bluuebunny/crossref_metadata_2025_split huggingface dataset:
Nonmetastatic Rhabdomyosarcoma in Children and Adolescents: Overall Results of the European Pediatric Soft Tissue Sarcoma Study Group RMS2005 Study
Authors: Bisogno Gianni , Minard-Colin Veronique , Zanetti Ilaria , Ferrari Andrea , Gallego Soledad , Dávila Fajardo Raquel , Mandeville Henry , Kelsey Anna , Alaggio Rita , Orbach Daniel , Terwisscha van Scheltinga Sheila , Guillén Burrieza Gabriela , Ben-Arush Myriam , Glosli Heidi , Mudry Peter , Ferman Sima , Devalck Christine , Defachelles Anne Sophie , Merks Johannes Hendrikus Maria , Jenney Meriel 
Published: February 2023

Clinical trials frequently include multiple end points that mature at different times. The initial report, typically based on the primary end point, may be published when key planned co-primary or secondary analyses are not yet available. Clinical Trial Updates provide an opportunity to disseminate additional results from studies, published in JCO or elsewhere, for which the primary end point has already been reported. The RMS2005 study included two phase III randomized trials for high-risk (HR) and observational trials for low (LR), standard (SR), and very high-risk (VHR) patients who have been partially reported. Herein, we present a comprehensive report of results achieved for the complete unselected nonmetastatic cohort and analyze the evolution of treatment in comparison with previous European protocols. After a median follow-up of 73.1 months, the 5-year event-free survival (EFS) and overall survival (OS) of the 1,733 patients enrolled were 70.7% (95% CI, 68.5 to 72.8) and 80.4% (95% CI, 78.4 to 82.3), respectively. The results by subgroup: LR (80 patients) EFS 93.7% (95% CI, 85.5 to 97.3), OS 96.7% (95% CI, 87.2 to 99.2); SR (652 patients) EFS 77.4% (95% CI, 73.9 to 80.5), OS 90.6% (95% CI, 87.9 to 92.7); HR (851 patients) EFS 67.3% (95% CI, 64.0 to 70.4), OS 76.7% (95% CI, 73.6 to 79.4); and VHR (150 patients) EFS 48.8% (95% CI, 40.4 to 56.7), OS 49.7% (95% CI, 40.8 to 57.9). The RMS2005 study demonstrated that 80% of children with localized rhabdomyosarcoma could be long-term survivors. The study has established the standard of care across the European pediatric Soft tissue sarcoma Study Group countries with the confirmation of a 22-week vincristine/actinomycin D regimen for LR patients, the reduction of the cumulative ifosfamide dose in the SR group, and for HR disease, the omission of doxorubicin and the addition of maintenance chemotherapy.
Serum Succinate/Fumarate Ratio in Patients With Paraganglioma/Pheochromocytoma Attending an Endocrine Oncogenetic Unit
Authors: Bancel Léo-Paul , Masso Vincent , Dessein Anne-Frederique , Aubert Sébastien , Leteurtre Emmanuelle , Coppin Lucie , Odou Marie-Françoise , Cao Christine Do , Cardot-Bauters Catherine , Pigny Pascal 
Published: February 2023

Abstract

Context
Pheochromocytomas and paragangliomas (PPGLs) with SDHx pathogenic variants (PVs) are characterized by a higher intratissular succinate/fumarate ratio (RS/F) than non-SDHx–mutated ones. Also, an increase in serum succinate levels has been reported in patients with germline SDHB or SDHD PV.

Objective
To assess whether measurement of serum succinate, fumarate levels, and RS/F might aid identification of an SDHx germline PV/likely pathogenic variant (LPV) in patients with PPGL or in asymptomatic relatives; and to guide identification of a PV/LPV among the variants of unknown significance (VUS) identified in SDHx by next-generation sequencing.

Methods
This prospective monocentric study included 93 patients attending an endocrine oncogenetic unit for genetic testing. Succinate and fumarate were measured in serum by gas chromatography coupled to mass spectrometry. The RS/F was calculated to assess SDH enzymatic function. Diagnostic performance was assessed by receiver operating characteristic analysis.

Results
RS/F had a higher discriminant power than succinate alone to identify an SDHx PV/LPV in patients with PPGL. However, SDHD PVs/LPVs are frequently missed. Only RS/F differed between asymptomatic SDHB/SDHD PV/LPV carriers and SDHB/SDHD-linked patients with PPGL. Finally RS/F could be helpful to easily evaluate the functional impact of VUS in SDHx.

Conclusion
Measurement of serum RS/F in patients with PPGL and in asymptomatic relatives is a valuable initial workup tool to detect those carrying a germline PV/LPV in SDHx. Its discriminative power is equal or superior to those of succinate measured alone. SDHD PVs/LPVs are less frequently identified by these biochemical tools. Use of RS/F for SDHx VUS reclassification needs to be evaluated further.
Crip Genealogies
Authors: Pickens Therí Alyce 
Published: February 2023

The contributors to Crip Genealogies reorient the field of disability studies by centering the work of transnational feminism, queer of color critique, and trans scholarship and activism. They challenge the white, Western, and Northern rights-based genealogy of disability studies, showing how a single coherent narrative of the field is a mode of exclusion that relies on logics of whiteness and imperialism. The contributors examine how disability justice activists work in concert with other social justice projects, explore crip environments, create alternate disciplinary genealogies, and reject notions of the model minority. Throughout, they demonstrate how the mandate for a single genealogy of the discipline whitewashes disability and continues forms of violence. By cripping disability studies, the contributors allow for divergent histories, the coexistence of anti-ableist and antiracist theorizing, and a radically just and capacious understanding of disability.
Contributors. Suzanne Bost, Mel Y. Chen, Sony Coráñez Bolton, Natalia Duong, Lezlie Frye, Magda García, Alison Kafer, Eunjung Kim, Yoo-suk Kim, Kateřina Kolářová, James Kyung-Jin Lee, Stacey Park Milbern, Julie Avril Minich, Tari Young-Jung Na, Therí A. Pickens, Leah Lakshmi Piepzna-Samarasinha, Jasbir K. Puar, Sami Schalk, Faith Njahîra Wangarî
Translational landscape in human early neural fate determination
Authors: Yan Chenchao , Meng Yajing , Yang Jie , Chen Jian , Jiang Wei 
Published: February 2023

ABSTRACT
Gene expression regulation in eukaryotes is a multi-level process, including transcription, mRNA translation and protein turnover. Many studies have reported sophisticated transcriptional regulation during neural development, but the global translational dynamics are still ambiguous. Here, we differentiate human embryonic stem cells (ESCs) into neural progenitor cells (NPCs) with high efficiency and perform ribosome sequencing and RNA sequencing on both ESCs and NPCs. Data analysis reveals that translational controls engage in many crucial pathways and contribute significantly to regulation of neural fate determination. Furthermore, we show that the sequence characteristics of the untranslated region (UTR) might regulate translation efficiency. Specifically, genes with short 5′UTR and intense Kozak sequence are associated with high translation efficiency in human ESCs, whereas genes with long 3′UTR are related to high translation efficiency in NPCs. In addition, we have identified four biasedly used codons (GAC, GAT, AGA and AGG) and dozens of short open reading frames during neural progenitor differentiation. Thus, our study reveals the translational landscape during early human neural differentiation and provides insights into the regulation of cell fate determination at the translational level.
The irritable bowel syndrome among adults in Qatif, Saudi Arabia: prevalence and impact on health-related quality of life, by gender and age
Authors: Alnasser Ali Hassan A. , Al Kalif Mohammed Sheker H. , Alrowaila Muneera Ahmed O. , Alhomaidi Fatimah Mahdi A. , Alalsayedsalih Hasheem Murtada S. , Alkabah Ali Ahmad A. , Almanyan Ali Jaffar Q. , Al Zawwad Ali Hasan M. , Alalwan Hussain Tawfeeq A. , Bu Hassan Ahmed Tariq T. , Al Abbad Komail Abdulaziz S. , Alibrahim Hassan Ayman H. , Awkar Ali Abdullah D. , Altawfiq Kauthar Jaffar A. , Almosa Fadel Ali M. 
Published: February 2023

(1) Background: Even though irritable bowel syndrome (IBS) is not life-threatening, it can have a significant impact on a patient's daily activities and quality of life. Studies indicate that IBS is more prevalent in women than in men. The mechanism, etiology, and signs of IBS are not well understood, but there is evidence that gender and age is a biological characteristic that affects these subjects. This influence on the quality of life of Saudi adults by gender and age has not previously been compared. (2) Methods: A snowball sampling techniques of 279 people in Qatif, Saudi Arabia, is surveyed online to assess health-related quality of life (HRQoL). The Short SF-36 was self-administered to assess HRQoL; logistic regression models, Mann-Whitney, and the Kruskal-Wallis were used. SPSS version 23.0 was used to store and analyze all data. (3) Results: The prevalence of IBS among Saudi adults was 17.6%. IBS has a statistically significant association with age and gender which is more common among older women. IBS participants have poorer HRQoL than the overall population, particularly in Physical Component Score (PCS). (4) Conclusions: IBS is more prevalent among Saudi adults in Qatif and has a significant negative impact on the HRQoL, particularly among elderly women.
Experimental assessment of barely visible impact damage carbon fibre reinforced epoxy composite using ultrasound method
Authors: Tan Yee Vien , Lai Wei Liang , Saeedipour Hamid , Adul Majid Mohd Shukry , Goh Kheng Lim 
Published: February 2023

Background: Carbon fibre reinforced epoxy (CFRP) is susceptible to impact damage which could resulted in reduction of the mechanical properties. This paper studies the architecture of barely visible impact damage (BVID) to comprehend the extent of damage on quasi-isotropic CFRP laminates of varying thickness (i.e. 16, 24 and 32-ply laminates of 3, 4 and 5 mm respectively). Methods: Quasi-static indentation is chosen to produce BVID on CFRP laminates, followed by using non-destruction evaluation method, namely conventional contact-type ultrasonic testing (UT) and C-mode scanning acoustic microscopy (C-SAM) method. Results: The findings revealed (1) the size and shapes of the BVID on CFRP laminates, (2) no damage found at the point of damage, and (3) the bridging between the point of impact to the outer damaged diameter due to the consequence of diverse orientation of carbon fibre strips which exhibit excellent mechanical properties before structural failure. Conclusions: The results concluded that the UT and C-SAM method can identify both the pristine region and the internal damaged structures in CFRP laminates.
A hitchhikers’ guide to the terminology of accreditation processes for health professionals and institutions
Authors: Sousa Nuno , Santa-Cruz Andre , Melo Antonio , Sousa Camila , Marques Fernanda , Leite-Almeida Hugo , Souza Lucimara , Marangoni Marco , Raia Marcia , Santos Maurilio , Goncalves Nuno , Morgado Pedro , Souza Pedro , Matos Sousa Rita , Gomes Sara , Santos Wilfredo , Araújo Beatriz , Amaral Eliana , Pereira Vitor , Scoles Peter ,   MAPS
Published: February 2023

Accreditation processes for health care professions are designed to ensure that individuals and programs in these fields meet established standards of quality and effectiveness. The accelerating pace of globalization in the health care professions has increased the need for a shared understanding of the vocabulary of evaluation, assessment, and accreditation. The psychometric principles of valid and reliable assessment are commonly accepted, but the terminology is confusing. We believe that all stakeholders – evaluators, faculty, students but also the community – will benefit from a shared language and common set of definitions. We recognize that not all readers will agree with the definitions we propose, but we hope that this guide will help to ensure clarity, consistency, transparency, and fairness, and that it will promote through the stimulation of a debate greater collaboration across national and international boundaries.
as práticas adultocêntricas nas políticas públicas destinadas às infâncias no brasil: pistas para aproximações de um devir-erê
Authors: Fim Matheus magno Dos santos , César Janaína Mariano 
Published: February 2023

This article problematizes work practices with children, analyzing especially adult-centric practices and the ways in which they characterize public policies aimed at childhood in Brazil. Our study was based on the cartography method, which made it possible to monitor the uncertain psychosocial landscapes caused by the Covid-19 pandemic. It used methodological concepts from Institutional Analysis and the Sociology of Childhood, which intersected with elements of Afroperspectivist philosophy, such as represented among the Ibeji and the Erês. The methodological tool adopted was the Conversation Circle, carried out online, which operated as a group apparatus characterized by welcoming and sharing, where it was possible to gather and analyze data on adult-centric practices among workers from different public agencies in health, social assistance and education, aimed at children in the state of Espírito Santo. Research diaries were also produced, which were referred to in the analytic process. The text identifies several aspects of Erean adult’s views of childhood, and offers insight into the knowledge and practices that contribute to the process of becoming-Erê, a possibility that can be exercised at any stage of life.
Tchinguilile: infâncias da Ganda Crianças “de lá”
Authors: Souza Adelaide Rezende 
Published: February 2023

Recently, an increasing number of researchers in different parts of the world have been studying childhood from a point of view that highlights children who are considered “peripheral” – far from the ideal model associated with Western countries. We began with the intention of getting to know African children in Angola, informed by the belief that the preservation of languages is important in maintaining social knowledge and traditions. This text describes a preliminary study carried out in a culturally rich location with strong traditions, but which is also marked by processes of cultural denial and devaluation. According to Ndombele and Timbane (2020), most Angolan children learn Portuguese at age seven, when they start school, especially in rural areas. This fact hinders the literacy learning process, and presents the risk of the disappearance of some native languages. Many schools teach children only in Portuguese. When visiting a school in Ganda, a rural area of Benguela, we found a group of children who, primarily through the games they played, keep the Umbundu language alive, and with that the identity and culture of their ancestors. Building on this, these children and their teachers created a syllable association game based on the music sung as they played. The game had two modalities - in the Umbundu language and in the Portuguese language – and functioned as a bilingual pedagogical tool. In the initial moment of offering the game for testing with the children, it was discovered that by singing the song of the game and discussing the possible solutions by themselves, they were able to make correct associations of the syllables in Umbundu, which they then translated into Portuguese. Thus, a resource was created together with the teachers, which, in addition to valuing the native language of the population, allowed the use of children's knowledge to create a didactic resource. In addition, it was possible to encounter the concept of childhood in the current world, deconstructing the idea of a single and ideal model, especially in light of those children who bring habits from home that do not always harmonize with the school routine, and often clash with Euro/Western model, which is based on age separation of classes as a requirement to guarantee learning, and tend to devalue other exchanges that help children learn.
por um horizonte público na educação das crianças: um diálogo com Hannah Arendt
Authors: Araújo Vania Carvalho de , Auer Franceila 
Published: February 2023

This paper documents an encounter with some issues that deal with the education of children as mentioned by Hannah Arendt in her essay "The crisis in education." Arendt’s invocation of "the obligation that the existence of children imposes on the whole society" is a key element of an ethical and political commitment to the education of new generations—especially in a world disillusioned by the estrangement of the human, and in which the undeniable criteria that the past – as authority – bequeathed to us as support for action and judgment on the world has faltered. If education should not be entirely responsible for the world, at least it should be able to insert children into a public culture that allows them, in turn, to appropriate it. The issues addressed by Arendt, although full of criticism and redolent with paradoxes, move us to think that only an education founded on a public horizon is able to educate children in a way that allows them the opportunity to act in the world, and to be able to discern civilization from barbarism, the fair from the unfair, truth from lies, and good from evil. On the one hand, her reflections reveal the utopian aspect of her thought, while on the other they make clear the collective responsibility that the arrival of children in a pre-existing world imposes on every human society.
Identity erasure: women’s experiences of living with domestic violence and abuse
Authors: Neale Jo 
Published: February 2023

This article explores the process of ‘identity erasure’ that is a feature of domestic violence and abuse. Data are taken from semi-structured narrative-style interviews with 14 women who had experienced abuse from a male partner.
I draw on Erving Goffman’s (1968) work on ‘total institutions’. Goffman uses the term ‘mortification’ in describing the attacks on identity and self that occur in ‘total institutions’ such as psychiatric hospitals, prisons and concentration camps. These attacks take the form of: loss of contact with the outside world, ritual degradation, the removal of possessions, and lack of control. Their effect is to erase the inmate’s prior identity, and render them compliant.
For the women who participated in this study, their abusers attempted to achieve compliance by adopting many of the tactics that Goffman describes. Drawing on participants’ words, I discuss some of the behaviours adopted by their abusive partners: isolation from support networks, surveillance, deprivation of privacy, and dispossession. I argue that, for women, the home/abusive relationship becomes a total institution. Understanding the abusive household as a ‘total institution’ can help friends, family and professionals to more fully appreciate, and therefore provide women with more appropriate help to overcome, the barriers to leaving.
Photon propagation control on laser-written photonic chips enabled by composite waveguides
Authors: Li Ze-Zheng , Tian Zhen-Nan , Li Zhong-Tian , Ouyang Yang , Chen Qi-Dai , Sun Hong-Bo 
Published: February 2023

Femtosecond laser direct writing (FsLDW) three-dimensional (3D) photonic integrated circuits (PICs) can realize arbitrary arrangement of waveguide arrays and coupling devices. Thus, they are capable of directly constructing arbitrary Hamiltonians and performing specific computing tasks crucial in quantum simulation and computation. However, the propagation constant β is limited to a narrow range in single-mode waveguides by solely changing the processing parameters, which greatly hinders the design of FsLDW PICs. This study proposes a composite waveguide (CWG) method to increase the range of β, where a new single-mode composite waveguide comprises two adjacent circular waveguides. As a result, the photon propagation can be controlled and the variation range of β can be efficiently enlarged by approximately two times (Δβ∼36  cm−1). With the CWG method, we successfully realize the most compact FsLDW directional couplers with a 9 μm pitch in a straight-line form and achieve the reconstruction of the Hamiltonian of a Hermitian array. Thus, the study represents a step further toward the fine control of the coupling between waveguides and compact integration of FsLDW PICs.
A systematic review of influences on implementation of supported self-management interventions for people with severe mental health problems in secondary mental health care settings
Authors: Islam Samihah , Appleton Rebecca , Hutchings-Hay Chloe , Lloyd-Evans Brynmor , Johnson Sonia 
Published: February 2023

PurposeThere is robust evidence for offering supported self-management interventions for people with severe mental illness (SMI) throughout secondary mental health services, but their availability remains patchy. The aim of this systematic review is to synthesise the evidence on barriers and facilitators to implementing self-management interventions for people with SMI in secondary mental health care settings.MethodsThe review protocol was registered with PROSPERO (CRD42021257078). Five databases were searched to identify relevant studies. We included full-text journal articles with primary qualitative or quantitative data on factors which affect the implementation of self-management interventions for people with SMI in secondary mental health services. The included studies were analysed using narrative synthesis, using the Consolidated Framework for Implementation Research and an established taxonomy of implementation outcomes.ResultsTwenty-three studies from five countries met eligibility criteria. The barriers and facilitators identified in the review were mainly on the organisational level, but included some individual-level influences. Facilitators included high feasibility, high fidelity, a strong team structure, sufficient number of staff, support from colleagues, staff training, supervision, the presence of an implementation champion and adaptability of the intervention. Barriers to implementation include high staff turnover, staff shortage, lack of supervision, lack of support for staff delivering the programme, staff struggling with their increased workload, a lack of senior clinical leadership, and programme content perceived as irrelevant.ConclusionThe findings from this research suggest promising strategies to improve implementation of self-management interventions. For services providing support for people with SMI, organisational culture should be considered, as well as the adaptability of interventions.