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Ketogenic diet therapy in children with epilepsy caused by SLC2A1 mutations:a single-center single-arm retrospective study

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World Journal of Pediatrics 2024年第5期20卷 517-524页

作者： Ying-Yan Wang Yun-Qing Zhou li-Juan Luo cui-Jin Wang Nan Shen Hao li Ji-Wen Wang Department of Neurology Shanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong UniversityShanghaiChina Department of Infectious Diseases Shanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong UniversityShanghaiChina Clinical Research Ward Clinical Research CenterShanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong UniversityShanghaiChina

Background This retrospective study assessed the efficacy and safety of ketogenic diet therapies in children with epilepsy caused by SLC2A1 genetic mutations and glucose transporter type 1 deficiency syndrome.Methods Pediatric patients with epilepsy symptoms admitted to our medical center between January 2017 and October 2021 were included if they presented with an SLC2A1 genetic mutation on whole-exome sequencing.We analyzed the patients’convulsions and treatment with antiepileptic drugs.The patients were followed up at different time periods after ketogenic diet therapies.Results Six patients with SLC2A1 mutations were included in this study.The patients had seizures of different types and frequencies,and they took antiepileptic drugs to relieve their symptoms.They were then treated with a ketogenic diet for at least four months.We analyzed epilepsy control rates at 1,2,3,6,and 12 months after ketogenic diet treatment.All patients were seizure-free within a month of receiving the diet therapy.All patients were followed up for six months,three were followed up for 12 months after the treatment,and there was no recurrence of epilepsy during this period.After antiepileptic drug withdrawal,none of the patients experienced seizure relapse when receiving ketogenic diet treatment alone.No severe adverse events occurred during the therapy.Conclusions Ketogenic diet therapy is very effective and safe for the treatment of epilepsy caused by SLC2A1 mutations.Therefore,patients with glucose transporter type 1 deficiency syndrome caused by SLC2A1 mutations should begin ketogenic diet treatment as soon as possible.

关键词： Epilepsy Glucose transporter type 1 Ketogenic diet SLC2A1

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Investigating effect of coke porosity on blast furnace performance based on multi-physical fields

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Journal of Iron and Steel Research(International) 2024年第2期31卷 357-367页

作者： Ji Chen Zhao Lei Zhe Yao li Wang Qiang ling Ping cui Anhui Province Key Laboratory of Coal Clean Conversion and High Valued Utilization School of Chemistry and Chemical EngineeringAnhui University of TechnologyMa’anshan243002AnhuiChina Shanxi Academy of Printing Technology Research Institute Co. Ltd.Xi’an710077ShaanxiChina

Reducing coke use is an effective measure to reduce carbon emission and energy consumption in the blast furnace(BF)ironmaking.Essentially,BF is a high-temperature moving bed reactor,where complex physical transformations coupled with complicated reactions occur.This makes it challenging to investigate the factors determining BF performance with the conventional method.A multi-physical field coupling mathematical model of BF was thus developed to describe its mass and heat transfer as well as its intrinsic reactions.Then,the proposed model was validated with the production data.Under coupling conditions,influences of dominating reactions on BF performance(temperature distribution,gas distribution,iron formation reaction,and direct reduction degree)were revealed.The results indicated that coke combustion,indirect reduction,and direct reduction of iron ore mainly took place nearby the shaft tuyere,cohesive zone,and dripping zone,respectively.Besides,the rate of coke solution loss reaction was increased with the rising coke porosity in the cohesive zone.Considering the effect of coke porosity on the efficiency and stability of BF,the coke porosity of 0.42 was regarded as a reasonable value.

关键词： Blast furnace Mathematical model Multi-physical field Simulation Coke porosity

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Sn-decorated CeO_(2) with different morphologies for direct dehydrogenation of ethylbenzene

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Journal of Rare Earths 2024年第1期42卷 102-109,I0004页

作者： Kai Zhang Guoju cui Mengzhen Yuan Huiwen Huang Ning li Jiale Xu Guowei Wang Chunyi li State Key Laboratory of Heavy Oil Processing China University of Petroleum(East China)Qingdao266580China Petrochemical Plant of Sinopec Shengli Oilfield Company Dongying257237China College of Chemical and Biological Engineering Shandong University of Science and TechnologyQingdao266590China

Three Sn-decorated ceria catalysts with various morphologies(rods,particles,and cubes)were prepared and applied to the direct dehydrogenation of ethylbenzene.Multi-technology characterizations,including X-ray photoelectro n spectroscopy(XPS),H_(2)-tempe rature programmed reduction(H_(2)-TPR),and Raman spectroscopy,prove that the oxygen vacancies are the active sites for ethylbenzene dehydrogenation,which can be regulated by engineering CeO_(2) morphology and enhanced via introducing metal Sn.Given the results of activity test,the catalytic activities for ethylbenzene dehydrogenation over different samples are closely dependent on the amount of oxygen vacancies.The reduced Sn-decorated CeO_(2) catalyst with nanoparticles morphology exhibits better dehydrogenation performance than the other two studied catalysts at 600℃.This work provides an effective approach to regulate the active oxygen vacancies and further enhance the dehydrogenation activities through engineering the surface morphology of the catalyst and introducing suitable additives.

关键词： Ethylbenzene dehydrogenation CeO_(2) Sn Morphology Oxygen vacancy Rare earths

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Recent progress in functional metal-organic frameworks for bio-medical application

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Regenerative Biomaterials 2024年第2期3卷 37-54页

作者： Wenwen Chai Xiaochen Chen Jing liu liyan Zhang Chunyu liu li li John Robert Honiball Haobo Pan Xu cui Deping Wang School of Materials Science and Engineering Tongji UniversityShanghai 201804China Shenzhen Institute of Advanced Technology Chinese Academy of SciencesShenzhen 518055China Department of Orthopaedics&Traumatology School of Clinical MedicineLi Ka Shing Faculty of MedicineThe University of Hong KongHong KongChina

Metal-organic frameworks(MOFs)have a high specific surface area,adjustable pores and can be used to obtain functional porous materials with diverse and well-ordered structures through coordination and self-assembly,which has intrigued wide interest in a broad range of disciplines.In the arena of biomedical engineering,the functionalized modification of MOFs has produced drug carriers with excellent dispersion and functionalities such as target delivery and response release,with promising applications in bio-detection,disease therapy,tissue healing,and other areas.This review summa-rizes the present state of research on the functionalization of MOFs by physical binding or chemical cross-linking of small molecules.***.bioma romolecules.and hvdrogels and evaluates the role and approach of MOFs functionalization in boosting the reactivity of materials.On this basis,research on the application of functionalized MOFs composites in biomedical engineering fields such as drug delivery,tissue repair,disease treatment,bio-detection and imaging is surveyed,and the development trend and application prospects of functionalized MOFs as an important new class of biomedical materials in the biomedical field are anticipated,which may provide some inspiration and reference for further development of MOF for bio-medical applications.

关键词： metal-organic frameworks porous functionalization tissue repair disease treatment

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Effect of mutations on acetohydroxyacid synthase(AHAS)function in Cyperus difformis L.

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Journal of Integrative Agriculture 2024年第1期23卷 177-186页

作者： Xiaotong Guo Xiangju li Zheng li licun Peng Jingchao Chen Haiyan Yu Hailan cui State Key Laboratory for Biology of Plant Diseases and Insect Pests Institute of Plant ProtectionChinese Academy of Agricultural SciencesBeijing 100193China College of Resources and Environmental Sciences China Agricultural UniversityBeijing 100193China

Cyperus difformis L.is a troublesome weed in paddy fields and has attracted attention due to its resistance to acetohydroxyacid synthase(AHAS)inhibitors.It was found that the amino acid mutation in AHAS was the primary cause for the resistance of Cyperus difformis.However,the effect of different mutations on AHAS function is not clear in Cyperus difformis.To confirm the effect of mutations on AHAS function,six biotypes were collected,including Pro197Arg,Pro197Ser,Pro197Leu,Asp376Glu,Trp574Leu and wild type,from Hunan,Anhui,Jiangxi and Jiangsu provinces,China and the function of AHAS was characterized.The AHAS in vitro inhibition assay results indicated that the mutations decreased the sensitivity of AHAS to pyrazosulfuron-ethyl,in which the I_(50)(the half maximal inhibitory concentration)of wild type AHAS was 0.04μmol L^(-1)and Asp376Glu,Pro197Leu,Pro197Arg,Pro197Ser and Trp574Leu mutations were 3.98,11.50,40.38,38.19 and 311.43μmol L^(-1),respectively.In the determination of enzyme kinetics parameters,the Km and the maximum reaction velocity(Vmax)of the wild type were 5.18 mmol L^(-1)and 0.12 nmol mg^(-1)min^(-1),respectively,and the Km values of AHAS with Asp376Glu,Trp574Leu,Pro197Leu and Pro197Ser mutations were 0.38-0.93 times of the wild type.The Km value of the Pro197Arg mutation was 1.14times of the wild type,and the Vmax values of the five mutations were 1.17-3.33-fold compared to the wild type.It was found that the mutations increased the affinity of AHAS to the substrate,except for the Pro197Arg mutation.At a concentration of 0.0032-100 mmol L^(-1)branched-chain amino acids(BCAAs),the sensitivity of the other four mutant AHAS biotypes to feedback inhibition decreased,except for the Pro197Arg mutation.This study elucidated the effect of different mutations on AHAS function in Cyperus difformis and provided ideas for further study of resistance development.

关键词： acetohydroxyacid synthase(AHAS) mutation enzyme function Cyperus difformis

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Challenges in li-ion battery high-voltage technology and recent advances in high-voltage electrolytes

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Journal of Energy Chemistry 2024年第4期91卷 73-98页

作者： Jianguo liu Baohui li Jinghang Cao Xiao Xing Gan cui Shandong Key Laboratory of Oil&Gas Storage and Transportation Safety College of Pipeline and Civil EngineeringChina University of Petroleum(East China)Qingdao 266580ShandongChina

The electrolyte directly contacts the essential parts of a lithium-ion battery,and as a result,the electrochemical properties of the electrolyte have a significant impact on the voltage platform,charge discharge capacity,energy density,service life,and rate discharge performance.By raising the voltage at the charge/discharge plateau,the energy density of the battery is increased.However,this causes transition metal dissolution,irreversible phase changes of the cathode active material,and parasitic electrolyte oxidation reactions.This article presents an overview of these concerns to provide a clear explanation of the issues involved in the development of electrolytes for high-voltage lithium-ion batteries.Additionally,solidstate electrolytes enable various applications and will likely have an impact on the development of batteries with high energy densities.It is necessary to improve the high-voltage performance of electrolytes by creating solvents with high thermal stabilities and high voltage resistance and additives with superior film forming performance,multifunctional capabilities,and stable lithium salts.To offer suggestions for the future development of high-energy lithium-ion batteries,we conclude by offering our own opinions and insights on the current development of lithium-ion batteries.

关键词： lithium-ion battery High voltage Electrolyte additive Solid electrolyte

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Effectivenss of electroacupuncture for skeletal muscle pain in Parkinson's disease:a Clinical randomized controlled trial

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Journal of Traditional Chinese Medicine 2024年第2期44卷 388-395页

作者： WANG Shaosong SUN Jingqing FENG Qingyin li Bin WANG Xin YUAN Fan cui Yingxue Department of acupuncture Beijing Hospital of Traditional Chinese MedicineBeijing 100010China

OBJECTIVE:To explore the effect of electroacupuncture on skeletal muscle pain in Parkinson's disease(PD).METHODS:A single-center randomized controlled trial was conducted with sixty patients with Parkinson's disease with skeletal muscle pain were randomly divided into electroacupuncture group and sham acupuncture control group with 30 patients each.The electric acupuncture group was treated with electric acupuncture,while the control group was treated with Park needle pseudoacupuncture.Both groups were treated 5 times a week for a total of 4 weeks,and both groups completed 20 treatments.King's Parkinson's Pain Scale(KPPS)and visual analog scale(VAS)were used before and after treatment to evaluate the pain degree of patients.Realtime shear wave elastography(SWE)and modified Ashworth score(MAS)were used to evaluate the changes of muscle tone.Parkinson's comprehensive Score Scale(MDS-UPDRS,including UPDRS Ⅱ and UPDRS Ⅲ)was used to evaluate exercise ability.Hamilton Depression Scale(HAMD)score was used to evaluate the emotional changes of patients.Spearman correlation analysis was used to explore the correlation between pain degree and muscle tone,exercise ability and emotion.RESULTS:During the study,one case fell off in the control group,and 30 cases were eventually included in the analysis and treatment group and 29 cases in the control group.After treatment,Young's modulus of biceps and quadriceps and shear wave velocity of biceps were decreased in electroacupuncture group compared with before treatment,while KPPS score,VAS score,UPDRS Ⅱ,UPDRS Ⅲ and modified Ashworth score were decreased,with statistical significance(P0.05).After treatment,KPPS score,VAS score,UPDRS Ⅱ and UPDRS Ⅲ,MAS,HAMD score,Young's modulus of biceps and shear wave velocity in electroacupuncture group were significantly lower than those in control group(P<0.05).Spearman correlation analysis showed that KPPS score was positively correlated with UPDRS Ⅲ(r=0.414,P<0.05).KPPS score was

关键词： Parkinson disease muscle skeletal pain electroacupuncture muscle tention mood disorders randomized controlled trial

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SAM Era:Can It Segment Any Industrial Surface Defects?

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Computers, Materials & Continua 2024年第3期78卷 3953-3969页

作者： Kechen Song Wenqi cui Han Yu Xingjie li Yunhui Yan National Key Laboratory of Advanced Casting Technologies Shenyang110022China School of Mechanical Engineering&Automation Northeastern UniversityShenyang110819China

Segment Anything Model(SAM)is a cutting-edge model that has shown impressive performance in general object segmentation.The birth of the segment anything is a groundbreaking step towards creating a universal intelligent model.Due to its superior performance in general object segmentation,it quickly gained attention and interest.This makes SAM particularly attractive in industrial surface defect segmentation,especially for complex industrial scenes with limited training data.However,its segmentation ability for specific industrial scenes remains unknown.Therefore,in this work,we select three representative and complex industrial surface defect detection scenarios,namely strip steel surface defects,tile surface defects,and rail surface defects,to evaluate the segmentation performance of SAM.Our results show that although SAM has great potential in general object segmentation,it cannot achieve satisfactory performance in complex industrial scenes.Our test results are available at:https://***/VDT-2048/SAM-IS.

关键词： Segment anything SAM surface defect detection salient object detection

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Distributed Stochastic Scheduling of Massive Backup Batteries in Cellular Networks for Operational Reserve and Frequency Support Ancillary Services

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Journal of Modern Power Systems and Clean Energy 2024年第2期12卷 393-404页

作者： Kun li Jiakun Fang Xiaomeng Ai Shichang cui Rongkang Zhao Jinyu Wen State Key Laboratory of Advanced Electromagnetic Engineering and Technology School of Electrical and Electronic EngineeringHuazhong University of Science and TechnologyWuhan 430074China IEEE

Base station(BS)backup batteries(BSBBs),with their dispatchable capacity,are potential demand-side resources for future power systems.To enhance the power supply reliability and post-contingency frequency security of power systems,we propose a two-stage stochastic unit commitment(UC)model incorporating operational reserve and post-contingency frequency support provisions from massive BSBBs in cellular networks,in which the minimum backup energy demand is considered to ensure BS power supply reliability.The energy,operational reserve,and frequency support ancillary services are co-optimized to handle the power balance and post-contingency frequency security in both forecasted and stochastic variable renewable energy(VRE)scenarios.Furthermore,we propose a dedicated and scalable distributed optimization framework to enable autonomous optimizations for both dispatching center(DC)and BSBBs.The BS model parameters are stored and processed locally,while only the values of BS decision variables are required to upload to DC under the proposed distributed optimization framework,which safeguards BS privacy effectively.Case studies on a modified IEEE 14-bus system demonstrate the effectiveness of the proposed method in promoting VRE accommodation,ensuring post-contingency frequency security,enhancing operational economics,and fully utilizing BSBBs'energy and power capacity.Besides,the proposed distributed optimization framework has been validated to converge to a feasible solution with near-optimal performance within limited iterations.Additionally,numerical results on the Guangdong 500 kV provincial power system in China verify the scalability and practicality of the proposed distributed optimization framework.

关键词： Base station backup battery operational reserve frequency security distributed optimization privacy

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Eutectogel-based self-powered wearable sensor for health monitoring in harsh environments

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Nano Research 2024年第6期17卷 5559-5568页

作者： Junpeng Wu Xinru Teng Lu liu Hongzhi cui Xiaoyi li College of Materials Science and Engineering Ocean University of ChinaQingdao 266100China Key Lab for Special Functional Materials of Ministry of Education School of Materials Science and EngineeringHenan UniversityKaifeng 475004China

Triboelectric nanogenerators(TENG)have emerged as a highly promising energy harvesting technology,attracting significant attention in recent years for their broad applications.Gel-based TENGs,with superior stretchability and sensitivity,have been widely reported as wearable sensors.However,the traditional hydrogel-based TENGs suffer from freezing at low temperatures and drying at high temperatures,resulting in malfunctions.In this study,we introduce an anti-freezing eutectogel,which uses a deep eutectic solvent(DES),to improve the stability and electrical conductivity of TENGs in harsh environmental conditions.The eutectogel-based TENG(E-TENG)produces an open-circuit voltage of 776 V,a short-circuit current of 1.54μA,and a maximum peak power of 1.1 mW.Moreover,the E-TENG exhibits exceptional mechanical properties with an elongation at a break of 476%under tension.Importantly,it maintains impressive performances across a wide temperature range from−18 to 60℃,with conductivities of 2.15 S/m at−10℃and 1.75 S/m at−18℃.Based on the excellent weight stability of the E-TENG sensor,motion sensing can be achieved in the air,and even underwater.Finally,the versatility of the E-TENG can serve as a wearable sensor,by integrating it with Bluetooth technology.The self-powered E-TENG can monitor various human motion signals in real-time and send the health signals directly to mobile phones.This research paves a new road for the applications of TENGs in harsh environments,offering wireless flexible sensors with real-time health signal monitoring capabilities.

关键词： triboelectric nanogenerators self-powered harsh environment health monitoring Eutectogel

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