bluuebunny/crossref_metadata_2025_split huggingface dataset:
Expected or unexpected clinical findings in liver glycogen storage disease type IX: distinct clinical and molecular variability
Authors: İnci Aslı , Kılıç Yıldırım Gonca , Cengiz Ergin Filiz Başak , Sarı Sinan , Eğritaş Gürkan Ödül , Okur İlyas , Biberoğlu Gürsel , Bükülmez Ayşegül , Ezgü Fatih Süheyl , Dalgıç Buket , Tümer Leyla 
Published: January 2022

Abstract

Objectives
To reveal the different clinical presentations of liver glycogen storage disease type IX (GSD IX), which is a clinically and genetically heterogeneous type of glycogenosis.

Methods
The data from the electronic hospital records of 25 patients diagnosed with liver GSD IX was reviewed. Symptoms, clinical findings, and laboratory and molecular analysis were assessed.

Results
Of the patients, 10 had complaints of short stature in the initial presentation additionally other clinical findings. Elevated serum transaminases were found in 20 patients, and hepatomegaly was found in 22 patients. Interestingly, three patients were referred due to neurodevelopmental delay and hypotonia, while one was referred for only autism. One patient who presented with neurodevelopmental delay developed hepatomegaly and elevated transaminases during the disease later on. Three of the patients had low hemoglobin A1C and fructosamine values that were near the lowest reference range. Two patients had left ventricular hypertrophy. Three patients developed osteopenia during follow-up, and one patient had osteoporosis after puberty. The most common gene variant, PHKA2, was observed in 16 patients, 10 variants were novel and six variants were defined before. Six patients had variants in PHKG2, two variants were not defined before and four variants were defined before. PHKB variants were found in three patients. One patient had two novel splice site mutations in trans position. It was revealed that one novel homozygous variant and one defined homozygous variant were found in PHKB.

Conclusions
This study revealed that GSD IX may present with only hypotonia and neurodevelopmental delay without liver involvement in the early infantile period. It should be emphasized that although liver GSDIX is thought of as a benign disease, it might present with multisystemic involvement and patients should be screened with echocardiography, bone mineral densitometry, and psychometric evaluation.
Forgiveness as an Approach to the History of Philosophy
Authors: Gazit Yael 
Published: January 2022

AbstractIn the past, Robert Brandom’s philosophy has provided fruitful grounds for the development of an approach to the history of philosophy. InA Spirit of Trust (2019), however, this approach takes a new form; one that corresponds to a shift of focus in Brandom’s philosophy, from his earlier inferentialism to its later developments in the thesis of rational recollection. This article aims to elucidate and explicate this new approach, which Brandom refers to asforgiveness. By looking into the thesis of rational recollection, the article examines the back-looking attitude that Brandom argues for and how it can be applied to past philosophy. To better understand the novelty of this approachas forgiveness, the article considers other names that Brandom ascribes to this process, which interestingly correspond to different genres in the historiography of philosophy. Lastly, the article discusses whether such a forgiving approach leaves room for criticism and mistakes.
The appliability of systemic functional linguistics and its role in discipline integration
Authors: Zhang Delu , Hu Ruiyun 
Published: January 2022

Abstract
The present article aims to investigate the model and significance of discipline integration based on the appliability of systemic functional linguistics. To this end, it first describes the inevitability of the emergence of social semiotics; then it explores the six appliable characteristics of social semiotics: sociality, systemicness, applicability, transdisciplinarity, dynamicity, and multimodality. It next studies the basic conditions of discipline integration and the models of integration. After that, it investigates the basic model of discipline integration in systemic functional linguistics, which includes four basic types: direct application, borrowing, complementarity, and backwashing. It finally examines the model of the discipline integration process, including choice of a theme, choice of problems to be solved, choice of applicable disciplines, the contextualization of disciplines to be integrated; and the effect of the discipline integration in solving problems is also discussed. It is hoped that this study will shed light on cross-disciplinary or transdisciplinary research.
Photonic neuromorphic technologies in optical communications
Authors: Argyris Apostolos 
Published: January 2022

Abstract
Machine learning (ML) and neuromorphic computing have been enforcing problem-solving in many applications. Such approaches found fertile ground in optical communications, a technological field that is very demanding in terms of computational speed and complexity. The latest breakthroughs are strongly supported by advanced signal processing, implemented in the digital domain. Algorithms of different levels of complexity aim at improving data recovery, expanding the reach of transmission, validating the integrity of the optical network operation, and monitoring data transfer faults. Lately, the concept of reservoir computing (RC) inspired hardware implementations in photonics that may offer revolutionary solutions in this field. In a brief introduction, I discuss some of the established digital signal processing (DSP) techniques and some new approaches based on ML and neural network (NN) architectures. In the main part, I review the latest neuromorphic computing proposals that specifically apply to photonic hardware and give new perspectives on addressing signal processing in optical communications. I discuss the fundamental topologies in photonic feed-forward and recurrent network implementations. Finally, I review the photonic topologies that were initially tested for channel equalization benchmark tasks, and then in fiber transmission systems, for optical header recognition, data recovery, and modulation format identification.
Block copolymer gyroids for nanophotonics: significance of lattice transformations
Authors: Park Haedong , Jo Seungyun , Kang Byungsoo , Hur Kahyun , Oh Sang Soon , Ryu Du Yeol , Lee Seungwoo 
Published: January 2022

Abstract
A gyroid crystal possesses a peculiar structural feature that can be conceptualized as a triply periodic surface with a constant mean curvature of zero. The exotic optical properties such as the photonic bandgap and optical chirality can emerge from this three-dimensional (3D) morphological feature. As such, gyroid crystals have been considered as the promising structures for photonic crystals and optical metamaterials. To date, several methods have been proposed to materialize gyroid crystals, including 3D printing, layer-by-layer stacking, two-photon lithography, interference lithography, and self-assembly. Furthermore, the discovery of Weyl points in gyroid crystals has further stimulated these advancements. Among such methods, the self-assembly of block copolymers (BCPs) is unique, because this soft approach can provide an easy-to-craft gyroid, especially at the nanoscale. The unit-cell scale of a gyroid ranging within 30–300 nm can be effectively addressed by BCP self-assembly, whereas other methods would be challenging to achieve this size range. Therefore, a BCP gyroid has provided a material platform for metamaterials and photonic crystals functioning at optical frequencies. Currently, BCP gyroid nanophotonics is ready to take the next step toward topological photonics beyond the conventional photonic crystals and metamaterials. In particular, the intrinsic lattice transformations occurring during the self-assembly of BCP into a gyroid crystal could promise a compelling advantage for advancing Weyl photonics in the optical regime. Lattice transformations are routinely considered as limitations, but in this review, we argue that it is time to widen the scope of the lattice transformations for the future generation of nanophotonics. Thus, our review provides a comprehensive understanding of the gyroid crystal and its lattice transformations, the relevant optical properties, and the recent progress in BCP gyroid self-assembly.
Nonlinear thermal lensing of high repetition rate ultrafast laser light in plasmonic nano-colloids
Authors: Agiotis Leonidas , Meunier Michel 
Published: January 2022

Abstract
We report on experimental observations of phenomenological self-trapping in plasmonic colloids of varying plasmon peaks in the visible/near infrared. A femtosecond (fs) oscillator is used in both pulsed (35 fs, 76 MHz) and continuous wave (cw) operation for comparison. We show that for both modes and for all examined colloids (and under typically applied external focusing conditions in self-trapping studies in colloidal media) nonlinear propagation is governed by thermal defocusing of the focused beam, which precedes the steady-state regime reached by particle diffusion, even far from the plasmon resonance (or equivalently for non-plasmonic colloids, even for low absorption coefficients). A strategy for the utilization of high repetition fs pulses to mitigate thermal lensing and promote gradient force-induced self-trapping is discussed. Notably, nonlinear thermal lensing is further accompanied by natural convection due to the horizontal configuration of the setup. Under resonant illumination, for both fs and cw cases, we observe mode break-up of the beam profile, most likely due to azimuthal modulation instability. Importantly, time-resolved observations of the break-up indicate that in the fs case, thermal convection heat transfer is reduced in magnitude and significantly decoupled in time from thermal conduction, presumably due to temperature increase confinement near the particles. We anticipate that our findings will trigger interest toward the use of high repetition fs pulses for self-channeling applications in nano-colloids.
Weltverhalten
Authors: Eickhoff Hajo 
Published: January 2022

Abstract
Erst, wenn Menschen ihr Wissen und Können so weit entwickelt haben, dass sie so stark in sich und in die Welt eingreifen können, dass sie zur zerstörerischen Naturkraft werden und die Gegenwart zum Anthropozän machen, und erst, wenn krankmachende Mikroorganismen ebenso rasch um die Welt reisen wie Waren und Millionen von Menschen, können Pandemien entstehen. Umweltzerstörung, Klimawandel und Pandemie gehören zusammen. Doch gegen das entfremdete Handeln des Menschen entwickelt sich ein neues Weltverhalten: Als Folge der unheilvollen Entwicklung für das Leben auf der Erde macht es eine praktische und poetische Haltung des Respekts, der Verantwortung und des aktiven Lassens möglich.
„Als das kleine Virus tanzte ...“
Authors: Stroß Annette Miriam 
Published: January 2022

Abstract
Am Beginn des Jahres 2020 führten die Worst-Case-Szenarien von Virologen innerhalb weniger Wochen dazu, in Deutschland eine – politische – Ausnahmesituation zu erzeugen. Unterstützt wurde diese durch eine weitgehend einheitliche mediale Berichterstattung. So schienen Angst und Panik binnen kürzester Zeit zur normalen wie auch (einzig) moralisch legitimen Reaktionsweise von Millionen von Menschen zu gehören. Und dies, obwohl der weitaus überwiegende Teil der Bevölkerung gar nicht zur Gruppe der an diesem Virus schwer oder schwerst Erkrankten gehört(e). Der nachstehende Essay schildert Eindrücke vom Beginn der sog. Corona-Pandemie kurz vor und nach dem ersten „Lockdown“ in Deutschland im März 2020. Er verweist auf die ersten öffentlichen Beiträge von Wissenschaftler.innen aus nichtmedizinischer Sicht und erinnert an die Aufgabe der Bildungswissenschaften, auch und gerade in Krisenzeiten eine den Menschen in seiner Menschlichkeit stärkende Perspektive anzubieten. Worin diese konkret bestehen kann, soll im Folgenden näher beleuchtet werden.
The Work of Art in the Age of its Sanitized Fruition
Authors: Desideri Fabrizio , Zingaro Vincenzo , Portera Mariagrazia 
Published: January 2022

Abstract
For almost two years now the COVID-19 pandemic impacted in most different forms habits, models of organization, socio-political dynamics and economic assets. Arrangements and orders taking decades to reach stabilization have demonstrated an unsuspected precarity, demanding a profound reorganization of dynamics we had been long accustomed to. As the distant, sanitized character of interaction, transmission, fruition and creation processes has turned from a contingent measure into the unamenable norm of these days’ routine, every aspect of social interaction is changing accordingly. “Contact”, “contagion”, “proximity” and “distance” are not features of a transient period anymore: they are keywords of today’s experience. Indeed, while these concepts were already at stake in masscommunication and social media, it was not before nowadays that their precipitate on art forms could be taken into account more pristinely, for especially performative arts never had to amend their “familiar ground” in order to pursue their goals. Hence, how did artforms adjust themselves to wholly new forms of participation? What are we supposed to expect next? The main aim of this paper is to sketch out, relying on some classical loci of the history of Western aesthetics such as Plato, Aloïs Riegl, Walter Benjamin, this new, re-configured notion of aesthetic and artistic experience in the time of the COVID-19 pandemic.
Combating the Education Pandemic in Africa
Authors: Omolewa Michael , Adejo Adetola 
Published: January 2022

Abstract
The disruptions in Africa including underdevelopment, poverty, disease, ignorance, instability, are said to have constituted a serious pandemic, and the causes of the pandemic have been variously described as consequences of colonialism, neo-colonialism, military incursion, poor leadership and corruption. This paper argues that the absence of mass education may be considered as a pandemic. It further contends that the failure to offer the population mass education, beginning with the basic literacy programmes is serious educational pandemic. That pandemic could explain much of the problems and afflictions in Africa and the breakdown of the society, long before the contemporary COVID-19. The study relied on interviews, articles and books using the descriptive approach as a methodology. The authors found out that the absence of mass education has consequences such as unemployment, low life expectancy, poverty trap and exploitation among others. The study recommends that training structures at all levels need to ensure there is always a built-in link between literacy and students’ professional activities, including the different elements of their environment. It also recommends that the vision for mass education itself needs to be more inclusive of and responsive to other areas of life to improve the guarantee of adjustment to globalization, rapid change and new challenges.
Pandemie und Freiheit
Authors: Bernecker Roland 
Published: January 2022

AbstractWir haben die Covid-19 Pandemie nicht nur als eine medizinische Krise erlebt, sondern auch als eine Krise der Freiheit. Die Maßnahmen zur Eindämmung des Corona-Virus waren Ergebnisse politischer Abwägungen, in denen auch Grundfreiheiten zur Disposition standen. Die Pandemie und die auf sie reagierenden politischen Strategien der Gegenwehr haben auf eine ungewohnte Weise in unser Erleben von Räumen individueller und interpersonaler Freiheit eingewirkt. Der Beitrag geht der Frage nach, wie die Strategien zur Bewältigung der Pandemie politisch vermittelt wurden, welche Wahrnehmungen sie hervorriefen und was sich an ihnen über die globale Krise des Liberalismus und ein global sich wandelndes Verständnis von Governance ablesen lässt.
Behaviour of DGA and Ln resin with alpha radiation dose
Authors: Malmbeck Rikard , Banik Nidhu Lal 
Published: January 2022

Abstract
In this work the separation efficiency of the DGA and Ln extraction chromatographic resins has been investigated as a function of absorbed dose induced by alpha radiation. It was found that the resins show good stability towards alpha radiolysis, both maintaining high weight distribution ratios (and resin capacity factors) with the absorbed dose, calculated as if the complete alpha energy would be absorbed only in the resin material. However, the results obtained for Ln resin indicates that the method used in this work (extractive distribution ratio) to investigate alpha radiolytic behaviour is not appropriate. The reason is likely the strong acid dependency of this particular extraction system. The behaviour of the DGA resin towards alpha radiolysis was compared to alpha radiolysis in ordinary liquid-liquid extraction of an organic solvent based on the same extractant, TODGA, dissolved in n-dodecan. It was found that the alpha radiolysis rate of TODGA is about 5 times higher in liquid-liquid extraction where the complete energy of the alpha particle is deposited within the solvent, compared to the DGA resin. This indicates that in the heterogeneous DGA resin system, only 20% of the alpha energy is actually deposited to the organic solvent layered onto the particles of the DGA resin.
Common Fixed Point Theorems for T-Hardy-Rodgers Contraction Mappings in Complete Cone b-Metric Spaces with an Application
Authors: Shashi Pauline , Kumar Santosh 
Published: January 2022

Abstract
This paper presents some fixed point theorems for T-Hardy-Rodgers contraction mappings in complete cone b-metric spaces without the assumption of normality conditions. We prove the existence of the common fixed point in complete cone b-metric spaces for the continuous self mappings. Our results generalize many recent known results in metric spaces, b-metric spaces and cone metric spaces in the literature.
A new compilation method of general standard test load spectrum for aircraft engine
Authors: Lu Qi , Sun Zhigang , Xu Cong , Zhao Sheng , Song Yingdong 
Published: January 2022

Abstract
Based on the existing compilation technology of load spectrum, this paper proposed a new method for the compilation of the general standard test load spectrum of aircraft engine. On the premise of meeting operating requirements of the actual condition and the basic life test, it simplifies the test load conditions and overcomes the shortage of the existing technology. According to the actual flight of aircraft engine, the load spectrum of a flight mission profile was divided into three parts: the take-off phase, the middle-flight phase, and the landing phase. The proposed compilation method ensures that the generated load spectrum of the take-off phase and landing phase is consistent with the actual flight, and the load spectrum of the middle-flight phase is programmed according to a certain method so that a simple load cycle sequence can be implemented. On that basis, the load spectrum was analyzed and compiled by subsection. The general standard test load spectrum lays the foundation for carrying out the life test and reduces the error between the test and practical use. Since the parameters of the engine load are standardized in the process of compiling, the method can be widely used in various types of engines.
Nachhaltigkeit im genossenschaftlichen Wertesystem
Authors: Bouten Christiane , Wiedemann Arnd 
Published: January 2022

Zusammenfassung
Das genossenschaftliche Geschäftsmodell ist aufgrund seiner Wertestruktur in besonderem Maße vom Zusammenspiel ökonomischer, ökologischer und sozialer Nachhaltigkeit geprägt. Der Wertewandel in der Gesellschaft und die sich daraus ergebenden Veränderungen sind für Genossenschaftsbanken daher grundsätzlich vorteilhaft. Durch das CSR-Richtlinie-Umsetzungsgesetz (CSR-RUG) sind auch große Kreditinstitute dazu verpflichtet, ihre externe Berichterstattung um eine nichtfinanzielle Erklärung zu erweitern. Der vorliegende Beitrag untersucht die Nachhaltigkeitsberichterstattung von 20 CSR-RUG-pflichtigen Genossenschaftsbanken über den Zeitraum von 2017 bis 2019. Die Analyse zeigt, dass zumeist ein standardisiertes Berichtsformat und ein hoher Berichtsumfang gewählt werden. Dadurch wird das Potenzial einer adressatengerechten Kommunikation noch nicht völlig ausgeschöpft.
Ethics, Risk and Recovery
Authors: Ferencz Joseph 
Published: January 2022

The practice of recovery-oriented care with individuals who have been found unfit to stand trial or not criminally responsible, and who are subject to review board dispositions, presents a variety of ethical tensions. The assessment and management of risk in a rehabilitative context raises issues related to autonomy, confidentiality and conflicting roles. Awareness of and, where possible, resolution of these conflicts is necessary for the success of the recovery paradigm in this context.
Dopamine Release in Nucleus Accumbens Is under Tonic Inhibition by Adenosine A1Receptors Regulated by Astrocytic ENT1 and Dysregulated by Ethanol
Authors: Roberts Bradley M. , Lambert Elizabeth , Livesey Jessica A. , Wu Zhaofa , Li Yulong , Cragg Stephanie J. 
Published: January 2022

Striatal adenosine A1receptor (A1R) activation can inhibit dopamine release. A1Rs on other striatal neurons are activated by an adenosine tone that is limited by equilibrative nucleoside transporter 1 (ENT1) that is enriched on astrocytes and is ethanol sensitive. We explored whether dopamine release in nucleus accumbens core is under tonic inhibition by A1Rs, and is regulated by astrocytic ENT1 and ethanol. Inex vivostriatal slices from male and female mice, A1R agonists inhibited dopamine release evoked electrically or optogenetically and detected using fast-scan cyclic voltammetry, most strongly for lower stimulation frequencies and pulse numbers, thereby enhancing the activity-dependent contrast of dopamine release. Conversely, A1R antagonists reduced activity-dependent contrast but enhanced evoked dopamine release levels, even for single optogenetic pulses indicating an underlying tonic inhibition. The ENT1 inhibitor nitrobenzylthioinosine reduced dopamine release and promoted A1R-mediated inhibition, and, conversely, virally mediated astrocytic overexpression of ENT1 enhanced dopamine release and relieved A1R-mediated inhibition. By imaging the genetically encoded fluorescent adenosine sensor [GPCR-activation based (GRAB)-Ado], we identified a striatal extracellular adenosine tone that was elevated by the ENT1 inhibitor and sensitive to gliotoxin fluorocitrate. Finally, we identified that ethanol (50 mm) promoted A1R-mediated inhibition of dopamine release, through diminishing adenosine uptake via ENT1. Together, these data reveal that dopamine output dynamics are gated by a striatal adenosine tone, limiting amplitude but promoting contrast, regulated by ENT1, and promoted by ethanol. These data add to the diverse mechanisms through which ethanol modulates striatal dopamine, and to emerging datasets supporting astrocytic transporters as important regulators of striatal function.SIGNIFICANCE STATEMENTDopamine axons in the mammalian striatum are emerging as strategic sites where neuromodulators can powerfully influence dopamine output in health and disease. We found that ambient levels of the neuromodulator adenosine tonically inhibit dopamine release in nucleus accumbens core via adenosine A1receptors (A1Rs), to a variable level that promotes the contrast in dopamine signals released by different frequencies of activity. We reveal that the equilibrative nucleoside transporter 1 (ENT1) on astrocytes limits this tonic inhibition, and that ethanol promotes it by diminishing adenosine uptake via ENT1. These findings support the hypotheses that A1Rs on dopamine axons inhibit dopamine release and, furthermore, that astrocytes perform important roles in setting the level of striatal dopamine output, in health and disease.