bluuebunny/crossref_metadata_2025_split huggingface dataset:
Accelerated adulthood, extended adolescence and the care cliff: Supporting care leavers' transition from care to independent living
Authors: Palmer Angela , Norris Michelle , Kelleher Joanne 
Published: March 2022

AbstractThe pressures of ‘accelerated adulthood’ are a critical challenge for young people ageing out of the care system. Despite the trauma related to their placement history, young people ‘aging out’ are expected to adapt to adulthood at younger age and faster pace than their non‐care peers, who enjoy an ‘extended adolescence’ a far more gradual progression into adulthood than previous generations. This article draws on first‐person narratives of care leavers in Ireland who have aged out of care and transitioned into independent living in a dedicated social housing programme to examine their strategies for coping with these competing pressures. It examines their worries about the sudden withdrawal of supports after they reach 18 years, which they characterized as a ‘care cliff’. The emotional and practical challenges they faced when transitioning to independent living at a comparatively young age are explored, and strategies for mitigating these are identified. These include better preparing care leavers for this transition, maintaining supports for longer and withdrawing them in a more gradual way, which is tailored to meet the specific needs of each care leaver and grounded in more comprehensive after care planning than has been the norm in Ireland.
El‐Hattab‐Alkuraya syndrome caused by biallelic WDR45B pathogenic variants: Further delineation of the phenotype and genotype
Authors: Almannai Mohammed , Marafi Dana , Abdel‐Salam Ghada M. H. , Zaki Maha S. , Duan Ruizhi , Calame Daniel , Herman Isabella , Levesque Felix , Elbendary Hasnaa M. , Hegazy Ibrahim , Chung Wendy K. , Kavus Haluk , Saeidi Kolsoum , Maroofian Reza , AlHashim Aqeela , Al‐Otaibi Ali , Al Madhi Asma , Abou Al‐Seood Hager M. , Alasmari Ali , Houlden Henry , Gleeson Joseph G. , Hunter Jill V. , Posey Jennifer E. , Lupski James R. , El‐Hattab Ayman W. 
Published: March 2022

AbstractHomozygous pathogenic variants in WDR45B were first identified in six subjects from three unrelated families with global development delay, refractory seizures, spastic quadriplegia, and brain malformations. Since the initial report in 2018, no further cases have been described. In this report, we present 12 additional individuals from seven unrelated families and their clinical, radiological, and molecular findings. Six different variants in WDR45B were identified, five of which are novel. Microcephaly and global developmental delay were observed in all subjects, and seizures and spastic quadriplegia in most. Common findings on brain imaging include cerebral atrophy, ex vacuo ventricular dilatation, brainstem volume loss, and symmetric under‐opercularization. El‐Hattab‐Alkuraya syndrome is associated with a consistent phenotype characterized by early onset cerebral atrophy resulting in microcephaly, developmental delay, spastic quadriplegia, and seizures. The phenotype appears to be more severe among individuals with loss‐of‐function variants whereas those with missense variants were less severely affected suggesting a potential genotype–phenotype correlation in this disorder. A brain imaging pattern emerges which is consistent among individuals with loss‐of‐function variants and could potentially alert the neuroradiologists or clinician to consider WDR45B‐related El‐Hattab‐Alkuraya syndrome.
Conceptualization of quality of life in autistic individuals
Authors: Evers Kris , Maljaars Jarymke , Schepens Hadewych , Vanaken Gert‐Jan , Noens Ilse 
Published: March 2022

AbstractThe present study examines to what extent two core characteristics of the quality of life (QoL) construct were incorporated in the field of autism: (1) its subjective nature; and (2) its multidimensionality. Therefore, we reviewed 174 articles examining QoL in individuals with autism. The review showed parents reporting a lower QoL compared with autistic individuals themselves, especially on internal domains. This may suggest different expectations about what a good QoL may entail. Such an underestimation of QoL by others is commonly observed in individuals with disabilities (the so‐called ‘disability paradox’). For the multidimensionality of the QoL construct, our findings suggest that the narrower (and more unidimensional) construct of health‐related QoL is often measured instead of QoL. Additionally, a substantial proportion of items did not measure QoL, but they evaluated characteristics that may or may not have an impact on QoL. Researchers and clinicians should be aware that QoL domains are selected and operationalized differently by different instruments. QoL may benefit from an exclusive focus on subjective aspects, which can be measured alongside more normative, objective characteristics of individuals or their environment.
Modelling quality of life in children with intellectual disability using regression trees
Authors: Jacoby Peter , Williams Katrina , Reddihough Dinah , Leonard Helen , Whitehouse Andrew , Downs Jenny 
Published: March 2022

AimTo identify factors associated with quality of life (QoL) in children with intellectual disability. We aimed to identify patterns of association not observable in previous hypothesis‐driven regression modelling using the same data set from a cross‐sectional observational study.MethodA questionnaire was completed by 442 caregivers of children with confirmed intellectual disability and a diagnosis of autism spectrum disorder, cerebral palsy, Down syndrome, or Rett syndrome. The Quality of Life Inventory‐Disability (QI‐Disability) questionnaire was used to assess child QoL. Independent variables described the child's health, functional abilities, community participation, and sociodemographics. The R package rpart was used to build the regression trees.ResultsThe mean total QI‐Disability score was 69.2 out of a maximum 100. The subgroup with the lowest QoL scores comprised children with a high degree of daytime sleepiness (n=74, mean 57.5) while the subgroup with the highest QoL scores (n=91, mean 80.3) comprised children with little daytime sleepiness who participated more frequently in community activities and displayed good eye contact while listening.InterpretationRegression tree analysis provides insights into the relative importance of associated factors. Sleep problems and community participation were more important than functional abilities in accounting for differences in QoL.What this paper adds

A hypothesis‐free regression tree analysis enables examination of multiple factors potentially influencing quality of life (QoL) in children with intellectual disability.
Functional abilities were less strongly associated with QoL than sleep problems and community participation.
Identifying indicators of community participation‐promoting efforts within coordinated specialty care: A modified e‐Delphi study of stakeholder perspectives
Authors: Thomas Elizabeth C. , Jones Nev , Shern David L. , Salzer Mark S. 
Published: March 2022

AbstractAim: Community participation in occupational, social, recreational, and other domains is critically important during young adulthood. Coordinated Specialty Care (CSC) programs provide developmentally tailored care to young adults experiencing early psychosis within the United States, but little is known about the breadth of efforts to promote community participation. This study aimed to develop and evaluate indicators of these efforts based on the perspectives of a national multi‐stakeholder group.Methods: Seventeen stakeholders (i.e., young adults with early psychosis, family members, experts by profession) participated in a modified e‐Delphi study, conducted in two rounds. The purpose of round one was to generate a comprehensive list of community participation‐promoting indicators. During round two, stakeholders rated the importance and feasibility of the implementation of each indicator. Descriptive statistics and percentage of agreement regarding round two ratings were assessed.Results: During round one, 186 indicators of activities and/or practices designed to promote community participation were identified; this list was reduced to 44 by eliminating redundancies or indicators not related to community participation. In round two, we found broad agreement regarding the importance, but significant variation in perceived feasibility of indicators. The highest‐rated indicators in both categories pertained to staff knowledge (regarding barriers and supports to participation and the importance of participation to health) and strategies for addressing participation barriers.Conclusions: This study is expected to facilitate the identification and development of promising CSC activities and practices designed to promote community participation among young adults while potentially also enhancing engagement in services and improving clinical outcomes.
Functional investigation of two simultaneous or separately segregating DSP variants within a single family supports the theory of a dose‐dependent disease severity
Authors: Vermeer Mathilde C. S. C. , Andrei Daniela , Kramer Duco , Nijenhuis Albertine M. , Hoedemaekers Yvonne M. , Westers Helga , Jongbloed Jan D. H. , Pas Hendri H. , van den Berg Maarten P. , Silljé Herman H. W. , van der Meer Peter , Bolling Maria C. 
Published: March 2022

AbstractDesmoplakin (DP) is an important component of desmosomes, essential in cell–cell connecting structures in stress‐bearing tissues. Over the years, many hundreds of pathogenic variants in DSP have been associated with different cutaneous and cardiac phenotypes or a combination, known as a cardiocutaneous syndrome. Of less than 5% of the reported DSP variants, the effect on the protein has been investigated. Here, we describe and have performed RNA, protein and tissue analysis in a large family where DSPc.273+5G>A/c.6687delA segregated with palmoplantar keratoderma (PPK), woolly hair and lethal cardiomyopathy, while DSPWT/c.6687delA segregated with PPK and milder cardiomyopathy. hiPSC‐derived cardiomyocytes and primary keratinocytes from carriers were obtained for analysis. Unlike the previously reported nonsense variants in the last exon of DSP that bypassed the nonsense‐mediated mRNA surveillance system leading to protein truncation, variant c.6687delA was shown to cause the loss of protein expression. Patients carrying both variants and having a considerably more severe phenotype were shown to have 70% DP protein reduction, while patients carrying only c.6687delA had 50% protein reduction and a milder phenotype. The analysis of RNA from patient cells did not show any splicing effect of the c.273+5G>A variant. However, a minigene splicing assay clearly showed alternative spliced transcripts originating from this variant. This study shows the importance of RNA and protein analyses to pinpoint the exact effect of DSP variants instead of solely relying on predictions. In addition, the particular pattern of inheritance, with simultaneous or separately segregating DSP variants within the same family, strongly supports the theory of a dose‐dependent disease severity.
Parental socialization of mental health in Chinese American families: What parents say and do, and how youth make meaning
Authors: Yasui Miwa , Choi Yoonsun , Chin Marshall , Miranda Samuels Gina , Kim Karen , Victorson David 
Published: March 2022

AbstractParental mental health socialization is a process by which parents shape how youth develop and maintain beliefs, attitudes, and behaviors regarding mental health and help‐seeking behaviors. Although culture shapes parental mental health socialization, few studies have examined specific parental socialization practices regarding mental health and help‐seeking, especially as a culturally anchored process. Using a qualitative approach, this study explores youth‐reported parental socialization of mental health within Chinese American families by examining focus group data from 69 Chinese American high school and college students. Findings revealed that youth received parental messages that conveyed culturally anchored conceptualizations of mental health that included stigmatized views of mental illness and perceptions of mental distress as not a legitimate problem. Parents responded to youth distress in culturally consonant ways: by encouraging culturally specific coping methods, dismissing or minimizing distress, or responding with silence. Youth engaged in the active interpretation of parental messages through cultural brokering, bridging the gap between their parents’ messages and mainstream notions of mental health and help‐seeking. Overall, our findings point to the significant role of culture in parental mental health socialization in Chinese American families and the need to integrate culturally specific understandings of mental health into future interventions for Asian American youth.
Impact of social engagement on the development of sarcopenia among community‐dwelling older adults: A Kashiwa cohort study
Authors: Tanaka Tomoki , Son Bo‐Kyung , Lyu Weida , Iijima Katsuya 
Published: March 2022

AimHow older adults develop sarcopenia in the community setting is unclear. Focusing on social engagement, we aimed to validate our hypothesized model of sarcopenia development with various contributing factors, such as physical activity, oral function, psychological status and nutritional status. We also clarified direct and indirect effects of social engagement, physical activity, nutritional status, oral function and psychological status on new‐onset sarcopenia.MethodsWe analyzed 1483 participants' (72.6 ± 5.4 years) longitudinal data from the Kashiwa study. Sarcopenia was assessed in all the surveys in the Kashiwa study. Measures regarding social engagement, physical activity, oral function, psychological status and nutritional status were assessed at baseline. Structural equation modeling was used to analyze the efficiency of the hypothesized model, and calculate direct and indirect effects of factors affecting new‐onset sarcopenia.ResultsOver the follow‐up period (median 6 years [interquartile range 4–6 years]), 12% of individuals developed new‐onset sarcopenia. Our structural hypothesis model starting from social engagement to new‐onset sarcopenia was suitable (root mean square error of approximation = 0.031, goodness‐of‐fit index = 0.967, adjusted goodness‐of‐fit index = 0.954, comparative fix index = 0.911, parsimonious comparative fit index = 0.755; all paths were significant), showing direct effects of social engagement on psychological status, physical activity and oral function, and indirect effects on nutritional status through oral function and psychological status.ConclusionsThe present results showed that social engagement could potentially decrease new‐onset sarcopenia risks by influencing multidimensional factors, such as physical activity, oral function, and psychological and nutritional status. To prevent sarcopenia, it might be essential to promote social engagement through populational approaches. Geriatr Gerontol Int 2022; 22: 384–391.
Epstein–Barr virus infection patterns in nodular lymphocyte‐predominant Hodgkin lymphoma
Authors: Gerhard‐Hartmann Elena , Jöhrens Korinna , Schinagl Lisa‐Marie , Zamó Alberto , Rosenwald Andreas , Anagnostopoulos Ioannis , Rosenfeldt Mathias 
Published: March 2022

AimsTo investigate Epstein‐Barr virus (EBV) latency types in 19 cases of EBV‐positive nodular lymphocyte‐predominant Hodgkin lymphoma (NLPHL), as such information is currently incomplete.Methods and resultsImmunohistochemistry (IHC) for CD20, CD79a, PAX5, OCT2, CD30, CD15, CD3 and programmed cell death protein 1 was performed. For EBV detection, in‐situ hybridisation (ISH) for EBV‐encoded RNA (EBER) was employed combined with IHC for EBV‐encoded latent membrane protein (LMP)‐1, EBV‐encoded nuclear antigen (EBNA)‐2, and EBV‐encoded BZLF1. In 95% of the cases, neoplastic cells with features of Hodgkin and Reed–Sternberg (HRS) cells were present, mostly showing expression of CD30. In all cases, the B‐cell phenotype was largely intact, and delineation from classic Hodgkin lymphoma (CHL) was further supported by myocyte enhancer factor 2B (MEF2B) detection. All tumour cells were EBER‐positive except in two cases. EBV latency type II was most frequent (89%) and type I was rare. Cases with latency type I were CD30‐negative. Five cases contained some BZLF1‐positive and/or EBNA‐2‐positive bystander lymphocytes.ConclusionsAs HRS morphology of neoplastic cells and CD30 expression are frequent features of EBV‐positive NLPHL, preservation of the B‐cell transcription programme, MEF2B expression combined with NLPHL‐typical architecture and background composition facilitate distinction from CHL. EBER ISH is the method of choice to identify these cases. The majority present with EBV latency type II, and only rare cases present with latency type I, which can be associated with missing CD30 expression. The presence of occasional bystander lymphocytes expressing BZLF1 and/or EBNA‐2 and the partial EBV infection of neoplastic cells in some cases could indicate that EBV is either not primarily involved or is only a transient driver in the pathogenesis of EBV‐positive NLPHL.
WHAT IS THE POLITICAL? A VIEW FROM THE “GLOBAL SOUTH”*
Authors: Majumdar Rochona 
Published: March 2022

ABSTRACTIn Elementary Aspects of the Political: Histories from the Global South, Prathama Banerjee challenges the ontological givenness of the category of the political in South Asian histories. The claim is surprising since the most productive interventions that heralded a postcolonial turn in the field have been preoccupied with analyses of colonialism, nationalism, political economy, and sovereignty. I situate Banerjee's claims in the context of her arguments about the meaning and appositeness of a decolonial historiography of the global South. This book is an attempt to break out from the postcolonial into a decolonial mode of history writing. While seemingly simple, stable, universal, and singular, the political, she argues, is anything but. How, then, do we recognize it? The political shows itself through a contest or comparison with that which is not identical to it, the nonpolitical. The semantic field of the political always includes “nonpolitical” and “extrapolitical” aspects such as the economic or the spiritual. And the concept of the political comes undone when we follow up on these connections. This essay analyzes the intellectual stakes of the book as a conceptual history of the political by analyzing its constituent “elements”: Self, Action, Idea, and People. I conclude with an assessment of it as a model for decolonial histories of the global South.
Dopamine toxicity contributes to melanocyte loss via melanocytorrhagy: an in vitro study
Authors: Tanwar Sushma , Thakur Vishal , Parsad Davinder 
Published: March 2022

AbstractBackgroundCatecholamines (epinephrine, norepinephrine, and dopamine) have been proposed as a possible cause of melanocyte loss. Dopamine has been observed to cause apoptosis in melanocytes via reactive oxygen species development, but the effect on melanocyte adhesion and proliferation still remains to be elucidated. Thus, we explored the dose‐ and time‐dependent toxicity of catecholamines and the effect of dopamine on the proliferation and adhesion potential of melanocytes.Materials and methodsPrimary culture of melanocytes was investigated in vitro for toxic effects of epinephrine, norepinephrine, and dopamine on metabolic activity via MTT [3‐(4,5‐dimethylthiazol‐2‐yl)‐2,5 diphenyl tetrazolium bromide) assay. Cells were observed microscopically for any phenotypic changes. Further cell proliferation, cell adhesion, and cell death pathway were explored under dopamine toxicity in dose‐ and time‐dependent manner with RNase/PI method of cell cycle analysis, cell adhesion assay, and Annexin V‐FITC/PI assay, respectively. Altered gene expressions were confirmed with a real‐time polymerase chain reaction.ResultsMetabolic activity of cells varied with time and different doses of epinephrine, norepinephrine, and dopamine. Dopamine was observed to be more toxic than epinephrine and norepinephrine. Melanocytes were observed to follow different cell death pathways at comparatively lower and higher concentrations of dopamine. Persistent exposure to dopamine resulted in decreased cell proliferation and adhesion potential with apoptotic changes. Gene expression changes also confirmed the weak adhesion and survival potential of cells under the toxic effects of dopamine.ConclusionDopamine can alter melanocytes' adhesion and survival potential, leading to apoptotic cell death or melanocytorrhagic loss.
The object allocation problem with favoring upper ranks
Authors: Feizi Mehdi 
Published: March 2022

AbstractWe introduce (strict) favoring upper ranks, which is an extension of favoring higher ranks for random assignments. We demonstrate that ex post favoring ranks implies (strict) favoring upper ranks, and envy‐freeness implies favoring upper ranks. Moreover, for at least four agents, no mechanism satisfies strict favoring upper ranks and either equal division lower bound or equal treatment of equals and lower invariance. Finally, we proved that the (modified) eating algorithm provides a random assignment that is (strict) favoring upper ranks.
Effect of Opuntia pulp as a clean label ingredient on techno‐functional properties of meat‐free burger
Authors: Shahiri Tabarestani Parisa , Kashiri Mahboobeh , Maghsoudlou Yahya , Shahiri Tabarestani Hoda , Ghorbani Mohammad 
Published: March 2022

SummaryThere is a universal increase from an animal‐based diet towards healthy plant‐based foods. Opuntia fruit pulp (OFP) as a pigment/hydrocolloid complex provides excellent functional properties. The aim of this study was the reformulation of the meat‐free burgers by using OFP (0.5, 1.5 and 2.5%) as a stabilised natural pigment and analysing techno‐functional characteristics of uncooked and cooked meat‐free burgers. The OFP was a rich source of polyphenols (35.3 ± 2.07 mgGAE/g) and carbohydrates (68.22 ± 0.5%). All uncooked treated burgers exhibited higher water‐holding capacity and redness as well as lower thiobarbituric acid reactive substance (TBARS) values. Results confirmed a higher cooking loss in the control sample (22.2 ± 0.63%) compared with the OFP‐treated burger at 1.5 and 2.5% content. Incorporation of OFP as a great source of natural pigments and phenolic components had considerably influenced cooking yield, moisture retention, juiciness and oxidative stability of meat‐free burgers. The lowest total colour difference value with resemble meat burger was observed in the treated burgers at the highest content of OFP. According to sensory evaluation, the overall acceptability of cooked burgers with 1.5% OFP was more satisfactory than other samples. Using this clean label ingredient provides a sustainable burger, which is beneficial to the public health, environment and animal welfare.