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Special electromyographic features in a child with paramyotonia congenita: A case report and review of literature

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World Journal of Clinical Cases 2024年第3期12卷 587-595页

作者： hui Yi Chen-xiang liu Shu-Xin Ye Yu-Lin liu Department of Neuroelectrophysiology Qilu Children’s Hospital of Shandong UniversityJinan 250000Shandong ProvinceChina Department of Neuroelectrophysiology University of Health and Rehabilitation Sciences(Qingdao Municipal Hospital)Qingdao 266000Shandong ProvinceChina

BACKGROUND Paramyotonia congenita(PMC)stands as a rare sodium channelopaty of skeletal muscle,initially identified by Eulenburg.The identification of PMC often relies on electromyography(EMG),a diagnostic technique.The child’s needle EMG unveiled trains of myotonic discharges with notably giant amplitudes,alongside irregular wave trains of myotonic discharges.This distinctive observation had not surfaced in earlier studies.CASE SUMMARY We report the case of a 3-year-old female child with PMC,who exhibited la-ryngeal stridor,muffled speech,myotonia from birth.Cold,exposure to cool water,crying,and physical activity exacerbated the myotonia,which was relieved in warmth,yet never normalized.Percussion myotonia was observable in bilateral biceps.Myotonia symptoms remained unchanged after potassium-rich food consumption like bananas.Hyperkalemic periodic paralysis was excluded.Cranial magnetic resonance imaging yielded normal results.Blood potassium remained within normal range,while creatine kinase showed slight elevation.Exome-wide genetic testing pinpointed a heterozygous mutation on chromosome SCN4A:c.3917G>A(p.G1306E).After a six-month mexiletine regimen,symptoms alleviated.CONCLUSION In this case revealed the two types of myotonic discharges,and had not been documented in other studies.We underscore two distinctive features:Giant-amplitude potentials and irregular waves.

关键词： Paramyotonia congenita Channelopathy Electromyography Child Case report

Atroposelective Synthesis of 2-Arylindoles via Chiral Phosphoric Acid-Catalyzed Direct Amination of Indoles

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Chinese Journal of Chemistry 2024年第7期42卷 731-735页

作者： Wen Bao Ye-hui Chen Yu-Wei liu Shao-Hua xiang Bin Tan School of Pharmacy&StateKey Laboratory of Applied Organic Chemistry Lanzhou UniversityLanzhouGansu 730000China Shenzhen Grubbs Institute Department of ChemistrySouthern University of Science and TechnologyShenzhenGuangdong 518055China

Indole-based atropisomers are a very important class of axially chiral compounds.However,the atroposelective synthesis of axially chiral 2-arylindole remains largely unexplored.In this study,we report the successful synthesis of atropisomeric 2-arylindoles using direct amination of indoles with p-quinonediimines in the presence of chiral phosphoric acid as a catalyst.Quinonediimine acts as an aminating reagent through formal polarity inversion of imine.The malonate group on the 2-aryl of 2-indoles was found to be essen-tial for high enantioselectivity of the products.This could be due to the additional interaction between the ester group and the cata-lyst,as well as the intramolecular hydrogen bonding.Our findings provide a new strategy for the asymmetric construction of 2-arylindole atropisomers.

关键词： Chiral phosphoric acid Quinonediimines Direct amination Nucleophilic addition Asymmetric catalysis Enantioselectivity Axially chiral 2-arylindole Atroposelective synthesis

A Review of Type 1 and Type 2 Intraductal Papillary Neoplasms of the Bile Duct

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Current Medical Science 2024年第3期44卷 485-493页

作者： Xia-hui HUANG Tian-xiang CHEN Hong-liang liu Ming-wen HUANG Department of General Surgery The Second Affiliated Hospital of Nanchang UniversityNanchang330006China

Intraductal papillary neoplasm of the bile duct(IPNB)is a heterogeneous disease similar to intraductal papillary mucinous neoplasm of the pancreas.These lesions have been recognized as one of the three major precancerous lesions in the biliary tract since 2010.In 2018,Japanese and Korean pathologists reached a consensus,classifying IPNBs into type l and type 2 IPNBs.IPNBs are more prevalent in male patients in East Asia and are closely related to diseases such as cholelithiasis and schistosomiasis.From a molecular genetic perspective,IPNBs exhibit early genetic variations,and different molecular pathways may be involved in the tumorigenesis of type 1 and type 2 IPNBs.The histological subtypes of IPNBs include gastric,intestinal,pancreaticobiliary,or oncocytic subtypes,but type 1 IPNBs typically exhibit more regular and well-organized histological features than type 2 IPNBs and are more commonly found in the intrahepatic bile ducts with abundant mucin.Due to the rarity of these lesions and the absence of specific clinical and laboratory features,imaging is crucial for the preoperative diagnosis of IPNB,with local bile duct dilation and growth along the bile ducts being the main imaging features.Surgical resection remains the optimal treatment for IPNBs,but negative bile duct margins and the removal of lymph nodes in the hepatic hilum significantly improve the postoperative survival rates for patients with IPNBs.

关键词： intraductal papillary neoplasm of the bile duct subclassification neoplasms precancerous tissue prognosis

Removal of Cr(Ⅵ)by glutaraldehyde-crosslinked chitosan encapsulating microscale zero-valent iron:Synthesis,mechanism,and longevity

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Journal of Environmental Sciences 2024年第8期142卷 115-128页

作者： Yijun Duan Fang liu xiang liu Miao Li School of Environment Tsinghua UniversityBeijing 100084China Transportation Institute of Inner Mongolia University Hohhot 010070China

Microscale zero-valent iron(mZVI)has shown great potential for groundwater Cr(Ⅵ)remediation.However,low Cr(Ⅵ)removal capacity caused by passivation restricted the wide use of mZVI.We prepared mZVI/GCS by encapsulating mZVI in a porous glutaraldehydecrosslinked chitosan matrix,and the formation of the passivation layer was alleviated by reducing the contact between zero-valent iron particles.The average pore diameter of mZVI/GCS was 8.775 nm,which confirmed the mesoporous characteristic of this material.Results of batch experiments demonstrated that mZVI/GCS exhibited high Cr(Ⅵ)removal efficiency in a wide range of pH(2-10)and temperature(5-35℃).Common groundwater coexisting ions slightly affected m ZVI/GCS.The material showed great reusability,and the average Cr(Ⅵ)removal efficiency was 90.41%during eight cycles.In this study,we also conducted kinetics and isotherms analysis.Pseudo-second-order model was the most matched kinetics model.The Cr(Ⅵ)adsorption process was fitted by both Langmuir and Freundlich isotherms models,and the maximum Langmuir adsorption capacity of m ZVI/GCS reached243.63 mg/g,which is higher than the adsorption capacities of materials reported in most of the previous studies.Notably,the column capacity for Cr(Ⅵ)removal of a mZVI/GCS-packed column was 6.4 times higher than that of a mZVI-packed column in a 50-day experiment.Therefore,mZVI/GCS with a porous structure effectively relieved passivation problems of mZVI and showed practical application prospects as groundwater Cr(Ⅵ)remediation material with practical application prospects.

关键词： Chitosan Glutaraldehyde mZVI Cr(Ⅵ) Groundwater

A Novel Design of a Pre-separation Panel Structure to Protect Against the Impact of an Explosion in an Aircraft Plenum Chamber

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Acta Mechanica Solida Sinica 2024年第1期37卷 166-180页

作者： Zongxing liu xiang Yang Jun liu School of Aeronautics Northwestern Polytechnical UniversityXi'an 710072China COMAC Shanghai Aircraft Design and Research Institute Shanghai 201210China

To reduce the damage of the pressurizing panel structure of a fuselage caused by an explosion at the“least risk bomb location”in an aircraft structure,a new pre-separation panel structure was designed to resist blast loading.First,the dynamic strain response and morphology of impact damage of the new pre-separation panel were measured in an impact damage test.Second,the commercial software LS-DYNA was used to calculate the propagation of the blast shock wave,and the results were compared with empirical equations to verify the rationality of the numerical calculation method.Finally,the fluid–structure coupling method was used to calculate the damage process of the pre-separation panel structure under the impact of an explosion wave and an impact block.The calculated results were in good agreement with the test results,which showed the rationality of the calculation method and the model.The residual strength of the damaged pre-separation panel was significantly higher than that of the original damaged panel.The results show that the new pre-separation panel structure is reasonable and has certain significance for guiding the design of plenum chambers with strong resistance to implosion for aircraft fuselages.

关键词： Pre-separation panel structure Explosive response Residual strength Aircraft plenum chamber Least risk bomb location

High-throughput and simultaneous inertial separation of tumor cells and clusters from malignant effusions using spiral-contraction-expansion channels

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Microsystems & Nanoengineering 2024年第2期10卷 91-99页

作者： Zhixian Zhu hui Ren Dan Wu Zhonghua Ni Nan xiang School of Mechanical Engineering and Jiangsu Key Laboratory for Design and Manufacture of Micro-Nano Biomedical InstrumentsSoutheast University211189NanjingChina School of Mechanical Technology Wuxi Institute of TechnologyNo.1600 Gaolang West Road214129WuxiChina Department of Oncology Jiangyin People’s Hospital214400JiangyinChina

Tumor cell clusters are regarded as critical factors in cancer pathophysiology,and increasing evidence of their higher treatment resistance and metastasis compared to single tumor cells has been obtained.However,existing cell separation methods that are designed for single tumor cells cannot be used to simultaneously purify tumor cell clusters.To address this problem,we demonstrated a microfluidic approach for the high-throughput,continuous-flow ternary separation of single tumor cells,tumor cell clusters,and WBCs from clinical pleural or abdominal effusions by coupling slanted spiral channels and periodic contraction-expansion arrays.We first systematically explored the influence of particle size and flow rate on particle focusing.The separation performance indicated that 94.0%of WBCs were removed and more than 97%of MDA-MB-231 tumor cells were recovered at a high flow rate of 3500µL/min.Moreover,more than 90%of tumor cell clusters were effectively preserved after separation.Finally,we successfully applied our device for the ternary separation of single tumor cells,tumor cell clusters,and WBCs from different malignant effusions collected from patients with metastatic cancer.Thus,our spiral-contraction-expansion device has potential as a sample pretreatment tool for the cytological diagnosis of malignant effusions.

关键词： separation contraction removed

Review of dynamics and active control of large-scale space membrane antenna

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Astrodynamics 2024年第1期8卷 1-26页

作者： xiang liu Guoping Cai Department of Engineering Mechanics MOE Key Laboratory of HydrodynamicsChongqing Research InstituteShanghai Jiao Tong UniversityShanghai200240China

Large-scale space membrane antennas have significant potential in satellite communication,space-based early warning,and Earth observation.Because of their large size and high flexibility,the dynamic analysis and control of membrane antenna are challenging.To maintain the working performance of the antenna,the pointing and surface accuracies must be strictly maintained.Therefore,the accurate dynamic modeling and effective active control of large-scale space membrane antennas have great theoretical significance and practical value,and have attracted considerable interest in recent years.This paper reviews the dynamics and active control of large-scale space membrane antennas.First,the development and status of large-scale space membrane antennas are summarized.Subsequently,the key problems in the dynamics and active control of large membrane antennas,including the dynamics of wrinkled membranes,large-amplitude nonlinear vibration,nonlinear model reduction,rigid-flexible-thermal coupling dynamic modeling,on-orbit modal parameter identification,active vibration control,and wave-based vibration control,are discussed in detail.Finally,the research outlook and future trends are presented.

关键词： large-scale space membrane antenna dynamicmodeling active control

Modeling of LF refining process:a review

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Journal of Iron and Steel Research(International) 2024年第2期31卷 289-317页

作者： Zi-cheng Xin Jiang-shan Zhang Kai-xiang Peng Jun-guo Zhang Chun-hui Zhang Qing liu State Key Laboratory of Advanced Metallurgy University of Science and Technology BeijingBeijing100083China School of Automation and Electrical Engineering University of Science and Technology BeijingBeijing100083China Tangshan Branch Hebei Iron and Steel Co.Ltd.Tangshan063000HebeiChina Xinjiang Bayi Iron and Steel Co. Ltd.Baosteel GroupWulumuqi830022XinjiangChina

With the increasing demand for energy conservation and emission reduction,more attentions have been paid to the intelligentization,greenization and low carbonization during the transformation and upgrading of steelmaking plants.Ladle furnace(LF)refining is one of the key procedures in steelmaking process and has been widely used in steelmaking plants for its high equipment matching degree,low equipment investment and outstanding refining performance.According to the main tasks of LF refining process,the modeling methods of temperature prediction model,slag-making model,alloying model,argon blowing model and model of inclusions behavior were systematically reviewed,and the advantages and disadvantages of each modeling method were summarized.In addition,the technical framework for the future has also been proposed based on existing works,including classification of raw materials,graphic representation of knowledge,introduction,upgradation and management of device/equipment,customization of steelmaking,modeling of refining process,synergy of models,intelligentization of decision-making,automation of control,and digitization of processes and operations,aiming to provide a reference for the modeling and intelligent development of LF refining process.

关键词： LF refining Temperature prediction Slag-making Alloying Argon blowing Inclusion behavior Modeling method

Vanadium(V) Complexes Containing Unsymmetrical N-Heterocyclic Carbene Ligands: Highly Efficient Synthesis and Catalytic Behavior towards Ethylene/Propylene Copolymerization

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Chinese Journal of Polymer Science 2024年第1期42卷 32-41,I0006页

作者： Yi-Cong Wang hui Zhat Pei-Yi Cheng Shu Zhang xiang-Wei liu Yi-Xian Wu State Key Laboratory of Chemical Resource Engineering Beijing University of Chemical TechnologyBejing 100029China

The highly efficient method has been developed for the synthesis of NHC·VOCl_(3) containing symmetrical or unsymmetrical Nheterocyclic carbene(NHC) ligands by the transmetallation reaction of NHC·AgCl with VOCl_(3).The total isolated yield of VOCl_(3)[1,3-(2,4,6-Me_(3)C_(6)H_(2))_(2)(NCH=)_(2)C:](V4') reached 86% by transmetallation reaction,which is much higher than that(48%) by direct coordination method.This methodology has also been used to synthesize the novel vanadium complexes containing unsymmetrical NHC ligands of VOCl_(3)[PhCH_(2)NCH=CHNR)C:](V5',R=2,4,6-Me_(3)C_(6)H_(2);V6',R=2,4-Me_(2)-6-Ph-C_(6)H_(2);V7',R=2,6-^(i)Pr_(2)-C_(6)H_(3)) with high yield,which could not be obtained by direct coordination method.The catalytic activity and copolymerization ability would be improved by introducing unsymmetrical NHC ligands due to their less steric bulky effect.The vanadium complex V5' containing unsymmetrical NHC ligand exhibits higher catalytic activity(3.7×10^(5)g_(copolymer)·mol^(-1) of V·h^(-1)) than that of V4' containing symmetrical NHC ligand.Moreover,the higher propylene incorporation ratio(45.6 mol%) in the copolymers of ethylene with propylene could be obtained by using V5' than that(39.9%) by using V4'.The results would provide a highly efficient strategy for the synthesis of early transition metal complexes containing versitile NHC ligands,affording the catalyst with both high catalytic activity and copolymerization ability for the synthesis of high performance polyolefin elastomers.

关键词： Vanadium complex N-heterocyclic carbene ligand Transmetallation reaction Copolymerization of ethylene with propylene