bluuebunny/crossref_metadata_2025_split huggingface dataset:
Genetic Basis of Childhood Cardiomyopathy
Authors: Bagnall Richard D. , Singer Emma S. , Wacker Julie , Nowak Natalie , Ingles Jodie , King Ingrid , Macciocca Ivan , Crowe Joshua , Ronan Anne , Weintraub Robert G. , Semsarian Christopher 
Published: October 2022

Background:
The causes of cardiomyopathy in children are less well described than in adults. We evaluated the clinical diagnoses and genetic causes of childhood cardiomyopathy and outcomes of cascade genetic testing in family members.

Methods:
We recruited children from a pediatric cardiology service or genetic heart diseases clinic. We performed Sanger, gene panel, exome or genome sequencing and classified variants for pathogenicity using American College of Molecular Genetics and Genomics guidelines.

Results:

Cardiomyopathy was diagnosed in 221 unrelated children aged ≤18 years. Children mostly had hypertrophic cardiomyopathy (n=98, 44%) or dilated cardiomyopathy (n=89, 40%). The highest genetic testing diagnostic yields were in restrictive cardiomyopathy (n=16, 80%) and hypertrophic cardiomyopathy (n=65, 66%), and lowest in dilated cardiomyopathy (n=26, 29%) and left ventricular noncompaction (n=3, 25%). Pathogenic variants were primarily found in genes encoding sarcomere proteins, with
TNNT2
and
TNNI3
variants associated with more severe clinical outcomes. Ten children (4.5%) had multiple pathogenic variants. Genetic test results prompted review of clinical diagnosis in 14 families with syndromic, mitochondrial or metabolic gene variants. Cascade genetic testing in 127 families confirmed 24 de novo variants, recessive inheritance in 8 families, and supported reclassification of 12 variants.

Conclusions:
Genetic testing of children with cardiomyopathy supports a precise clinical diagnosis, which may inform prognosis.
Revisiting the Passive Revolution
Authors: Sotiris Panagiotis 
Published: October 2022

Abstract
Passive revolution is one of the most debated notions to come out of the Prison Notebooks. It belongs to the notions that have been used as ‘established’ descriptions of historical and political sequences. However, a reading of Gramsci’s texts suggests that passive revolution is not a ‘historical phase’ and is not limited to the historical interpretation of a particular historical period. Nor is it part of an historical ‘canon’ that would suggest that, in the absence of a ‘proper’ Jacobin revolution, the only alternative is passive revolution. Rather it points to the experimental character of the Prison Notebooks and Gramsci’s confrontation with the profound changes in state apparatuses (and hegemonic apparatuses) and practices of politics and hegemony after WWI, in particular the new ‘mass’ forms of politics and ideological interpellation, and his attempt to elaborate a strategic–theoretical thinking suited to revolutionary politics in such a context.
The Godly “I Am” of Trans Identity
Authors: Sanchinel Sam 
Published: October 2022

Abstract
To many, reading trans identity through a Christian lens only makes sense if the goal is to deny trans people of rights. As such, trans persons have generally been understood through a secular lens, thus erasing the value held in Biblical texts on understanding humanity. In this paper the author reads trans identity as emerging from what they describe as “the Godly ‘I am.’ ” With a framework rooted in the works of Winnicott, de Certeau, and trans theological practice as well as referring to the Tower of Babel narrative and the various declarations of “I am” throughout the Bible, the author presents a view of trans embodiment as the expression of human possibility where one may truly “come into being” in the enunciation of “I am”.
Analysis of Multiyear Blowing Snow Occurrences in the French Alps
Authors: Xie Zhipeng , Ma Yaoming , Ma Weiqiang , Hu Zeyong , Sun Genhou , Wang Yongjie 
Published: October 2022

Abstract
Wind-driven snow transport has important implications for spatial–temporal heterogeneity of snow distribution and snowpack evolution in mountainous areas, such as the French Alps. Due to the paucity of near-surface observations, our knowledge on the spatiotemporal variability of blowing snow occurrences is rather limited. Based on multiyear in situ observations, the spatial–temporal variability in the occurrence of blowing snow events in the French Alps was presented to investigate potential links with ambient meteorological conditions. Statistical analysis of the observations demonstrates that blowing snow events are frequently observed with substantial spatiotemporal variability. Most stations experienced snow transport one out of every five days throughout winter, and the corresponding cumulative hours with blowing snow occurrence accounted for 8% of the month in winter. Blowing snow events generally last 4–8 h in winter and early spring. The likelihood of blowing snow occurrences increases with wind speed but with divergent patterns across snow types. The frequency of blowing snow occurrences with concurrent snowfall is substantially higher than that without concurrent snowfall, although high spatiotemporal variability was found. The considerable variation in snow transport frequency can be explained by contrasting meteorological conditions, local climate, snowpack properties, and topography (elevation and aspect). The temperature-based empirical scheme failed to recognize individual occurrence of blowing snow events because of the significantly overestimated threshold wind speeds, highlighting the importance of validation using in situ observations. Our results contribute to the understanding of spatiotemporal occurrence of blowing snow events and facilitate the development of blowing snow models.
Analysis and prediction of the joint strength of friction stir welded Aluminium 5754 to polyamide using response surface methodology and artificial neural network
Authors: S J Adarsh , Natarajan Arivazhagan 
Published: October 2022

Lightweight hybrid structures are developing these days due to increased demand for fuel economy and lower emissions in the automotive and aerospace industries. This study aims to analyse and optimise the influence of friction stir welding (FSW) process parameters on the tensile shear strength of the aluminium-polyamide hybrid joint. The study on the influence of each parameter on the joint strength helps define the bonding mechanism while joining aluminium-polymer hybrid structures. Optical microscopy and scanning electron microscopy (SEM) were used for microstructural examination. A SEM image of the weld’s cross-sectional area shows micro and macro mechanical interlocks with a small interfacial gap which indicates better joint strength. An elemental area mapping investigation of the weld zone reveals fine polymer and aluminium mixing along the interaction region. In addition, FSW parameters have been optimized to maximize the tensile shear strength of aluminium-polyamide hybrid joints. A mathematical model for tensile shear strength in terms of FSW parameters is developed using response surface methodology (RSM). A predictive model was developed using an Artificial Neural Network (ANN) to validate RSM predicted results. The analysis of variance (ANOVA) shows that the actual and predicted values have a satisfactory correlation. ANN methods are better than regression models in predicting tensile shear strength within input welding parameter ranges. The process variables were optimised using the desirability function analysis. The maximum joint tensile shear strength of about 19.74 MPa and attained at optimal FSW parameters, i.e. rotational tool speed of 1421 r/min, welding speed of 27 mm/min, and tool tilt angle of 1°. The regression coefficient for the ANN model was 0.988 for the test data set, indicating that the developed model is appropriate for predicting tensile shear strength.
Patients with heat-related illness admitted to an intensive care unit in Hong Kong: A single-centre retrospective study (2009–2020)
Authors: Chan Carol Cheuk-Yan , Shum HP , Wu P , Leung NYW , Chang LL , Yan WW 
Published: October 2022

Introduction: In Hong Kong, heat-related illness (HRI) represents an important cause of morbidity and mortality among healthy adults engaging in outdoor activities during summer. It can result in intensive care unit (ICU) admission. This retrospective study aimed to profile critically ill patients with HRI and identify potential risk factors for mortality. Methods: This retrospective case series included adults with HRI from 1 January 2009 to 30 June 2020 admitted to the ICU of Pamela Youde Nethersole Eastern Hospital (PYNEH). Demographics, admission severity, co-morbidities, and laboratory investigations were analysed. Outcome data studied included mortality, ICU length of stay (LOS), ventilator days, and hospital LOS. Results: Among the 38 individuals included, 97% were hikers. About 92.1% of patients survived, while 7.9% expired. The median age of the patients was 48 years, and 78.9% were men. Hypertension was the most frequent concurrent disorder. Most cases were presented during the hot summer months of May (29%), July (24%) and August (18%). The localities’ median peak temperature and humidity were 33.2°C and 88%, respectively. Ninety-two percent of patients were airlifted to the hospital by helicopters. The median peak body temperature was 40.3°C. Central nervous system derangement (Glasgow Coma Scale (GCS) score ⩽ 8) was the top presenting feature (65.7%). About 57.9% of patients required vasopressor support, followed by mechanical ventilation (50%) and renal replacement therapy (31.6%). Conclusions: Most critically ill patients suffering from HRI were hikers and middle-aged adults. Mortality was 7.9% despite advanced life support in ICU. Raising public awareness of the associated risk with hiking in hot summer is essential for the prevention of HRI.
The failure of four bootstrap procedures for estimating confidence intervals for predicted-to-expected ratios for hospital profiling
Authors: Austin Peter C. 
Published: October 2022

Abstract
Background
Healthcare provider profiling involves the comparison of outcomes between patients cared for by different healthcare providers. An important component of provider profiling is risk-adjustment so that providers that care for sicker patients are not unfairly penalized. One method for provider profiling entails using random effects logistic regression models to compute provider-specific predicted-to-expected ratios. These ratios compare the predicted number of deaths at a given provider given the case-mix of its patients with the expected number of deaths had those patients been treated at an average provider. Despite the utility of this metric in provider profiling, methods have not been described to estimate confidence intervals for these ratios. The objective of the current study was to evaluate the performance of four bootstrap procedures for estimating 95% confidence intervals for predicted-to-expected ratios.

Methods
We used Monte Carlo simulations to evaluate four bootstrap procedures: the naïve bootstrap, a within cluster-bootstrap, the parametric multilevel bootstrap, and a novel cluster-specific parametric bootstrap. The parameters of the data-generating process were informed by empirical analyses of patients hospitalized with acute myocardial infarction. Three factors were varied in the simulations: the number of subjects per cluster, the intraclass correlation coefficient for the binary outcome, and the prevalence of the outcome. We examined coverage rates of both normal-theory bootstrap confidence intervals and bootstrap percentile intervals.

Results
In general, all four bootstrap procedures resulted in inaccurate estimates of the standard error of cluster-specific predicted-to-expected ratios. Similarly, all four bootstrap procedures resulted in 95% confidence intervals whose empirical coverage rates were different from the advertised rate. In many scenarios the empirical coverage rates were substantially lower than the advertised rate.

Conclusion
Existing bootstrap procedures should not be used to compute confidence intervals for predicted-to-expected ratios when conducting provider profiling.
Consultation of parents and healthcare professionals in end-of-life decision-making for neonates and infants: a population-level mortality follow-back physician survey
Authors: Dombrecht Laure , Cools Filip , Cohen Joachim , Deliens Luc , Goossens Linde , Naulaers Gunnar , Beernaert Kim , Chambaere Kenneth 
Published: October 2022

Abstract
Background
End-of-life decisions with potential life-shortening effect in neonates and infants are common. We aimed to evaluate how often and in what manner neonatologists consult with parents and other healthcare providers in these cases, and whether consultation is dependent on the type of end-of-life decision made.

Methods
Based on all deaths under the age of one that occurred between September 2016 and December 2017 in Flanders, Belgium, a nationwide mortality follow-back survey was performed. The survey asked about different types of end-of-life decisions, and whether and why parents and/or other healthcare providers had or had not been consulted.

Results
Response rate was 83% of the total population. End-of-life decisions in neonates and infants were consulted both with parents (92%) and other healthcare providers (90%), and agreement was reached between parents and healthcare providers in most cases (96%). When medication with an explicit life-shortening intent was administered parents were always consulted prior to the decision; however when medication without explicit life-shortening intention was administered parents were not consulted in 25% of the cases.

Conclusions
Shared decision-making between parents and physicians in case of neonatal or infant end-of-life decision-making is the norm in daily practice. All cases without parental consultation concerned non-treatment decisions or comfort medication without explicit life-shortening intention where physicians deemed the medical situation clear and unambiguous. However, we recommend to at least inform parents of medical options, and to explore other possibilities to engage parents in reaching a shared decision. Physicians consult other healthcare providers before making an end-of-life decision in most cases.
Health-Related Quality of Life and mental health of families with children and adolescents affected by rare diseases and high disease burden: the perspective of affected children and their siblings
Authors: Wiegand-Grefe Silke , Liedtke Anna , Morgenstern Lydia , Hoff Antonia , Csengoe-Norris Anikó , Johannsen Jessika , Denecke Jonas , Barkmann Claus , Grolle Benjamin , Daubmann Anne , Wegscheider Karl , Boettcher Johannes 
Published: October 2022

Abstract
Background
Advances in genetic and pharmaceutical technology and pediatric care have enabled treatment options for an increasing number of rare diseases in affected children. However, as current treatment options are primarily of palliative nature, the Health-Related Quality of Life (HRQoL) and mental health of this impaired population and their siblings are of increasing importance. Among children and adolescents with rare diseases, those who are technology-dependent carry a high disease burden and are selected as the target population in our study. In a cross-sectional observational design, the children’s HRQoL was assessed with the DISABKIDS (DCGM-37) as well as KIDSCREEN-27, while mental health was assessed with the Strengths and Difficulties Questionnaire (SDQ) by both the affected children, their parents, and siblings.

Results
Results of the study sample were compared to normative data. Affected children scored significantly lower than the norm on almost all HRQoL subscales as reported by parent and child. From the parental perspective, more mental health subscales were significantly impaired compared to the child’s perspective. Siblings showed no impairment in HRQoL as well as significantly fewer behavioral problems and higher prosocial behavior regarding their mental health compared to the norm.

Conclusion
Children and adolescents with rare diseases seem particularly impaired in social and emotional aspects of HRQoL and mental health. Interventions may focus primarily on promoting social skills, fostering prosocial behavior and peer relationships.
Type, density, and healthiness of food-outlets in a university foodscape: a geographical mapping and characterisation of food resources in a Ghanaian university campus
Authors: Mensah Daniel O. , Yeboah Godwin , Batame Michael , Lillywhite Rob , Oyebode Oyinlola 
Published: October 2022

Abstract
Introduction
Food environments are viewed as the interface where individuals interact with the wider food system to procure and/or consume food. Institutional food environment characteristics have been associated with health outcomes including obesity and nutrition-related non-communicable diseases (NR-NCDs) in studies from high-income countries. The objectives of this study were (1) to map and characterise the food-outlets within a Ghanaian university campus; and (2) to assess the healthiness of the food outlets.

Methods
Data collection was undertaken based on geospatial open-source technologies and the collaborative mapping platform OpenStreetMap using a systematic approach involving three phases: remote mapping, ground-truthing, and food-outlet survey. Spatial analyses were performed using Quantum Geographical Information System (QGIS) and comprised kernel density, buffer, and average nearest neighbour analyses to assess outlet distribution, density, and proximity. A classification system was developed to assess the healthiness of food-outlets within the University foodscape.

Results
Food-outlets were unevenly distributed over the University foodscape, with many outlets clustered closer to student residencies. Informal food-outlets were the most frequent food-outlet type. Compared to NCD-healthy food-outlets, NCD-unhealthy food-outlets dominated the foodscape (50.7% vs 39.9%) with 9.4% being NCD-intermediate, suggesting a less-healthy university foodscape. More NCD-unhealthy food outlets than NCD-healthy food outlets clustered around student residences. This difference was statistically significant for food outlets within a 100-m buffer (p < 0.001) of student residence and those within 100 and 500 m from departmental buildings/lecture halls (at 5% level of significance).

Conclusion
Further action, including research to ascertain how the features of the University’s food environment have or are influencing students’ dietary behaviours are needed to inform interventions aimed at creating healthier foodscapes in the study University and other campuses and to lead the way towards the creation of healthy food environments at the home, work, and community levels.
Evaluation of biofilm formation on acrylic resins used to fabricate dental temporary restorations with the use of 3D printing technology
Authors: Mazurek-Popczyk Justyna , Nowicki Adam , Arkusz Katarzyna , Pałka Łukasz , Zimoch-Korzycka Anna , Baldy-Chudzik Katarzyna 
Published: October 2022

Abstract
Background
Temporary implant-retained restorations are required to support function and esthetics of the masticatory system until the final restoration is completed and delivered. Acrylic resins are commonly used in prosthetic dentistry and lately they have been used in three-dimensional (3D) printing technology. Since this technology it is fairly new, the number of studies on their susceptibility to microbial adhesion is low. Restorations placed even for a short period of time may become the reservoir for microorganisms that may affect the peri-implant tissues and trigger inflammation endangering further procedures. The aim of the study was to test the biofilm formation on acrylamide resins used to fabricate temporary restorations in 3D printing technology and to assess if the post-processing impacts microbial adhesion.

Methods
Disk-shaped samples were manufactured using the 3D printing technique from three commercially available UV-curable resins consisting of acrylate and methacrylate oligomers with various time and inhibitors of polymerization (NextDent MFH bleach, NextDent 3D Plus, MazicD Temp). The tested samples were raw, polished and glazed. The ability to create biofilm by oral streptococci (S. mutans, S. sanguinis, S. oralis, S. mitis) was tested, as well as species with higher pathogenic potential: Staphylococcus aureus, Staphylococcus epidermidis and Candida albicans. The roughness of the materials was measured by an atomic force microscope. Biofilm formation was assessed after 72 h of incubation by crystal violet staining with absorbance measurement, quantification of viable microorganisms, and imaging with a scanning electron microscope (SEM).

Results
Each tested species formed the biofilm on the samples of all three resins. Post-production processing resulted in reduced roughness parameters and biofilm abundance. Polishing and glazing reduced roughness parameters significantly in the NextDent resin group, while glazing alone caused significant surface smoothing in Mazic Temp. A thin layer of microbial biofilm covered glazed resin surfaces with a small number of microorganisms for all tested strains except S. oralis and S. epidermidis, while raw and polished surfaces were covered with a dense biofilm, rich in microorganisms.

Conclusions
UV-curing acrylic resins used for fabricating temporary restorations in the 3D technology are the interim solution, but are susceptible to adhesion and biofilm formation by oral streptococci, staphylococci and Candida. Post-processing and particularly glazing process significantly reduce bacterial biofilm formation and the risk of failure of final restoration.
When cultural values meets professional values: a qualitative study of chinese nurses’ attitudes and experiences concerning death
Authors: Tu Jiong , Shen Manxuan , Li Ziying 
Published: October 2022

Abstract
Background
In China, there is a culture of death-avoidance and death-denying. Influenced by this distinctive socio-cultural views surrounding death, nurses often find it challenging to handle death and care for dying patients. This study explores the nurses’ attitudes and coping strategies concerning death and caring for dying patients in a cultural context of death taboo.

Methods
This research is a qualitative study that employs in-depth, semi-structured interviews with nurses from two major hospitals in Guangzhou, China. Overall, 28 nurses from four departments with high patient death rate were recruited and interviewed. All of the interviews were analyzed thematically.

Results
The nurses who participated in this study expressed attitudes toward death and caring for dying patients from both a personal dimension and a professional dimension. The personal dimension is influenced by traditional culture and societal attitudes towards death and dying, while their professional dimension is congruent with the nursing and palliative care values concerning death and dying. With an obvious discrepancy between these two dimensions, Chinese nurses adopt three strategies in their practice to solve this tension: boundary-drawing to separate their personal and professional life, complying with the existing cultural values at work, and constructing positive meanings for end-of-life care.

Conclusion
In a society that traditionally avoids making any reference to death, it is useful to reduce cultural taboo and construct positive meanings in end-of-life care, death education and the development of palliative care. Meanwhile, nurses also need institutional support, education and training to transition smoothly from a novice to a mature professional when handling patient death.