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Sturge–Weber syndrome:an update for the pediatrician

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World Journal of Pediatrics 2024年第5期20卷 435-443页

作者： Emilie Dingenen Damien Segers Hannelore de Maeseneer Dirk Van Gysel Ghent University Faculty of Medicine and Health Sciences GhentBelgium department of Pediatrics O.L.Vrouw Hospital AalstMoorselbaan 1649300AalstBelgium Interdisciplinary Unit of Pediatric dermatology Vrije Universiteit Brussel(VUB)Universitair Ziekenhuis Brussel(UZ Brussel)BrusselsBelgium

Sturge–Weber syndrome(SWS)is a rare congenital neurocutaneous disorder characterized by the simultaneous presence of both cutaneous and extracutaneous capillary *** usually presents as a facial port-wine birthmark,with a varying presence of leptomeningeal capillary malformations and ocular vascular *** latter may lead to significant neurological and ocular morbidity such as epilepsy and *** is most often caused by a somatic mutation involving the G protein subunit alpha Q or G protein subunit alpha 11 gene causing various alterations in downstream signaling *** specifically conducted a comprehensive review focusing on the current knowledge of clinical practices,the latest pathophysiological insights,and the potential novel therapeutic avenues they *** sources A narrative,non-systematic review of the literature was conducted,combining expert opinion with a balanced review of the available literature.A search of PubMed,Google Scholar and Embase was conducted,using keywords“Sturge–Weber Syndrome”OR“SWS”,“Capillary malformations”,“G protein subunit alpha 11”OR“G protein subunit alpha Q”.Results One of the hallmark features of SWS is the presence of a port-wine birthmark at birth,and forehead involvement is most indicative for *** most common ocular manifestations of SWS are glaucoma and choroidal *** presents in either in infancy(0–3 years of age)or later in *** complications are common in SWS,occurring in about 70%–80%of patients,with seizures being the most common *** significantly impacts the quality of life for patients and their families,and requires a multidisciplinary approach for diagnosis and ***,no disease-modifying therapies exist,and treatment is mostly focused on symptoms or complications as they *** SWS remains a complex and heterogeneous *** research is needed to optimize diagnostic and therapeutic strategies,and to transl

关键词： Capillary malformations G-protein Gq-G11 Port-wine birthmark Neurocutaneous disorder Sturge-Weber syndrome

Numerical investigation of the coupled aero-hydrodynamic performances of a semi-submersible floating offshore wind turbine with inclined columns

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Journal of Hydrodynamics 2024年第2期36卷 316-330页

作者： Ying-jie Xue Xiao-long Yang Wei-wen Zhao de-cheng Wan Computational Marine Hydrodynamics Lab(CMHL) School of Naval ArchitectureOcean and Civil EngineeringShanghai Jiao Tong UniversityShanghai200240China Offshore Oil Engineering Co.Ltd. Tianjin300461China

Numerical investigations of floating platforms with different outer column inclined angles under two operating conditions of regular wave and irregular wave are presented in this paper.A coupled aero-hydrodynamic computational fluid dynamics in-house solver FOWT-UALM-SJTU is applied for the ***,the validation for wave and wind generation are conducted to determine mesh distribution *** on these,the hydrodynamic motion response,aerodynamic performance and wake flow are analyzed to explore the impact of inclined *** spectral analysis on the motion response under wave action,discuss the aerodynamic attack angle and inflow wind velocity along the blade spanwise direction in detail,reveal different trends in wake development and *** results show that for the regular wave condition with the increase of inclined angles,the equilibrium position of surge motion is constantly rising,while pitch is *** maximum root mean square(rms)value occurs at angle=30°,compared with the original OC4 FOWT,the rms in power and thrust increase 0.35%,0.71%.And there are two low regions of attack angle and high regions of axial inflow velocity,corresponding to aerodynamic *** spectral analysis indicates that the natural frequency of pitch motion will increase with inclined ***,from the middle to far region of wake flow,the velocity recovery of FOWT with inclined angle will become faster,which is beneficial for downstream turbines to enhance more wind energy.

关键词： Floating offshore wind turbine numerical simulation aero-hydrodynamic coupled analysis floating platform with inclinedcolumns wake flow recovery

Prediction of high-embankment settlement combining joint denoising technique and enhanced GWO-v-SVR method

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Journal of Rock Mechanics and Geotechnical Engineering 2024年第1期16卷 317-332页

作者： Qi Zhang Qian Su Zongyu Zhang Zhixing deng de Chen School of Civil Engineering Southwest Jiaotong UniversityChengduChina MOE key Laboratory of High Speed Railway Engineering Southwest Jiaotong UniversityChengduChina

Reliable long-term settlement prediction of a high embankment relates to mountain infrastructure *** study developed a novel hybrid model(NHM)that combines a joint denoising technique with an enhanced gray wolf optimizer(EGWO)-n-support vector regression(n-SVR)***-embankment field measurements were preprocessed using the joint denoising technique,which in-cludes complete ensemble empirical mode decomposition,singular value decomposition,and wavelet packet ***,high-embankment settlements were predicted using the EGWO-n-SVR *** this method,the standard gray wolf optimizer(GWO)was improved to obtain the EGWO to better tune the n-SVR model *** proposed NHM was then tested in two case ***,the influences of the data division ratio and kernel function on the EGWO-n-SVR forecasting performance and prediction efficiency were *** results indicate that the NHM suppresses noise and restores details in high-embankment field ***,the NHM out-performs other alternative prediction methods in prediction accuracy and *** demonstrates that the proposed NHM is effective in predicting high-embankment settlements with noisy field ***,the appropriate data division ratio and kernel function for EGWO-n-SVR are 7:3 and radial basis function,respectively.

关键词： High embankment Settlement prediction Joint denoising technique Enhanced gray wolf optimizer Support vector regression

Numerical simulation of liquid sloshing in a spherical tank by MPS method

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Journal of Hydrodynamics 2024年第2期36卷 232-240页

作者： Cong-yi Huang Ji-fei Wang Wei-wen Zhao de-cheng Wan Computational Marine Hydrodynamic Lab(CMHL) School of Naval ArchitectureOcean and Civil EngineeringShanghai Jiao Tong UniversityShanghai200240China Shanghai Aerospace System Engineering Institute Shanghai201109China

This paper investigates the sloshing phenomena in a spherical liquid tank using the moving particle semi-implicit(MPS)method,a crucial study in fluid *** from previous research focused on rectangular or LNG tanks,this work explores the unique motion patterns inherent to spherical *** accuracy of our in-house MPS solver MLParticle-SJTU is validated against experimental data and finite volume method(FVM).And the MPS method reveals a closer alignment with experimental outcomes,which suggests that MPS method is particularly effective for modeling complex,non-linear fluid *** the fluid’s response to excitation at its natural frequency is simulated,showcasing vigorous sloshing and rotational *** analyses of the fluid motion are conducted by drawing streamline diagrams,velocity vector diagrams,and vorticity *** fluid’s motion response is explored using both time-domain and frequency-domain curves of the fluid centroid,as well as the sloshing force.

关键词： Moving particle semi-implicit(MPS)method spherical tank liquid sloshing model experiment

Rheological and stability analysis of cement pastes incorporating silica-based wastes

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Particuology 2024年第6期89卷 144-152页

作者： Julia da Rosa Martins Janaíde Cavalcante Rocha Dachamir Hotza Luciano Senff Graduate Program in Materials Science and Engineering(PPGMAT) Federal University of Santa Catarina(UFSC)Florianópolis88040-900SCBrazil Graduate Program in Civil Engineering(PPGEC) Federal University of Santa Catarina(UFSC)Florianópolis88040-900SCBrazil Graduate Program in Chemical Engineering(POSENQ) Federal University of Santa Catarina(UFSC)Florianópolis88040-900SCBrazil department of Mobility Engineering Federal University of Santa Catarina(UFSC)Joinville89219-600SCBrazil

This study investigates the influence of waste characteristics,especially zeta potential,on the properties of cement pastes and *** focus is to evaluate the impact of the zeta potential of cement particles and waste materials on the sedimentation speed,rheology,and hardening time of stabilized cement *** Cement II F 40,retarder additive,silica,and fly ash were used in the *** pastes were prepared,and during the stabilization period,their rheological properties and pH were *** zeta potential and sedimentation speed of the cement and waste particles were measured at the pH that the pastes presented during the entire stabilization *** the stabilization period,the pastes were subjected to the hardening time *** zeta potential analyses revealed diverse values for the different powder types,with the cement particles exhibiting a zeta potential of−3.0 mV,the silica particles exhibiting−10.5 mV,and the fly ash particles exhibiting−20.3 *** influence of the high zeta potential modulus was observed on the sedimentation speed,with the solution containing fly ash exhibiting a speed of 40.01μm/s,whereas the solution containing only cement exhibited a speed of 99.38μm/*** the pastes,the results indicate that the presence of fly ash particles with a significantly negative zeta potential led to a 16%reduction in hardening time compared to particles with a lower modulus of zeta *** tests showed that the inclusion of fly ash particles prevented the formation of *** the zeta potential influenced agglomerate formation and hardening time,it was found to have no effect on yield stress or viscosity.

关键词： Wastes Zeta potential Setting time Rheometry

Effect of FeSi additive in dual-chamber sample cup on thermal analysis characteristic values and vermiculating rate of compacted graphite iron

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China Foundry 2024年第1期21卷 91-100页

作者： de-quan Shi Ze-yu Liu Gui-li Gao Yi-cheng Feng School of Materials and Chemistry University of Shanghai for Science and TechnologyShanghai 200093China School of Materials Science and Chemical Engineering Harbin University of Science&TechnologyHarbin 150040China School of Mechanical and Energy Engineering Shanghai Technical Institute of Electronics and InformationShanghai 201411China

Thermal analysis plays a key role in the online inspection of molten iron *** solidification process of molten iron can be reflected by thermal analysis curves,and silicon is one of important elements affecting the solidification of molten *** this study,FeSi75 was added in one chamber of the dual-chamber sample cup,and the influences of FeSi75 additive on the characteristic values of thermal analysis curves and vermiculating rate were *** results show that with the increase of FeSi75,the start temperature of austenite formation TALfirstly decreases and then increases,but the start temperature of eutectic growth TSEF,the lowest eutectic temperature TEU,temperature at maximum eutectic reaction rate TEM,and highest eutectic temperature TERkeep always an *** temperature at final solidification point TEShas little *** FeSi75 additive has different influences on the vermiculating rate of molten iron with different vermiculation,and the vermiculating rate increases for lower vermiculation molten iron while decreases for higher *** to the thermal analysis curves obtained by a dual-chamber sample cup with 0.30wt.%FeSi75 additive in one chamber,the vermiculating rate of molten iron can be evaluated by comparing the characteristic values of these *** time differenceΔtERcorresponding to the highest eutectic temperature TERhas a closer relationship with the vermiculating rate,and a parabolic regression curve between the time differenceΔtERand vermiculating rateηhas been obtained within the range of 65%to 95%,which is suitable for the qualified melt.

关键词： FeSi75 additive characteristic value vermiculating rate thermal analysis compacted graphite iron

Changes in calcium accumulation and utilization efficiency and their impact on recycling,immobilization,and export across the oil palm cycle

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Oil Crop Science 2024年第3期9卷 143-150页

作者： Ismael de Jesus Matos Viégas Luma Castro de Souza Eric Victor de Oliveira Ferreira Milton Garcia Costa Glauco André dos Santos Nogueira Vitor Resende do Nascimento Candido Ferreira de Oliveira Neto Capanema Campus Federal Rural University of the Amazon(UFRA)Av.Barao de CapanemaCaixa D'agua5514-6434ParaBrazil Center of Agricultural and Environmental Sciences Federal University of Maranhao(UFMA)ChapadinhaBR-222km 04SNBoa Vista65500000MaranhaoBrazilc Capitao Poço Campus Federal Rural University of the Amazon(UFRA)Tv.Pau AmareloS/n-Vila Nova68650-000Capitao PoçoParaBrazil department of Soil Science Sao Paulo State University(Unesp)School of Agricultural and Veterinarian SciencesJaboticabalVia de Acesso Prof.Paulo Donato CastelaneCastellane-S/N-Vila Industrial14884-900Sao PauloBrazil Institute of Agricultural Sciences(ICA) Federal Rural University of the Amazon(UFRA)Estr.Principal da Ufra2150Curio-Utinga66077-580BelemParaBrazil

Effective calcium(Ca)management is crucial for optimizing oil palm cultivation and enhancing crop *** study aimed to gain insights into the dynamics of Ca concentration,accumulation,exportation,immobilization,and recycling in various oil palm organs relative to plant *** experiment was conducted at the Agropalma enterprise site in the northeastern region of Para State,Brazil,evaluating seven plant age treatments:2,3,4,5,6,7,and 8 years *** a completely randomized design with four *** results demonstrated an age-related increase in Ca concentration in petioles,rachis,arrows,male inflorescences,peduncles,and ***,Ca accumulation exhibited an upward trend in all organs with progressing plant ***,the study revealed an enhanced Ca use efficiency across all plant organs in correlation with the age of oil palm *** findings underscore the dynamic nutritional demands of oil palm,influencing Ca immobilization,cycling,and export throughout its developmental stages.

关键词： Elaeis guineensis Jacq Ca cycling Ca export Ca immobilization Ca use efficiency Plant nutrition Amazon

Unraveling the enigma:A comprehensive review of solid pseudopapillary tumor of the pancreas

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World Journal of Gastrointestinal Oncology 2024年第3期16卷 614-629页

作者： Ye-Cheng Xu de-Liang Fu Feng Yang department of Pancreatic Surgery Pancreatic Disease InstituteHuashan HospitalFudan UniversityShanghai 200040China

Solid pseudopapillary tumor of the pancreas(SPTP)is a rare neoplasm predom-inantly observed in young ***,CTNNB1 mutations,β-catenin nuclear accumulation,and subsequent Wnt-signaling pathway activation are the leading molecular *** preoperative diagnosis often relies on imaging techniques and endoscopic *** resection remains the mainstay *** models,such as the Fudan Prognostic Index,show promise as predictive tools for assessing the prognosis of *** three types of metachronous liver metastasis can be beneficial in tailoring individu-alized treatment and follow-up *** advancements,challenges persist in understanding its etiology,establishing standardized treatments for unresectable or metastatic diseases,and developing a widely recognized grading *** comprehensive review aims to elucidate the enigma by consolidating current knowledge on the epidemiology,clinical presentation,pathology,molecular characteristics,diagnostic methods,treatment options,and prognostic factors.

关键词： Pancreas Solid pseudopapillary tumor β-catenin Endoscopic ultrasound Surgery Recurrence Liver metastasis Prognostic prediction

Skin-inspired interface modification strategy toward a structurefunction integrated hybrid smart fabric system with self-powered sensing property for versatile applications

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Nano Research 2024年第9期17卷 8200-8208页

作者： Xiang Cheng Teng Chen de Gong Pengcheng Ma Bo Chen Jun Cai Aerospace Information Research Institute Chinese Academy of SciencesBeijing 100190China School of Mechanical Engineering and Automation Beihang UniversityBeijing 100191China Beijing U-Precision Tech Co. Ltd.Beijing 100176China University of Chinese Academy of Sciences Beijing 100049China

Fabric-based composites with superior mechanical properties and excellent perceptive function are highly ***,it remains a huge challenge to attain structure-function integration,especially for hybrid fabric ***,a skin-inspired interface modification strategy is proposed toward this target by constructing a hybrid smart fabric system consisting of two types of smart fabrics:carbon nanotube(CNT)/MXene-modified aramid fabrics and zinc oxide nanorod(ZnO NR)-modified carbon *** on that,flexible piezoelectric pressure sensors with skin-like hierarchical perception interfaces are fabricated,which demonstrate superb sensitivity of 2.39 V·kPa^(-1)and are capable of various wearable monitoring ***,the interface-modified hybrid fabric reinforced plastics can also be fabricated,which are proven to possess 13.6%higher tensile strength,10.1%elastic *** impressively,their average energy absorption can be improved by 111.9%,accompanied with inherent damage alert *** offers a paradigm to fabricate structure-function integrated hybrid smart fabric composites for the smart clothing and intelligent aerial vehicles.

关键词： hybrid fabrics interface modification piezoelectric pressure sensors self-diagnostic composites structure-function integration