bluuebunny/crossref_metadata_2025_split huggingface dataset:
Genetics in Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency and Clinical Implications
Authors: Concolino Paola , Falhammar Henrik 
Published: January 2025

Abstract
Of all congenital adrenal hyperplasia (CAH), 95% to 99% is 21-hydroxylase deficiency (21OHD), an autosomal recessive disease. 21OHD is due to an insufficiency of 21-hydroxylase enzyme, which is encoded by the CYP21A2 gene and involved in cortisol and aldosterone production. The clinical presentation differs widely from severe classic to mild nonclassic CAH. 21OHD represents one of the most complex and at the same time intriguing topics in human genetics and its molecular diagnosis involves ongoing challenges. To provide a meticulous presentation of the topic, we searched the past and present literature, including original articles and reviews from PubMed, ScienceDirect, Web of Science, Embase, and Scopus, using search terms for genetics of 21OHD, 21OHD variants, molecular diagnosis of 21OHD, and 21OHD genetic testing. We offer a comprehensive review focusing on recent developments, new concepts, and conclusions.
Robotic Anatomical Segment VIII Resection for Hepatocellular Carcinoma Using the Scissor Hepatectomy Technique
Authors: Birgin Emrullah , Rahbari Nuh N. 
Published: January 2025

Abstract

Background
Robotic hepatectomy has been increasingly adopted for the treatment of hepatocellular carcinoma (HCC). However, the ideal technique of parenchymal transection in robotic hepatectomy has been a matter of ongoing debate in literature.

Patients and Methods
In this video, we demonstrate the technique of robotic anatomical segment VIII resection using the scissor hepatectomy technique1 for parenchymal transection on a 75-year-old male patient with a solitary HCC lesion. The patient had viral hepatitis (chronic hepatitis B infection) with a compensated liver cirrhosis and a new solitary 40 mm lesion on cross-sectional imaging. The AFP-negative lesion was biopsied, and the diagnosis of HCC was confirmed. A primary surgery was considered by a multidisciplinary tumor conference. We performed a pure robotic segmentectomy on the DaVinci Xi-system.

Results
An intrahepatic Glissonean pedicle approach was performed. Segment VIII pedicles were accessed and taken down with clips. Parenchymal transection was performed using robotic scissors with the intermittent Pringle maneuver and indocyanine green-fluorescence guiding. The middle hepatic vein and the right hepatic vein were preserved. There was a total blood loss of 300 ml. The postoperative course was uneventful. The patient was discharged on postoperative day 6 with no signs of recurrence on 3-month follow-up.

Conclusions
Robotic anatomical resection of segment VIII using the scissor hepatectomy technique is safe and feasible. Prospective studies are warranted to compare robotic parenchymal transection techniques using various devices.
Monacolin K as a Natural Alternative for Synthetic Statins
Authors: Kozakiewicz Julia , Okońska Aleksandra , Ziemba Michał , Kościelecki Kamil , Kalisz Agnieszka , Krakowiak Magdalena , Kaczmarek Aleksandra 
Published: January 2025

Introduction: This review paper aims to verify the safety of monacolin K, examine the effect on blood cholesterol levels, discuss the contribution to the reduction of cardiovascular risk and indicate examples of pleiotropic effects of this substance.
Materials and methods: A review of chosen literature in the PubMed database was conducted, using the following key words: ,,Monacolin K”, ,,Hypercholesterolemia’’, ,,Red yeast rice’’, ,,Supplementation’’.
Summary: Monacolin K is considered to be natural statin contained in fermented red rice or oyster mushroom. The use of monacolin K may contribute to the development of mild muscle pain or rhabdomyolysis, but it is noticed less frequently than in treatment with synthetic statins. In addition, there are also concerns about liver and/or kidney damage, headaches and/or dizziness, musculoskeletal symptoms and gastrointestinal disturbances in some people, especially when high doses of monacolin K are used. Monacolin K lowers the level of cholesterol in the blood and contributes to reducing the risk of cardiovascular diseases. Furthermore, monacolin K has anti-inflammatory effects and anti-cancer mechanisms.
Conclusions: Monacolin K improves the lipid profile by lowering low-density lipoprotein cholesterol (LDL-C), triglycerides (TG) and total cholesterol (TC) levels which indirectly reduces the cardiovascular risk with less frequently observed side effects. Monacolin K has many pleiotropic effects like anti-inflammatory and anti-cancer mechanisms by inhibiting cancer cell proliferation and stimulating apoptosis. It should be emphasized that the long-term safety of regular use of these preparations has not been fully documented. Some people taking these supplements experience side effects similar to those seen with statins. Both health benefits and side effects should be further investigated in future studies.
PODER NAVAL E O CLUSTER MARÍTIMO DA REGIÃO SUL DO BRASIL
Authors: Pinto Guilherme Penha , Ribeiro Erika Cristina Barbosa de Almeida , Ternus Cássia Heloísa 
Published: January 2025

O presente estudo objetiva identificar e analisar as potencialidades dos investimentos do Poder Naval no desenvolvimento dos nascentes clusters na região sul do Brasil, especialmente em Itajaí, Santa Catarina. Parte-se do levantamento da literatura sobre o tema, com foco nas economias em desenvolvimento como o Brasil e sua região costeira e, em um segundo momento, analisam-se alguns dados sobre a indústria de grandes embarcações no país e mais especificamente na região sul. Diversos estudos sobre Economia de Defesa e Economia do Mar servirão como base para analisar o estágio de desenvolvimento da “clusterização” na indústria de Defesa Naval da região, tendo em vista os recentes investimentos do Programa Fragatas Classe Tamandaré, sendo uma análise seminal para ensejar o acompanhamento dos impactos das alocações de Defesa nos médio e longo prazos pelo meio acadêmico especializado.
Efeitos espaciais da incerteza e desenvolvimento regional: o caso do Rio de Janeiro - 2000 a 2010
Authors: Gama Fabio , Bastos Suzana , Azevedo Caio , De Paula Teófilo 
Published: January 2025

A partir do arcabouço teórico pós-keynesiano da moeda endógena, este trabalho avalia os efeitos espaciais da incerteza – preferência pela liquidez dos bancos e do público – na atividade econômica de 87 municípios do Estado do Rio de Janeiro (RJ), entre 2000 a 2010. São utilizadas técnicas de análise fatorial, análise de agrupamento e painel espacial para estimar os efeitos espaciais da incerteza sobre o desempenho econômico de diferentes grupos de municípios. As evidências indicam que os municípios periféricos, quando comparados aos municípios centrais, se mostram mais vulneráveis às variações internas e externas na incerteza bancária.
confronting adultcentrism: childist and decolonial interventions in educational philosophies and institutions
Authors: Biswas Tanu , Rollo Toby 
Published: January 2025

'Confronting Adultcentrism: Childist and Decolonial Interventions in Educational Philosophies and Institutions' explores the transformative potential of childism as an orientation to critique and transform adultism and coloniality within education. Edited by Tanu Biswas and Toby Rollo, the special dossier builds on the Childism Institute’s discussions to center children as epistemic agents and challenge the structural marginalization of childhood in socio-political and philosophical spheres. Childism deconstructs and critiques adult-centric biases, proposing a profound shift toward recognizing the interdependence of childhood and adulthood, and unique relational perspectives as vital to intergenerational justice. The articles examine how traditional (euro-centric) educational systems reinforce adultist hierarchies, viewing children as incomplete beings in a preparatory phase called 'childhood'. They advocate for reimagining educational systems with age-inclusive pedagogical relationships where children participate as active contributors. Key themes include democratic education and liberation as an intergenerational project,, decolonial critiques of childhood philosophies underlying educational practices, and the role of children as epistemic agents and activists. Some contributions argue for dismantling adultist structures and integrating children and childhood into democratic and liberatory frameworks. Others explicitly link adultism to coloniality, showing how Eurocentric colonial projects infantilized colonized peoples. The articles extend childism’s reach to address historical legacies of racialized and epistemic oppression including explorations of racialized imageries of childhood. Collectively the dossier advances childism as a timely intervention for reshaping education, philosophy, and policy, embracing children’s present contributions and fostering intergenerational equity.
Sensing characteristics of integrated fiber Bragg grating sensors for high-temperature strain measurement
Authors: Wang Chen , Zhang Yumin , Huang Qisheng , Luo Zhiwei , Gao Yi 
Published: January 2025

This study explored the response characteristics of fiber optic
high-temperature strain sensors to axial strains under varying thermal
conditions. The sensor assembly integrated a Type II fine-core pure
silica fiber Bragg grating (FBG) with a Type I Ge-doped regenerative
fiber Bragg grating (RFBG). The matrix’s condition number was
optimized by leveraging differential sensitivities to temperature and
strain, combined with 2×2 matrix demodulation techniques,
resulting in a substantial improvement in measurement accuracy. The
sensor was found to exhibit good linearity and repeatability in strain
response under conditions up to 550°C. Below 550°C, the
temperature uncertainty was determined to be 5.49°C, and the
strain uncertainty was 38.05 µɛ. However, the
repeatability significantly decreased when the temperature exceeded
550°C, likely due to the viscoelastic behavior of the FBG at
high temperatures. Therefore, for reliable measurements, it is
recommended to use the high-temperature strain sensor for simultaneous
strain and temperature monitoring in environments not exceeding
550°C.
Tunable broadband terahertz absorber based on a simple design of a vanadium dioxide resonator
Authors: Wang Yunji , Gu Yao , Liu Fei , Chen Lin , Wang XingChao , Ji Ke 
Published: January 2025

A tunable broadband terahertz absorber based on five square rings of vanadium dioxide (VO2) is proposed in this paper. We use a CST software to simulate the absorption characteristics of the absorber. The results show that the bandwidth of the absorber, with absorption exceeding 90%, reached 2.51 THz from 2.71 to 5.22 THz. The electric and surface current distributions reveal that the high absorption is due to the dipole resonance and coupling of the square rings. The absorption peak can be approximately tuned from 2% to 99.5% by varying the conductivity of VO2 from 200 S/m to 200,000 S/m. In addition, the absorber has polarization insensitivity and can maintain good absorption performance over a wide range of incident angles. The designed absorber is expected to be widely used in areas of terahertz communication, imaging, and detection.
The international trial of nasal oxygen therapy after cardiac surgery (NOTACS) in patients at high risk of postoperative pulmonary complications: Economic evaluation protocol and analysis plan
Authors: Shetty Siddesh , Duckworth Melissa , Norman Richard , Affandi Jacquita , Dawson Sarah , Fox-Rushby Julia ,   On behalf of NOTACS investigators,   On behalf of NOTACS Trial Steering Committee,   On behalf of NOTACS DMEC Committee
Published: January 2025

Introduction
High-Flow Nasal Therapy (HFNT) is an innovative non-invasive form of respiratory support. Compared to standard oxygen therapy (SOT), there is an equipoise regarding the effect of HFNT on patient-centred outcomes among those at high risk of developing postoperative pulmonary complications after undergoing cardiac surgery. The NOTACS trial aims to determine the clinical and cost-effectiveness of HFNT compared to SOT within 90 days of surgery in the United Kingdom, Australia, and New Zealand. This protocol describes the methods and analyses planned for economic evaluation embedded within the ongoing NOTACS trial.

Methods and analysis
The economic evaluation will identify, measure and value resources and health outcomes in both trial arms and compare changes in costs with ‘days alive and at home’ and EQ-5D-5L quality adjusted life years (QALYs) from the perspective most relevant to the decision-making country. Results from pooling data across the trial will use health and social care sector perspective. All patient-specific data including hospital/community care and health outcomes will be collected prospectively. Unit costs will be sourced from national, published or local data. Missing data will be assessed, with values replaced depending on assumed mechanism of missingness, and impact of replacement on cost-effectiveness assessed. Costs and outcomes by trial arm will be presented as components and totals per patient using a range of descriptive statistics. Regression models for costs and effects will account for patient characteristics, quality of life and health service utilization at baseline. Uncertainty in parameters, sampling and heterogeneity will be addressed through deterministic, probabilistic and subgroup analyses to assess the impact of varying methods and assumptions for costs, outcomes and approaches used in base-case analysis. Results will be interpreted using recommended national cost-effectiveness thresholds.

Registration details
The study is registered with ISRCTN (ISRCTN14092678) on 13/05/2020. ISRCTN is a primary registry of the WHO ICTRP network and includes all items from the WHO Trial Registration data set.
Assessing the influence of the modifiable areal unit problem on Bayesian disease mapping in Queensland, Australia
Authors: Jahan Farzana , Haque Shovanur , Hogg James , Price Aiden , Hassan Conor , Areed Wala , Thompson Helen , Cameron Jessica , Cramb Susanna M. 
Published: January 2025

Background
Spatial data are often aggregated by area to protect the confidentiality of individuals and aid the calculation of pertinent risks and rates. However, the analysis of spatially aggregated data is susceptible to the modifiable areal unit problem (MAUP), which arises when inference varies with boundary or aggregation changes. While the impact of the MAUP has been examined previously, typically these studies have focused on well-populated areas. Understanding how the MAUP behaves when data are sparse is particularly important for countries with less populated areas, such as Australia. This study aims to assess different geographical regions’ vulnerability to the MAUP when data are relatively sparse to inform researchers’ choice of aggregation level for fitting spatial models.

Methods
To understand the impact of the MAUP in Queensland, Australia, the present study investigates inference from simulated lung cancer incidence data using the five levels of spatial aggregation defined by the Australian Statistical Geography Standard. To this end, Bayesian spatial BYM models with and without covariates were fitted.

Results and conclusion
The MAUP impacted inference in the analysis of cancer counts for data aggregated to coarsest areal structures. However, area structures with moderate resolution were not greatly impacted by the MAUP, and offer advantages in terms of data sparsity, computational intensity and availability of data sets.
Burnout and organisational stressors among healthcare staff working with adults with intellectual disabilities in Ireland
Authors: Clancy Patrick , Cassarino Marica 
Published: January 2025

Background
The associations between organisational stressors and burnout among healthcare staff working with adults with intellectual disabilities are underexplored. This study investigated rates of burnout and associated stressors among Irish healthcare workers during the COVID-19 pandemic.

Materials and methods
A convenience sample of 329 Irish frontline staff supporting adults with intellectual disabilities completed a survey assessing personal, work-related, and client-related burnout, and organisational stressors. Quantitative correlational analysis assessed bivariate and multi-variate associations, while qualitative accounts were analysed thematically.

Results
Compared to international data, we observed very high levels of personal and work-related burnout, with lower levels of client-related burnout. “Lack of resources” was the stressor holding the strongest association with burnout, followed by “bureaucracy” and “work-home conflict”. Qualitative responses highlighted the negative impact of the pandemic on workload, service quality, and staff wellbeing.

Discussion
Our findings highlight an important association between organisational stressors and burnout among frontline staff, suggesting the potential benefit of designing organisationally focused interventions to reduce stress and promote staff wellbeing.
The liminal space between hope and grief: The phenomenon of uncertainty as experienced by people living with relapsing-remitting multiple sclerosis
Authors: van Reenen Eva C. , van Nistelrooij Inge A. M. , Visser Leo H. , de Beukelaar Janet W. K. , Frequin Stephan T. F. M. , Niemeijer Alistair R. 
Published: January 2025

Background
People with the chronic disease Multiple Sclerosis are subjected to different degrees of profound uncertainty. Uncertainty has been linked to adverse psychological effects such as feelings of heightened vulnerability, avoidance of decision-making, fear, worry, anxiety disorders, and even depression. Research into Multiple Sclerosis has a predominant focus on the scientific, practical, and psychosocial issues of uncertainty. In comparison, existential uncertainty has been under-researched, even though it might pose a greater burden to those experiencing it.

Objective
To understand the lived experience of uncertainty for people living with relapsing-remitting Multiple Sclerosis.

Methods
This study followed a phenomenological research design, employing elements of both the Reflective Lifeworld Approach and Phenomenology of Practice. Seventeen people with a recent (<1 year) diagnosis of relapsing-remitting Multiple Sclerosis were included. In-depth interviews were conducted immediately after inclusion.

Results
The lived experience of uncertainty can be described as a stumbling motion across the liminal space between hope and grief while dealing with oscillating feelings of unrest concerning the body, self, and others. The following four constituents further illuminate the meaning of the phenomenon: Having to constantly (re)define unfamiliar and intangible bodily changes on one’s own; Unsteady navigating amidst a destabilization of the imagined life; Relating to others as a source of, mirror or buffer for uncertainty; Going through overwhelming fears and worries while clinging to one’s own logic.

Conclusion
Adding to existing qualitative and phenomenological research into MS and theories on uncertainty, this study portrays uncertainty as a multifaceted experience. The findings imply the need for a continuous attunement of healthcare practitioners to the expectations, fears, avoidance techniques, and other uniquely personal circumstances of people with Multiple Sclerosis.
Racial disparities in extended venous thromboembolism prophylaxis after hysterectomy
Authors: Wu Wenbo , Wu Sherry , Berlene Mariano Sim , Burney Richard E. , Kuriakose Jonathan P. 
Published: January 2025

Background
Venous thromboembolism (VTE) is a significant preventable cause of postoperative morbidity and mortality after major abdominopelvic surgery that calls for extended VTE prophylaxis (eVTEp). Literature suggests that significant racial disparities may exist in post-operative care.

Objective
The study sought to examine if racial disparities exist in the administration of eVTEp after hysterectomy in a statewide collaborative.

Methods
We conducted a retrospective cohort study of post-hysterectomy patients across 69 hospitals in the Michigan Surgical Quality Collaborative from January 2016 to February 2020. The variable of interest was race (Black/African or White American). The primary outcome was administration or absence of eVTEp. Descriptive statistics and mixed effects logistic regression were performed for risk adjustment with covariates such as age, cancer occurrence, inflammatory bowel disease, American Society of Anesthesiologists physical status classification, perioperative VTE prophylaxis, postoperative VTE prophylaxis, surgical approach, and surgical duration, among other variables.

Results
In total, 24,513 patients underwent hysterectomy. Of these patients, 1,107 (4.45%) received eVTEp, 153 (13.24%) of which were Black and 954 (82.53%) of which were White. Mixed effects logistic regression analysis suggested that Black patients were significantly less likely to receive eVTEp than White patients (odds ratio = 0.776; 95% CI: 0.615–0.979; P = 0.039). Additionally, tobacco use, coronary artery disease, bleeding disorder, cancer occurrence, functional status, perioperative VTE prophylaxis, surgical duration, length of stay, and surgical approach were associated with a higher likelihood of receiving eVTEp.

Conclusion
eVTEp is recommended for the prevention of post-discharge VTE in select patients after hysterectomy. Regression analysis showed that, compared to their White counterparts, Black females were significantly less likely to receive eVTEp. The underlying reasons for this disparity require further investigation into possible socioeconomic influences and inherent biases.
The Rise of the Graphic Novel: Computational Criticism and the Evolution of Literary Value, Alexander Dunst (2023)
Authors: Williams Paul 
Published: January 2025

Review of: The Rise of the Graphic Novel: Computational Criticism and the Evolution of Literary Value, Alexander Dunst (2023)
Cambridge: Cambridge University Press, xiv + 240 pp.,
ISBN 978-1-00918-293-5, h/bk, £85.00
Az első lépések az irodalmi tanulás területén
Authors: Szilvássy Orsolya 
Published: January 2025

A vizuális és irodalmi literáció kezdeti szakaszában megjelenő képeskönyv-befogadásra akkor nyílik lehetőség, amikor a kisgyermek bizonyos figyelmi képességekkel rendelkezik. A tanulmány bevezetésében először áttekintem a figyelmi folyamatok és a képeskönyvbefogadás kapcsolatát a legfiatalabb befogadók körében, mert már itt találhatók olyan összefüggések, amelyek a későbbiek során is szerepet játszanak (habituáció - diszhabituáció, a figyelem alakításának, motiválásának társas, kulturális keretei). Majd a figyelemirányítás médiumspecifikus eljárásaival foglalkozom a narratíva reprezentációja, illetve a foregrounding és az irodalmiság összefüggései vonatkozásában. Ezen belül abból indulok ki, hogy a képeskönyvben a kép és a szöveg külön-külön is hozzájárulnak a kognitív hangsúlyok megteremtéséhez, a közöttük lévő kapcsolatok a stilisztikailag semleges párhuzamos elbeszéléstől a stilisztikailag előtérbeállított ellenpontozó vagy ellentmondó kapcsolatokig terjed. Mivel az utóbbiak esetében az eltérő narrátori szólamokhoz ellentétes nézőpontok is társulnak, a sajátos nézőpontok alkalmazása az egyik legelterjedtebb eszköze a gyerekképeskönyveknek arra, hogy a célközönségben jelentős kognitív változást érjenek el.
A vizsgált példák egyrészt azt támasztják alá, hogy a képeskönyvek általában igyekeznek a még tapasztalatlan befogadó figyelmét egyértelmű módon irányítani, ugyanakkor egyes darabok arra is törekszenek, hogy a befogadói elvárásokat, rutinokat szokatlanabb technikák használatával újítsák meg és nagyobb teret engedjenek a befogadói aktivitásnak. Mivel a szokatlan stiláris elemek figyelemkoncentrációt váltanak ki a befogadókban, jelentős szerepet játszanak az értelmezői készségek, az irodalmi kompetenciák elsajátításában is.