To achieve consensus on the definition and clinical approach of Monogenic Inflammatory Immune Dysregulation Disorders (MIIDDs), a collective term for rare conditions marked by inflammation, immune dysregulation, and infection susceptibility. These consensus guidelines specifically apply to pathogenic (or likely pathogenic) gene mutations affecting both innate and adaptive immunity, excluding variants of unknown significance (VUS). A multi-step, evidence-based, multidisciplinary consensus process was employed, consisting of: (1) a systematic literature review across four electronic databases (Cochrane Library, Web of Science, Scopus, and MEDLINE via PubMed), updated through December 31, 2024; (2) a pre-Delphi electronic survey completed by 95 international adult and pediatric immunologists and rheumatologists; and (3) a modified online Delphi process with an international multidisciplinary expert panel, where statements were iteratively analyzed and refined until achieving consensus (≥ 80% agreement among panelists). Fifteen experts from 12 countries participated in two rounds of the Delphi process, resulting in the development of eight overarching principles and 10 consensus statements. These were categorized into five domains: (1) definitions and conceptual framework, (2) diagnostic and monitoring considerations, (3) treatment and therapeutic strategies, (4) multidisciplinary and collaborative care, and (5) patient education and support. This consensus defines MIIDDs and provides a structured clinical framework to streamline research efforts and improve patient outcomes.

In this article, we review the extensive and complex fabric of literature concerning the ontogenesis of spatial representations from earliest childhood to the elderly, including normal and abnormal aging (dementia and Alzheimer’s disease). We also revisit fundamental concepts of the neuronal representations of space, egocentric vs. allocentric reference frames, and path integration. We highlight a thread of contradictions in spatial cognition from infant cognition to the first breakthrough at around the age of four. The contradictions reemerge in the literature on age-related decline in spatial cognition. We argue that these contradictions derive from the incorrect assumption that path integration is exclusively associated with allocentric frames of references, hence, signatures of path integration are often taken as evidence for allocentric perspective-taking. We posit that several contradictions in the literature can be resolved by acknowledging that path integration is agnostic to the type of reference frame and can be implemented in both egocentric and allocentric frames of reference. By freeing the frames of reference from path integration, we arrive at a developmental trajectory consistent across cognitive development studies, enabling us to ask questions that may dissolve the obscurity of this topic. The new model also sheds light on the very early stage of spatial cognition.

In this study, polyphenolic compounds from pomegranate peel (Punica granatum) were extracted using different extraction methods. Three techniques were applied for polyphenol extraction: Soxhlet extraction, ultrasound-assisted extraction, and maceration. These methods varied in the time required for extraction and the yield of dry extract. For Soxhlet and ultrasound-assisted extraction, two solvents were used: methanol and ethanol. While maceration is simple and cost-effective, it was found to be the least efficient method for extraction. The removal of ethanol and methanol from the extracts was successfully achieved through evaporation, ensuring the purity of the extracts.The results obtained showed that Soxhlet extraction with methanol gave the highest yield of 33.5% compared to the ethanol solvent with 30.45%.Ultrasound-assisted extraction also yielded significant results, but the difference in yield was more pronounced depending on the solvent used. The goal of this study was to determine and present the efficiency of each extraction method. Further research will focus on assessing the antioxidant capacity of the extracted polyphenolic compounds.

This article provides an overview of Direct Energy Deposition – Arc technology (DED-Arc), also known as Wire Arc Additive Manufacturing (WAAM), which involves the deposition of metal wire using an arc power source and a CNC or robotic manipulator. The high deposition rate of WAAM justifies its use for the manufacturing of small to large-size components with lower resolution and less complex geometry. However, the use of wire as feedstock in the WAAM process has certain advantages and disadvantages, which are explained in detail. The WAAM specialties are in-situ alloying and the production of functionally graded materials (FGMs). Various sensors, path planning, process control, and FEM simulation from WAAM are used to reduce material and energy consumption and make the process more sustainable. Post-processing techniques are also discussed as a method of improving the quality of the final product. Finally, the prospects of the WAAM process are presented.

Objectives Generative artificial intelligence (GAI) tools can enhance the quality and efficiency of medical research, but their improper use may result in plagiarism, academic fraud and unreliable findings. Transparent reporting of GAI use is essential, yet existing guidelines from journals and institutions are inconsistent, with no standardised principles. Design and setting International online Delphi study. Participants International experts in medicine and artificial intelligence. Main outcome measures The primary outcome measure is the consensus level of the Delphi expert panel on the items of inclusion criteria for GAMER (Rreporting guideline for the use of Generative Artificial intelligence tools in MEdical Research). Results The development process included a scoping review, two Delphi rounds and virtual meetings. 51 experts from 26 countries participated in the process (44 in the Delphi survey). The final checklist comprises nine reporting items: general declaration, GAI tool specifications, prompting techniques, tool’s role in the study, declaration of new GAI model(s) developed, artificial intelligence-assisted sections in the manuscript, content verification, data privacy and impact on conclusions. Conclusion GAMER provides universal and standardised guideline for GAI use in medical research, ensuring transparency, integrity and quality.

This study presents a digital twin approach to quantifying the durability and failure risk of concrete gravity dams by integrating advanced numerical modelling with field monitoring data. Building on a previously developed finite element model for dam–reservoir interaction analysis, this research extends its application to the assessment of existing, fully operational dams by using digital twin technology. One such case study of a digital twin is given for the concrete gravity dam, Salakovac. The numerical model combines finite element formulations representing the dam as a nonisothermal saturated porous medium and the reservoir water as an acoustic fluid, ensuring realistic simulation results of their interactions. The selected finite element discrete approximations enable the detailed analysis of the dam failure mechanisms under varying extreme conditions, while simultaneously ensuring the consistent transfer of all fields (displacement, temperature, and pressure) at the dam–reservoir interface. A key aspect of this research is the calibration of the numerical model through the systematic definition of boundary conditions, external loads, and material parameters to ensure that the simulation results closely align with observed behaviour, thereby reflecting the current state of the ageing concrete dam. For the given case study of the Salakovac Dam, we illustrate the use of the digital twin to predict the failure mechanism of an ageing concrete dam for the chosen scenario of extreme loads.

The saddle-point solutions for strong-laser-field-induced high-order above-threshold ionization, the complete classification of which was recently presented in , are considered classically. In the limit of vanishing ionization potential the system of saddle-point equations simplifies, allowing a semi-analytical treatment. For a monochromatic field, the analytical nonlinear equations obtained this way allow one to determine the maximum (cutoff) photoelectron energies for the backward- and forward-scattering saddle-point solutions for all values of the multi-indices introduced by our classification scheme. These cutoffs are determined for all photoelectron momenta and it is shown how the backward-scattering solutions from one half of the momentum plane are related to the forward-scattering solutions from the other. The case of a bichromatic linearly polarized field is analyzed in detail. The results are rederived with the help of a simple graphical method, which can be used to qualitatively discuss the effect of varying the field parameters. Published by the American Physical Society 2025

Pertaining to goal orientation and achievement, agency is a fundamental aspect of human cognition and behavior. Accordingly, detecting and quantifying linguistic encoding of agency are critical for the analysis of human actions, interactions, and social dynamics. Available agency-quantifying computational tools rely on word-counting methods, which typically are insensitive to the semantic context in which the words are used and consequently prone to miscoding, for example, in case of polysemy. Additionally, some currently available tools do not take into account differences in the intensity and directionality of agency. In order to overcome these shortcomings, we present BERTAgent, a novel tool to quantify semantic agency in text. BERTAgent is a computational language model that utilizes the transformers architecture, a popular deep learning approach to natural language processing. BERTAgent was fine-tuned using textual data that were evaluated by human coders with respect to the level of conveyed agency. In four validation studies, BERTAgent exhibits improved convergent and discriminant validity compared to previous solutions. Additionally, the detailed description of BERTAgent's development procedure serves as a tutorial for the advancement of similar tools, providing a blueprint for leveraging the existing lexicographical data sets in conjunction with the deep learning techniques in order to detect and quantify other psychological constructs in textual data. (PsycInfo Database Record (c) 2025 APA, all rights reserved).

In competitive organizations and projects, assessing risks related to human capital is essential for improving workplace conditions and ensuring project success. This study evaluates primary, secondary, and residual human capital risks in urban water transfer projects using an innovative hybrid DEMATEL–MARCOS approach. The DEMATEL method was employed to analyze causal relationships and interdependencies among risks, while the MARCOS method ranked their significance. The key findings reveal that “accidents during material transportation” (primary risk), “corrosion” (secondary risk), and “pipeline pressure” (residual risk) are the most critical factors influencing human capital in such projects. The study provides a structured framework for prioritizing risk mitigation strategies, offering actionable insights for policymakers and project managers to enhance safety, efficiency, and workforce well-being. By integrating multi-criteria decision-making techniques, this research bridges a gap in the water industry’s risk management practices and contributes to safer, more sustainable infrastructure development.

Introduction The aim of the TALENT project is to promote equality in education, prevent exclusion, support dual careers (sport and school), create new role models for the benefit of young talents and prepare them for lifelong learning and professional sport from an early age. It is promoted by a European consortium of 7 partner institutions and runs from December 2022 to May 2025. It consists of five work packages. In the first work phase, developing the WP2 (from December 2022 to October 2023), under the coordination of UNIPA, NIS University, KMOP and EAS standards for talent recognition were identified and validated. Methods Initially, 12 focus groups were conducted with teachers (77 teachers) and coaches (73 coaches) on creating talent identification standards; subsequently, workshops were held with dual career experts to validate these standards. This was a key piece of work that enabled the establishment of clear guidelines and protocols to identify and support talented young people in their dual careers. Results A final list of 41 shared statements was identified: 20 related to teachers and 21 related to coaches. For example, teachers emphasized the need for multidisciplinary approaches and early identification of talent, while coaches underlined the importance of psychological readiness and collaboration with schools and families. Discussion These statements not only provide structured reference points for talent identification but also highlight actionable needs across educational and sport systems. As such, they represent a solid foundation for developing standard operating procedures in talent recognition and dual career support.

A practical method is presented for determining root and pole cancellation functions of a matrix function $Q(z)$ meromorphic on the extended complex plane $\bar{\mathbb{C}}:=\mathbb{C} \cup \left\{ \infty \right\}$. This method is applied to solve a nonlinear system of $n\in \mathbb{N}$ differential equations of order $l\in \mathbb{N}$ with $n $ unknown functions $u_{i}\left( t \right)$, where $i=1,\, \mathellipsis ,\,n $. For a function $Q\in \mathcal{N}_{\kappa}(\mathcal{H}) ,\, \kappa \in \mathbb{N} \cup \lbrace 0 \rbrace$, posesing a pole at infinity of order $m \in \mathbb{N}$, the following factorization is establish \[ Q(z)=(z-\beta)^{m}\tilde{Q}(z), \, z\in \mathcal{D}(Q), \] where $\beta \in \mathbb{R}$ is a regular point of $Q$, and $\tilde{Q}\in \mathcal{N}_{\kappa'}(\mathcal{H})$ is holomotphic at $\infty$. Unlike the Krein-Langer representation of $Q$, which involves a linear relation $A$, this representation employs a bounded operator $\tilde{A}$ in the Krein-Langer representation of $\tilde{Q}$. The operator $\tilde{A}$ and the relation $A$ have identical spectra, except at $\beta$ and $\infty$. We demonstrate how to obtain this representation for a given meromorphic function $Q\in \mathcal{N}_{\kappa}^{n \times n}$ using the root functions developed in this work.

OBJECTIVES Brain tumor-related epilepsy management poses significant challenge in clinical practice. Healthcare providers must tailor treatment based on each patient's unique circumstances. Different antiepileptic drugs can be used, including oxcarbazepine. Several studies show this drug's efficacy and safety in brain tumor-related epilepsy. METHODS Observational, prospective study, monitoring the efficacy and safety of the drug oxcarbazepine in the prevention of epileptic seizures, included adult patients of both sexes with a supratentorial tumor and a risk of epileptic seizures after neurosurgery. RESULTS The study included 153 hospitalized patients. The percentages of amplified waves, sharp waves, and spike waves decreased in the second and third compared with the first visit. Significantly lower percentages of sharp waves (P = 0.028) on the second compared with the first measurement and spike waves (P = 0.002) on the third compared with the first measurement were determined. Deterioration from normal to low hemoglobin concentration was observed in 40 (26%) patients at the second visit and 17 (12%) at the third visit, compared with the first visit. However, mean corpuscular volume, mean corpuscular hemoglobin, and mean corpuscular hemoglobin concentration values did not change significantly during the 6 months of follow-up. A transient drop in the number of thrombocytes was observed on the second visit. Adverse reactions to the drug were mild. Therapeutic adherence was low, as measured by the Morisky Medication Adherence Scale (MMAS-4). CONCLUSIONS The drug oxcarbazepine has shown good efficacy and safety in the prevention of epileptic attacks after neurosurgery in patients with supratentorial tumors. Additional education of patients on the importance of taking regular therapy is crucial.