UK European Research Articles

Autosomal recessive VWA1-related disorder: comprehensive analysis of phenotypic variability and genetic mutations.

A newly identified subtype of hereditary axonal motor neuropathy, characterized by early proximal limb involvement, has been discovered in a cohort of 34 individuals with biallelic variants in von Willebrand factor A domain-containing 1 (VWA1). This study further delineates the disease characteristics in a cohort of 20 individuals diagnosed through genome or exome sequencing, incorporating neurophysiological, laboratory and imaging data, along with data from previously reported cases across three different studies. Newly reported clinical features include hypermobility/hyperlaxity, axial weakness, dysmorphic signs, asymmetric presentation, dystonic features and, notably, upper motor neuron signs. Foot drop, foot deformities and distal leg weakness followed by early proximal leg weakness are confirmed to be initial manifestations. Additionally, this study identified 11 novel VWA1 variants, reaffirming the 10 bp insertion-induced p.Gly25ArgfsTer74 as the most prevalent disease-causing allele, with a carrier frequency of ∼1 in 441 in the UK and Western European population. Importantly, VWA1-related pathology may mimic various neuromuscular conditions, advocating for its inclusion in diverse gene panels spanning hereditary neuropathies to muscular dystrophies. The study highlights the potential of lower quality control filters in exome analysis to enhance diagnostic yield of VWA1 disease that may account for up to 1% of unexplained hereditary neuropathies.

Exploring breed differences in discrimination, reversal learning, and resistance to extinction in the domestic dog (Canis familiaris)

Learning is crucial for shaping domestic dogs’ behaviour through life experiences, yet not all breeds exhibit the same learning aptitude towards a particular task. The current study’s objective was to identify differences in behaviour and learning performance across and within five breed clades and elucidate the underlying factors contributing into these variations. Dogs (n = 111) from five breed clades (UK Rural, Retrievers, Asian Spitz, European Mastiff, and New World) participated in a virtual learning task with their owners. Owners completed validated questionnaires of Impulsivity and Reward Responsiveness. The learning task comprised of reinforcing an arbitrary behaviour (hand-touch) through multiple sessions of Acquisition (reinforcing the hand-touch), Discrimination (reinforcing the hand-touch on one of two hands) and Reversal Learning (reinforcing the hand-touch on the opposite hand), followed by a single session of Extinction (hand-touch not reinforced). Results showed notable differences across the studied breed clades in certain learning and behavioural components. However, the observed disparities may not be entirely attributed to inherent cognitive differences among the breed clades but rather potentially influenced by contextual factors such as the human-dog communication dynamics associated with breeds’ cooperativity. Furthermore, breed clades differed in the contributing factors predicting individual learning performances, which could highlight the potential effect of breeds’ historical function.

Does Local Area Social Mobility Affect Political Alienation?

While existing research has considered how individual-level social mobility experiences affect a person’s political outlook, less attention has been paid to how historic levels of social mobility in local areas influence political attitudes and political behaviour. We link individual-level data from the UK Household Longitudinal Study to small area estimates of social class mobility derived from the decennial census. We find that living in a low absolute social mobility area was associated with a higher probability of voting ‘Leave’ in the 2016 UK European Union membership referendum. However, we find no evidence that historical social mobility rates in the local area predict abstention in general elections or attitudinal indicators of political alienation. Given declining rates of upwards mobility, and increasing levels of downwards mobility, our results have important implications for understanding geographies of political discontent.

Short communication: Five ways UK European Capitals and cities of culture have connected cultural activities with nature and their impacts on health and wellbeing, wider determinants of health and inequality

ObjectiveTo rapidly synthesise evidence for local practice on what initiatives UK European Capitals and Cities of Culture (UKEUCoCs) have implemented connecting cultural activities with green, blue, or outdoor space (culture-nature initiatives) and their impacts on planetary health outcomes: personal health and wellbeing, wider determinants of health particularly the environment, and existing inequality. Study designRapid evidence review. MethodsA rapid review of published articles and evaluation reports. Published articles were identified through database searches (Proquest, OVID, Scopus, Web of Science, MEDLINE) in January–February 2024. Data was extracted directly into a table and findings synthesised narratively by theme. ResultsPublished evidence about UKEUCoC culture-nature initiatives was limited but five initiative types were identified: 1) growing-focused activities; 2) activities exploring human-nature relationships; 3) targeted nature-based wellbeing activities; 4) activities connecting cultural engagement with environmental activism; and 5) use of outdoor spaces for artworks, performances and festivals. UKEUCoC culture-nature initiatives may contribute to short-term improvements in mental health and wellbeing (confidence, self-esteem, subjective wellbeing), community health (community relations, civic pride), cultural participation, and local environmental quality and use, but risk widening existing inequalities. Co-creating initiatives at hyper-local levels with marginalised groups and trusted Community Champions, active involvement, and creating equitable access to livelihood opportunities may mitigate inequality risks. ConclusionsEvidence is limited but suggests UKEUCoC culture-nature initiatives could positively support planetary health outcomes in the short-term. Equity in these outcomes appears to rely however, on action to ensure the involvement of and sustainable livelihood creation for marginalised groups. It is unclear how outcomes are generated across the initiative types identified, including through interactions between them, where they are implemented. The five initiative types identified in this work could be targeted for further investigation in research and practice on culture-nature initiatives for health more generally, using a complex systems approach to evaluation.

Christine and the Queens at the BBC: hybridity, relation, tilting

This article reads 2016 as a breakthrough year for Christine and the Queens in the United Kingdom, particularly England. Through analyzing a select corpus of interviews and performances, I argue that the artist’s personal breakthroughs occur in a context of changing UK–European relations, and indeed of the ever-changing persona of Christine, who changes constantly and yet stays the same in crucial regards. I read such evolving identities and relations with reference to the thought of the Martinican theorist Édouard Glissant.

To stay, remain or leave: how verbal concepts as response options in political referendums such as the Brexit polls might bias voting outcomes

Referendums allow people to participate in political decision-making. However, they also come with the challenge of presenting complex issues to the electorate in a concise and comprehensible way. In order to simplify the decision-making process, referendum questions are often tailored to yes/no response options. In comparison, the UK European Union membership referendum of 2016, in contrast to the 1975 referendum on the same issue, discarded this option, and a verbal and conceptually more complex alternative response option was brought into play (remain vs. leave). This potentially relevant change in the voting tradition justifies a linguistic consideration of the suitability of the choice of verbs. Verbal response options such as stay, remain and leave might activate different framing effects due to their underlying etymology. Our goal was therefore to examine whether the choice of verbs can have a biasing effect, which might be the case due to their frame-inherent structure. This investigation attempts, through both a linguistic analysis and an experimental analysis using a version of the Implicit Association Test (IAT), to bridge the research gap between the awareness that there are linguistic factors that can influence decision-making processes and the lack of inclusion of framing effects. Overall, the data of two IAT studies (n = 185 and n = 355) suggest that the exact wording of dichotomic response options has the potential to influence response choice based on evaluative associations of the verbs. Specifically, when compared to leave, we found relatively more positive evaluation for stay than for remain. Furthermore - independent of the Brexit referendum - our study raises the question whether verbs are at all suitable to replace yes/no response options due to inherent framing effects. This linguistic aspect requires more attention in the design of response options in future referendums.

Was the extreme rainfall that caused the August 2022 flood in Pakistan predictable?

Pakistan suffered from severe floods in the past, but in August 2022, the country experienced exceptional extreme rainfall events that caused widespread and catastrophic flooding. The 2022 flood affected all aspects of socio-economic lives including agriculture, infrastructure, and mortality of humans and livestock. The two-day accumulated extreme rainfall on 17–18 August was anomalous and contributed the most to the flood in the southern provinces of Pakistan. The damage caused by extreme rainfall and the subsequent flooding has raised questions regarding the predictability of extreme rainfall by the existing weather forecasting models. Here, we use ensemble forecasts from four numerical weather prediction models under THORPEX Interactive Grand Global Ensemble datasets to examine the predictability of extreme rainfall at a six-day lead. The extreme precipitation during 17–18 August 2022 was predictable a week before the event that contributed the most to the flooding. All the forecast models provided an early warning at a six-day lead time. UK Meteorological Office and European Centre for Medium-Range Weather Forecasts models produced comparable results to observations at all the lead times. Our findings highlight that an integrated framework of extended quantitative precipitation forecasts and hydrological modeling can help reduce the country’s flood vulnerability and risk associated with it.

The status of the UK breeding European Turtle Dove Streptopelia turtur population in 2021

ABSTRACT Capsule The first UK European Turtle Dove Streptopelia turtur survey, in 2021, estimated the breeding population at 2092 territories. Aims Changes in European Turtle Dove abundance in the UK have been monitored through schemes like the BTO/JNCC/RSPB Breeding Bird Survey, however, sample size is now too low to calculate robust trends going forward. The aims of this volunteer-based survey were to provide a new UK population estimate and create a baseline to monitor future population trends. Methods A stratified random sample of one-kilometre squares were surveyed within the core counties for the species. Elsewhere, one-kilometre squares containing recent European Turtle Dove records were targeted. The survey used a two-visit territory mapping approach, with observers asked to get within 200 metres of potentially suitable nesting or foraging habitat. Visits were undertaken in the early morning, to coincide with peak vocal activity, from 11 May to 31 July. Results The UK population estimate in 2021 was 2092 territories (95% confidence limits, 1559–2782). The species has become increasingly restricted to eastern and southeastern England, with 62.5% of the population estimated to occur in three counties: Kent (682 territories; 32.6%), Suffolk (326; 15.6%) and Essex (300; 14.3%). Additional hotspots occurred in other counties in eastern England, up to North Yorkshire. Conclusion This result suggests a 98% decline in abundance since the 1968–1972 breeding atlas, similar to trends identified from UK bird monitoring schemes, and a substantial contraction in range since the 2007–2011 bird atlas. The temporary cessation of hunting along their European western flyway provides a vital window of opportunity to scale up the delivery of high-quality breeding habitat and increase food availability in the UK. The survey should be repeated in 2026, and regularly thereafter, to help monitor the effectiveness of conservation interventions.

Police and criminal justice co-operation after Brexit

ABSTRACT The article will evaluate the current state of UK-EU police and criminal justice cooperation and its implications for UK criminal justice policy. The article will place current developments within the broader historical and constitutional context of the ambivalent UK participation in the EU area of criminal justice before Brexit and highlight the significance of criminal justice and police practitioners in shaping UK European policy in the field. The article will map the constitutional evolution of the UK position in the field and assess the relevance of pre-Brexit challenges to the post-Brexit era. Looking at both how UK policy has been influenced – and has in turn influenced – EU criminal justice policy, the article will evaluate the provisions of the TCA and focus on key current challenges, including the constitutional constraints underpinning UK participation in EU police and criminal justice structures after Brexit, the challenge of regulatory divergence in the field (in particular regarding data protection) and the development of mutual trust and credible benchmarks of collaboration (including compliance with European human rights norms) after Brexit.

Time for Brexit? Temporalities in the 2019 UK European election campaign

AbstractAlthough the importance of time in political discourse cannot be denied, few recent studies address the representation of time as a factor in election campaigns. This discourse analytical study focuses on the role of time in the campaign literature produced by the nine main parties in the 2019 EU election in the UK, which resulted in a landslide victory for the newly-formed Brexit Party. The corpus consisted of the manifestos or leaflets produced specifically for this election by the nine parties in question, amounting to 57,226 words. The timelines of the different parties are analysed, showing how the parties envisioned different timelines: some capitalized on public frustration by offering immediate satisfaction, while others legitimized their aspirations through timelines that reached across and beyond the uncertainties of the Brexit phase. Representations of linear time, cyclical time and radical rupture are contrasted, and the key significance of time in the populist performance of crisis is discussed.

It was the best of times, it was the worst of times - Charles Dickens.

Great North Trauma and Emergency Care Centre, Newcastle upon Tyne, UK European Society Emergency Medicine, Antwerp, Belgium Received 14 November 2022 Accepted 15 November 2022. Correspondence to Jim Connolly, MBBS, Emergency Department, Great North Trauma and Emergency Care Centre, Newcastle-upon-Tyne, UK Tel: +44 191 233 6161; e-mail: [email protected]

Severe acute hepatitis of unknown causes in children - Current findings, questions, opinions, and recommendations, a mini-review.

ABSTRACTSince October 2021 in Alabama, the United States, and March 2022 in central Scotland, the United Kingdom, the number of cases of severe acute hepatitis of unknown etiology/causes in children was found to increase, and the total number of cases has reached 920 worldwide by June 22 this year, 45 cases (5%) required liver transplantation, and 18 cases (2%) died according to World Health Organization (WHO). To understand the basic characteristics of this disease/syndrome, a literature search was performed at PubMed, websites of WHO, UK Health Security Agency, and US and European Centers for Disease Control and Prevention, and more than 20 reports were enrolled as references for this review. The main clinical manifestations are anorexia, vomiting, fatigue, jaundice, and so forth. Most of the cases seemed to have a self‐limited course of the disease, about 6% of cases may develop life‐threatening acute liver failure. The disease seems to be transmissible from person to person. Human adenovirus was detected in up to 75% of cases, but this virus seems not to be the only and major etiologic agent, other cofactors probably are involved. Researchers proposed many hypotheses concerning the etiology and pathogenesis, and many important works and studies are ongoing. This mini‐review is aimed at summarizing, reviewing, and further understanding the characteristics of the disease, raising some clinically relevant questions, and trying to discuss some questions that may be related to the treatment of the disease for consideration.

The Association between Serum Lipids and Intraocular Pressure in 2 Large United Kingdom Cohorts

Serum lipids are modifiable, routinely collected blood test features associated with cardiovascular health. We examined the association of commonly collected serum lipid measures (total cholesterol [TC], high-density lipoprotein cholesterol [HDL-C], low-density lipoprotein cholesterol [LDL-C], and triglycerides) with intraocular pressure (IOP). Cross-sectional study in the UK Biobank and European Prospective Investigation into Cancer and Nutrition (EPIC)-Norfolk cohorts. We included 94 323 participants from the UK Biobank (mean age, 57 years) and 6230 participants from the EPIC-Norfolk (mean age, 68 years) cohorts with data on TC, HDL-C, LDL-C, and triglycerides collected between 2006 and2009. Multivariate linear regression adjusting for demographic, lifestyle, anthropometric, medical, and ophthalmic covariables was used to examine the associations of serum lipids with corneal-compensated IOP (IOPcc). Corneal-compensated IOP. Higher levels of TC, HDL-C, and LDL-C were associated independently with higher IOPcc in both cohorts after adjustment for key demographic, medical, and lifestyle factors. For each 1-standard deviation increase in TC, HDL-C, and LDL-C, IOPcc was higher by 0.09 mmHg (95% confidence interval [CI], 0.06-0.11 mmHg; P < 0.001), 0.11 mmHg (95% CI, 0.08-0.13 mmHg; P < 0.001), and 0.07 mmHg (95% CI, 0.05-0.09 mmHg; P < 0.001), respectively, in the UK Biobank cohort. In the EPIC-Norfolk cohort, each 1-standard deviation increase in TC, HDL-C, and LDL-C was associated with a higher IOPcc by 0.19 mmHg (95% CI, 0.07-0.31 mmHg; P= 0.001), 0.14 mmHg (95% CI, 0.03-0.25 mmHg; P= 0.016), and 0.17 mmHg (95% CI, 0.06-0.29 mmHg; P= 0.003). An inverse association between triglyceride levels and IOP in the UK Biobank (-0.05 mmHg; 95% CI, -0.08 to -0.03; P<0.001) was not replicated in the EPIC-Norfolk cohort (P= 0.30). Our findings suggest that serum TC, HDL-C, and LDL-C are associated positively with IOP in 2 United Kingdom cohorts and that triglyceride levels may be associated negatively. Future research is required to assess whether these associations are causal in nature.

The Characteristics of the University Admission System and Their Infl uence on the Accessibility of Higher Education

The study provides a review of the admission to universities models in different countries and also a comparative analysis of the educational systems characteristics from an international perspective. The aim of the research is to assess the possible impact of the educational system characteristics on the accessibility of higher education. There is also proposed an analytical model which explains how the level of autonomy and selectivity of the educational system, along with the characteristics of the student financial support influence the level of accessibility of higher education. The framework of the model is supported by the cases of selected groups of countries: post-Soviet countries, the USA and the UK, Scandinavian countries and Western European countries. Based on the classification of higher education systems, countries with the highest level of access to higher education tend to have low levels of autonomy and selectivity, or they also can be characterized by highly developed system of financial support. As a result, it was found that high selectivity and autonomy level can negatively influence the accessibility of higher education, while high level of the financial support system may neutralize this effect and positively influence on the access to higher education. Results of the study can be useful while reforming the selection system in Russia, since comparative analysis shows which tools make it possible to increase the accessibility of higher education and, as a result, to reduce educational inequality among different social groups.

Abdul, G., A., &amp; Sehar, S. (2015). Conflict management and organizational performance: A case study of Askari Bank Ltd. Research Journal of Finance and Accounting. 6(11), 201. Adhiambo, R., &amp; Simatwa, M. (2011). Assessment of conflict management and resolution in public secondary schools in Kenya: A case study of Nyakach District. International Research Journal 2(4), 1074-1088. Adomi, E., &amp; Anie,

The Constitution of Kenya specifically recognizes the freedom of association to form and belong to trade unions. However, despite the adoption of the Labour Relations Act, union practice is still hampered by excessive restrictions. The EPZ companies are labor intensive requiring a large amount of labor to produce its goods or service and thus, the welfare of the employees play a key role in their functions. This study sought to determine the effect of trade union practices on employees’ welfare at export processing zones industries in Athi River, Kenya. The specific objectives sought to determine the effect of collective bargaining agreements, industrial action, dispute resolution and trade union representation on employees’ welfare at export processing zones industries in Athi River, Kenya. The study employed a descriptive research design. Primary data was collected by means of a structured questionnaire. The target population of the study was employees in EPZ companies in Athi River, Kenya with large employees enrolled in active trade unions. The unit of observation was the employees in the trade unions. The findings indicated that collective bargaining agreements had a positive and significant coefficient with employees’ welfare at the EPZ industries. Industrial action had a positive but non-significant effect with employees’ welfare at Export Processing Zones industries. Dispute resolution had a positive and significant coefficient with employees’ welfare at the EPZ industries. Trade union representation had a positive and significant coefficient with employees’ welfare at the EPZ industries. The study recommended that trade union should avoid the path of confrontation but continue dialogue through the collective bargaining process and demands should be realistic in nature with what is obtainable in the related industry. An existence of a formal two way communication between management and trade unions will ensure that right message is properly understood and on time too. Keywords: Collective Bargaining Agreements, Industrial Action, Dispute Resolution, Trade Union Representation, Employees Welfare &amp; Export Processing Zones

Organisational forms of science communication: the UK and Spanish European higher education systems as paradigms

As key elements in research and development systems, higher education institutions have been taking a leading role when it comes to communicating science and technology, but their performance has been inconsistent so far. In this critical and comparative study of the UK public engagement model and the Spanish scientific culture model, eighteen practitioners from higher education institutions across both regions were interviewed. A mixed qualitative data analysis has been performed identifying similarities and differences that unravelled the science communication management model in the two different higher education systems. This article provides evidence on how the institutionalisation of science communication is strongly influenced by key driving forces in the higher education context as well as the policies of administrations and other agents.

Visual Impairment and Risk of Dementia in 2 Population-Based Prospective Cohorts: UK Biobank and EPIC-Norfolk.

Visual impairment has emerged as a potential modifiable risk factor for dementia. However, there is a lack of large studies with objective measures of vision and with more than 10 years of follow-up. We investigated whether visual impairment is associated with an increased risk of incident dementia in UK Biobank and European Prospective Investigation into Cancer in Norfolk (EPIC-Norfolk). In both cohorts, visual acuity was measured using a “logarithm of the minimum angle of resolution” (LogMAR) chart and categorized as no (≤0.30 LogMAR), mild (>0.3 to ≤0.50 LogMAR), and moderate to severe (>0.50 LogMAR) impairment. Dementia was ascertained through linkage to electronic medical records. After restricting to those aged ≥60 years, without prevalent dementia and with eye measures available, the analytic samples consisted of 62 206 UK Biobank and 7 337 EPIC-Norfolk participants, respectively. In UK Biobank and EPIC-Norfolk, respectively, 1 113 and 517 participants developed dementia over 11 and 15 years of follow-up. Using multivariable Cox proportional-hazards models, the hazard ratios for mild and moderate to severe visual impairment were 1.26 (95% confidence interval [CI]: 0.92–1.72) and 2.16 (95% CI: 1.37–3.40), in UK Biobank, and 1.05 (95% CI: 0.72–1.53) and 1.93 (95% CI: 1.05–3.56) in EPIC-Norfolk, compared to no visual impairment. When excluding participants censored within 5 years of follow-up or with prevalent poor or fair self-reported health, the direction of the associations remained similar for moderate impairment but was not statistically significant. Our findings suggest visual impairment might be a promising target for dementia prevention; however, the possibility of reverse causation cannot be excluded.

Standing the Test of Time: The Level Playing Field and Rebalancing Mechanism in the UK–EU Trade and Cooperation Agreement (TCA)

ABSTRACT This article explores the dispute settlement elements of the UK–European Union Trade and Cooperation Agreement’s (TCA) novel Level Playing Field (LPF) rebalancing obligations, consisting of a requirement not to lower standards in areas of labour, the environment and subsidy policy such that an adverse material impact results to trade or investment flows between the parties. The test for violations of these provisions is narrowly framed, requiring strong evidence of significant harms and carefully calibrated retaliation. The system contemplates several avenues of dispute settlement including unilateral ex ante retaliation which may be levied against another sector. The article suggests that the substantive and procedural features of the TCA’s LPF mechanism affords reasonably wide opportunity for parties to practice regulatory divergence while evincing a commitment to shared values.

Bahrain's transnational Arab Spring: repression, oil and human rights activism

Abstract Studies of the Middle East following the Arab Spring have concluded that ‘repression works’, especially in the oil- and gas-rich countries of the Gulf. Drawing on primary materials collected during fieldwork trips to Bahrain, the United States and Britain, this article nuances the ‘repression effect’ by tracing the emergence of a transnational Bahraini opposition, mapping the relationships and joint activities between domestic and exiled Bahraini groups, international NGOs and western policy-makers. It finds that even in the context of domestic repression and continuing ideological divides within Bahrain's opposition, transnational networks have not only sustained opposition organizations, but also maintained access to foreign policy-makers, producing repeated criticisms of the Bahraini regime and legal challenges to ruling elites who visit western states. The successes of this advocacy are modest: while it has not drastically reshaped the domestic state–society relationship, it has created significant costs for the Bahraini regime and damaged Bahrain's international brand. As a result, state and opposition networks now compete for influence over western policy-makers: international ‘arenas of advocacy’—such as the UK parliament, US Congress, UNHRC and European Parliament—have now become ‘arenas of contestation’, as state and opposition narratives of Bahraini politics, filtered through western policy-makers, play out during debates over foreign policy towards Bahrain. The article positions transnational activism as a direct outcome of the ‘repression effect’, highlighting ongoing contests for influence between state and society occurring in international and transnational spaces, even as the domestic scope for opposition mobilization remains highly restricted.

Multi-objective optimization of CCUS supply chains for European countries with higher carbon dioxide emissions

Abstract This research work wants to overcome the gap present in the literature, reformulating our single optimization problems of a CCUS supply chain for Germany, Italy and the UK (European countries with higher carbon dioxide emissions) as bi-objective problems. The amount of captured carbon dioxide is maximized while total costs are minimized at the same time. Results show that, for solving this problem, the augmented ε-constraint method is more efficient than the traditional ε-constraint method, and the respective Pareto fronts with environmentally and economically efficient solutions are obtained. These plots are utilized to suggest scenarios for a decision maker, considering only the total costs objective function (the scenario with the minimum value of net total cost is selected) or both objective functions (the scenario with the shortest distance from the Utopia point is chosen). In the second option, the CCUS supply chain of Germany is that closest to the ideal conditions, even if the system has the highest costs.