0.074~0.180 mm粒级石墨回收效果较好,该粒级主要是晶质石墨单体以及富连生体。闪速浮选工艺能提前回收分级沉砂中的有用矿物,降低了过磨概率,实现对鳞片的保护,该工艺有望在晶质石墨矿浮选中实现工业应用。

关键词：晶质石墨闪速浮选叶轮转速充气速率泡沫层厚度

Mobile mapping with ubiquitous point clouds

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Geo-Spatial Information Science 2016年第3期19卷 169-170页

作者： Bisheng Yang jinling wang State Key Laboratory of Information Engineering in Surveying Mapping and Remote SensingWuhan UniversityWuhanChina School of Civil and Environmental Engineering The University of New South WalesSydneyAustralia

The 9th International Symposium on Mobile Mapping Technology(MMT 2015)was successfully held in Sydney,Australia on 9−11 December,***2015 attracted about 300 registered participants from 35 countries/regions,and received 156 full paper submissions,with topics ranging from new mapping concepts,the state of the art of technology,multi-disciplinary approaches,new applications,to future ***,a large number of submissions explore the new methods of point cloud processing for many applications.

关键词： Sydney mapping MMT