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Homozygous deletion, c. 1114-1116del, in exon 8 of the CRPPA gene causes congenital muscular dystrophy in Chinese family: A case report

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World Journal of Clinical Cases 2021年第19期9卷 5226-5231页

作者： mi yang Ru-Xin Xing Department of Neurology The Fourth Affiliated HospitalZhejiang University School of MedicineYiwu 322000Zhejiang ProvinceChina Department of Neurosurgery The Fourth Affiliated HospitalZhejiang University School of MedicineYiwu 322000Zhejiang ProvinceChina

BACKGROUND Congenital muscular dystrophy(CMD)is a clinically and genetically heterogeneous group of inherited muscle *** in the CRPPA gene(encoding CDPLribitol pyrophosphorylase A)are recognized as causative factors of dystroglycanopathies,a subtype of CMD with defects in *** SUMMARY The present study examined a Chinese family,whose proband presented mainly with muscle weakness in both lower limbs but without brain and eye *** this family,a homozygous deletion,c.1114-1116del(p.V372del),was identified in exon 8 of CRPPA in the proband,while a heterozygous deletion was identified in the proband’s father and mother,who lacked symptoms.A mild dystroglycanopathy of CMD was *** The findings of this study expanded the clinical and mutational spectrum of patients with CMD associated with CRPPA mutations.

关键词： Congenital muscular dystrophy CRPPA Mutation Dystroglycanopathy Case report

Numerical Modelling for Dynamic Instability Process of Submarine Soft Clay Slopes Under Seismic Loading

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Journal of Ocean University of China 2021年第5期20卷 1109-1120页

作者： mi yang WANG Jianhua CHENG Xinglei YAN Xiaowei State Key Laboratory of Hydraulic Engineering Simulation and Safety Tianjin UniversityTianjin 300072China Geotechnical Engineering Institute Tianjin UniversityTianjin 300072China

Marine geological disasters occurred frequently in the deep-water slope area of the northern South China Sea,especially submarine landslides,which caused serious damage to marine *** cyclic elastoplastic model that can describe the cyclic stress-strain response characteristic for soft clay,is embedded into the coupled Eulerian-Lagrangian(CEL)algorithm of ABAQUS by means of subroutine interface *** the basis of CEL technique and undrained cyclic elastoplastic model,a method for analyzing the dynamic instability process of marine slopes under the action of earthquake load is *** rationality for cyclic elastoplastic constitutive model is validated by comparing its calculated results with those of von mises model built in *** dynamic instability process of slopes under different conditions are *** results indicate that the deformation accumulation of soft clay have a significant effect on the dynamic instability process of submarine slopes under earthquake *** cumulative deformation is taken into our model and this makes the calculated final deformation of the slope under earthquake load larger than the results of conventional numerical *** different contact conditions are used for analysis,the smaller the friction coefficient is,the larger the deformation of slopes will be.A numerical analysis method that can both reflect the dynamic properties of soft clay and display the dynamic instability process of submarine landslide is proposed,which could visually predict the topographies of the previous and post failure for submarine slope.

关键词： submarine slope saturated soft clay coupled Eulerian-Lagrangian cyclic elastoplastic model dynamic instability process

Frequency control of a wind-diesel system based on hybrid energy storage

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Protection and Control of Modern Power Systems 2022年第1期7卷 446-458页

作者： yang mi Boyang Chen Pengcheng Cai Xingtang He Ronghui Liu Xingwu yang School of Electric Power Engineering ShangHai University of Electric PowerShanghai 200090China School of Electric Power Engineering Tianjin UniversityTianjin 300072China

To improve the stability of a wind-diesel hybrid microgrid,a frequency control strategy is designed by using the hybrid energy storage system and the adjustable diesel generator with load frequency control(LFC).The objective of frequency control is to quickly respond to the disturbed system to reduce system frequency deviation and restore *** evaluating the area control error,the disturbance state of the system can be divided into four different areas by a corresponding control strategy for precise *** the diesel generator,an adaptive sliding mode(SM)algorithm is used to design LFC that can participate in frequency *** frequency coordination con-trol strategy proposed in this paper can realize the partition adjustment according to different resources,and ensure frequency *** proposed control strategy is verified by RTDS simulations in multiple scenarios.

关键词： Wind-diesel microgrid Hybrid energy storage Load frequency control Sliding mode adaptive algorithm

Multi-Scenario millimeter Wave Wireless Channel Measurements and Sparsity Analysis

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China Communications 2022年第11期19卷 16-31页

作者： Hang mi Bo Ai Ruisi He Xin Zhou Zhangfeng Ma mi yang Zhangdui Zhong Ning Wang State Key Laboratory of Rail Traffic Control and Safety Beijing Jiaotong UniversityBeijing 100044China Henan Joint International Research Laboratory of Intelligent Networking and Data Analysis Zhengzhou UniversityZhengzhou 450001China PengCheng Laboratory Shenzhen 518000China National Institute of Metrology of China Beijing 100029China School of Information Engineering Zhengzhou UniversityZhengzhou 450001China

Wireless channel characteristics have significant impacts on channel modeling,estimation,and communication *** the channel sparsity is an important characteristic of wireless *** the sparse nature of wireless channels can reduce the complexity of channel modeling and estimation,and improve system design and performance *** with the traditional sub6 GHz channel,millimeter wave(mmWave)channel has been considered to be more sparse in existing ***,most research only assume that the mmWave channel is sparse,without providing quantitative analysis and ***,this paper evaluates the sparsity of mmWave channels based on mmWave channel measurements.A vector network analyzer(VNA)-based mmWave channel sounder is developed to measure the channel at 28 GHz,and multi-scenario channel measurements are *** Gini index,Rician𝐾factor and rootmean-square(RMS)delay spread are used to measure channel ***,the key factors affecting mmWave channel sparsity are *** is found that antenna steering direction and scattering environment will affect the sparsity of mmWave *** addition,the impact of channel sparsity on channel eigenvalue and capacity is evaluated and analyzed.

关键词： channel sparsity channel measurement mmWave channel measures of sparsity

Robust Load Frequency Control for Isolated microgrids Based on Double-loop Compensation

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CSEE Journal of Power and Energy Systems 2023年第4期9卷 1359-1369页

作者： yang mi Yuchen Ma Xingtang He Xingwu yang Jinxia Gong Yao Zhao Ronghui Liu Wei Wei Shanghai University of Electric Power Shanghai 200090China. State Grid Luoyang Power Supply Company Henan 410000China Tianjin University Tianjin 300072China

Frequency is an important indicator for the oper-ation of ***,the randomness and uncertainty of renewable energy and load variability may lead to frequency ***,a robust load frequency control(LFC)is pro-posed for isolated wind-diesel microgrids considering time delay and parameter *** control strategy can suppress frequency fuctuation and optimize frequency dynamic ***,the double compensation loop,including feedforward control and integral sliding mode control(SMC),is devised to provide anti-disturbance compensation for the diesel generator system and ameliorate the frequency stability of independent ***,a dynamic fuzzy controller,composed of wind speed and load demand,is designed to provide real-time response reference power for doubly fed induction generator systems(DFIGs),which can promote the effective participation of a wind turbine system for frequency ***,the proportional differential(PD)parameters of a dynamic fuzzy controller and the frequency adjustment compensation of DFIGs can be obtained by using a particle swarm optimization(PSO)***,load demand is an important index of the robust dynamic load frequency control method;the radial basis function(RBF)neural network observer(NNO)based on the LFC model is presented to obtain more accurate load deviations and improve the control precision of *** performance of the proposed LFC method is tested under different operation *** Terms-Load frequency control,microgrid,neural network observer,sliding mode,time delay and parameter uncertainty.

关键词： Load frequency control microgrid neural network observer sliding mode time delay and parameter uncertainty.

Multi-Frequency Channel Characterization for Massive miMO Communications in Lobby Environment

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China Communications 2019年第9期16卷 79-92页

作者： Jianzhi Li Bo Ai Ruisi He mi yang Zhangdui Zhong State Key Laboratory of Rail Traffic Control and Safety Beijing Jiaotong University

In this paper,a massive multiple input multiple output(miMO)channel measurement campaign with two setups is conducted in an indoor lobby *** the first setup,two types of 256-element virtual uniform rectangular arrays(URAs),i.e.,the 4×64 virtual URA and the 64×4 virtual URA are *** carrier frequency is 11 GHz;in the second setup,measurements are performed at 4,6,11,13,15,18 GHz at two different user *** channel characterization is presented by investigating the typical channel parameters,including average power delay profile(APDP),K factor,root mean square(RMS)delay spread,and coherence ***,the channel characteristics in angular domain are investigated by applying the space-alternating generalized expectation-maximization(SAGE)*** extracted multipath components(MPCs)are preliminarily clustered by visual inspection,and related to the interacting objects(IOs)in physical *** structures at multiple frequency bands are *** spread of departure is estimated to evaluate the directional dispersion at the base station(BS)*** results in this paper can help to reveal the propagation mechanisms in massive miMO channels,and provide a foundation for the design and application of the practical massive miMO system.

关键词： 5G Massive miMO channelmeasurement virtual array SAGE K factor RMS delay spread coherence bandwidth scatterer localization direction spread

Noncovalent cross-linked engineering hydrogel with low hysteresis and high sensitivity for flexible self-powered electronics

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Journal of Energy Chemistry 2024年第7期94卷 136-147页

作者： Hang Yuan Shaowei Han Jia Wei Songwei Li Peipei yang Hao-yang mi Chuntai Liu Changyu Shen National Engineering Research Center for Advanced Polymer Processing Technology Key Laboratory of Materials Processing and Mold(Ministry of Education)Zhengzhou UniversityZhengzhou450002HenanChina Yunnan TobaccoQuality Inspection&Supervision Station Kunming 650106YunnanChina

In this study,the hydrogel network was reinforced by covalent-like hydrogen bonding,and the strong binding ability of boron-nitrogen coordination served as the main driving *** them,acrylamide(AM)and 3-acrylamidophenylboronic acid(AAPBA)were the main body,and the numerous hydroxyl groups in the trehalose(Treh)molecule and other polymer groups formed strong hydrogen bonding interactions to improve the mechanical properties of the PAM/PAAPBA/Treh(PAAT)hydrogel and ensured the simplicity of the synthesis *** hydrogel possessed high strain at break(1239%),stress(64.7 kPa),low hysteresis(100%to 500%strain,corresponding to dissipation energy from 1.37 to 7.80 kJ/m^(3)),and outstanding cycling stability(retained more than 90%of maximum stress after 200 ten-sile cycles).By integrating carbon nanotubes(CNTs)into PAAT hydrogel(PAATC),the PAATC hydrogel with excellent strain response performance was successfully *** PAATC conductive hydro-gel exhibited high sensitivity(gauge factor(GF)=10.58 and sensitivity(S)=0.304 kPa^(-1)),wide strain response range(0.5%-1000%),fast response time(450 ms),and short recovery time(350 ms),excellent fatigue resistance,and strain response ***,the PAATC-based triboelectric nanogener-ator(TENG)displayed outstanding energy harvesting performance,which shows its potential for appli-cation in self-powered electronic devices.

关键词： Low hysteresis Covalent-like hydrogen bonding Boron-nitrogen coordination Hydrogel sensor Triboelectric nanogenerator

The variable structure controller design for a class of nonlinear uncertain systems based on robust observer

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控制理论与应用（英文版） 2011年第4期9卷 548-552页

作者： yang mi Juntao LI Wangqing ZHU Wenlin LI Electric Power and Automation Engineering Shanghai University of Electric Power Shanghai 200090 China Department of Information and Mathematics Henan Normal University Xinxiang Henan 453002 China

The variable structure controller is designed for a class of nonlinear uncertain time-delay system by using robust observer, and incorporating H-infinity control technique, the controller can guarantee the H-infinity performance of sliding mode dynamics and satisfy the reaching condition, which also does not require uncertainties to satisfy matching condition and linear boundary condition. The simulation example is given to illustrate the method.

关键词： Robust observer Variable structure control H-infinity control Time-delay system Linear matrix inequalities

Clinical analysis of 114 cases of bronchiolitis in infants

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World Journal of Clinical Cases 2023年第35期11卷 8284-8290页

作者： Cheng Shi Meng-Hua Wu An Zuo mi-mi yang Rong-Rong Jiang Department of Pediatrics Tongling Maternal and Child Health HospitalTongling 244000Anhui ProvinceChina

BACKGROUND Bronchiolitis is a common lower respiratory tract infection in infants and young *** cases may be accompanied by obvious dyspnea and oxygen saturation *** To summarize the clinical features,standard diagnosis,and treatment of *** This is a retrospective analysis of 114 pediatric patients(74 males,40 females)who were first diagnosed as having bronchioles at the Department of Pediatrics of Tongling Maternal and Child Health Hospital from January 2019 to December *** clinical features,imaging features,treatment,and other clinical data were recorded and *** The age of onset of the disease was mainly from 1 mo to 6 mo(75.4%),and the time to hospital visit was mostly from the 2nd day to the 4th day of the course of the disease(75.4%).Lung imaging examination showed increase in lung texture,fuzzy(93.8%).The main treatment was atomization therapy:Budesonide combined with terbutaline(45.6%)and budesonide combined with salbutamol(38.5%).The average hospitalization time was 7.1±2.4 d,and the overall cure rate was 94.7%.In patients without bacterial infection,the use of antibiotics significantly prolonged the length of hospital stay(7.8±2.5 d vs 5.7±1.8 d)and improved the cure rate(98.3%vs 87.9%,P<0.05).CONCLUSION Infants with bronchiolitis are mainly male and tend to have a good ***,the unneeded use of antibiotics may prolong the length of hospital stay significantly,which imposes the burden both on the patients and hospital *** Bronchiolitis is a common acute respiratory infectious disease in infants and young *** mainly affects male children and the age onset is between 1 to 6 *** standard use of antibiotics should be emphasized in view of the prolonged average length of hospital stay between the antibiotic treatment group and the non-antibiotic treatment *** the course of disease is more than 7 d or the treatment effect is poor,active anti-infective treatment is

关键词： Bronchiolitis Diagnosis Treatment Infants Antibiotics Retrospective analysis