bluuebunny/crossref_metadata_2025_split huggingface dataset:
Identification of novel compound heterozygous LRBA mutations associated with recurrent hemophagocytic lymphohistiocytosis and CNS manifestations
Authors: Sham Marina , Merico Daniele , Pereira Myra , Roifman Chaim M. , Roifman Maian 
Published: March 2023

Background: Lipopolysaccharide-responsive beige-like anchor (LRBA) is an intracellular protein that regulates the recycling of cytotoxic T lymphocyte-associated protein 4 (CTLA4), an immune checkpoint molecule which prevents ongoing activation of T cells. Deficiency of LRBA results in increased trafficking and degradation of CTLA4, and consequently, uncontrolled T cell responses. The phenotypic spectrum of LRBA deficiency arising from biallelic loss-of-function typically includes recurrent infections, autoimmunity, lymphoproliferation, chronic diarrhea, hypogammaglobulinemia, and cytopenia. Aim: To report an atypical presentation of LRBA deficiency arising from a set of compound heterozygous LRBA variants, encompassing recurrent hemophagocytic lymphocytosis (HLH) and neurological manifestations. Methods: Clinical data was gathered through retrospective chart review. Expanded genetic analysis including whole exome sequencing was performed. Results: Our patient initially presented at age 15 months with fever, seizures, and encephalopathy. HLH-work-up showed bicytopenia, elevated ferritin and triglyceride, and low fibrinogen, however, he did not yet meet the diagnostic criteria for HLH. MRI brain and EEG at diagnosis was suggestive of acute necrotizing encephalopathy of childhood. He responded to pulsed IV methylprednisolone treatment with minimal residual neurological deficit on follow-up. At 36 months of age, he had a repeat presentation and rapidly deteriorated. He developed severe encephalopathy with fixed dilated pupils. Whole exome sequencing revealed a set of compound heterozygous missense variants in the LRBA gene, a novel c.2206A>T (p.R736W) and c.5989C>T (p.R1997C) variant. Conclusion: Compound heterozygous mutations in the LRBA gene caused an atypical presentation of recurrent HLH with central nervous system (CNS) manifestations in our patient. Statement of Novelty: We herein report a novel set of compound heterozygous mutations in LRBA with atypical presentation of recurrent HLH with CNS manifestations, thus expanding the known phenotypic spectrum of LRBA deficiency.
Partial information maximum principle for optimal control problem with regime switching in the conditional mean-field model
Authors: Tamer Lazhar , Ben Abdallah Hani 
Published: March 2023

Abstract
This paper is concerned with a stochastic optimal control problem
for a Markov regime switching in the conditional mean field model.
Sufficient and necessary maximum principles for optimal control under
partial information are obtained. Finally, we illustrate our result through a model which gives an explicit solution.
Por los caminos de La Aurora: apuntes y reflexiones sobre procesos de inserción comunitaria
Authors: León-Páez Brealey Diego 
Published: March 2023

The goal of this systematization paper is to think over the experiences as an academic, researcher, and extensionist of the School of Psychology (EPS) of the National University of Costa Rica (UNA) about the project Community Action for Health Promotion: an experience in the Heredia-Virilla Health Area (ACOPOS), UNA code NFAG02, and the Graduation Seminar Health Needs and Resources of La Aurora de Heredia, focusing on the path between the phases of proposal and planning, to that of insertion and community intervention. It starts from a theoretical-methodological reflection from community social psychology, which delves into the experiences and concrete actions of this important step from the classrooms to the community. The methodological approach used in the mentioned works were the qualitative research and the participatory action research methodology. Research techniques such as observation, in-depth interviews, discussion groups and psychosocial mapping were used. This document also tries to demystify any reductionist -simplistic view of community social psychology that is taught in some university classroomsand in institutions that work with communities, in which, although it is considered essential to rely on solid epistemological-methodological references, they result insufficient to carry out field work. For writing this article, an exhaustive review of the reports of the ACOPOS project was carried out, as well as the memory of the Graduation Seminar document. In-depth interviews and discussion processes were carried outwith the student-researchers involved in the Graduation Seminar. As a result of the thought procedure, it is found that although it is essential to carry out an adequate planning of a project of action and intervention in the communities from the institutions, this is only the beginning of a complex process of involvement in the socio-historical– cultural -psychological reality of the community, its institutions, groups, families and inhabitants. This allows us to conclude that it is essential that the training of students and professionalswho carry out research and community action, is not only in theoreticalmethodological elements, but also in aptitudes, attitudes and competences when inserting, interacting and intervening in and with the communities.
El Jornal das Senhoras: un proyecto periodístico femenino para la emancipación de las mujeres brasileñas
Authors: Simon da Silva Regina 
Published: March 2023

Argentinian Juana Paula Manso (1819-1875) was born and lived most of her life in one of the most troubled periods in the history of Argentina, and her ideals, daring for the mentality of the time, brought her, among many problems, political persecutions by Juan Manuel de Rosas, which culminated in the family’s exile, first to Uruguay and then to Brazil. In this country, Manso founded and directed, in 1852, Jornal das Senhoras, considered the first Brazilian feminist newspaper, with the clear intention of promoting theemancipation of women. This article, dedicated to the female press, analyzes the dialogue established between the Jornal das Senhoras and the Brazilian readers, seeking to identify elements that foster their illustration and emancipation.
Revistas criollas e intelectualidad criollista: el amplio debate por la tradición nacional argentina
Authors: Minelli Ivia 
Published: March 2023

The purpose of this essay is to review the discussion on the Argentinian national tradition between 1890 and 1910 from the analysis of Creole and its branches. Creole magazines being a part of popular journalism during the independence centenary will be analyzed. These magazines are mainly preserved in Robert Lehmann-Nitsche archives (Ibero-Amerikanisches Institut, Berlin) classified as folklore since 1897. So the analysis of their programs is the main purpose of this essay in order to study their editorial context and its specificity; the result is the recovery of a speech that was overshadowed.
TENSÃO ENTRE MEMÓRIA COLONIAL E A MEMÓRIA DECOLONIAL NA CONSTRUÇÃO DO CURRÍCULO ESCOLAR
Authors: Silva Janssen Felipe da , Santos Aline Renata dos 
Published: March 2023

Este texto tem como base e coluna argumentativa a ruptura com as metanarrativas epistêmicas e políticas da história e da memória única e, por conseguinte, universal gerada na e pela modernidade-colonial-capitalista-patriarcal-racista, como também o rompimento com a ideia universal de ser humano, de humanidade e de sociedade, por consequência, de cultura, de educação e de currículo. Assim, objetivamos, com esse artigo, evidenciar que tal ruptura nos abre caminhos para questionar ideias hegemônicas como civilização, cultura, ciência, conhecimento e principalmente história e memória e suas relações com educação escolarizada e currículo. Seguimos pelo caminho da revisão bibliográfica, atrelado à pesquisa qualitativa, propomos um diálogo sobre estas rupturas, compreendendo-as como essenciais para a reconstrução-produção de currículos descoloniais. Apoiamo-nos nas Epistemologias do Sul, principalmente, nos Estudos Pós-coloniais e Decoloniais. Nessa direção, entendemos que desvelar a Memória Colonial possibilitaria a construção da Memória Decolonial enquanto elemento fundante dos currículos e de sua materialização no chão da escola, contribuindo, assim, para o esfacelamento das heranças coloniais, ainda presentes nos currículos.
A Szent László-ének mint zarándokének
Authors: Ács Pál 
Published: March 2023

Szent László kultuszának és tiszteletének magyar nyelvű irodalmi emléke a Mátyás korában, talán 1470 körül keletkezett, latin–magyar versszakpárból álló Szent László-ének, amely az egyik legszebb késő középkori magyar versünk. Mi volt az eredeti funkciója ennek a kiemelkedő esztétikai értékű versnek?Ez a probléma régóta érdekelte az irodalomtörténészeket. Ki, mikor, milyen körülmények között, hol, és hogyan énekelte a Szent László-éneket? A korábbi kutatások megállapították, a költeményt két, világi környezetben keletkezett kódex, a Peer-kódex és a Gyöngyösi kódex őrizte meg, egymástól lényeges különbségeket mutató másolatokban. Arra is felhívták a figyelmet, hogy a mű latin és magyar strófái egyetlen szöveget alkotnak, azaz a vers kétnyelvű, felelgetős énekként élt a késő középkori gyakorlatban. A vers alapos olvasása megmutatja, hogy az egész Szent László-ének szorosan összefügg a szent magyar király, a 11. századi jámbor lovag-szent, Szent László kegyhelyével. Várad (=Nagyvárad), a Szent László-kultusz központja, a leghíresebb középkori búcsújáró hely Magyarországon. Sajnos a váradi székesegyház, Szent László sírjával együtt, elpusztult a város oszmán ostroma során. 1660-ban.Jelen tanulmány Szent László-ének eredetét igyekszik feltárni, és az egykor széles körben ismert vers eredeti funkcióját. Ha a verset zarándokdalként értelmezzük, nemcsak a Szent László-kultusz központjára tett gazdag utalások lesznek érthetők, hanem mindazon megállapítások, amelyek a mű félig világi jellegére, politikai célkitűzéseire, paraliturgikus funkciójára vonatkoznak, és megmagyarázza a vers kétnyelvűségét is.
A Magyar Királyság és az Erdélyi Nagyfejedelemség könyvtári rendszerének felépítése új birodalmi keretben (1686/1690–1815)
Authors: Monok István 
Published: March 2023

A nyelv mellett, talán még annál is fontosabb egy birodalom összetartásában a közös kultusz kiépítése, benne a könyvtárak szerepével. A gazdasági érdekek mentén ez a kultikus közösség soha nem épült fel. A gazdaság működtetésének közös elvei természetesen részévé válhatnak egy közös kultusznak, de soha nem a kultikus alapokhoz tartoznak.
A Magyar Királyság és az Erdélyi Nagyfejedelemség könyv-, könyvtár-, olvasmány- és olvasástörténetének vizsgálatában az Oszmán Birodalom elleni harc utolsó évszázadában – Bécs felmentésétől a napóleoni háborúkig – az alapvető szempont az a tény kell, hogy legyen, miszerint egy olyan országról beszélünk, amely ténylegesen három: államjogilag kettő, gazdasági és társadalmi valóságában pedig három. A 16. század első harmada végén a törvényhozó és államigazgatási központ csakúgy, mint a kultikus centrum, elköltözött arról a helyéről, ahol évszázadok alatt felépült (Buda, Esztergom), majd ezeknek a központi helyeknek török hatalom alóli felszabadítása után másfél évszázad kellett ahhoz, hogy újra elfoglalják egykori helyüket. A két ország – a Magyar Királyság és Erdély – újjáépítését, gazdasági, társadalmi és kulturális újraszervezését másként szándékozott megvalósítani a birodalmi központ (amelynek részeivé rendeltettek az 1713-as utrechti békében létrejött nagyhatalmi konszenzus által) és másként a helyi hatalom képviselői.
A szándékok és az anyagi lehetőségek különbözőségei közötti kompromisszumok mentén zajlott le a két ország könyves infrastruktúrájának a kiépítése, vagyis a könyvkiadási és a könyvkereskedelmi rendszer felépítése, ennek a politikai és kultikus hatalmi oldalról történő ellenőrzési mechanizmusaival együtt. Ebben az erőtérben egy sajátos könyvtári rendszer épült fel, követni próbálva a birodalom más országainak mintáját. Közben az országokon belül is eltérő gazdasági, egyházi és kulturális (ezen belül nemzetiségi) érdekek mentén zajlott egy szellemi, gondolkodásbeli változási folyamat, közvetlen német és itáliai, közvetett francia hatásokkal. Más kulturális hatások sporadikusan jelentek csak meg. Számottevő angol (’insularis’) hatásról ekkor még nem beszélhetünk.
Összegezve azonban megállapítható, hogy a török uralom megszűntét követő bő egy évszázad alatt a Magyar Királyságban és Erdélyben a Habsburg Birodaloméval konform könyvtári rendszer jött létre. Az egy további kérdés, hogy a könyvtárak tartalmi és nyelvi összetétele mennyiben volt más, mint a birodalom más területeinek gyűjteményeié.
Dermatomyositosis: a rare case report
Authors: Behera Sunil Kumar , Divya Amaravadi  , Merugoju Alekhya  , Rakham Sneha  
Published: March 2023

Dermatomyositis (DM) is a connective tissue disorder and is an idiopathic inflammatory myopathy characterized by skin manifestations. Diagnosis is based on progressive muscle weakness, skin rashes, elevated serum muscle enzymes, muscle biopsy, and abnormal electromyography. In this case study we report a case of classic DM without co-existing malignancy. Timely diagnosis and administration of corticosteroids and physiotherapy led to a better prognosis for the patient. Corticosteroids are the foundation of the treatment. However, the patient remained generally weak and treatment was advised to be continued on discharge.
A systematic analytical study on ocular manifestations of pregnancy
Authors: Halder Abhijit , Maiti Nupur Nandi , Singha Raju , Biswas Atanu 
Published: March 2023

During pregnancy, ocular changes are very commonly observed. Ocular changes during pregnancy are categorized as physiological or pathological. Ocular manifestations during pregnancy could also lead to diplopia, scotomata, retrobulbar pain and visual obstruction. According to many studies we analyzed, it has been stated that an estimate of 14 percent of pregnant women reports experience visual changes during pregnancy.A systemic self-study and meta analysis was planned to analyze and study the various ocular manifestations of pregnancy. With the application of Electronic databases we searched PubMed, Google Scholar, Web of Science, Medline Plus, Health line & Cleveland Clinic web which were published in English Language. This systemic self-study have reviewed the facts which were published earlier to determine the current scenario by vast study of statistics and derivation of facts. Vast analysis and along with proper examination of data were made to evaluate with final conclusion.: Based on our systematic self study and vast analysis on ophthalmic changes occurring during pregnancy we derive to a fact that any ocular changes could be categorized as physiological ocular changes or pathological ocular changes occurring during pregnancy. Physiological changes are those changes which occur normally during pregnancy whereas pathological changes are those changes which are present for the very first time in pregnancy or it could be modification in existed disease affected by pregnancy.: We conclude that pathological manifestations are very common in pregnancy which accounts for majority of pregnant women could be managed by general practitioners and optometrists. Pathological preexisting ocular condition could be referred to ophthalmologist for further management. Pregnant women with systemic disease would require hospital care with supportive antenatal service.
Peran Dynamic Geometry Software Untuk Meningkatkan Kemampuan Spasial Peserta Didik Dalam Belajar Descriptive Geometry: Sebuah Review Literatur
Authors: Wijaksana Aris Hadiyan , Kusumah Yaya S. 
Published: March 2023

Peserta didik dalam memahami Descriptive Geometry perlu memiliki kemampuan spasial. Karakteristik dari Descriptive Geometry adalah proyeksi dimana dalam pembelajaran siswa dituntut untuk mampu merepresentasikan objek-objek 3D ke dalam bentuk 2D dengan aturan-aturan proyeksi yang ketat. Oleh karena itu peserta didik hendaknya memiliki kemampuan spasial yang mumpuni apabila mengikuti pembelajaran Descriptive Geometry, sehingga penelitian ini memiliki tujuan untuk melihat sejauh mana peran Dynamic geometry software dalam meningkatkan kemampuan spasial peserta didik dalam belajar Descriptive Geometry. Penelitian ini merupakan sebuah review literatur yang mengkaji berbagai macam referensi melalui penelusuran artikel internasional maupun nasional secara daring. Studi ini diperuntukkan untuk mendapatkan kekuatan kajian ilmiah tentang kemampuan spasial peserta didik dalam mempelajari Geometry Descriptive. Setelah melakukan kajian terhadap artikel-artikel yang relevan, dapat disimpulkan bahwa Dynamic geometry software memiliki peran yang sangat penting dalam meningkatkan kemampuan spasial peserta didik dalam belajar Descriptive Geometry.
Tight-Rock RTA: Global Band of Shale Producers Joins Forces To Improve Crucial Diagnostic
Authors: Jacobs Trent 
Published: March 2023

More than three dozen oil and gas producers are working together to solve one of the grand challenges faced by all in the increasingly global unconventional sector. That is the ability to better predict tight-oil and -gas production using rate transient analysis (RTA).
Considered an “unconventional diagnostic” when first introduced in the 1970s, RTA relies on fluid rates and flowing pressures to inform engineers on what their reservoirs will ultimately yield. Unfortunately, the low permeability of tight rocks and a myriad of dynamics stemming from hydraulic fracturing have undercut the simplicity of the tool.
This has given rise to arguments that RTA is not a fit for unconventional reservoirs. But a joint industry project with 37 operators from around the world is betting against that notion.
Those taking part represent the biggest and most active shale plays in the US—a list that includes Apache Corp., BP’s shale unit BPx. Devon Energy, EQT, Hess Corp., and Ovintiv.
Others hold assets in Canada’s Montney and Duvernay formations, Argentina’s Vaca Muerta Shale, and the emerging Jafurah tight-gas basin that Saudi Aramco is in the early stages of developing.
The organizer behind the joint project is petroleum engineering software and consultancy Whitson. The Trondheim-based firm said the client consortium is likely the largest of its kind to focus squarely on improving RTA for tight reservoirs.
In October, the multinational group wrapped up its first phase of study with a set of best practices and the release of new add-ons for Whitson’s software service. Why this might evolve into a notable development is because the deliverables are all designed to help standardize a recently debuted alternative called the numerically enhanced RTA workflow.
Introduced by reservoir experts at Houston-based Apache and IHS Markit, this “enhanced” version of numerical RTA has caught the industry’s attention for its ability to account for the effects of multiphase flow in tight wells. The first details about the approach were shared with the industry in 2020 in URTeC 2967.
Whitson reports that a newly established workflow for numerical RTA created with operator clients delivers consistent well analysis in seconds to a few minutes, and importantly, was proven to work across their disparate geologies.
Mathias Carlsen is a general manager at Whitson and has been at the center of the joint industry project from the beginning. Here, he helps explain what the industry should know about the RTA joint project and where it is heading.
Assembling a Trifecta Carlsen, a reservoir engineering expert, described the overarching goal of the joint project not as a mission to discover a panacea for RTA but one designed to fill in its big gaps with some recent innovations.
“What we’ve been successful at through the joint industry project is getting the new tools ready so that the workflows can be easily used and in standardizing them for a wide range of wells found in every single unconventional basin in the world,” he said.
An Analytical Model for The Capacitance of Stainless Steel Braided Reinforced Teflon Aviation Hoses
Authors: Yao Jing , Li Xinhao , Ren Yihan , Li Mandi , Yang Shuai , Wang Dingyu 
Published: March 2023

Abstract
Stainless Steel Braided Reinforced Teflon Hose（SSBRTH), as an extensively used component in the aviation hydraulic system, has a huge impact on system performance. Because of its elasticity, the calculation of hose deformation and capacitance is difficult, and inappropriate hose parameters likely degrade the system and even affect the aircraft’s normal operation. In this study, the theory of corrugated plate is introduced for deformation prediction of fluctuant steel wire, then a new analytical model is established to describe the deformation condition of each regular braided SSBRTH layer with its capacitance calculation. In addition, the immediate establishment of SSBRTH 3D models by Rhino & Grasshopper parametric modeling for FEA is proposed and compared with the analytical model of capacitance, whose accuracies are all verified by experiments. The results emphasize that the actual deformation of the hose was in accordance with the prediction, and steel wire diameter, braided angle and the number of steel wires are negatively correlated with the capacitance value.
Outpatient Geriatric Health Care in a Rural Region in Germany: A Population-Based Spatial Analysis of Secondary Data
Authors: Pfeuffer Nils , Radicke Franziska , Leiz Maren , Moon Kilson , Hoffmann Wolfgang , Berg Neeltje van den 
Published: March 2023

Abstract
Background and objective: Due to unrecognized and unaddressed geriatric care needs, elderly patients have a higher risk for developing chronic conditions and acute medical complications. Early geriatric screenings and assessments help to timely identify geriatric needs. Holistic and coordinated therapeutic approaches maintain the independence of elderly patients and avoid adverse effects of several uncoordinated treatments. General practitioners’ practices are important for the timely identification of geriatric needs. The aims of this study are to examine the spatial distribution of the utilization of outpatient geriatric services in the Northeast of Germany and to identify regional disparities.
Methods: Geographical analysis and cartographic visualization of the spatial distribution of outpatient geriatric services of patients who are eligible to receive basic or specialized geriatric care were carried out. Claims data of the Association of Statutory Health Insurance Physicians in Mecklenburg-Western Pomerania were analysed on the level of postcode areas. A Moran’s I analysis was carried out to identify clusters of low or high utilization rates.
Results: Of all patients who were eligible for outpatient geriatric care in 2017, 58.3% (n=129,283/221,654) received at least one basic outpatient geriatric service. 77.2% (n=73,442/95,171) of the patients who were also eligible for specialized services, received any geriatric service (basic or specialized). 0.4% (n=414/95,171) of this group, actually received specialized outpatient geriatric care. Among the postcode areas in the study region, the proportion of patients who received a basic geriatric assessment ranged from 3.4% to 86.7%. Several regions with statistically significant Clusters of utilization rates were identified.
Conclusions: Considering the entire federal state, about half of the eligible population received basic geriatric care. Of the subgroup eligible for specialized geriatric care, only 0.4% received specialized services. The wide range of regional variations in the utilization rates and the local segregation of high and low rates indicate that the provision of outpatient geriatric care may depend to a large extent on local structures (e.g., multiprofessional, integrated networks or innovative projects or initiatives). Considering specialized outpatient care, utilization is limited to a few regions. Innovative solutions to improve access to both basic and specialized geriatric outpatient care are necessary.