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Pediatrics Jul 2024Maple syrup urine disease (MSUD), a life-threatening metabolic disorder, is included in newborn screening (NBS) programs worldwide. The study aims to evaluate the impact...
OBJECTIVE
Maple syrup urine disease (MSUD), a life-threatening metabolic disorder, is included in newborn screening (NBS) programs worldwide. The study aims to evaluate the impact of NBS on the long-term outcome of MSUD patients.
METHODS
We performed a prospective, national, multicenter, observational study.
RESULTS
In the studied NBS cohort (N = 33; 22 classic MSUD [cMSUD], 11 variant MSUD [vMSUD]; median age at last visit 10.4 years), 32 (97%) patients survived, 58% of them had normal cognitive functions (median IQ 87). Initial peak leucine increased linearly with age in cMSUD (median: 1712 µmol/L), but not in vMSUD. Global IQ correlated inversely with the initial peak leucine concentration (P = .04; β = -0.0081) and the frequency of decompensations (P = .02; β = -9.133). A cluster analysis identified 2 subgroups differing in their long-term metabolic control (median leucine concentration: 162 vs 278 µmol/L; P < .001). In cMSUD, lower leucine concentrations were associated with a higher IQ (95.5 vs 80; P = .008). Liver transplantation (median age 5.8 years) was not associated with better cognitive outcome. NBS is highly sensitive for cMSUD, but vMSUD might be missed (N = 2 missed by NBS).
CONCLUSIONS
NBS and the early start of treatment improve survival and long-term outcome in individuals with cMSUD. Disease severity is an important modifier of outcome; however, the time to NBS report and the quality of long-term metabolic control had an independent impact on cognitive outcome, highlighting the importance of an early diagnosis and the quality of treatment.
PubMed: 38957900
DOI: 10.1542/peds.2023-064370 -
Physical Chemistry Chemical Physics :... Jul 2024Uranium is considered as a very important nuclear energy material because of the huge amount of energy it releases. As the main product of the spontaneous decay of...
Uranium is considered as a very important nuclear energy material because of the huge amount of energy it releases. As the main product of the spontaneous decay of uranium, it is difficult for helium to react with uranium because of its chemical inertness. Therefore, bubbles will be formed inside uranium, which could greatly reduce the performance of uranium or cause safety problems. Additionally, nuclear materials are usually operated in an environment of high-temperature and high-pressure, so it is necessary to figure out the exact state of helium inside uranium under extreme conditions. Here, we explored the structural stability of the U-He system under high pressure and high temperature by using density functional theory calculations. Two metastable phases are found between 50 and 400 GPa: UHe with space group and UHe with space group 1̄. Both are metallic and adopt layered structures. Electron localization function calculation combined with charge density difference analysis indicates that there are covalent bonds between U and U atoms in both -UHe and 1̄-UHe. Regarding the elastic modulus of α-U, the addition of helium has certain influence on the mechanical properties of uranium. Besides, first-principles molecular dynamics simulations were carried out to study the dynamical behavior of -UHe and 1̄-UHe at high-temperature. It was found that -UHe and 1̄-UHe undergo one-dimensional superionic phase transitions at 150 GPa. Our study revealed the exotic structure of U-He compounds beyond the formation of bubbles under high-pressure and high-temperature, which might be relevant to the performance and safety issues of nuclear materials under extreme conditions.
PubMed: 38957898
DOI: 10.1039/d4cp02037e -
Reports on Progress in Physics.... Jul 2024A test of lepton flavor universality inB±→K±μ+μ-andB±→K±e+e-decays, as well as a measurement of differential and integrated branching fractions of a...
A test of lepton flavor universality inB±→K±μ+μ-andB±→K±e+e-decays, as well as a measurement of differential and integrated branching fractions of a nonresonantB±→K±μ+μ-decay are presented. The analysis is made possible by a dedicated data set of proton-proton collisions ats=13TeVrecorded in 2018, by the CMS experiment at the LHC, using a special high-rate data stream designed for collecting about 10 billion unbiased b hadron decays. The ratio of the branching fractionsB(B±→K±μ+μ-)toB(B±→K±e+e-)is determined from the measured double ratioR(K)of these decays to the respective branching fractions of theB±→J/ψK±withJ/ψ→μ+μ-ande+e-decays, which allow for significant cancellation of systematic uncertainties. The ratioR(K)is measured in the range1.1
PubMed: 38957892
DOI: 10.1088/1361-6633/ad4e65 -
Epigenomics Jul 2024We investigate the genome-wide DNA methylation (DNAm) patterns of term low birth weight (TLBW) neonates. In the discovery phase, we assayed 32 samples...
We investigate the genome-wide DNA methylation (DNAm) patterns of term low birth weight (TLBW) neonates. In the discovery phase, we assayed 32 samples (TLBW/control:16/16) using the EPIC 850k BeadChip Array. Targeted pyrosequencing of in 60 samples (TLBW/control:28/32) using targeted pyrosequencing during the replication phase. The 850K array identified TLBW-associated 144 differentially methylated positions (DMPs) and 149 DMRs. Nearly 77% DMPs exhibited hypomethylation, located in the opensea and gene body regions. The most significantly enriched pathway in KEGG is sphingolipid metabolism (hsa00600), and the genes and related to this pathway both show hypomethylation. Our analysis provides evidence of genome-wide DNAm alterations in TLBW. Further investigations are needed to elucidate the functional significance of these DNAm changes.
PubMed: 38957889
DOI: 10.1080/17501911.2024.2358744 -
Frontiers in Psychology 2024Using convenience sampling and snowball sampling methods, data from 432 Chinese international students in 10 countries, including the United Kingdom, South Korea, and...
Using convenience sampling and snowball sampling methods, data from 432 Chinese international students in 10 countries, including the United Kingdom, South Korea, and the United States, were collected to construct a multivariate sequential-mediated mixed model for cross-cultural adaptation. SPSS 23.0 and AMOS 23.0 were employed for aggregated validity, discriminant validity, and sequential-mediated effects analysis. The study found that: Cultural adaptation stress is negatively correlated with positive coping and positively correlated with negative coping, with negative coping having a significant negative impact during the cross-cultural adaptation process. Positive coping is positively correlated with sports participation, while negative coping is negatively correlated with sports participation. Sports participation is positively associated with in-group identification and negatively associated with out-group bias. In-group identification has a positive impact on cross-cultural adaptation, whereas out-group bias cannot effectively predict cross-cultural adaptation.
PubMed: 38957887
DOI: 10.3389/fpsyg.2024.1386044 -
Frontiers in Psychology 2024As a topic of widespread concern in the fields of mental health and public health, social anxiety has many negative impacts on the physical and mental health of...
OBJECTIVE
As a topic of widespread concern in the fields of mental health and public health, social anxiety has many negative impacts on the physical and mental health of contemporary college students. Therefore, this study aims to provide new ideas for solving the problem of social anxiety among college students by exploring the potential mediating role of social support and psychological capital in the relationship between physical activity and social anxiety.
METHODS
A cross-sectional survey was conducted on 874 college students from five universities in Shandong Province. Various self-report tools such as physical activity rating scale, social support scale, positive psychological capital scale, and social anxiety scale were used to collect information needed for this study. Related data. Use this to conduct descriptive statistical analysis, correlation analysis, and mediation effect analysis.
RESULTS
The results showed that physical activity was significantly positively correlated with college students' social support ( = 0.354, < 0.01) and psychological capital ( = 0.448, < 0.01), and social support was significantly positively correlated with psychological capital ( = 0.556, < 0.01), the above three are significantly negatively correlated with social anxiety ( = -0.326, -0.381, -0.432, < 0.01); the mediation effect analysis shows that physical activity has a significant direct effect on college students' social anxiety The effect value is -0.136, accounting for 43.31% of the total effect; social support and psychological capital both play a separate mediating role between physical activity and social anxiety among college students, with effect values of -0.064 and -0.073 respectively, accounting for the total effect. 20.38, 23.25%, and also played a chain intermediary role, with an effect value of -0.041, accounting for 13.05% of the total effect.
CONCLUSION
Current research shows that physical activity can not only directly reduce social anxiety among college students, but also indirectly alleviate social anxiety among college students by increasing the level of social support and psychological capital. This has important reference significance for helping college students in China and even around the world overcome social anxiety.
PubMed: 38957885
DOI: 10.3389/fpsyg.2024.1406452 -
Frontiers in Psychology 2024The testing of visuocognitive development in preterm infants shows strong interactions between perinatal characteristics and cognition, learning and overall...
INTRODUCTION
The testing of visuocognitive development in preterm infants shows strong interactions between perinatal characteristics and cognition, learning and overall neurodevelopment evolution. The assessment of anticipatory gaze data of object-location bindings via eye-tracking can predict the neurodevelopment of preterm infants at the age of 3 years; little is known, however, about the early cognitive function and its assessment methods during the first year of life.
METHODS
The current study presents data from a novel assessment tool, a Delayed Match Retrieval (DMR) paradigm via eye-tracking was used to measure visual working memory (VWM) and attention skills. The eye-tracking task that was designed to measure infants' ability to actively localize objects and to make online predictions of object-location bindings. 63 infants participated in the study, 39 preterm infants and 24 healthy full term infants - at a corrected age of 8-9 months for premature infants and similar chronological age for full term infants. Infants were also administered the Bayley Scales of Infant and Toddler Development.
RESULTS
The analysis of the Bayley scores showed no significant difference between the two groups while the eye-tracking data showed a significant group effect on all measurements. Moreover, preterm infants' VWM performance was significantly lower than full term's. Birth weight affected the gaze time on all Areas Of Interest (AOIs), overall VWM performance and the scores at the Cognitive Bayley subscale. Furthermore, preterm infants with fetal growth restriction (FGR) showed significant performance effects in the eye-tracking measurements but not on their Bayley scores verifying the high discriminatory value of the eye gaze data.
CONCLUSION
Visual working memory and attention as measured via eye-tracking is a non-intrusive, painless, short duration procedure (approx. 4-min) was found to be a significant tool for identifying prematurity and FGR effects on the development of cognition during the first year of life. Bayley Scales alone may not pick up these deficits. Identifying tools for early neurodevelopmental assessments and cognitive function is important in order to enable earlier support and intervention in the vulnerable group of premature infants, given the associations between foundational executive functional skills and later cognitive and academic ability.
PubMed: 38957884
DOI: 10.3389/fpsyg.2024.1384486 -
Frontiers in Psychology 2024The development of advanced sewage technologies empowers the industry to produce high-quality recycled water, which greatly influences human's life and health. Thus,...
INTRODUCTION
The development of advanced sewage technologies empowers the industry to produce high-quality recycled water, which greatly influences human's life and health. Thus, this study investigates the mechanism of individuals' adoption of recycled water from the technology adoption perspective.
METHODS
Employing the mixed method of structural equation modeling and artificial neural network analysis, we examined a research model developed from the extended Unified Theory of Acceptance and Use of Technology (UTAUT2) framework. To examine the research model, this study employs a leading web-survey company (Sojump) to collect 308 valid samples from the residents in mainland China.
RESULTS
The structural equation modeling results verified the associations between the six predictors (performance expectancy, effort expectancy, social influence, facilitating conditions, environmental motivation, and price value), individuals' cognitive and emotional attitudes, and acceptance intention. The artificial neural network analysis validates and complements the structural equation modeling results by unveiling the importance rank of the significant determinants of the acceptance decisions.
DISCUSSION
The study provides theoretical implications for recycled water research and useful insights for practitioners and policymakers to reduce the environmental hazards of water scarcity.
PubMed: 38957883
DOI: 10.3389/fpsyg.2024.1384635 -
Frontiers in Psychology 2024There are increasing demands for Participatory Arts-Based (PAB) programs involved in health research to better evidence outcomes using robust quantitative evaluation...
There are increasing demands for Participatory Arts-Based (PAB) programs involved in health research to better evidence outcomes using robust quantitative evaluation methodologies taken from science, such as standardized questionnaires, to inform commissioning and scale-up decisions. However, for PAB researchers trying to do this, barriers arise from fundamental interdisciplinary differences in values and contexts. Researchers are required to navigate the tensions between the practice-based evidence produced by the arts and the evidence-based practice sought by psychologists. Consequently, there is a need for interdisciplinary arts-science collaborations to produce alternative methods of evaluation that are better aligned to PAB approaches, and which combine systematic rigor with a sensitivity to the values, contexts and strengths of this approach. The current article centers on the development of an alternative transdisciplinary analytic tool, the Participatory arts Play Framework (PP-Framework), undertaken as part of an arts-psychology collaboration for a UK AHRC-funded PAB research project: Playing A/Part: Investigating the identities and experiences of autistic girls. We present details of three stages in the development of the PP-Framework: 1. preliminary emergence of the framework from initial video analysis of observational data from participatory music and sound workshops run for 6 adolescent autistic girls (aged 11-16); 2. identification and application of modes of engagement; and 3. further testing of the framework as an evaluation tool for use in a real-world setting, involving professional musicians engaged in delivery of a creative music project at a center for homeless people. The PP-Framework maps types of participation in terms of performative behaviors and qualities of experience, understood as modes of play. It functions as a vehicle for analyzing participant engagement, providing a tool predicated on the processes of working in creative participatory contexts while also being sensitive to the esthetic qualities of what is produced and capable of capturing beneficial changes in engagement. It offers a conceptual approach for researchers to undertake observation of participatory arts practices, taking account of embodied engagement and interaction processes. It is informed by understandings of autistic performativity and masking in conjunction with an ecological understanding of sense making as being shaped by environments, social relations and sensing subjectivity. The framework has the potential to be a bi-directional tool, with application for both practitioners and participants.
PubMed: 38957882
DOI: 10.3389/fpsyg.2024.1324036 -
Frontiers in Psychology 2024Placeness is believed to play a significant role in enhancing the well-being and place-use of individuals, contributing profoundly to how spaces are experienced and...
Placeness is believed to play a significant role in enhancing the well-being and place-use of individuals, contributing profoundly to how spaces are experienced and interacted with. Despite its perceived importance, there is ongoing debate and insufficient clarity about how exactly placeness influences people's behavior. This study aims to bridge this gap by theorizing and investigating the pathways from placeness to people's behavioral intentions, emphasizing the roles of personal place attachment and public place image as pivotal mediators in this relationship. To explore these dynamics, we conducted a survey in Japan, examining the complex interplay between placeness and behavioral intentions, given their rich cultural heritage and modern urban pressures. We employed the Partial Least Squares Structural Equation Modeling (PLS-SEM) approach for path analysis. The analysis suggests that placeness influences behavioral intention through personal place attachment. While placeness does affect public place image, this public image does not have an impact on behavioral intention. The results demonstrated that an individual's activities, experiences, and cognition of a place are significant factors in creating the intention to engage in word-of-mouth, recommendation, and revisiting behaviors. Policymakers, urban planners, and designers need to understand how to foster people's behavioral intentions when creating a place imbued with placeness.
PubMed: 38957880
DOI: 10.3389/fpsyg.2024.1394930