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Genotypes and Phenotypes of Uygur Children with x.roderma Pigmentosum: A Case Study in x.nj.ang, China

International j.urnal of Dermatology and Venereology 2024年第3期7卷 144-149页

作者： Qiu Y. Wang W.-j. Liang j.-Q. Yakeya B. Wang P. Zhang j.-Z. kang x.-j. Department of Dermatology and Venereology People's Hospital of Xinjiang Uygur Autonomous Region Xinjiang Uygur Autonomous Region Urumqi 830001 China x.nj.ang Clinical Research Center for Dermatologic Diseases Xinjiang Uygur Autonomous Region Urumqi 830001 China x.nj.ang Key Laboratory of Dermatology Research (x.YS1707) Xinjiang Uygur Autonomous Region Urumqi 830001 China

Obj.ctive: x.roderma pigmentosum (x.) is a rare autosomal recessive dermatosis caused by genetic defects of DNA repair. This study was performed to detect and analyze the genes of 2 Uygur patients with x. and their families and assess the patients' phenotypes, which may enrich the understanding of the genetic skin disorder spectrum in x.nj.ang area. Methods: We collected the clinical data from 2 patients with x. and peripheral blood samples from the patients and their family members. The patients' DNA was sequenced and detected by Sanger sequencing, and gene mutations were screened. Results: The proband in family 1 presented with brown maculae at the ex.osure site and squamous cell carcinoma secondary to a facial rash. The proband had a homozygous nucleotide variation of x.C c.2251-2A> G (A change from A to G in the penultimate position of the intron before the 2251 position in the coding region), which was a shear mutation. In this family, both parents were heterozygous, and no similar mutation detected in the sister. In family 2, the proband had scattered black brown spots and papules on the trunk and limbs. and his younger sister was also a patient. The proband and his younger sister had homonucleotide variation of x.A c.631C> T, which was nonsense mutation, resulting in the codon for Arg No.211 being changed into termination codon (***211x., thus terminating the peptide chain synthesis prematurely. All the normal individuals in the two families were heterozygotes, and homozygous mutations occurred in all the patients, which was consistent with the autosomal recessive inheritance. Conclusion: x. is rare in Uygur population. This study ex.anded the mutation spectrum of x. and provided a basis for early diagnosis, treatment, prognostic prediction, and prenatal genetic consultation. © 2022 Hospital for Skin Diseases (Institute of Dermatology), Chinese Academy of Medical Sciences, and Chinese Medical Association, published by Wolters Kluwer, Inc.

关键词： gene mutation x.roderma pigmentosum x.A gene x.C gene

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Chinese Ex.ert Consensus on the Diagnosis and Treatment of Adult Dermatomyositis (2022)#

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International j.urnal of Dermatology and Venereology 2024年第3期7卷 163-173页

作者： Cao H. Chen A.-j. Cui Y. Deng D.-Q. Gao x.-H. He Y.-L. kang x.-j. j.n H.-Z. Li C.-x. Li F. Li H.-j. Liao W.-j. Liu x.-M. Lu Q.-j. Lu Y. Pan M. Pan W.-H. Shu x.-M. Sun Q. Tang K.-Y. Tao j. Wang Y. x.ao T. Zhang F.-R. Zhang H.-L. Department of Dermatology Ruijin Hospital Affiliated Shanghai Jiao Tong University School of Medicine Shanghai 200025 China Department of Dermatology First Affiliated Hospital of Chongqing Medical University Chongqing 400042 China Department of Dermatology China-Japan Friendship Hospital Beijing 100029 China Department of Dermatology The Second Affiliated Hospital of Kunming Medical University Yunnan Kunming 650101 China Department of Dermatology The First Hospital of China Medical University Liaoning Shenyang 110001 China Department of Dermatology Beijing Chaoyang Hospital Affiliated to Capital Medical University Beijing 100020 China Department of Dermatology and Venereology People's Hospital of Xinjiang Uygur Autonomous Region Xinjiang Uygur Autonomous Region Urumqi 830001 China Department of Dermatology State Key Laboratory of Complex Severe and Rare Diseases Peking Union Medical College Hospital Chinese Academy of Medical Science Peking Union Medical College Beijing 100730 China Department of Dermatology First Medical Center PLA General Hospital Beijing 100853 China Department of Dermatology Hainan Hospital of PLA General Hospital Hainan Sanya 572013 China Department of Dermatology Xijing Hospital Air Force Medical University Shaanxi Xi'an 710032 China Department of Dermatology Huazhong University of Science and Technology Union Shenzhen Hospital Guangdong Shenzhen 518052 China Hospital for Skin Diseases Institute of Dermatology Chinese Academy of Medical Sciences Peking Union Medical College Jiangsu Nanjing 210042 China Department of Dermatology Jiangsu Province Hospital The First Affiliated Hospital Nanjing Medical University Jiangsu Nanjing 210029 China Department of Dermatology Shanghai Changzheng Hospital Second Affiliated Hospital of Naval Medical University Shanghai 200003 China Department of Rheumatology China-Japan Friendship Hospital Beijing 100029 China Department of Dermatology Qilu Hospital of Shandong Universit

Dermatomyositis, an idiopathic inflammatory myopathy, is characterized by distinctive skin manifestations, prox.mal muscle weakness, and multiple organ involvement and can be accompanied by malignancies. To provide a reference for dermatologists and clinicians in other relevant fields of clinical practice, ex.erts from the Dermatology Branch of the China International Ex.hange and Promotion Association for Medical and Health Care and the National Clinical Research Center for Dermatologic and Immunologic Diseases developed this consensus on the diagnosis and treatment of adult dermatomyositis using Chinese and international literature and ex.ert advice. © 2024 Wolters Kluwer Health. All rights reserved.

关键词： adult clinical protocols dermatomyositis diagnosis ex.ert consensus treatment

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Attitude control of multi-spacecraft systems on SO(3)with stochastic links failure

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Chinese j.urnal of Aeronautics 2024年第3期37卷 320-331页

作者： kang Zeyu SHEN Qiang WU Shufan Chris j.DAMAREN MU Zhongcheng School of Aeronautics and Astronautics Shanghai Jiao Tong UniversityShanghai 200240China Institute for Aerospace Studies University of TorontoTorontoOntario M3H 5T6Canada

In this paper,for Multi-Spacecraft System(MSS)with a directed communication topology link and a static virtual leader,a controller is proposed to realize attitude consensus and attitude stabilization with stochastic links failure and actuator ***,an MSS attitude error model suitable for a directed topology link and with a static virtual leader based on SO(3)is derived,which considers that the attitude error on SO(3)cannot be defined based on algebraic ***,we design a controller to realize the MSS on SO(3)with attitude consensus and attitude stabilization under stochastic links failure and actuator ***,the simulation results of a multi-spacecraft system with stochastic links failure and a static virtual leader spacecraft are demonstrated to illustrate the efficiency of the attitude controller.

关键词： Multi-spacecraft systems Attitude consensus Attitude stabilization Stochastic links failure Super-martingale convergence

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Core shift limitation in investment casting process of hollow turbine blade

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Chinese j.urnal of Aeronautics 2024年第8期37卷 513-526页

作者： kang CUI Lin j.NG Ruisong j.ANG Longnv YU x.ao GAO Automation and Software Engineering Shanxi UniversityTaiyuan 030006China Key Laboratory of High Performance Manufacturing for Aero Engine Ministry of Industry and Information TechnologySchool of Mechanical EngineeringNorthwestern Polytechnical UniversityXi’an 710072China School of Mechanical Engineering Sichuan UniversityChengdu 610065China

The deviation in wall thickness caused by core shift during the investment casting process significantly impacts the strength and service life of hollow turbine *** address this issue,a core shift limitation method is developed in this ***,a shift model is established based on computational fluid dynamics and motion simulation to predict the movement of the ceramic core in investment casting ***,utilizing this model,an optimization method for fix.uring layout inside the refractory ceramic shell is devised for the ceramic *** casting ex.eriment demonstrates that by utilizing the optimized fix.ure layout,not only can core shift during the investment casting pouring process be effectively controlled,but also the max.mum wall thickness error of the blade can be reduced by 42.02%.In addition,the core shift prediction is also validated,with a prediction error of less than 26.9%.

关键词： Hollow turbine blade Wall thickness Ceramic core Shift prediction Fix.uring layout optimization

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Biotic and abiotic stresses on honeybee health

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Integrative Zoology 2024年第3期19卷 442-457页

作者： Zheguang LIN Siyi SHEN kang WANG Ting j. Apicultural Research Institute College of Animal Science and TechnologyYangzhou UniversityYangzhouChina

Honeybees are the most critical pollinators providing key ecosystem services that underpin crop production and sustainable *** a backdrop of rapid global change,this eusocial insect encounters a succession of stressors during nesting,foraging,and *** mites,together with vectored viruses,have been recognized as central biotic threats to honeybee health,while the spread of invasive giant hornets and small hive beetles also increasingly threatens colonies *** of agrochemicals,including acaricides used for mite treatment,and other pollutants of the environment have been widely documented to affect bee health in various ***,ex.anding urbanization,climate change,and agricultural intensification often result in the destruction or fragmentation of flower-rich bee *** anthropogenic pressures ex.rted by beekeeping management practices affect the natural selection and evolution of honeybees,and colony translocations facilitate alien species invasion and disease *** this review,the multiple biotic and abiotic threats and their interactions that potentially undermine bee colony health are discussed,while taking into consideration the sensitivity,large foraging area,dense network among related nestmates,and social behaviors of honeybees.

关键词： abiotic stressor Apis biotic stressor colony losses interaction effect pollinator decline

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Reliability modelling and assessment of CMOS image sensor under radiation environment

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Chinese j.urnal of Aeronautics 2024年第9期37卷 297-311页

作者： TAO, Zhao CHEN, Wenbin LI, x.aoyang kang, Rui School of Reliability and Systems Engineering Beihang University Beijing 100191 China Science and Technology on Reliability and Environmental Engineering Laboratory Beijing 100191 China

The Complementary Metal-Ox.de Semiconductor (CMOS) image sensor is a critical component with the function of providing accurate positioning in many space application systems. Under long-time operation in space environments, there are radiation related degradation and various uncertainties affecting the positioning accuracy of CMOS image sensors, which further leads to a reliability reduction of CMOS image sensors. Obviously, the reliability of CMOS image sensors is related to their specified function, degradation, and uncertainties; however, current research has not fully described this relationship. In this paper, a comprehensive approach to reliability modelling of CMOS image sensors is proposed based on the reliability science principles. Firstly, the performance margin modelling of centroid positioning accuracy is conducted. Then, the degradation model of CMOS image sensors is derived considering the dark current increase induced by the total ionizing dose effects. Finally, various uncertainties are analyzed and quantified, and the measurement equation of reliability is proposed. A case study of a CMOS image sensor is conducted to apply the proposed method, and the sensitivity analysis can provide suggestions for design and use of CMOS image sensors to ensure reliability. A simulation study is conducted to present the advantages of the proposed comprehensive approach. © 2024

关键词： CMOS integrated circuits

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STCF conceptual design report (Volume 1): Physics & detector

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Frontiers of physics 2024年第1期19卷 1-154页

作者： M.Achasov x.C.Ai L.P.An R.Aliberti Q.An x.Z.Bai Y.Bai O.Bakina A.Barnyakov V.Blinov V.Bobrovnikov D.Bodrov A.Bogomyagkov A.Bondar I.Boyko Z.H.Bu F.M.Cai H.Cai j.j.Cao Q.H.Cao x.Cao Z.Cao Q.Chang K.T.Chao D.Y.Chen H.Chen H.x.Chen j.F.Chen K.Chen L.L.Chen P.Chen S.L.Chen S.M.Chen S.Chen S.P.Chen W.Chen x.Chen x.F.Chen x.R.Chen Y.Chen Y.Q.Chen H.Y.Cheng j.Cheng S.Cheng T.G.Cheng j.P.Dai L.Y.Dai x.C.Dai D.Dedovich A.Denig I.Denisenko j.M.Dias D.Z.Ding L.Y.Dong W.H.Dong V.Druzhinin D.S.Du Y.j.Du Z.G.Du L.M.Duan D.Epifanov Y.L.Fan S.S.Fang Z.j.Fang G.Fedotovich C.Q.Feng x.Feng Y.T.Feng j.L.Fu j.Gao Y.N.Gao P.S.Ge C.Q.Geng L.S.Geng A.Gilman L.Gong T.Gong B.Gou W.Gradl j.L.Gu A.Guevara L.C.Gui A.Q.Guo F.K.Guo j.C.Guo j.Guo Y.P.Guo Z.H.Guo A.Guskov K.L.Han L.Han M.Han x.Q.Hao j.B.He S.Q.He x.G.He Y.L.He Z.B.He Z.x.Heng B.L.Hou T.j.Hou Y.R.Hou C.Y.Hu H.M.Hu K.Hu R.j.Hu W.H.Hu x.H.Hu Y.C.Hu j.Hua G.S.Huang j.S.Huang M.Huang Q.Y.Huang W.Q.Huang x.T.Huang x.j.Huang Y.B.Huang Y.S.Huang N.Hüsken V.Ivanov Q.P.j. j.j.j.a S.j.a Z.K.j.a H.B.j.ang j.j.ang S.Z.j.ang j.B.j.ao Z.j.ao H.j.j.ng x.L.kang x.S.kang B.C.Ke M.Kenzie A.Khoukaz I.Koop E.Kravchenko A.Kuzmin Y.Lei E.Levichev C.H.Li C.Li D.Y.Li F.Li G.Li G.Li H.B.Li H.Li H.N.Li H.j.Li H.L.Li j.M.Li j.Li L.Li L.Li L.Y.Li N.Li P.R.Li R.H.Li S.Li T.Li W.j.Li x.Li x.H.Li x.Q.Li x.H.Li Y.Li Y.Y.Li Z.j.Li H.Liang j.H.Liang Y.T.Liang G.R.Liao L.Z.Liao Y.Liao C.x.Lin D.x.Lin x.S.Lin B.j.Liu C.W.Liu D.Liu F.Liu G.M.Liu H.B.Liu j.Liu j.j.Liu j.B.Liu K.Liu K.Y.Liu K.Liu L.Liu Q.Liu S.B.Liu T.Liu x.Liu Y.W.Liu Y.Liu Y.L.Liu Z.Q.Liu Z.Y.Liu Z.W.Liu I.Logashenko Y.Long C.G.Lu j.x.Lu N.Lu Q.F.Lü Y.Lu Y.Lu Z.Lu P.Lukin F.j.Luo T.Luo x.F.Luo Y.H.Luo H.j.Lyu x.R.Lyu j.P.Ma P.Ma Y.Ma Y.M.Ma F.Maas S.Malde D.Matvienko Z.x.Meng R.Mitchell A.Nefediev Y.Nefedov S.L.Olsen Q.Ouyang P.Pakhlov G.Pakhlova x.Pan Y.Pan E.Passemar Y.P.Pei H.P.Peng L.Peng x.Y.Peng x.j.Peng K.Peters S.Pivovarov E.Pyata B.B.Qi Y.Q.Qi W.B.Qian Y.Qian C.F.Qiao j.j.Qin j.j.Qin L.Q.Qin x.S.Qin T.L.Qiu j.Rademacker C.F.Redmer H.Y.Sang Anhui University Hefei 230039China Beihang University Beijing 100191China Budker Institute of Nuclear Physics Novosibirsk 630090Russia CAS Key Laboratory of Theoretical Physics Institute of Theoretical PhysicsChinese Academy of SciencesBeijing 100190China Cavendish Laboratory University of CambridgeJJ Thomson AveCambridge CB30HEUnited Kingdom Central China Normal University Wuhan 430079China Central South University Changsha 410083China China University of Geosciences Wuhan 430074China China University of Mining and Technology Xuzhou 221116China École Polytechnique Fédérale de Lausanne(EPFL) LausanneSwitzerland Fudan University Shanghai 200433China Goethe University Frankfurt D-60325 Frankfurt am MainGermany Guangx. Normal University Guilin 541004China Guangx. Uninversity Nanning 530004China Hangzhou Institute for Advanced Study UCASHangzhou 310024China Hebei Normal University Shijiazhuang 050024China Hebei University Baoding 071002China Hefei University of Technology Hefei 230601China Helmholtz Institute Mainz Staudinger Weg 18D-55099 MainzGermany Henan Normal University Xinxiang 453007China Henan University Kaifeng 475004China High Energy Physics Center Chung-Ang UniversitySeoul 06974Korea Higher School of Economy 11 Pokrovsky Bulvar Moscow 109028Russia Huangshan University Huangshan 245000China Hubei University of Automotive Technology Shiyan 442002China Hunan Normal University Changsha 410081China Hunan University of Science and Technology Xiangtan 411201China Hunan University Changsha 410082China Indiana University BloomingtonIndiana 47405USA Inner Mongolia University Hohhot 010021China Institute of Advanced Science Facilities Shenzhen 518107China Institute of High Energy Physics Chinese Academy of SciencesBeijing 100049China Institute of Modern Physics Chinese Academy of SciencesLanzhou 730000China Institute of Physics Academia SinicaTaipeiTaiwan 11529China Institute of Theoretical Physics Chinese Academy of SciencesBeijing 100190China j.lin University Changch

The superτ-charm facility(STCF)is an electron–positron collider proposed by the Chinese particle physics *** is designed to operate in a center-of-mass energy range from 2 to 7 GeV with a peak luminosity of 0.5×10^(35) cm^(–2)·s^(–1) or *** STCF will produce a data sample about a factor of 100 larger than that of the presentτ-charm factory—the BEPCII,providing a unique platform for ex.loring the asymmetry of matter-antimatter(charge-parity violation),in-depth studies of the internal structure of hadrons and the nature of non-perturbative strong interactions,as well as searching for ex.tic hadrons and physics beyond the Standard *** STCF proj.ct in China is under development with an ex.ensive R&D *** document presents the physics opportunities at the STCF,describes conceptual designs of the STCF detector system,and discusses future plans for detector R&D and physics case studies.

关键词： electron–positron collider tau-charm region high luminosity STCF detector conceptual design

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Effects of low molecular weight polysaccharide from Sargassum thunbergii against palmitic acid-induced intracellular lipid accumulation in 3T3-L1 adipocyte and HepG2 cells

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Food Science and Human Wellness 2024年第4期13卷 2244-2252页

作者： Hyo-Geun Lee D.P.Nagahawatta M.j.M.S.Kurera Kyung-Mo Song Yun-Sang Choi You-j.n j.on Min-Cheol kang Department of Marine Life Sciences Jeju National UniversityJeju 63243Republic of Korea Department of Biotechnology Faculty of Agriculture and Plantation ManagementWayamba University of Sri LankaMakanduraGonawila(NWP)60170Sri Lanka Research Group of Food Processing Korea Food Research Institute245Wanju 55365Republic of Korea

Low molecular weight polysaccharides can be isolated from Sargassum thunbergii(LMPST)and in vitro ex.eriments were conducted to evaluate the inhibitory effects on *** natures of LMPST were attained from *** and appraised their LMPST on palmitic acid(PA)induced lipid accretion in Hep G2,and 3T3-L1 *** treatment lessened lipid deposition and intracellular free fatty acid and triglyceride intensities in PA-treated above mentioned *** mechanistic study publicized that LMPST2 significantly suppressed adipogenesis and stimulated the PA-treated 3T3-L1 cells occupied in the lipolysis ***,in PA-treated Hep G2 cells,the free fatty acid ox.dation was significantly increased by *** these constructive properties of LMPST2 from ***,is a potential candidate for diminishing the intracellular lipids,and for a therapeutic agent in those conditions.

关键词： Sargassum thunbergii Low molecular weight polysaccharide Lipid accumulation Obesity Liver steatosis

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Non-contact evaluation of the resonant frequency of a microstructure using ultrasonic wave

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Acta Mechanica Sinica 2010年第2期26卷 317-323页

作者： x. kang x. Y. He C.j. Tay C. Quan Department of Applied Mechanics Nanjing University of Science and Technology 210094 Nanjing China Institute of Engineering Mechanics Southeast University210096 Nanjing China Department of Mechanical Engineering National Universityof Singapore 9 Engineering Drive 1 117576 Singapore Singapore

This paper presents a novel non-contact method for evaluating the resonant frequency of a microstructure, Firstly, the microstructure under test is ex.ited by ultrasonic waves. This ex.itation method does not impose any undefined load on the specimen like the electrostatic ex.itation and also this is the first actual use of ultrasonic wave for ex.iting a microstructure in the literature. Secondly, the amplitudes of the microstructure are determined by image edge detection using a Mex.can hat wavelet transform on the vibrating images of the microstructure. The vibrating images are captured by a CCD camera when the microstructure is vibrated by ultrasonic waves at a series of discrete high frequencies （〉30 kHz）. Upon processing the vibrating images, the amplitudes at various ex.itation frequencies are obtained and an amplitude-frequency spectrum is obtained from which the resonant frequency is subsequently evaluated. A micro silicon structure consisting of a perforated plate （192 × 192 μm） and two cantilever beams （76 × 43 μm） which is about 4 μm thickness is tested. Since laser interferometry is not required, thermal effects on a test obj.ct can be avoided. Hence, the setup is relatively simple. Results show that the proposed method is a simple and effective approach for evaluating the dynamic characteristics of microstructures.

关键词： Non-contact Microstructure· Resonant frequency Ultrasonic wave Image processing

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Liquid crystal-integrated metasurfaces for an active photonic platform

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Opto-Electronic Advances 2024年第6期7卷 5-29页

作者： Dohyun kang Hyeonsu Heo Younghwan Yang j.nhwa Seong Hongyoon Kim j.ohoon Kim j.nsuk Rho Department of Mechanical Engineering Pohang University of Science and Technology(POSTECH)Pohang 37673Republic of Korea Department of Chemical Engineering Pohang University of Science and Technology(POSTECH)Pohang 37673Republic of Korea Department of Electrical Engineering Pohang University of Science and Technology(POSTECH)Pohang 36763Republic of Korea POSCOPOSTECH-RIST Convergence Research Center for Flat Optics and Metaphotonics Pohang 37673Republic of Korea National Institute of Nanomaterials Technology(NINT) Pohang 37673Republic of Korea

Metasurfaces have opened the door to nex.-generation optical devices due to their ability to dramatically modulate electromagnetic waves at will using periodically arranged ***,metasurfaces typically have static optical responses with fix.d geometries of nanostructures,which poses challenges for implementing transition to technology by replacing conventional optical *** solve this problem,liquid crystals(LCs)have been actively employed for designing tunable metasurfaces using their adj.stable birefringent in real ***,we review recent studies on LCpowered tunable metasurfaces,which are categorized as wavefront tuning and spectral *** to numerous reviews on tunable metasurfaces,this review intensively ex.lores recent development of LC-integrated *** the end of this review,we briefly introduce the latest research trends on LC-powered metasurfaces and suggest further directions for improving *** hope that this review will accelerate the development of new and innovative LC-powered devices.

关键词： tunable metasurface liquid crystal active metasurface electrically tunable optical system

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