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Clinical Analysis and Mental Health Survey of Hemophilia Carriers:a Cross-sectional Study

Current Medical Science 2024年第2期44卷 435-440页

作者： Wen WANG Li-juan JIANG Dong-yan CUI ai ZHANG Xiong WANG ai-guo liu Qun HU Department of Pediatrics Tongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430030China Department of Laboratory Medicine Tongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430030China

Objective:Hemophilia carriers(HCs),who are heterozygous for mutations in the clotting factor VIII/clotting factor IX gene(F8 or F9),may have a wide range of clotting factor levels,from very low,similar to afflicted males,to the upper limit of normal,and may experience mental health *** purpose of this study was to provide genetic information on mothers of hemophilia patients and to understand the clotting factor activity and phenotype of ***,we aimed to investigate the mental health status of HCs in ***:A total of 127 hemophilia mothers,including 93 hemophilia A(HA)mothers and 34 hemophilia B(HB)mothers,were enrolled in this *** distance PCR,multiplex PCR,and Sanger sequencing were used to analyze mutations in F8 or *** factor activity was detected by a one-stage clotting *** Symptom Checklist 90(SCL-90,China/Mandarin version)was given to HCs at the same time to assess their mental ***:A total of 90.6%of hemophilia mothers were diagnosed genetically as carriers,with inversion in intron 22 and missense mutations being the most common mutation types in HA and HB carriers,*** median clotting factor level in carriers was 0.74 IU/mL(ranging from 0.09 to 1.74 IU/mL)compared with 1.49 IU/mL(ranging from 0.93 to 1.89 IU/mL)in noncarriers,of which 14.3%of HCs had clotting factor levels of 0.40 IU/mL or below.A total of 53.8%(7/13)of HA carriers with low clotting factor levels(less than 0.50 IU/mL)had a history of bleeding,while none of the HB carriers displayed a bleeding *** total mean score and the global severity index of the SCL-90 for surveyed HCs were 171.00(±60.37)and 1.78(±0.59),respectively.A total of 67.7%of the respondents had psychological symptoms,with obsessive-compulsive disorder being the most prevalent and *** pooled estimates of all nine factors were significantly higher than those in the general population(P<0.05).Conclusions:The detection rate of gene mutations

关键词： hemophilia A hemophilia B mutation carrier mental health

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Genetic Analysis of Two Novel GPI Variants Disrupting H Bonds and Localization Characteristics of 55 Gene Variants Associated with Glucose-6-phosphate Isomerase Deficiency

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Current Medical Science 2024年第2期44卷 426-434页

作者： Bi-xin XI Si-ying liu Yu-ting XU De-dong ZHANG Qun HU ai-guo liu Department of Pediatrics Tongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430030China

Objective:Glucose-6-phosphate isomerase(GPI)deficiency is a rare hereditary nonspherocytic hemolytic anemia caused by GPI gene *** disorder exhibits wide heterogeneity in its clinical manifestations and molecular characteristics,often posing challenges for precise diagnoses using conventional *** this end,this study aimed to identify the novel variants responsible for GPI deficiency in a Chinese ***:The clinical manifestations of the patient were summarized and analyzed for GPI deficiency phenotype *** compound heterozygous variants of the GPI gene,c.174C>A(***58Lys)and c.1538G>T(***513Leu),were identified using whole-exome and Sanger *** AlphaFold program and Chimera software were used to analyze the effects of compound heterozygous variants on GPI ***:By characterizing 53 GPI missense/nonsense variants from previous literature and two novel missense variants identified in this study,we found that most variants were located in exons 3,4,12,and 18,with a few localized in exons 8,9,and *** study identified novel compound heterozygous variants associated with GPI *** pathogenic variants disrupt hydrogen bonds formed by highly conserved GPI amino ***:Early family-based sequencing analyses,especially for patients with congenital anemia,can help increase diagnostic accuracy for GPI deficiency,improve child healthcare,and enable genetic counseling.

关键词： glucose-6-phosphate isomerase deficiency whole-exome sequencing compound heterozygous variants genetic characterization hydrogen bond

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Research and Practice of Production Technology for High Performance Low Alloy Steels in WISCO

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Journal of Iron and Steel Research International 2011年第S1期18卷 99-103页

作者： FU Lian-chun,guo ai-ming,liu Ji-bin,guo Bin,CHEN Yan-tang [Wuhan Iron and Steel (Group) Corporation,Wuhan 430080,Hubei,China] Wuhan Iron & Steel Grp Corp Wuhan 430080 Hubei Peoples R China

The product system of high strength low alloy steels (HSLA) produced by Wuhan Iron and Steel (Group) Corporation (WISCO) was presented.A series of high performance structural steels have been developed for important construction projects around the world in different areas,such as bridge,pressure vessel,pipe line,shipbuilding,architecture,machinery and railway *** order to promote the development of large heat input welding steel,high grade thick pipeline steel and long span bridge steel,a lot of advanced production technologies have been achieved through intensive investigation,such as the control of high purity,high homogenization and free defects in steel slabs,the rules of high melting point particle formation and distribution in micro and nano scale during steel production,and the TMCP technology for ultra low carbon bainite steel for medium and heavy *** will carry on research and application of economic and weather-resistant steels and promote the developing and producing technologies of HSLA steel.

关键词： WISCO high performance low alloy steels production technology

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The Role of Endolymphatic Hydrops in Patients with Pantonal Idiopathic Sudden Sensorineural Hearing Loss:A Cause or Secondary Reaction

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Current Medical Science 2019年第6期39卷 972-977页

作者： Ye-xian ZHENG ai-guo liu Xing-long WANG Ying HU Yan-fei ZHANG Li-yan PENG Department of Otorhinolaryngology the Central Hospital of WuhanTongji Medical CollegeHuazhon University of Science and technologyWuhan 430014China Department of Otorhinolaryngology Tongji HospitalTongji Medical CollegeHucizhong University of Science and technologyWuhan 430030China

The purpose of this study was to investigate the presence of endolymphatic hydrops(EH)in both affected and unaffected ears of patients with pantonal unilateral idiopathic sudden sensorineural hearing loss(ISSNHL)using three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging(3D-FLaiR MRI)and further evaluate the significance of EH in this ***-seven ISSHL patients were enrolled in this study.3D-FLaiR MRI was performed 24 h after intratympanic injection of gadolinium-diethylenetriaminepentaacetic acid(Gd-DPTA).The incidences of EH in the affected ears and contralateral unaffected ears were compared and the correlations of EH with vertigo or prognosis were analyzed using the Chi-square *** results showed that the incidence of EH was 68.0%(17/25)in the affected ears and 34.8%(8/23)in the unaffected *** was a statistically significant difference between affected ears and unaffected ears in regard to the incidence of EH(Pairment of the inner ears with pantonal ISSNHL.

关键词： idiopathic sudden sensorineural hearing loss endolymphatic hydrops magnetic resonance imaging gadolinium-diethylenetriamincpentaacetic acid

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Screening of SARS-CoV-2 in 299 Hospitalized Children with Hematooncological Diseases:A Multicenter Survey in Hubei,China

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Current Medical Science 2020年第4期40卷 642-645页

作者： Song-mi WANG Fang TAO Yan HOU ai ZHANG Hao XIONG Jun-jie SUN Xiao-ping LUO Yan HAO Jian-xin LI Qun HU ai-guo liu Department of Peditrics Tongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430030China Department of Pediatric Hematology&Cncology Wuhan Children's HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430015China Department of Pediatrics Xiangyang Central HospitalAfiliated Hospital of Hubei University ofArt and ScienceXiangyang 441021China

Summary:The SARS-CoV-2 infection status of hospitalized children was surveyed in the department of pediatric hematology and oncology in three different hospitals of epidemic areas in Hubei,China.A cross-sectional study was performed to investigate the clinical characteristics,lung CT scan,SARS CoV-2 nucleic acid test and serum antibodics of hospitalized children with hemato-oncological diseases from January 23 to April 24,2020.299 children were enrolled in this study,including 176 males(58.9%)and 123 females(41.1%),aged from 2 months to 16 years.255 cases(85.3%)received chemotherapy or other immunosuppressive therapies,and there were 44 cases(14.7%)of other benign *** acid test was performed on 258 children(86.3%)and one case was positive.163 cases(54.5%)were tested for serum antibodies,and all of them were *** CT scan was performed on 247 children(82.6%),and 107 of them showed infectious *** one case(0.33%)of COVID-19 was diagnosed in the *** prevalence rate of COVID-19 in enrolled children with hemato-oncological diseases in Hubei was 0.33%.Immunosuppressed patients are not prone to produce related *** protective measures and ward management can reduce the risk of SARS-CoV-2 infection in the group patients.

关键词： children COVID-19 hemato-oncological diseases SARS-CoV-2

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Design of an automated robotic microinjection system for batch injection of zebrafish embryos and larvae

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Microsystems & Nanoengineering 2024年第1期10卷 89-103页

作者： Zhongyi guo Nana ai Wei Ge Qingsong Xu Department of Electromechanical Engineering Faculty of Science and TechnologyUniversity of MacaoMacaoChina Department of Biomedical Sciences and Centre of Reproduction Development and Aging(CRDA)Faculty of Health SciencesUniversity of MacaoMacaoChina

This paper presents the design of a vision-based automated robotic microinjection system for batch injection of both zebrafish embryos and larvae.A novel visual recognition algorithm based on an automatic threshold and excessive dilatation is introduced to accurately identify the center of zebrafish embryos and larval yolks.A corresponding software system is developed using the producer-consumer model as the framework structure,and a friendly user interface is designed to allow operators to choose from a range of desired functions according to their different *** addition,a novel microstructural agarose device is designed and fabricated to simultaneously immobilize mixed batches of embryos and ***,a prototype microinjection system is fabricated by integrating hardware devices with visual *** experimental study is conducted to verify the performance of the robotic microinjection *** results show that the reported system can accurately identify zebrafish embryos and larvae and efficiently complete batch microinjection tasks of the mixtures with an injection success rate of 92.05%in 13.88 s per *** with manual and existing microinjection systems,the proposed system demonstrates the merits of versatility,excellent efficiency,high success rate,high survival rate,and sufficient stability.

关键词： zebra automated hardware

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Control Strategies for Digital Twin Systems

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IEEE/CAA Journal of Automatica Sinica 2024年第1期11卷 170-180页

作者： guo-Ping liu IEEE the Center for Control Science and Technology Southern University of Science and TechnologyShenzhen 518055China

With the continuous breakthrough in information technology and its integration into practical applications, industrial digital twins are expected to accelerate their development in the near future. This paper studies various control strategies for digital twin systems from the viewpoint of practical *** make full use of advantages of digital twins for control systems, an architecture of digital twin control systems, adaptive model tracking scheme, performance prediction scheme, performance retention scheme, and fault tolerant control scheme are proposed. Those schemes are detailed to deal with different issues on model tracking, performance prediction, performance retention, and fault tolerant control of digital twin systems. Also, the stability of digital twin control systems is analysed. The proposed schemes for digital twin control systems are illustrated by examples.

关键词： Digital twin control systems fault tolerant control model tracking performance prediction performance retention

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Rapid tooth movement through distraction osteogenesis of the periodontal ligament in dogs

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Chinese Medical Journal 2008年第5期121卷 455-462页

作者： ai Hong XU Qing-feng LU Hong-fei Mai Zhi-hui AN ai-qun liu guo-ping Department of Stomatology Affiliated Hospital Guangzhou Sun Yat-sen University Third Guangdong 510630 China

Background Animal models are needed for the study of rapid tooth movement into the extraction socket through distraction osteogenesis of the periodontal ligament. Methods Modified distraction devices were placed on eight dogs between the first and third mandibular premolars on the left sides; similar placement of traditional straight wise appliances on the right sides served as the control. The experimental distractors were activated （0.25 mm/d） twice a day and the control devices were activated （100 g） for two weeks with consolidation periods at weeks two, three, six, and ten. Two dogs were sacrificed at each consolidation time point; rates and patterns of tooth movement, loss of anchorage, and periapical films were evaluated, and the affected premolars and surrounding periodontal tissues were decalcified and examined histologically. General observations, X-ray periapical filming and histology examination were performed. Results Distal movement （（3.66±0.14） mm） measured two weeks after modified distraction exceeded that achieved using the traditional device （（1.15±0.21） mm; P 〈0.05）. Loss of anchorage was minimally averaged （0.34±0.06） mm and （0.32±0.07） mm in the experimental and control sides, respectively. By radiography, apical and lateral surface root resorptions on both sides were minimal. Alveolar bone lesions were never evident. Fibroblasts were enriched in periodontal ligaments and bone spicules formed actively along directions of distraction. Conclusions The canine model is suitable for the study of rapid tooth movement through distraction osteogenesis of the periodontal ligament. The technique accelerates tooth movement, periodontal remodeling, alveolar bone absorption, and may induce fibroblast formation, as compared to the traditional orthodontic method, without adversely affecting root absorption, bone loss, tooth mobility and anchorage loss.

关键词： animal model distraction osteogenesis periodontal ligament rapid tooth movement bone formation

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The SLEGS beamline of SSRF

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Nuclear Science and Techniques 2024年第7期35卷 20-30页

作者： Long-Xiang liu Hong-Wei Wang Gong-Tao Fan Hang-Hua Xu Yue Zhang Zi-Rui Hao ai-guo Li Shanghai Advanced Research Institute Chinese Academy of SciencesShanghai201210China

The Shanghai Laser Electron Gamma Source(SLEGS, located in BL03SSID) beamline at the Shanghai Synchrotron Radiation Facility(SSRF) is a Laser Compton Scattering(LCS) gamma source used for the investigation of nuclear structure, which is in extensive demand in fields such as nuclear astrophysics, nuclear cluster structure, polarization physics, and nuclear energy. The beamline is based on the inverse Compton scattering of 10640 nm photons on 3.5 GeV electrons and a gamma source with variable energy by changing the scattering angle from 20° to 160°. γ rays of 0.25-21.1 MeV can be extracted by the scheme consisting of the interaction chamber, coarse collimator, fine collimator, and attenuator. The maximum photon flux for 180° is approximately 10~7 photons/s at the target at 21.7 MeV, with a 3-mm-diameter beam. The beamline was equipped with four types of spectrometers for experiments in( γ,γ'),( γ,n),( γ,p), and( γ,α). At present, Nuclear Resonance Fluorescence(NRF) spectrometry, Flat-Efficiency neutron Detector(FED) spectrometry, neutron Time-Of-Flight(TOF) spectrometry, and Light-Charged Particle(LCP) spectrometry methods have been developed.

关键词： Shanghai Synchrotron Radiation Facility SLEGS Nuclear astrophysics Nuclear structure

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A model reduction approach for discrete-time linear time-variant systems with delayed inputs

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Science China(Information Sciences) 2024年第4期67卷 197-217页

作者： ai-guo WU Guang-Ren DUAN Yu WANG Jie ZHANG School of Mechanical Engineering and Automation Harbin Institute of Technology(Shenzhen) HIT Wuhu Robot Technology Research Institute Center for Control Science and Technology Southern University of Science and Technology

A model reduction approach is presented for discrete-time linear time-variant input-delayed systems. According to this proposed approach, a dynamical variable is constructed by taking advantage of the current state and historical information of input. It is revealed that the behavior of this dynamical variable is governed by a discrete-time linear delay-free system. It is worth noting that the presented variable transformation does not require the system matrix to be invertible. Based on the reduced delay-free models,stabilizing control laws can be easily obtained for the original delayed system. For the case with a single input delay, the constructed variable is an exact prediction for the future state, and thus the stabilizing control law could be designed by replacing the future state with its prediction. Finally, three discrete-time periodic systems with delayed input are employed to illustrate how to utilize the presented model reduction approaches.

关键词： reduction approach predictor-based feedback time-varying systems delayed input periodic system

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