bluuebunny/crossref_metadata_2025_split huggingface dataset:
Organic acidemias in the neonatal period: 30 years of experience in a referral center for inborn errors of metabolism
Authors: Unsal Yagmur , Yurdakok Murat , Yigit Sule , Celik Hasan Tolga , Dursun Ali , Sivri Hatice Serap , Tokatli Aysegul , Coskun Turgay 
Published: October 2022

Abstract

Objectives
Neonatal-onset organic acidemias (OAs) account for 80% of neonatal intensive care unit (NICU) admissions due to inborn errors of metabolism. The aim of this study is to analyze clinical features and follow-up of neonates diagnosed with OAs in a metabolic referral center, focusing on perinatal characteristics and the impact of first the metabolic crisis on long-term outcome.

Methods
Perinatal features, clinical and laboratory characteristics on admission and follow-up of 108 neonates diagnosed with OAs were retrospectively analyzed. Global developmental delay, abnormal electroencephalogram (EEG) or brain magnetic resonance imaging (MRI), chronic complications, and overall mortality. Associations between clinical findings on admission and outcome measures were evaluated.

Results
Most prevalent OA was maple syrup urine disease (MSUD) (34.3%). Neonates with methylmalonic acidemia (MMA) had significantly lower birth weight (p<0.001). Metabolic acidosis with increased anion gap was more frequent in MMA and propionic acidemia (PA) (p=0.003). 89.1% of OAs were admitted for recurrent metabolic crisis. 46% had chronic non-neurologic complications; 19.3% of MMA had chronic kidney disease. Abnormal findings were present in 26/34 of EEG, 19/29 of MRI studies, and 32/33 of developmental screening tests. Metabolic acidosis on admission was associated with increased incidence of abnormal EEG (p=0.005) and overall mortality (p<0.001). Severe hyperammonemia in MMA was associated with overall mortality (33.3%) (p=0.047). Patients diagnosed between 2007–2017 had lower overall mortality compared to earlier years (p<0.001).

Conclusions
Metabolic acidosis and hyperammonemia are emerging predictors of poor outcome and mortality. Based on a large number of infants from a single center, survival in neonatal-onset OA has increased over the course of 30 years, but long-term complications and neurodevelopmental results remain similar. While prompt onset of more effective treatment may improve survival, newer treatment modalities are urgently needed for prevention and treatment of chronic complications.
(Im)politeness as a tool to categorize interactive discourse markers of Arabic in radio shows
Authors: Jarrah Marwan , Alghazo Sharif , Asad Arwa 
Published: October 2022

AbstractLittle research has related DMs to (im)politeness, and none in Jordanian Arabic (JA). This study provides evidence that (im)politeness can categorize interactive DMs into polite DMs and impolite DMs in JA. It aims to explore the use of DMs in JA in relation to (im)politeness dichotomy. Twenty-two episodes of three different Jordanian radio shows which use vernacular JA were recorded and transcribed. With the help of prosodic cues and native speakers’ judgement and comprehension of the relevant utterances, this study finds that DMs can be categorized into polite DMs and impolite DMs. For instance,ʕafwanعفوا ‘excuse me’ andja:si:di: يا سيدي ‘sir’ are exclusively used as mitigating polite DMs whilstwbaʕdi:n maʕakوبعدين معك ‘hey stop!’ andeṯlaʕ min ra:si:اطلع من راسي ‘get out of my head’ are impolite DMs which are primarily used in complaints and expressions of state of anger. This is all interpreted as evidence that interactive DMs can be strongly flavored by politeness which regulates their distribution and manifestation. The study contributes to research on DMs by presenting a categorization based on (im)politeness and thus strengthening the link between discourse and pragmatics.
Konjunkturzyklen in Deutschland – die Datierung durch den Sachverständigenrat
Authors: Breuer Sebastian , Elstner Steffen , Kirsch Florian , Wieland Volker 
Published: October 2022

Zusammenfassung
In diesem Papier stellen Sebastian Breuer, Steffen Elstner, Florian Kirsch und Volker Wieland, ehemaliges Mitglied des Sachverständigenrates zur Begutachtung der gesamtwirtschaftlichen Entwicklung (SVR), die Konjunkturdatierung des Sachverständigenrates vor. Die Autoren arbeiten stilisierte Fakten für die sich ergebenden Zyklen heraus und präsentieren Besonderheiten der Rezessionsphasen. Methodisch orientiert sich die Datierung an den Verfahren des National Bureau of Economic Research (NBER) und des Centre for Economic Policy Research (CEPR). Eine Rezession wird als signifikanter Rückgang der gesamtwirtschaftlichen Aktivität definiert, der sich in weiten Teilen der Wirtschaft zeigt. Der Sachverständigenrat hat für Deutschland sechs Rezessions- und sieben Aufschwungphasen seit 1950 identifiziert. Der jüngste konjunkturelle Hochpunkt wird auf das vierte Quartal 2019 beziehungsweise Februar 2020 datiert. Die darauffolgende Corona-Rezession ist die siebte Nachkriegsrezession. Eine Datierung des konjunkturellen Tiefpunktes steht noch aus.
A singular case of hyposmia and transient audiovestibular post-vaccine disorders: case report and literature review
Authors: Fantin Francesco , Frosolini Andrea , Tundo Isabella , Inches Ingrid , Fabbris Cristoforo , Spinato Giacomo , de Filippis Cosimo 
Published: October 2022

Abstract

Introduction
Rare and mild adverse effects on cranial nerves have been reported after vaccination. Here, we report a singular case of smell and taste disorder associated with tinnitus that occurred after Oxford-AstraZeneca vaccination together with a review of the available literature.

Case presentation
A 76-year-old patient experienced smell disorder, ear fullness and tinnitus 2 days after the first dose of Oxford-AstraZeneca vaccine. The patient then underwent a complete audiological and Ear, Nose and Throat evaluation, nasal endoscopy, Sniffin’Sticks battery, audiometric test battery, and cerebral magnetic resonance imaging (MRI). The exams revealed hyposmia and bilateral reduction of the volume of the olfactory bulbs (OB). At the follow-up, tinnitus was completely resolved while olfactory dysfunction only partially reduced.

Review of the literature
A PubMed search was conducted on olfactory and gustatory dysfunctions after COVID-19 vaccination resulting in four case reports with a total of 10 patients. The main symptoms were hyposmia, parosmia, and dysgeusia developed after 1–9 days from vaccination with complete resolution occurring within 1 month. Notably, none of the considered articles reported reduction of OB volumes at cerebral MRI.

Discussion
So far, no definitive cause–effect relationship has been established between anti-COVID19 vaccination and otolaryngologic adverse reactions. The persistence of hyposmia in our patient could possibly be explained by the reduction in OB volume, even though also the advanced age of the patient needs to be taken into account. This is a first indication of a cause–effect relation between hyposmia and Covid19 vaccination, even though a more robust study is needed to confirm the autoimmunological mechanisms responsible for these rare adverse reactions. However, it is worth highlighting that benefits of the anti-COVID-19 vaccination clearly outweigh the risk of rare adverse events.
Corticotroph tumor progression speed after adrenalectomy
Authors: Bessiène Laura , Moutel Sandrine , Lataud Marine , Jouinot Anne , Bonnet-Serrano Fidéline , Guibourdenche Jean , Villa Chiara , Baussart Bertrand , Gaillard Stephan , Barat Maxime , Dohan Anthony , Bertagna Xavier , Dousset Bertrand , Bertherat Jérôme , Assié Guillaume 
Published: October 2022

Abstract

Objectives
After bilateral adrenalectomy in Cushing's disease, corticotroph tumor progression occurs in one-third to half of patients. However, progression speed is variable, ranging from slow to rapid. The aim was to explore corticotroph progression speed, its consequences and its risk factors.

Design
A retrospective single-center observational study.

Methods
In total,103 patients with Cushing's disease who underwent bilateral adrenalectomy between 1990 and 2020 were included. Clinical, biological, histological and MRI features were collected. Median duration of follow-up after bilateral adrenalectomy was 9.31 years.

Results
In total,44 patients progressed (43%). Corticotroph tumor progression speed ranged from 1 to 40.7 mm per year. Progression speed was not different before and after bilateral adrenalectomy (P  = 0.29). In univariate analyses, predictive factors for rapid corticotroph tumor progression included the severity of Cushing's disease before adrenalectomy as the cause of adrenalectomy, high ACTH in the year following adrenalectomy and high Ki67 immunopositivity in the tumor. During follow-up, early morning ACTH absolute variation was associated with corticotroph tumor progression speed (P-value = 0.001). ACTH measurement after dynamic testing did not improve this association.

Conclusion
After adrenalectomy, corticotroph progression speed is highly variable and manageable with MRI and ACTH surveillance. Progression speed does not seem related to bilateral adrenalectomy but rather to intrinsic properties of highly proliferative and secreting tumors.
The Return of Religion and the Problem of Radical Evil: A Case Study of Russia’s War in Ukraine
Authors: Bacevičiūtė Danutė 
Published: October 2022

The article analyses the case of Russia’s war in Ukraine by focusing on the ambivalent relationship between radical evil and the “return of the religious”. First, the revival of politicised religious fundamentalism in contemporary Russia is examined by discussing the relationship between the state and the church and its concretisation in the field of foreign policy – the “Russian World” teaching. The article shows how the Russian Orthodox Church legitimises a project that pursues geopolitical goals and justifies the started war as a metaphysical fight against evil. Attention is drawn to the fact that in this case we are dealing with a kind of “secular religion” or “inverted secularism”, when ecclesial forms are used to revive the Soviet imperialism, and faith is replaced by beliefs. Next, when considering the analysed case in the context of the “return of the religious” reflected by Jacques Derrida and Gianni Vattimo, the emphasis is not only on the principle of violence (which is born from the fusion of political and religious discourses), but also on the positive possibilities of this return, which aforementioned thinkers elaborated by formulating the concepts of elementary faith and ethics of charity. At the same time, the article shows that these concepts lack an existential focus that would allow the problem of radical evil to be grasped at the level of individual existence. Therefore, to highlight the existential aspect of radical evil, the concepts of the perverse will, “grey zone” and impossible hope are used.
Emily Dickinson in the Work of Polish Translators. Continuation of Research
Authors: Krzysztofik Małgorzata , Wzorek Anna 
Published: October 2022

This article deals with the Polish translation of Emily Dickinson’s poetry and correspondence. It is a continuation of the reflection in The Emily Dickinson Journal’s pages, which started in 2022. This time we present translations of Kazimiera Iłłakowiczówna, Andrzej Szuba, Krystyna Lenkowska, Ryszard Mierzejewski, Tadeusz Sławek, Teresa Pelka, Artur Międzyrzecki, Lilla Latus, Agnieszka Osiecka, Agnieszka Kreczmar and Ewa Kuryluk. We discuss a selection of Dickinson’s letters translated by Danuta Piestrzyńska. We emphasize the characteristic features of each of the discussed translations. Although Iłłakowiczówna discovered Dickinson for Polish readers, her translations are perceived as archaic. Szuba mainly translates aphoristic texts. Lenkowska makes an effort to be faithful to the original. The newest translators introduce Poles to unknown poems and make new translations of the texts previously rendered into Polish.
Causal Mediation for Survival Data: A Unifying Approach via GLM
Authors: Taddeo Marcelo M. , Amorim Leila D. 
Published: October 2022

Mediation analysis has been receiving much attention from the scientific community in the last years, mainly due to its ability to disentangle causal pathways from exposures to outcomes. Particularly, causal mediation analysis for time-to-event outcomes has been widely discussed using accelerated failures times, Cox and Aalen models, with continuous or binary mediator. We derive general expressions for the Natural Direct Effect and Natural Indirect Effect for the time-to-event outcome when the mediator is modeled using generalized linear models, which includes existing procedures as particular cases. We also define a responsiveness measure to assess the variations in continuous exposures in the presence of ediation. We consider a community-based prospective cohort study that investigates the mediation of hepatitis B in the relationship between hepatitis C and liver cancer. We fit different models as well as distinct distributions and link functions associated to the mediator. We also notice that estimation of NDE and NIE using different models leads to non-contradictory conclusions despite their effect scales. The survival models provide a compelling framework that is appropriate to answer many research questions involving causal mediation analysis. The extensions through GLMs for the mediator may encompassa broad field of medical research, allowing the often necessary control for confounding.
Towards hermeneutics of substantial change. Between conciliar continuity and tendency to break
Authors: Napiórkowski Andrzej 
Published: October 2022

In the interpretation of Revelation, as well as, documents of the Magisterium of the post-conciliar Catholic theology, strong tensions and differences have been exposed.They are a consequence of various hermeneutics. Each of the sides claims the right to define “a reform”.However, a careful and critical analysis of the three functioning types of hermeneutics (of absolute continuity, of a break, and of absolute fidelity to the tradition) is not fully satisfactory.In its external form of expression the symbols and dogmas of faith are historical, so with the flow of history they become anachronistic, despite the fact that the essence (the core) of the doctrine is not changed, because it comes from the Divine Revelation and is a subject of personal experience of the faithful. Therefore, a fundamental basis of faith lies in the event of Revelation, namely, the self-giving of the Triune God. Hence the Revelation must be understood not as a statement of truth, but as an event of truth. It is about personal experience of the Resurrected, and not just knowledge or information about Him. Therefore, the language and the manner of communication must be constantly verified and contemporized. While the truth is always not completely grasped. This is why the presented article is a small contribution in developing a fourth type of hermeneutics – a hermeneutics of substantial change, which though would allow to break the continuity at some points, nevertheless would not destroy the identity of faith.
The Relationship between the First Proclamation and a Kerygmatic type of Catechesis
Authors: Sultana Carl-Mario 
Published: October 2022

The aim of this paper is to try and highlight the fecund relationship between the first proclamation and a kerygmatic type of catechesis as this is found in the Directory for Catechesis promulgated by the Pontifical Council for Promoting the New Evangelization. In Evangelii Gaudium, the first proclamation has been given a renewed meaning in that it is not only considered as the first proclamation in time, but it is extended as a primary proclamation throughout the life of the person. The kerygmatic type of catechesis posited in the Directory seeks to present Jesus Christ in a way in which is the Good News becomes an accompanying factor throughout the entire life of the person. It is this person-centredness which is at the basis of the relationship between the first proclamation and a kerygmatic type of catechesis.
Comparative assessment of the signs of instability of atherosclerotic plaques in the carotid arteries in elderly patients with acute coronary syndrome with duplex scanning and computed tomography angiography
Authors: Konstantinova E. V. , Sagatelyan A. A. , Bogdanova A. A. , Pershina E. S. , Shemenkova V. S. , Svet A. V. , Oganesyan A. A. , Gilyarov M. Yu. 
Published: October 2022

Aim. To study the signs of instability of atherosclerotic plaques (ASP) in the carotid arteries in elderly patients with acute coronary syndrome (ACS) in the comparative aspect of duplex ultrasound scanning (DUS) and computed tomography angiography (CT).Material and methods. 27 patients with ACS (75 years and over) were included in the study. The signs of instability of ASP were assessed according to DUS and CT.Results. The signs of instability of ASP according to DUS were detected in 85,7%, according to CT — in 84,6%. The following signs were detected with DUS and CT: the presence of irregular plaque surface including signs of ulceration — 6,4 and 11,6% (p=0,021), positive remodeling — 3,8 and 3,8% (p=0,998), signs of local calcification — 23 and 25,9% (p=0,536), heterogenous structure — 55,1 and 46,8% (p=0,045), hypoechogenic component and low-density areas — 11,5 and 11,6% (p= 0,998). The correlation analysis showed high comparability of DUS and CT: irregular plaque surface with ulceration (K=0,624, p=0,02), positive remodeling (K=1, p<0,001), calcification (K=0,858, p<0,001), heterogenous structure (K=0,754, p<0,001), the presence of hypoechogenic component and low-density areas (K=1, p<0,001).Conclusion. The study of elderly patients with ACS found high comparability of DUS and CT in the definition of the signs of instability of ASP in the carotid arteries. It is possible to use DUS as a routine method for assessing carotid atherosclerosis in patients of this group, which can reduce the risk of complications during CT, shorten the examination time, and minimize economic costs.
The Relationship between Native and Foreign Language Speaking Proficiency in University Students
Authors: Stankova Eva , Chlumska Renata , Zerzanova Dana 
Published: October 2022

Background. There are many factors that affect the development of speaking in a foreign language. Drawing on the theories that state that competencies established in a native language will transfer across foreign languages, this study examines whether there is a relationship between native and foreign language speaking proficiency.
Purpose. Although literature research indicates that native and foreign language acquisition processes are interrelated, there is a lack of studies comparing proficiency levels of native and foreign language speaking skills in adult learners. The purpose of this study was to examine the relationship between speaking competences in English as a Foreign Language and Czech as a Native Language in university students.
Methods. A between-group design was used to compare two groups of fifty university students at two different levels of their speaking proficiency in English. Both groups were tested in speaking in Czech. Each test was assessed by an analytical rating scale examining four speaking sub-skills: accuracy, discourse, content and paralinguistics. The scores were analyzed using the F-Test for Equality of Variances and T-Test for the Differences between the Means.
Results. The results showed that the group with the lower level of speaking proficiency in English achieved significantly worse scores for their speaking sub-skills in Czech than the group with the higher level of speaking proficiency in English.
Implications. The study offers another piece of empirical evidence in support of the theories that state that competencies established in a native language will transfer across foreign languages and suggests the importance of the development of native language competence with regard to later proficiency in a foreign language.
Relationships Between Language-Related Variations in Text Tasks, Reading Comprehension, and Students’ Motivation and Emotions: A Systematic Review
Authors: Wirth Lina , Kuhl Poldi , Ehmke Timo 
Published: October 2022

Background. There is consensus in research that students' motivation and emotions are important for learning and achievement processes in the educational context, as are language competencies that, related to the demands of academic language, enable participation in education. However, the interrelationships between these aspects have hardly been empirically investigated in depth.
Purpose and Methods. This systematic review addresses this research need, and aims to synthesise the existing evidence on the interrelationship between motivational/emotional and language-related variables. First, the relationship between learners’ motivation and emotions, and their language competencies is considered. Second, findings on how motivation and emotion depend on language-related factors are compiled.
Results. A systematic data search conducted for this purpose yields seven studies. Five studies relate to the first concern, and confirm the effects of motivational and emotional variables on reading comprehension. Emotions, in particular, emerge as strong predictors. Two studies relate to the second concern, and report significant effects of language-related variations in text tasks on students’ motivation; however, neither study considers emotions.
Implications. The findings are used to derive implications for language design in the educational context and identify important research gaps.
Mutation profile of diffuse large B-cell lymphoma with relapses in the central nervous system
Authors: Voropaeva E. N. , Pospelova T. I. , Karpova V. S. , Churkina M. I. , Vyatkin Yu. V. , Ageeva T. A. , Maksimov V. N. 
Published: October 2022

Introduction. The recurrence of diffuse large B-cell cell lymphoma in the central nervous system in the vast majority of cases is a fatal manifestation of the disease. The study of the lymphoma mutational profile can improve the accuracy of the prognosis of relapse in the central nervous system and justify the selection of patients for preventive treatment. Aim. To evaluate the mutational profile of cases of diffuse large B-cell cell lymphoma with central nervous system damage in relapse based on the results of our own experiment on high-performance sequencing.Materials and methods. On the Illumina platform, full-exome sequencing of diagnostic samples of diffuse large B-cell cell lymphoma with relapses in the central nervous system was performed. A panel including more than 70 genes was analyzed.Results. Four main groups of genetic events can be distinguished in the group of studied samples, namely: combined mutations in the NF-kB (MYD88, NOTCH1, CD79B, CARD11) and JAK-STAT (PIM1, STAT6) signaling pathways, as well as aberrations in the main oncosuppressor TP53 and chromatin remodeling system genes (ARID1A, KMT2D, EP300, SMARCA4). A recurrent mutation c. 794T>C, p.L265P MYD88 was detected in the study group. Among other findings, mutations in the CIITA and CD58 genes should be noted, which are important in avoiding tumor cells from immune surveillance.Conclusion. Despite the apparent heterogeneity of the mutational profile of diffuse large B-cell cell lymphoma with relapses in the central nervous system, in most cases, tumor cells were characterized by genetic disorders leading to the production of a large number of pro-inflammatory cytokines by malignant lymphocytes, as well as aberrations that reduce immunogenicity and contribute to the avoidance of immune surveillance by the tumor.