bluuebunny/crossref_metadata_2025_split huggingface dataset:
Homozygous variant of MLC1 results in megalencephalic leukoencephalopathy with subcortical cysts
Authors: Zha Jian , Chen Yong , Cao Fangfang , Xu Yuxin , Yang Zuozhen , Wen Shu , Liang Mengmeng , Wu Huaping , Zhong Jianmin 
Published: February 2024

AbstractBackgroundMegalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare, inherited disorder that causes epilepsy, intellectual disorders, and early onset macrocephaly. MLC1 has been identified as a main pathogenic gene.MethodsClinical data such as magnetic resonance imaging (MRI), routine blood tests, and physical examinations were collected from proband. Trio whole‐exome sequencing (WES) of the family was performed, and all variants with a minor allele frequency (<0.01) in the exon and canonical splicing sites were selected for further pathogenic evaluation. Candidate variants were validated using Sanger sequencing.ResultsHere, we report a new homozygous variant identified in two children from the same family in the MLC1 gene [NM_015166.4: c.838_843delinsATTTTA, (p.Ser280_Phe281delinsIleLeu)]. This variant is classified as variant of uncertain significance (VUS) according to the ACMG guidelines. Further experiments demonstrate that the newly identified variant causes a decrease of MLC1 protein levels when expressed in a heterologous expression system.ConclusionOur case expands on this genetic variation and provides new evidence for the clinical diagnosis of MLC1‐related MLC.
Individual traits are associated with pro‐environmental behaviour: Environmental sensitivity, nature connectedness and consideration for future consequences
Authors: Dunne Helen , Lionetti Francesca , Pluess Michael , Setti Annalisa 
Published: February 2024

Abstract

Despite growing concern about climate change, there remains a significant gap between individuals' environmental concern and their actual behaviour. Humans' personal relationship with nature is a key contributor to pro‐environmental behaviour (PEB), which may account for this gap. Those individuals with high levels of sensory processing sensitivity (SPS), approximately 25%–30% of the population, have a particularly strong connection with the natural world and have a ‘pause to check’ attitude that may make them more prone to consider future consequences of actions. We hypothesise that (1) a disposition to experience awe, characterizing high SPS, mediates the association between the trait and connectedness to nature (CN); (2) SPS is associated with PEB, and CN mediates the association; and (3) consideration for future consequences also mediates the association between high SPS and PEB.

807 participants completed an online survey including validated measures of SPS, CN, awe, PEB and consideration for future consequences. Correlational and mediational analyses were performed to test the hypotheses.

The study showed that the relationship between SPS and CN was mediated by awe. The relationship between SPS and PEB was independently mediated by CN and consideration for future consequences.

Policy implications. The UN Sustainable Development Goals were set to reduce inequalities (SDG 10) by considering individual differences, as well as improving climate action (SDG 13). This study shows that psychological dimensions such as sensory processing sensitivity (SPS) are linked to pro‐environmental behaviour (PEB) through future‐oriented thinking and feeling connected with nature. These results also suggest that cultivating connectedness to nature and training those with lower SPS to consider future consequences of actions may be a potential avenue to improve PEB.

Read the free Plain Language Summary for this article on the Journal blog.
Using photo editing to understand the impact of species aesthetics on support for conservation
Authors: Shaw Meghan , Dunn Matilda , Crowley Sarah , Owen Nisha , Veríssimo Diogo 
Published: February 2024

Abstract

Many threatened species suffer from a lack of conservation attention compared to others. Prioritisation of funding, research and conservation efforts seem to be driven by reasons beyond conservation need. This could be due to a ‘beauty bias’, whereby aesthetically pleasing species receive more attention.

We examined how editing an image to increase a species' aesthetic appeal may impact donation choices and public attitude towards that species. We posed two research questions; first, ‘do people make different donation choices when they see original images of a species compared to when they see images of the same species that have been edited to match aesthetic preferences?’ Using hypothetical donation experiments, we asked respondents to allocate funds to the conservation of three pictured species, one ‘aesthetically appealing’, one ‘aesthetically unappealing’, and one whose image was either edited to reflect common aesthetic preferences or left unedited. Our findings suggest that images edited to make an animal more visually appealing tend to receive higher hypothetical donation amounts than original images.

We also posed a second research question; ‘How do people of varying conservation expertise respond to original versus edited images of wildlife?’ To investigate this, we ran three focus groups with individuals unfamiliar with our test species, those familiar with two or more of our test species, and with conservation professionals, which showed mixed reactions both within and between groups. Focus group participants with less conservation expertise noted that edited images often seemed ‘cuter’ than unedited images, and were more likely to compare them to cartoon characters. Participants with more conservation expertise and species familiarity reported greater empathy towards unedited images, and noted that the edited images prompted an ‘uncanny valley’ response, highlighting the need for further scrutiny in how photo editing might be used in conservation messaging.

Our findings support the beauty bias hypothesis and highlight that decisions on conservation support should acknowledge that less aesthetically pleasing species are disadvantaged in public attention and funding. In addition, the findings highlight the role of conservation expertise in impacting viewer reactions, as well as the ethical implications of editing images of wildlife.

Read the free Plain Language Summary for this article on the Journal blog.
Optimization design of control charts: A systematic review
Authors: Haridy Salah , Maged Ahmed , Alherimi Nadin , Shamsuzzaman Mohammad , Al‐Ali Sondos 
Published: February 2024

AbstractIn today's competitive landscape, fulfilling customer expectations and achieving a competitive edge are crucial for business success. These objectives can be attained by effective monitoring in both manufacturing and service sectors to enhance quality, reduce variation, and augment productivity. The control chart, a widely used tool for this purpose, has attracted significant attention from researchers for its ability to detect anomalies and manage out‐of‐control situations. The optimization of control charts, a central focus of this review, not only enhances the detection effectiveness but also maintains the desired false alarm rate, thus ensuring efficient process control without additional cost, complexity, or operational challenges for shop floor personnel. The optimization process involves adjusting charting parameters like the sample size, sampling interval, and control limits within a hypothesis testing framework, thereby achieving optimal system performance. Numerous optimization models have been developed to enhance control chart performance. This paper introduces a classification scheme to analyze and categorize the existing research on control chart optimization. By conducting a thorough review of more than 240 articles, the study pinpoints research gaps and offers valuable insights, thereby advancing the future research in this domain.
Robust H2 filtering of phase‐type semi‐Markov jump linear systems with cluster observations
Authors: de Oliveira André M. , Costa Oswaldo L. V. 
Published: February 2024

AbstractIn this work we study the design of filters for phase‐type (PH) semi‐Markov jump linear systems, considering partial information on the system's operating mode and possible parameter uncertainties. With respect to the mode of operation, it is assumed that the state space of the semi‐Markov chain can be written as the union of disjoint sets, called clusters, and the only information available to the filter is which cluster the state of the semi‐Markov chain belongs to. A new linear matrix inequality (LMI) parameterization employing the slack variable technique is introduced for the design of switching full‐order filters according to the cluster observations so that suitable bounds on the norm of the estimation error are guaranteed for the uncertain system. If the system is restricted to be a Markov jump linear system and the Markov chain is assumed to be perfectly measured, the design conditions are shown to be also necessary leading to the optimal full‐order filter. Furthermore, the general filter can be particularized into an observer form for the case in which the system matrices are the same within each cluster and there are no parameter uncertainties except for possible uncertainties affecting the transition rates of the jumping process. The paper concludes with an illustrative example in the context of networked control systems.
Assessing venous congestion in cardiac surgery with extracorporeal circulation using point‐of‐care transesophageal ultrasonography
Authors: Longo Silvina , Pérez Illidge Luis Carlos , Cheong Issac , Del Pino Juan Pablo , Tamagnone Francisco Marcelo 
Published: February 2024

AbstractPurposeIndividualizing fluid resuscitation utilizing indicators that assess fluid tolerance could positively impact optimizing volemia while avoiding venous congestion in cardiac surgery. Our study aims to describe hemodynamic changes based on the assessment of venous congestion using transesophageal ultrasonography (TEUS), both before and after cardiopulmonary bypass (CPB) in patients with preserved right ventricle (RV) function.MethodsA retrospective study was conducted at the Hospital Privado Universitario de Córdoba, involving patients who underwent cardiac surgery with CPB. The study assessed ultrasonographic venous congestion parameters before and after CPB using TEUS. The measured parameters included the diameter of the inferior vena cava (IVC), the pattern of hepatic vein flow (HVF), and the pulsatility index of portal vein flow (PVF).ResultsA total of 43 patients who underwent cardiac surgery between January 2021 and January 2022 were enrolled. A significant difference in the IVC diameter was observed before and after initiation of CPB (p = .0001). Comparative measurements of the HVF pattern before and after CPB revealed a significant difference, indicating modifications in congestion patterns related to CPB initiation (p = .0001). Pre‐CPB, portal flow was normal in all patients, while more than 50% of patient showed altered post‐CPB PVF. A significant difference in the PVF pulsatility index related to CPB initiation was observed, suggesting increased portal venous congestion (p = .0001).ConclusionOur study demonstrates that extracorporeal circulation can induce signs of venous congestion, which are detectable by TEUS, in patients with preserved RV function preoperatively.
An EWMA sign chart for monitoring processes with fixed and variable sample sizes
Authors: Haq Abdul 
Published: February 2024

AbstractThis study addresses limitations in the nonparametric EWMA sign chart with fixed control limits (FCLs), particularly when facing time‐varying sample sizes. The FCLs‐based EWMA sign chart has a variable conditional false alarm rate (CFAR), especially at the startup of a process or after recovering from an out‐of‐control signal. To overcome these limitations, we propose a nonparametric EWMA sign chart based on dynamic probability control limits. This chart is capable of monitoring the process target with fixed, as well as time‐varying sample sizes. Monte Carlo simulations are used to estimate the CFARs, zero‐state (ZS) and steady‐state (SS) average run‐length profiles of the EWMA sign charts. It turns out that the proposed chart outperforms the existing chart, particularly in detecting shifts during the process startup, while maintaining the desired CFAR levels in both ZS and SS scenarios. A real data example is given to demonstrate the implementation of the EWMA sign charts.
Artificial intelligence for international business: Its use, challenges, and suggestions for future research and practice
Authors: Menzies Jane , Sabert Bianka , Hassan Rohail , Mensah Prince Kofi 
Published: February 2024

AbstractThe emergence of artificial intelligence (AI) has transformed global business, aiding operational efficiency and innovation. It utilizes machine learning and big data analytics, driving predictive market trends and strategic decision‐making. However, despite the rising discussion and accessibility of AI tools, understanding its impact on international business remains limited. This article explores AI's potential in international business strategies, practices, and activities. To address this aim, we reviewed 37 articles in the existing literature to critically explore AI within the context of international business. More specifically, we explored how AI can be applied to innovation approaches in international business, international market selection, entry modes, foreign exchange, international human resource management, international supply chains, managing across cultures, and more topics. AI has necessitated changes in workplace configurations and the need for organizational and employee adjustments in response to this technology. As a result of the foregoing issues on AI integration within international business, our analysis provided an exploratory discussion around its use, challenges, managerial implications, and suggested areas requiring future studies.
Optimizing indoor air quality: Investigating particulate matter exposure in household kitchens and source identification
Authors: Alhajeri Nawaf S. , Alrashidi Alanood M. , Yassin Mohamed F. , Al‐Awadi Layla 
Published: February 2024

AbstractIndoor environments pose a significant health risk due to exposure to particulate matter (PM), leading to various diseases and premature deaths, and raising public concerns about indoor air quality. Household kitchens are a major contributor to indoor air pollution, emitting particulate matter of varying sizes during cooking, which disperses throughout the house. Understanding the extent of PM exposure and identifying its sources is crucial. This study assesses PM presence in Kuwaiti household kitchens, measuring concentrations of different‐sized particles (PM 0.3, 0.5, 1.0, 2.5, 5.0, and 10 μm) under various conditions. Measurements were taken for 24 h using a particle/mass monitor device, considering different ventilation scenarios, cooking oils, and methods. Spearman's rank correlation and indoor/outdoor (I/O) ratios investigate the correlation between indoor and outdoor PM exposure. Principal component analysis (PCA) models identify specific sources contributing to PM exposure. A questionnaire survey in Kuwaiti households determines primary kitchen sources during cooking. Results show that kitchens with ventilation systems consistently reduce PM concentrations. PM emissions during cooking vary based on factors like cooking method, energy type, cooking oil, food, additives, temperature, and ventilation. The cooking stove is the primary emission source, but other household activities contribute. This study enhances our understanding of influential factors affecting cooking emissions and their concentrations, contributing valuable insights into mitigating indoor air pollution.
State and Markets: Not Whether But How
Authors: Coyle Diane 
Published: February 2024

AbstractThe public and political demand for simple answers to complex economic problems generates its own supply. Moreover, policy narratives or “missions” can play a useful role in aligning expectations and coordinating private sector actions. However, the standard historical examples of successful missions (such as the Apollo program or the smartphone) involve nuanced and contingent interaction between the state and the market. In the current context of a revival of strategic industrial policies, governments must avoid oversimplified rhetoric that obscures the need for an analytical framework assigning policy instruments to specific identified elements of the strategy. Without such a framework, responsibility and accountability for policy delivery are impossible to allocate.
Analyzing the Effectiveness of State-Guided Innovation
Authors: Yerger Rodney H. 
Published: February 2024

AbstractA crucial debate exists over the effectiveness of government-guided innovation efforts, which recently through economist Mariana Mazzucato’s arguments for an entrepreneurial state that encourages the public sector’s active role in technological change and value creation, is considerably shaping global policy. This essay addresses a key assertion by Mazzucato that the government is the boldest innovator accountable for the greatest value in society. Through use case analysis, I argue that Mazzucato’s claims of the state providing mission-oriented directionality that drives technology development do not survive the scrutiny of the Supply-Chain Fallacy, the belief that every item in a line of production or chain of events is necessary and causal. I do find occasions of public sector innovation success in the development of military technologies, particularly during times of war, which can have beneficial spillover effects. However, I show that the potentiality of such successes in a persistent peacetime environment is limited by the viability of the public sector entrepreneur.
A Case Study on DARPA: An Exemplar for Government Strategic Structuring to Foster Innovation?
Authors: Yerger Rodney H. 
Published: February 2024

AbstractAdvocates for a mission economy contend that government bureaucracy can be transformed through a strategic structuring that would improve upon the dynamic capabilities necessary to pursue and direct innovation. The Defense Advanced Research Projects Agency (DARPA) is touted as a model organization of strategic structuring for inducing public sector innovation of emerging technologies. Applying economic theory and employing empirical analysis, I objectively examine key factors that are attributed to DARPA’s success, such as the organization’s autonomy, small size, and limited tenure of its program managers, in order to assess the worthiness of the agency’s exemplar status of empowering a mission-oriented approach to innovation. I find that while DARPA undoubtedly provides value for national defense and has distinct advantages over other government organizations, it falls short in representing a sustainable and scalable source of strategic structuring that would befit the entrepreneurial state.
Imaging the Patient with Epilepsy or Seizures
Authors: Bargalló Núria , Krings Timo 
Published: February 2024

AbstractNeuroimaging plays an ever-increasing role in the workup of patients presenting with seizures, epilepsy, and, in particular in patients with medically refractory epilepsy. Abnormalities that may be amenable to surgery can be present in the latter group in up to 80% and thus the radiologist plays an important role in the interdisciplinary management of this patient population. In the current article, we are describing imaging protocols as well as typical pathologies and their imaging correlated to raise awareness of the spectrum of disorders typically encountered.
Demyelinating Diseases of the CNS (Brain and Spine)
Authors: Barkhof Frederik , Koeller Kelly K. 
Published: February 2024

AbstractMultiple sclerosis (MS) is the most important inflammatory demyelinating disorder that affects both the brain and spine. Dissemination in space and time on MRI is not limited to MS and can occur in neuromyelitis optica spectrum disorder (NMOSD) with aquaporin 4 antibodies, myelin oligodendrocyte glycoprotein-related antibody disease (MOGAD), and a series of other (inflammatory) demyelinating disorders. Spinal cord imaging is an important element of MS (differential) diagnosis and especially relevant in case of possible age-related vasculo-ischemic brain white matter lesions; a negative scan will help to rule out MS. Increasingly, MRI is used to monitor treatment and their complications such as progressive multifocal leukoencephalopathy (PML).
Evaluation of Myelopathy and Radiculopathy
Authors: Shah Lubdha M. , Ross Jeffrey S. 
Published: February 2024

AbstractMyelopathy and radiculopathy can be due to extrinsic causes, most often degenerative in origin. Imaging can elucidate an often-confusing clinical picture to guide management and provide prognostic information. For intrinsic causes of myelopathy, the differential diagnosis categories include demyelination, inflammation, infection, vascular, and neoplasm. The combination of clinical symptoms, timing of presentation, and imaging features can help narrow the differential diagnosis.
Spinal Trauma and Spinal Cord Injury (SCI)
Authors: van den Hauwe Luc , Flanders Adam E. 
Published: February 2024

AbstractThe imaging methods for evaluating patients with acute spinal trauma has dramatically changed in the last decade especially with the development of thin section multi-detector computed tomography (MDCT) and isotropic datasets that provide high-resolution sagittal and coronal reformats. MDCT allows for a comprehensive assessment of spinal column injury that has largely supplanted radiography except in the pediatric population. MRI has become the procedure of choice for evaluation of the spinal cord and surrounding soft tissues when there is a suspected SCI.
Intracranial Infection and Inflammation
Authors: Lim Tchoyoson , Thurnher Majda M. 
Published: February 2024

AbstractAlthough uncommon compared to traumatic and cerebrovascular disease, radiologists should recognize the typical imaging features of meningitis, abscess, and encephalitis; and be aware of autoimmune mimics. DWI, SWI, and vessel wall imaging are useful advanced MRI techniques for problem-solving.
Toxic and Metabolic Disorders
Authors: Van Cauter Sofie , Essig Marco 
Published: February 2024

AbstractMetabolic diseases are mostly congenital inborn errors leading to functional defects in metabolic pathways, whereas toxic and metabolic diseases in adults are usually acquired. MRI is the cornerstone in the assessment of these patients. The final diagnosis is often established in combination with laboratory findings and/or genetic analysis. Imaging patterns are almost invariably bilateral and often symmetric or nearly symmetric. The basal ganglia and thalami are often involved in acquired metabolic and toxic diseases. This chapter focuses on the most common inborn errors of metabolism that can present or persist into adulthood, as well as on the most common acquired metabolic and toxic disorders, relevant to daily clinical practice.