bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Clinical Spectrum of ANO3—Report of a New Family and Literature Review
Authors: Percetti Marco , Zini Michela , Soliveri Paola , Cogiamanian Filippo , Ferrara Mariarosa , Orunesu Eva , Ranghetti Alessandra , Ferrarese Carlo , Pezzoli Gianni , Garavaglia Barbara , Isaias Ioannis Ugo , Sacilotto Giorgio 
Published: January 2024

AbstractBackgroundMutations in ANO3 are a rare cause of autosomal dominant isolated or combined dystonia, mainly presenting in adulthood.CasesWe extensively characterize a new, large ANO3 family with six affected carriers. The proband is a young girl who had suffered from tremor and painful dystonic movements in her right arm since the age of 11 years. She later developed a diffuse dystonic tremor and mild extrapyramidal signs (ie, rigidity and hypodiadochokinesis) in her right arm. She also suffered from psychomotor delay and learning difficulties. Repeated structural and functional neuroimaging were unremarkable. A dystonic tremor was also present in her two sisters. Her paternal aunt, father, and a third older sister presented episodic postural tremor in the arms. The father and one sister also presented learning difficulties. The heterozygous p.G6V variant in ANO3 was identified in all affected subjects.Literature reviewStratification by age at onset divided ANO3 cases into two major groups, where younger patients displayed a more severe phenotype, probably due to variants near the scrambling domain.ConclusionsWe describe the phenotype of a new ANO3 family and highlight the need for functional studies to explore the impact of ANO3 variants on its phospholipid scrambling activity.
Can digital transformation reduce R&D investment disruption during the top executive transition period?
Authors: Deng Xiankun , Hou Qingsong , Shen Jin 
Published: January 2024

AbstractThis study examines whether digital transformation can reduce research and development (R&D) investment disruption during the top executive transition period. Using panel data of A‐listed firms in China from 2007 to 2021, we find that top executive turnover (TET) generally leads to a remarkable reduction in R&D investment, while digital transformation can significantly mitigate the negative impact of TET on R&D investment. Moreover, the smooth management transition effect of digital transformation is more pronounced for firms with outside succession and high organizational complexity. We further divide R&D investments into exploratory and exploitative R&D investments. Compared to exploitative R&D investment, exploratory R&D investment is more adversely affected by TET, and digital transformation has a positive moderating effect on the relationship between them. Our findings suggest that digital transformation enables companies to maintain continuous R&D investment during the top executive transition by improving communication efficiency and enhancing organizational learning.
Navigating the trade‐off between corporate social responsibility disclosure and the cost of financing: Evidence from BRICS economies
Authors: Gupta Jyotirani , Das Niladri 
Published: January 2024

AbstractThis study investigates the relationship between corporate social responsibility disclosure (CSRD) and the cost of financing for firms in emerging economies. Results show that CSRD initially reduces the cost of equity financing but increases equity costs beyond a certain threshold. Firms should weigh the trade‐offs of signal cost and benefits when choosing financing strategies. Moreover, investors in the debt market perceive a higher level of board independence as less risky, which consequently has a negative impact on the relationship between CSRD and the cost of debt. Thus, companies should streamline their CSR disclosures to reduce their cost of capital.
The nonlinear relationship between digital affordances and firm‐level export performance: The moderating role of organizational ambidexterity
Authors: Erdey László , Liu Laisha , Nagy Adrián 
Published: January 2024

AbstractA diminished trade cost has been ascribed to the advancement of digital transformation, particularly digital technologies and platforms. Nevertheless, whether this decline in trade costs manifests in the extensive or intensive margin of trade remains an unresolved query. This study conducts a comprehensive analysis of the influence of digital affordances on firm‐level export performance. Specifically, we posit that digital affordances will manifest a convex influence on firms' export decisions and intensive margin of export. Furthermore, we propose that the relationship between digital affordances and firm‐level export decisions will be contingent upon the degree of firms' organizational ambidexterity. To empirically test our hypotheses, we leverage a dataset comprising 13,251 firm‐level observations gathered by the World Bank Enterprise Surveys (WBES) unit across 19 Organization for Economic Cooperation and Development (OECD) countries. Our study substantiates a U‐shaped nexus between digital affordances and firm‐level export performance. Importantly, our analysis supports that this U‐shaped nexus is accentuated when firms present a high level of organizational ambidexterity.
Retrospective review of patients with myasthenia gravis switched from plasma exchange therapy to efgartigimod treatment
Authors: Mehrabyan Anahit , Traub Rebecca E. 
Published: January 2024

AbstractIntroduction/AimsTherapeutic plasma exchange (TPE) is sometimes used as maintenance therapy for the treatment of myasthenia gravis (MG). Efgartigimod is a newly approved monoclonal antibody targeting the neonatal Fc receptor, effectively reducing immunoglobulin G levels in the treatment of MG. The aim of this study was to describe the clinical experience of switching patients from maintenance TPE treatment to efgartigimod infusions.MethodsA retrospective review of medical records was performed on patients previously treated with maintenance TPE for the diagnosis of MG and subsequently switched to efgartigimod infusions. Clinical characteristics and response to treatment switch were described.ResultsFive of seven patients demonstrated improvement on Myasthenia Gravis Foundation of America‐post intervention status, one was unchanged and one was in pharmacological remission. This was reflected in pre‐ and postswitch MG activities of daily living and MG manual muscle testing scores. All patients have continued on efgartigimod therapy. The duration of treatment with efgartigimod at the time of this review ranged from 1 to 13 months. Recurrent uncomplicated infections were noted in two patients on efgartigimod therapy. Maintenance dosing regimens of efgartigimod varied based on clinical response to treatment and side effects.DiscussionIn this series, efgartigimod appeared effective and well tolerated in patients switched from TPE.
Roles of general and central adiposity in cardiometabolic multimorbidity: revisiting the obesity paradox using a multistate model
Authors: Xia Xue , Chen Shuohua , Tian Xue , Xu Qin , Zhang Yijun , Zhang Xiaoli , Li Jing , Wang Penglian , Wu Shouling , Wang Anxin 
Published: January 2024

AbstractObjectiveThe objective of this study was to evaluate the associations of general and central obesity with risk of first cardiometabolic disease (FCMD), cardiometabolic multimorbidity (CMM), and death.MethodsA total of 86,169 participants who were CMD‐free were included from the Kailuan cohort and categorized into four groups by quartiles of BMI, waist to hip ratio (WHR), weight‐adjusted waist index, and waist to height ratio. We defined FCMD as the first onset of diabetes, stroke, or myocardial infarction and CMM as co‐occurrence of at least two CMDs. Multistate models were used to estimate hazard ratios and 95% CI.ResultsA total of 18,461 participants developed FCMD, of whom 1476 progressed to CMM, and 10,009 died during follow‐ups. Both general and central adiposity indices increased the risk of transition from baseline to FCMD and from FCMD to CMM. However, compared with the first quartile, the hazard ratio (95% CI) of the fourth quartile of BMI was 0.86 (95% CI: 0.80–0.91) for transition from health to death and 0.66 (95% CI: 0.59–0.74) from FCMD to death, whereas the corresponding estimates of WHR were 1.22 (95% CI: 1.14–1.31) and 1.16 (95% CI: 1.02–1.32), respectively.ConclusionsCentral adiposity indices such as WHR were associated with an increased risk of CMD and mortality, showing no evidence for the obesity paradox and thereby supporting a shift of public focus from BMI only to both general obesity and adiposity distribution.
Adolescents and young adults with rhabdomyosarcoma: A report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group
Authors: Harrison Douglas J. , Qumseya Amira , Xue Wei , Arnold Michael , Lautz Timothy B. , Hiniker Susan M. , Thomas Stefanie M. , Venkatramani Rajkumar , Weiss Aaron R. , Mascarenhas Leo 
Published: January 2024

AbstractIntroductionThe impact of established prognostic factors on survival outcomes for childhood rhabdomyosarcoma (RMS) have not been well described in the adolescent and young adult (AYA) RMS patient population.MethodsThis is a retrospective analysis of patients with newly diagnosed RMS enrolled between 1997 and 2016 on seven previously reported Children's Oncology Group (COG) clinical trials. Demographics, clinical features, treatment details, and outcome data were collected. Five‐year event‐free survival (EFS) and overall survival (OS) were estimated for patients diagnosed at age 15–39 years and those diagnosed under age 15 years using the Kaplan–Meier method. Log‐rank test was used to compare prognostic factors for EFS and OS. Factors significant in the univariable analysis were included in a Cox proportional hazards regression model. Nonsignificant covariates were removed from the multiple regression model.ResultsTotal 2151 patients including 402 AYAs were analyzed. AYAs were more likely to present with primary tumors ≥5 cm in size, metastatic disease, alveolar histology, and have FOXO1 fusions compared to children. Five‐year EFS for the AYA cohort was 44.2% versus 67% for children (p < .001), and 5‐year OS was 52% for the AYA cohort versus 78% for children (p < .001). Multivariable analysis revealed tumor site, size and invasiveness, clinical group, and histology were prognostic in AYAs.ConclusionAYAs with RMS have a poorer prognosis compared to younger children due to multiple factors. Further research focused on AYAs to better understand RMS biology and improve treatments is critical to improve survival.
Improving resistance–strain effects of conductive polymer composites modified by multiscale fillers: Short carbon fiber and carbon nanotube
Authors: Niu Yi‐Fan , Zhang Yun‐Xuan , Yao Jia‐Wei , Wang Hai‐Nan 
Published: January 2024

AbstractConductive polymer composites (CPCs) have attracted much attention in strain sensing applications due to their excellent conductivities and high sensitivities. However, research on simultaneous modification of CPCs with multiple conductive fillers is lacking. This study aims to systematically investigate the electrical conductivities and strain‐sensing properties of two filler‐modified epoxy resin (EP) composites with different scale additives—short carbon fibers (SCFs) and carbon nanotubes (CNTs)—obtained by mixing and curing to obtain the samples. Both the SCF/EP and SCF/CNT/EP composite resistivities decreased with increasing filler content, and 1.35 wt% was the percolation threshold for SCF/EP composites; during monotonic tensile loading, the resistance change ratio (ΔR/R0) of the CPCs increased with increasing strain, and the sensitivities decreased with increasing conductive filler content. The CPCs containing SCFs and CNTs exhibited stable and repeatable resistance responses during cyclic tensile loading, among which the SCF/CNT_1.5/0.3 composite was the most suitable for use as a sensing material with a sensitivity of 0.149. According to the conductivity and resistance–strain response mechanism analysis, the CNTs supplementing and refining the CPC conductive network composed of SCFs were considered the main reason for the resistance response linearization and excellent dynamic response repeatability. CPCs can be widely used in strain sensing and damage monitoring.Highlights
Provided a strategy for multiscale conductive filler‐modified epoxy resins.
Realized monotonicity of resistance–strain response by adding multiscale conductive fillers.
Explored resistance‐strain effect under dynamic cycling loading.
Identified optimal materials for damage detection.
Analyzed the resistance–strain response mechanism of single and multiscale conductive filler‐modified CPCs.
Influence of aramid fibers modified by glycidyl polyhedral silsesquioxane assisted with supercritical carbon dioxide on the properties of aramid fiber‐reinforced styrene butadiene rubber composites
Authors: Li Yang , Shi Caiwen , Pan Xiaoli , Yang Le , Zhang Chun 
Published: January 2024

AbstractThe weak interfacial adhesion of aramid fibers (AFs) in rubber composites can be improved by modifying their surface structure. Herein, modified AFs with a high grafting rate were prepared by modification using glycidyl polyhedral silsesquioxane (POSS) assisted with supercritical carbon dioxide (scCO2). X‐ray photoelectron spectroscopy confirmed the chemical bonding between AFs and glycidyl POSS. Thermogravimetric analysis and scanning electron microscopy images showed that POSS was grafted uniformly and the surface roughness enhanced obviously. After grafting the rubber with 5 wt% glycidyl POSS assisted with scCO2, the surface grafting rate reached 12.26% and surface crystal spacing increased. The grafted layer on the modified AF surface was uniform, and the tensile strength of the modified fiber increased by 7.98%. Short AF‐reinforced carbon black/styrene butadiene rubber (AF/CB/SBR = 2/50/100 phr) composites were prepared to investigate the adhesion strength between modified AFs and SBR. The reduction in tan δ of the composites confirmed the improvement in the interfacial adhesion strength. The tensile strength and modulus at 100% elongation of the composites increased by 15.13% and 25.77%, respectively, and the relative interface slip increased by 23.26%. Moreover, the cutting resistance of the composites showed a dramatic improvement, particularly for grafting with 5% glycidyl POSS assisted with scCO2. © 2024 Society of Industrial Chemistry.
Women's empowerment and its role in household food security to achieve SDGs: Empirical evidence from rural Bangladesh
Authors: Haque Sadika , Salman Md. , Hasan Md. Mehedi , Prithi Aunjuman Ara , Hossain Md. Emran 
Published: January 2024

AbstractWomen in developing countries are often characterized by lower social status, less input into decision‐making, lack of access to resources, and lower mobility, while their inclusion is vital to achieving household food security. This concept is recognized as important for attaining sustainable development goals (SDGs), including SDG‐5 (gender equality). This study investigated the level of women's empowerment (WE) and its determinants, household food security, and the connections between WE and food security in farming households in Bangladesh. Using primary data collected from 350 households and applying the modified project‐level WE in Agriculture Index, this study found that less than 1% of women were empowered. Poisson regression evidenced that women's years of schooling, household size, farm type, and having household members working away were positively associated with WE, while the farm's distance from home was negatively related. The Household Food Insecurity Access Scale revealed that only 18% of households were food secure. Partial least squares structural equation modeling showed that three domains of empowerment—access to decisions on financial services, input in agricultural decisions, and ownership of assets—were negatively associated with household food insecurity, while mobility was positively related. This study shows, a realistic policy is needed to achieve food security through empowering rural women to achieve SDGs in a timely manner.
Optical Trapping and Fast Discrimination of Label‐Free Bacteriophages at the Single Virion Level
Authors: Villa Nicolas , Tartari Enrico , Glicenstein Simon , de Villiers de la Noue Hugues , Picard Emmanuel , Marcoux Pierre R. , Zelsmann Marc , Resch Grégory , Hadji Emmanuel , Houdré Romuald 
Published: January 2024

AbstractThere is a recent resurgence of interest in phage therapy (the therapeutic use of bacterial viruses) as an approach to eliminating difficult‐to‐treat infections. However, existing approaches for therapeutic phage selection and virulence testing are time‐consuming, host‐dependent, and facing reproducibility issues. Here, this study presents an innovative approach wherein integrated resonant photonic crystal (PhC) cavities in silicon are used as optical nanotweezers for probing and manipulating single bacteria and single virions with low optical power. This study demonstrates that these nanocavities differentiate between a bacterium and a phage without labeling or specific surface bioreceptors. Furthermore, by tailoring the spatial extent of the resonant optical mode in the low‐index medium, phage distinction across phenotypically distinct phage families is demonstrated. The work paves the road to the implementation of optical nanotweezers in phage therapy protocols.
Gallium‐Based Liquid Metals in Rechargeable Batteries: From Properties to Applications
Authors: Zeng Ziyue , Wang Chenyang , Zeng Mengqi , Fu Lei 
Published: January 2024

AbstractGallium‐based (Ga‐based) liquid metals have attracted considerable interest due to their low melting points, enabling them to feature both liquid properties and metallic properties at room temperature. In light of this, Ga‐based liquid metals also possess excellent deformability, high electrical and thermal conductivity, superior metal affinity, and unique self‐limited surface oxide, making them popular functional materials in energy storage. This provides a possibility to construct high‐performance rechargeable batteries that are deformable, free of dendrite growth, and so on. This review primarily starts with the property of Ga‐based liquid metal, and then focuses on the potential applications in rechargeable batteries by exploiting these advantages, aiming to construct the correlation between properties and structures. The glorious applications contain interface protection, self‐healing electrode construction, thermal management, and flexible batteries. Finally, the opportunities and obstacles for the applications of liquid metal in batteries are presented.
Science for our children: Othermothering leadership within an elementary science network
Authors: Marshall Stefanie L. , Forrester Jessica , Tilsen Jenny 
Published: January 2024

AbstractTransformational equity‐centered science education requires the fields of science education and school leadership to critically consider the limited preparation elementary principals are offered to lead for science education. Thus far, little effort has been made to foster a transdisciplinary curricula beyond traditional organizational theories related to school culture and climate; learning sciences; and supervision. School leadership programs are currently inadequately preparing elementary leaders in rigorous pedagogies involving science education. Although the role of principals is often not discussed concerning science implementation in elementary education, principals play a critical role in science decision‐making. In this study, the authors present a case study of one elementary principal who also served as an othermother. Othermothers have been described as those who share mothering responsibilities in Black communities. Through interview transcripts, field notes, and social network data, the authors examine how this othermother cared for her students and community by advocating for science instruction. By strategically navigating the socio‐political and policy climate and drawing on her authentic relationships, this othermother was critical in implementing a science agenda for elementary science. Three themes that emerged from the data analysis are, (1) othermothers view science as a potential means to transform lives and fulfill the needs of the local community, (2) science policies (i.e., federal, state, and local) can limit the potential of the vision of science an othermother has for the community, (3) othermothers draw on their community to guide equitable science instruction. Overall, othermothers have visions for what science can do. However, they cannot counter the status quo individually. Collective action among educators in various roles is one means of moving an equity agenda concerning science education forward.
Arecoline promotes Akt‐c‐Myc‐driven aerobic glycolysis in esophageal epithelial cells
Authors: Han Shuangze , Chen Yingji , Huang Yu , Jin Longyu , Ma Yuchao 
Published: January 2024

AbstractAerobic glycolysis is a typical metabolic rearrangement for tumorigenesis. Arecoline is of explicit carcinogenicity, numerous works demonstrate its mutagenicity, genotoxicity, and cytotoxicity. However, the effects of arecoline on aerobic glycolysis of esophageal epithelial cells remain unclear. In the present study, 5 μM arecoline efficiently increased HK2 expression to induce aerobic glycolysis in Het‐1A‐Are and NE2‐Are cells. The mechanistic analysis showed that arecoline activated the Akt‐c‐Myc signaling pathway and reduced the GSK3β‐mediated phosphorylation of c‐Myc on Thr58 to prevent its ubiquitination and destruction, subsequently promoting HK2 transcription and expression. Taken together, these results suggest that arecoline can induce aerobic glycolysis of esophageal epithelial cells and further confirm that arecoline is a carcinogen harmful to human health.
The quintuplet annihilation spectrum
Authors: Baumgart Matthew , Rodd Nicholas L. , Slatyer Tracy R. , Vaidya Varun 
Published: January 2024

Abstract
We extend the Effective Field Theory of Heavy Dark Matter to arbitrary odd representations of SU(2) and incorporate the effects of bound states. This formalism is then deployed to compute the gamma-ray spectrum for a 5 of SU(2): quintuplet dark matter. Except at isolated values of the quintuplet mass, the bound state contribution to hard photons with energy near the dark-matter mass is at the level of a few percent compared to that from direct annihilation. Further, compared to smaller representations, such as the triplet wino, the quintuplet can exhibit a strong variation in the shape of the spectrum as a function of mass. Using our results, we forecast the fate of the thermal quintuplet, which has a mass of ~13.6 TeV. We find that existing H.E.S.S. data should be able to significantly test the scenario, however, the final word on this canonical model of minimal dark matter will likely be left to the Cherenkov Telescope Array (CTA).
Effective field theories of axion, ALP and dark photon
Authors: Song Huayang , Sun Hao , Yu Jiang-Hao 
Published: January 2024

Abstract
With the help of Young tensor technique, we enumerate the complete and independent set of effective operators up to dim-8 for the extension of the standard model with a Goldstone boson by further imposing the Adler’s zero condition in the soft momentum limit. Such basis can be reduced to describe the axion or majoron effective Lagrangian if further (symmetry) constraints are imposed. Then reformulating dark photon as combination of Goldstone boson and transverse gauge boson, the effective operators of the Goldstone boson can be extended to effective chiral Lagrangian description of the dark photon. For the first time we obtain 0 (0), 6 (44), 1 (1), 44 (356), 32 (520) operators in Goldstone effective field theory, and 9 (49), 0 (0), 108 (676), 10 (426), 1904 (40783) operators in dark photon effective field theory at the dimension 4, 5, 6, 7, 8 for one (three) generation of fermions.