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CCNO mutation as a cause of primary ciliary dyskinesia:A case report

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World Journal of Clinical Cases 2022年第25期10卷 9148-9155页

作者： yun-yan zhang yan Lou Han yan Hao Tang Department of Respiratory and Critical Care Medicine Changzheng HospitalNaval Military Medical UniversityShanghai 200003China Department of Orthopedic Oncology Spine Tumor CenterChangzheng HospitalNaval Military Medical UniversityShanghai 200003China Department of Nephrology 905th Hospital of PLA NavyNaval Military Medical UniversityShanghai 200050China

BACKGROUND Primary ciliary dyskinesia(PCD)is an uncommon and genetically diverse *** to reports,most patients had more than 50 visits before being diagnosed with PCD,and the age at diagnosis was mostly in preschool,with an average age of about(10.9±14.4)years *** is a pathogenic gene that regulates the cell cycle,and its mutation is linked to the uncommon human genetic disorder *** the prevalence of the CCNO mutation is regarded to be exceptionally low,new reports of this mutation have increased in comparison to prior *** patients with CCNO are rare,and the incidence rate is no more than 2%in whole PCD *** SUMMARY Here,we report a case of a young Chinese woman diagnosed with PCD,who was found to carry the CCNO gene by whole exon gene *** this case,a young non-smoking Chinese female exhibiting recurrent cough and sputum at *** computed tomography(CT)showed bronchiectasis with infection,and sinus CT showed chronic ***,the patient had no visceral transposition and no history of *** electron microscope,it was found that cilia were short and reduced in number,and no power arm of cilia was *** exon sequencing analysis of the genome of the patient showed that the patient carried CCNO pathogenic gene,exon c.303C>A nonsense mutation and c.248_252dup frameshift *** clinical symptoms and CT images were improved after two months of treatment with aerosol inhalation and oral *** The results showed that CCNO is an important cause of *** mutant genes that may contribute to genetically diverse disorders like PCD have been discovered as sequencing technology has ***,the increase of genetic information makes it easier to diagnose uncommon diseases in clinical practice.

关键词： Primary ciliary immobility disorder CCNO gene Whole exon gene sequencing Clinical profiles Review of literature Case report

Multi-time Collaborative Restoration for Integrated Electrical-gas Distribution Systems Based on Rolling Optimization

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CSEE Journal of Power and Energy Systems 2024年第2期10卷 660-671页

作者： Jingyang yun Zheng yan yun Zhou Peichao zhang Weidong Hu Key Laboratory of Control of Power Transmission and Conversion of Ministry of Education Department of Electrical EngineeringShanghai Jiao Tong University

This paper proposes a multi-time collaborative restoration model for integrated electricity-gas distribution sys-tems(IEGDSs)considering multiple resources after extreme weather *** on the linearized power flow constraints of the unbalanced electrical distribution system(EDS)and gas distribution system(GDS),this problem can be formulated as a mixed-integer linear programming(MILP)*** improve the efficiency and veracity of the solution,a rolling optimiza-tion based two-stage method is developed with the first stage solved by a linear approximation model,and the second stage solved by real-time updated rolling *** solving the MILP problem using rolling optimization,the proposed model and solution method achieve efficient and reliable collaborative restoration of IEGDS considering multiple resources and unbal-anced operation characteristics of *** effectiveness of the proposed model and method is validated by using an IEGDS made of a 37-bus unbalanced EDS and 11-node *** Terms-Electricity-gas system,mix-integer linear programming,power system restoration.

关键词： Electricity-gas system mix-integer linear programming power system restoration

Harmonic Inversion of Recurrence Spectra of Nonhydrogen Atom in an Electric Field

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Communications in Theoretical Physics 2008年第5期49卷 1291-1294页

作者： GAO Song XU Xue-You LI Hong-yun zhang yan-Hui LIN Sheng-Lu College of Physics and Electronics Shandong Normal University Jinan 250014 China

An extended harmonic inversion method is analytically continued to approach bifurcation region of the dosed orbits thus to obtain highly resolved spectra of lithium atom in external field. The suitable band-limited signal is generated by a semielassieal uniform approximation. By decimating the selected signal window and solving the algebraic set of nonlinear equations the quantum eigenvalues are properly fitted, which reveal the fine resonance structure hidden in low resolution spectrum. The study is made at the sealed energy ε= -2.7, relevant bifurcation effects and corescattered impacts have to be taken into account. It is demonstrated that the present method is a useful technique for the semiclassieal quantization of system with mixed regular-chaotic classical dynamics.

关键词： harmonic inversion Stark effects recurrence spectrum

Clustering for Bivariate Functional Data

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Acta Mathematicae Applicatae Sinica 2024年第3期40卷 613-629页

作者： Shi-yun CAO yan-qiu ZHOU yan-ling WAN Tao zhang School of Science Guangxi University of Science and TechnologyLiuzhou 545006China School of Marxism Guangxi University of Science and TechnologyLiuzhou 545006China Key Laboratory of Intelligent Information Processing and Graph Processing Guangxi University of Science and TechnologyLiuzhou 545006China

In this paper,we consider the clustering of bivariate functional data where each random surface consists of a set of curves recorded repeatedly for each *** k-centres surface clustering method based on marginal functional principal component analysis is proposed for the bivariate functional data,and a novel clustering criterion is presented where both the random surface and its partial derivative function in two directions are *** addition,we also consider two other clustering methods,k-centres surface clustering methods based on product functional principal component analysis or double functional principal component *** results indicate that the proposed methods have a nice performance in terms of both the correct classification rate and the adjusted rand *** approaches are further illustrated through empirical analysis of human mortality data.

关键词： bivariate functional data -centres surface clustering functional principal component analysis partial derivative function

Advances in doping strategies for sodium transition metal oxides cathodes:A review

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Frontiers in Energy 2024年第2期18卷 141-159页

作者： Zhijing zhang Haoze zhang Yaopeng WU Wei yan Jiujun zhang yun ZHENG Lanting QIAN Department of Chemical Engineering Waterloo Institute of NanotechnologyUniversity of WaterlooWaterloo ON N2L 3G1Canada School of Mechanical and Automotive Engineering South China University of TechnologyGuangzhou 510640China Institute of New Energy Materials Science and Engineering School of Materials Science and EngineeringFuzhou UniversityFuzhou 350108China

The electrochemistry of cathode materials for sodium-ion batteries differs significantly from lithium-ion batteries and offers distinct ***,the progress of commercializing sodium-ion batteries is currently impeded by the inherent inefficiencies exhibited by these cathode materials,which include insufficient conductivity,slow kinetics,and substantial volume changes throughout the process of intercalation and deintercalation ***,numerous methodologies have been utilized to tackle these challenges,encompassing structural modulation,surface modification,and elemental *** paper aims to highlight fundamental principles and strategies for the development of sodium transition metal oxide ***,it emphasizes the role of various elemental doping techniques in initiating anionic redox reactions,improving cathode stability,and enhancing the operational voltage of these cathodes,aiming to provide readers with novel perspectives on the design of sodium metal oxide cathodes through the doping approach,as well as address the current obstacles that can be overcome/alleviated through these dopant strategies.

关键词： sodium-ion batteries transition metal cathode doping strategy

An adaptive physics-informed deep learning method for pore pressure prediction using seismic data

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Petroleum Science 2024年第2期21卷 885-902页

作者： Xin zhang yun-Hu Lu yan Jin Mian Chen Bo Zhou State Key Laboratory of Petroleum Resources and Prospecting China University of Petroleum(Beijing)Beijing102249China College of Petroleum Engineering China University of Petroleum(Beijing)Beijing102249China College of Artificial Intelligence China University of Petroleum(Beijing)Beijing102249China PetroChina Tarim Oilfield Company KorlaXinjiang841000China

Accurate prediction of formation pore pressure is essential to predict fluid flow and manage hydrocarbon production in petroleum *** deep learning technique has been receiving more interest due to the great potential to deal with pore pressure ***,most of the traditional deep learning models are less efficient to address generalization *** fill this technical gap,in this work,we developed a new adaptive physics-informed deep learning model with high generalization capability to predict pore pressure values directly from seismic ***,the new model,named CGP-NN,consists of a novel parametric features extraction approach(1DCPP),a stacked multilayer gated recurrent model(multilayer GRU),and an adaptive physics-informed loss *** machine training,the developed model can automatically select the optimal physical model to constrain the results for each pore pressure *** CGP-NN model has the best generalization when the physicsrelated metricλ=0.5.A hybrid approach combining Eaton and Bowers methods is also proposed to build machine-learnable labels for solving the problem of few *** validate the developed model and methodology,a case study on a complex reservoir in Tarim Basin was further performed to demonstrate the high accuracy on the pore pressure prediction of new wells along with the strong generalization *** adaptive physics-informed deep learning approach presented here has potential application in the prediction of pore pressures coupled with multiple genesis mechanisms using seismic data.

关键词： Pore pressure prediction Seismic data 1D convolution pyramid pooling Adaptive physics-informed loss function High generalization capability

Composition optimization and performance prediction for ultra-stable water-based aerosol based on thermodynamic entropy theory

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Defence Technology（防务技术） 2024年第3期33卷 437-446页

作者： Tingting Kang Canjun yan Xinying Zhao Jingru Zhao Zixin Liu Chenggong Ju Xinyue zhang yun zhang yan Wu College of Chemical Engineering and Materials Science Tianjin University of Science and TechnologyNo.29 13th AvenueEconomic and Technologic Development ZoneTianjin 300457China Tianjin Key Laboratory of Brine Chemical Engineering and Resource Eco-utilization Tianjin University of Science and TechnologyTianjin 300457China College of Meteorology and Oceanography National University of Defense TechnologyChangsha 410073China

Water-based aerosol is widely used as an effective strategy in electro-optical countermeasure on the battlefield used to the preponderance of high efficiency,low cost and ***,the stability of the water-based aerosol is always unsatisfactory due to the rapid evaporation and sedimentation of the aerosol *** efforts have been devoted to improve the stability of water-based aerosol by using additives with different composition and ***,the lack of the criterion and principle for screening the effective additives results in excessive experimental time consumption and *** the stabilization time of the aerosol is still only 30 min,which could not meet the requirements of the perdurable ***,to improve the stability of water-based aerosol and optimize the complex formulation efficiently,a theoretical calculation method based on thermodynamic entropy theory is *** the factors that influence the shielding effect,including polyol,stabilizer,propellant,water and cosolvent,are considered within *** ultra-stable water-based aerosol with long duration over 120 min is obtained with the optimal fogging agent composition,providing enough time for fighting the electro-optic *** design guideline for choosing the additives with high phase transition temperature and low phase transition enthalpy is also proposed,which greatly improves the total entropy change and reduce the absolute entropy change of the aerosol cooling process,and gives rise to an enhanced stability of the water-based *** theoretical calculation methodology contributes to an abstemious time and space for sieving the water-based aerosol with desirable performance and stability,and provides the powerful guarantee to the homeland security.

关键词： Ultra-stable Water-based aerosol Thermodynamic entropy Composition optimization Performance prediction

Self-Powered Flexible Multicolor Electrochromic Devices for Information Displays

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Research 2024年第2期2023卷 529-540页

作者： Wenzhao Xue yun zhang Feng Liu Yao Dou Mei yan Wenshou Wang School of Chemistry and Chemical Engineering University of JinanJinan 250022P.R.China

The development of self-powered flexible multicolor electrochromic(EC)systems that could switch different color without an external power supply has remained extremely ***,a new trilayer film structure for achieving self-powered flexible multicolor EC displays based on self-charging/discharging mechanism is proposed,which is simply assembled by sandwiching an ionic gel film between 2 cathodic nickel hexacyanoferrate(NiHCF)and Prussian blue(PB)nanoparticle films on indium tin oxide *** display exhibits independent self-powered color switching of NiHCF and PB films with fast responsive time and high reversibility by selectively connecting the Al wire as anodes with the 2 EC *** switching is thus achieved through a color overlay effect by superimposing the 2 EC films,including green,blue,yellow,and *** bleaching/coloration process of the displays is driven by the discharging/self-charging mechanism for NiHCF and PB films,respectively,ensuring the self-powered color switching of the displays reversibly without an external power *** is further demonstrated that patterns can be easily created in the self-powered EC displays by the spray-coating method,allowing multicolor changing to convey specific ***,a self-powered ionic writing board is demonstrated based on the self-powered EC displays that can be repeatedly written freehand without the need of an external power *** believe that the design concept may provide new insights into the development of self-powered flexible multicolor EC displays with self-recovered energy for widespread applications.

关键词： connecting repeatedly powered

Upregulation of circ0000381 atienuates microglial/macrophage pyroptosis after spinal cord injury

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Neural Regeneration Research 2024年第6期19卷 1360-1366页

作者： yan zhang Wenkai zhang Tao Liu Ziqian Ma Wenxiu zhang yun Guan Xueming Chen Central Laboratory Beijing Luhe HospitalCapital Medical UniversityBeijingChina Department of Orthopedics Beijing Luhe HospitalCapital Medical UniversityBeijingChina Department of Emergency Medicine Aerospace Center HospitalBeijingChina Department of Anesthesiology and Critical Care Medicine Johns Hopkins University School of MedicineBaltimoreMDUSA Department of Neurological Surgery Johns Hopkins University School of MedicineBaltimoreMDUSA

Neuroinflammation exacerbates secondary damage after spinal cord injury,while microglia/macrophage pyroptosis is important to *** RNAs(circRNAs)play a role in the central nervous ***,the functional role and mechanism of circRNAs in regulating microglia/macrophage pyroptosis after spinal cord injury are still poorly *** the present study,we detected microglia/macrophage pyroptosis in a female rat model of spinal cord injury,along with upregulated levels of circ0000381 in the spinal *** further experimental results suggest that circ0000381 may function as a sponge to sequester endogenous microRNA423-3p(miR-423-3p),which can increase the expression of NOD-like receptor 3(NLRP3),a pyroptosis ***,upregulation of circ0000381 may be a compensatory change after spinal cord injury to attenuate microglia/macrophage ***,knockdown of circ0000381 expression exacerbated microglia/macrophage ***,our findings provide novel evidence for the upregulation of circ0000381,which may serve as a neuroprotective mechanism to attenuate microglia/macrophage pyroptosis after spinal cord ***,circ0000381 may be a novel therapeutic target for the treatment of spinal cord injury.

关键词： circ0000381 inflammasome macrophage microglia miR-423-3p neuroinflammation neuroprotection NLRP3 pyroptosis RNA-Seq spinal cord injury