bluuebunny/crossref_metadata_2025_split huggingface dataset:
Caspase‐8‐driven apoptotic and pyroptotic crosstalk causes cell death and IL‐1β release in X‐linked inhibitor of apoptosis (XIAP) deficiency
Authors: Hughes Sebastian A , Lin Meng , Weir Ashley , Huang Bing , Xiong Liya , Chua Ngee Kiat , Pang Jiyi , Santavanond Jascinta P , Tixeira Rochelle , Doerflinger Marcel , Deng Yexuan , Yu Chien‐Hsiung , Silke Natasha , Conos Stephanie A , Frank Daniel , Simpson Daniel S , Murphy James M , Lawlor Kate E , Pearson Jaclyn S , Silke John , Pellegrini Marc , Herold Marco J , Poon Ivan K H , Masters Seth L , Li Mingsong , Tang Qin , Zhang Yuxia , Rashidi Maryam , Geng Lanlan , Vince James E 
Published: January 2023

AbstractGenetic lesions in X‐linked inhibitor of apoptosis (XIAP) pre‐dispose humans to cell death–associated inflammatory diseases, although the underlying mechanisms remain unclear. Here, we report that two patients with XIAP deficiency–associated inflammatory bowel disease display increased inflammatory IL‐1β maturation as well as cell death–associated caspase‐8 and Gasdermin D (GSDMD) processing in diseased tissue, which is reduced upon patient treatment. Loss of XIAP leads to caspase‐8‐driven cell death and bioactive IL‐1β release that is only abrogated by combined deletion of the apoptotic and pyroptotic cell death machinery. Namely, extrinsic apoptotic caspase‐8 promotes pyroptotic GSDMD processing that kills macrophages lacking both inflammasome and apoptosis signalling components (caspase‐1, ‐3, ‐7, ‐11 and BID), while caspase‐8 can still cause cell death in the absence of both GSDMD and GSDME when caspase‐3 and caspase‐7 are present. Neither caspase‐3 and caspase‐7‐mediated activation of the pannexin‐1 channel, or GSDMD loss, prevented NLRP3 inflammasome assembly and consequent caspase‐1 and IL‐1β maturation downstream of XIAP inhibition and caspase‐8 activation, even though the pannexin‐1 channel was required for NLRP3 triggering upon mitochondrial apoptosis. These findings uncouple the mechanisms of cell death and NLRP3 activation resulting from extrinsic and intrinsic apoptosis signalling, reveal how XIAP loss can co‐opt dual cell death programs, and uncover strategies for targeting the cell death and inflammatory pathways that result from XIAP deficiency.
Liver‐directed gene therapy for ornithine aminotransferase deficiency
Authors: Boffa Iolanda , Polishchuk Elena , De Stefano Lucia , Dell'Aquila Fabio , Nusco Edoardo , Marrocco Elena , Audano Matteo , Pedretti Silvia , Caterino Marianna , Bellezza Ilaria , Ruoppolo Margherita , Mitro Nico , Cellini Barbara , Auricchio Alberto , Brunetti‐Pierri Nicola 
Published: January 2023

AbstractGyrate atrophy of choroid and retina (GACR) is a chorioretinal degeneration caused by pathogenic variants in the gene encoding ornithine aminotransferase (OAT), an enzyme mainly expressed in liver. Affected patients have increased ornithine concentrations in blood and other body fluids and develop progressive constriction of vision fields leading to blindness. Current therapies are unsatisfactory and better treatments are highly needed. In two mouse models of OAT deficiency that recapitulates biochemical and retinal changes of GACR, we investigated the efficacy of an intravenously injected serotype 8 adeno‐associated (AAV8) vector expressing OAT under the control of a hepatocyte‐specific promoter. Following injections, OAT‐deficient mice showed reductions of ornithine concentrations in blood and eye cups compared with control mice injected with a vector expressing green fluorescent protein. AAV‐injected mice showed improved electroretinogram response and partial restoration of retinal structure up to one‐year post‐injection. In summary, hepatic OAT expression by AAV8 vector was effective at correction of hyperornithinemia and improved function and structure of the retina. In conclusion, this study provides proof‐of‐concept of efficacy of liver‐directed AAV‐mediated gene therapy of GACR.
Ecopipam for Tourette Syndrome: A Randomized Trial
Authors: Gilbert Donald L. , Dubow Jordan S. , Cunniff Timothy M. , Wanaski Stephen P. , Atkinson Sarah D. , Mahableshwarkar Atul R. 
Published: January 2023

BACKGROUND AND OBJECTIVES
All US Food and Drug Administration-approved medications for Tourette syndrome are antipsychotics, and their use is limited by the risk of weight gain, metabolic changes, and drug-induced movement disorders. Several small trials suggest that ecopipam, a first-in-class, selective dopamine 1 receptor antagonist, reduces tics with a low risk for these adverse events. This trial sought to further evaluate the efficacy, safety, and tolerability of ecopipam in children and adolescents with moderate to severe Tourette syndrome.

METHODS
This was a multicenter, randomized, double-blind, placebo-controlled, phase 2b trial. Subjects aged ≥6 to &lt;18 years with a baseline Yale Global Tic Severity Score Total Tic Score of ≥20 were randomly assigned 1:1 to ecopipam (n = 76) or placebo (n = 77). The primary endpoint was mean change over 12 weeks in the Yale Global Tic Severity Score Total Tic Score. The Clinical Global Impression of Tourette Syndrome Severity was the secondary endpoint. Safety and tolerability were evaluated at each study visit.

RESULTS
Total tic scores were significantly reduced from baseline to 12 weeks in the ecopipam group compared with placebo (least squares mean differences −3.44, 95% confidence interval −6.09 to −0.79, P = .01). Improvement in Clinical Global Impression of Tourette Syndrome Severity was also greater in the ecopipam group (P = .03). More weight gain was seen in subjects assigned to placebo. No metabolic or electrocardiogram changes were identified. Headache (15.8%), insomnia (14.5%), fatigue (7.9%), and somnolence (7.9%) were the most common adverse events.

CONCLUSIONS
Among children and adolescents with TS, ecopipam reduces tics to a greater extent than placebo, without observable evidence of common antipsychotic-associated side effects.
A COVID–19-járvány hatása a leggyakoribb légzőszervi megbetegedések lefolyására
Authors: Bogos Krisztina , Berta Judit , Cselkó Zsuzsa , Tisza Judit , Szilasi Mária , Simon Beatrix , Antus Balázs , Vizi Éva , Megyesfalvi Zsolt , Döme Balázs , Rózsás Anita , Török Szilvia 
Published: January 2023

Bevezetés: A SARS-CoV-2 által okozott fertőzés az elmúlt három
évben meghatározta mindennapi életünket, és nem várt terhet rótt az egészségügyi
ellátórendszerre, többek között azáltal, hogy komoly kockázati tényezőt
jelenthet a már meglévő, különböző légzőszervi megbetegedésekkel küzdő betegek
számára is. Célkitűzés: A COVID–19 és a fertőzéskor már
fennálló légzőszervi megbetegedések, elsősorban a krónikus obstruktív
tüdőbetegség (COPD), valamint az asztma összefüggéseinek feltárása.
Módszer: Hazai vizsgálatunkban közel 29 000 beteg adatait
dolgoztuk fel retrospektíven. Eredmények: Eredményeink alapján
elmondható, hogy a COPD mint társbetegség megléte a nemzetközi megállapítással
egybehangzóan összefüggést mutat a COVID–19-fertőzés súlyosságával, illetve
enyhén növeli az intenzív osztályos kezelés és a gépi lélegeztetés
szükségességének kockázatát a SARS-CoV-2 okozta megbetegedés során. Asztma
esetében mindezt nem sikerült kimutatnunk, vagyis sem a SARS-CoV-2-fertőzés
súlyosságát, sem az intenzív osztályos kezelés és a gépi lélegeztetés
szükségességét nem befolyásolta jelentősen az asztma mint társbetegség megléte.
Megbeszélés: Ahogy nemzetközi tanulmányokban is olvasható,
a COPD mint társbetegség megléte nem növeli jelentős mértékben a
SARS-CoV-2-fertőzés kockázatát. Ugyanakkor kijelenthető, hogy a COPD növeli a
COVID–19-pozitív betegek kórházba kerülésének esélyét, és emeli a megbetegedés
súlyosabb lefolyásának valószínűségét. Tekintettel a COPD-betegekben a tüdő
károsodása során végbemenő szerkezeti átépülésre és rendellenes regenerálódási
folyamatokra, e betegek a vírusfertőzés lezajlása után fokozott odafigyelést,
valamint személyre szabott rehabilitációt igényelnek.
Következtetés: Összességében elmondható, hogy a jövőben a
személyre szabott terápiás megközelítés bevezetéséhez elengedhetetlen a
különböző COPD-s fenotípusok (valamint egyéb krónikus tüdőbetegségek) és a
SARS-CoV-2-fertőzés klinikai megnyilvánulásainak mélyreható vizsgálata. Orv
Hetil. 2023; 164(2): 51–56.
Special abilities and qualities of young fencers aged 10-12 as criteria for success in sports
Authors: Klymenchenko Victoriya , Kryventsova Iryna , Chamera Michał 
Published: January 2023

Background and purpose. The success of young athletes depends on many personality characteristics and elements of the training system. The purpose of the study is to identify the most significant special abilities and qualities of young fencers aged 10-12 years old at the initial stage of training.
Material and methods. Leading coaches and qualified fencers of sports clubs participated in the study (n=21). The participants were selected according to their rating among Ukrainian specialists, most of whom are representatives of fencing clubs in Kharkiv (Ukraine). A questionnaire containing questions about the main elements of general training of young fencers was used.
Results. The main indicators of a fencer's general readiness are: intellectual development (knowledge, skills, abilities); physical development (anthropometric data and psychosomatics); physical qualities; technical readiness; tactical preparedness (understanding, ability, skills). The levels of significance of the specified indicators of the young fencer's personality have been established. The most important indicators are: intellectual skills (95.23%) and skills (90.47%); development of dexterity (61.90%) and speed (42.85%); the fencer's understanding of the purpose of performing technical techniques (71.42%).
Conclusions. At the initial stages of training young fencers, it is recommended to pay attention to the special abilities and qualities of young fencers. This approach makes it possible to promote the success of young fencers in sports.
Increasing the level of coordination abilities of young taekwondo athletes aged 13-14 under martial law
Authors: Lyuchkova Elizaveta , Nesen Olena , Bartik Pavol , Skrobecki Jerzy 
Published: January 2023

Background and Study Aim. Negative changes in the socio-economic environment in the territory of martial law (Ukraine) have a great impact on the ability to continue the training process for athletes. To a lesser extent, this affects experienced world-class athletes who have the opportunity to continue training in other countries. As for young athletes, they are closely connected with their family. This fact reduces the possibility of their preparation. The purpose of the work is to develop the coordination abilities of taekwondo fighters aged 13-14 under martial law
Material and methods. The study involved young taekwondo players from Ukraine (n=8, age - 13-14 years old), compactly living in the same area. A survey was conducted among experienced trainers (n=10), who had the opportunity to answer the questionnaire through electronic means of communication ( similar to the Likert scale.) Parents gave their consent to the participation of their children in the experiment. Participants were given homework in the form of exercises to develop the ability to maintain balance. The participants performed the Romberg test on the left and right leg with visual control, without control and Yarotsky's test. Participants conducted tests during a specially organized ZOOM video conference. This study was conducted by the Declaration of Helsinki and was approved by the Ethics Committee of University. All participants were aware during the informed consent process that the results of this study may be published.
Results. According to the coaches, the most important qualities for taekwondo athletes are speed and coordination. Among the coordination abilities trainers note as the most important the following: the sense of rhythm, the sense of time and distance, balance and vestibular stamina. Equilibrium indicators on an unstable support (Romberg test) have undergone significant positive changes (at p˂0.05). Although the Yarotsky test indicators had positive improvements, they did not have significant differences (at p˃0.05).
Conclusions. When declaring martial law, it is recommended to pay special attention to the life safety of participants in the education and training process. It is also recommended to use exercises on an unstable support, which are in the nature of imitation percussion and protective actions. This approach leads to a tangible improvement in the balance of young athletes.
Kingship and Covenant: Reconsidering the Oath of David and Jonathan
Authors: Johnson Sophia R. C. 
Published: January 2023

Abstract
Frank Moore Cross’s model of covenant as an early legal means of extending fictive kinship has dominated Anglo-American biblical scholarship for the last thirty years. His central case study is the covenant between David and Jonathan and the associated oath sworn by Jonathan in 1 Sam 20. In trying to distill a simple covenant concept, however, Cross collapses distinctions of genre and context, reducing the significance of secular covenant as a form of political bond to simply merging kinship groups. In this article, I reanalyze the oath in 1 Sam 20:12–17, contextualizing it with other material throughout 1 and 2 Samuel and comparing it to ancient Near Eastern treaty formulations to show its political significance in Israelite history. In contrast to Cross’s notion of imposing kinship obligations, the oath addresses questions of succession and dynasty, anticipating the political turmoil of David’s ascension to the throne. Deuteronomistic language and concern with Davidic succession shared between the oath and the divine promise in 2 Sam 7 point to a common vision of a united kingship. Far from the reiteration of a simple kinship bond, Jonathan’s oath of loyalty to their covenant becomes a keystone in the Deuteronomistic ideal of an eternal Davidic dynasty.
Assessment of the impact of polar bears on the grouping of pacific walruses at the Cape Vankarem rookery during the summer-autumn period in 2017–2021
Authors: Zagrebelniy S. V. 
Published: January 2023

The aim of this work is to assess the impact of polar bears (Ursus maritimus Phipps, 1774) on the distribution, abundance, and migration activity of Pacific walrus (Odobenus rosmarus divergens Illiger, 1815) on the coastal rookery Cape Vankarem (Chukotka Autonomous District) during the period from August to October 2017–2021.Also, based on the analysis of data on the number of walruses and bears on the rookery and analysis of photographs, we tried to assess the physical condition of predators, the degree of their attachment to the rookery, i. e. to determine how the age and sex composition of the bear group at the rookery changes during the development of walrus migration activity on the Cape Vankarem rookery. It has been established that: along with anthropogenic influence, polar bears are one of the main factors scaring walruses on the Cape Vankarem coastal rookery; immature bears spend significantly more time at the rookery than adult females with pups or adult males; the physical condition of the majority of bears is good, but the adult males are more frequently below average in fatness; while in the rookery, no food and space competition for food resources was observed between bears.
Epidemiology of tetanus in Canada, 1995–2019
Authors: Salem Nicole , Huang Grace , Squires Susan G. , Salvadori Marina I. , Li Y. Anita 
Published: January 2023

Abstract
Objectives
This report aims to use tetanus hospitalization data to describe the epidemiology in Canada from 1995 to 2019 and to assess progress on national reduction targets, including validating that Canada has eliminated maternal and neonatal tetanus (MNT).

Methods
Tetanus hospitalizations and fatalities occurring between 1995 and 2019 were retrieved from the Canadian Institute for Health Information (CIHI) and Statistics Canada. Cases coded with ICD-10 codes A33, A34, or A35 as the primary diagnosis (or ICD-9 equivalents) were included. The Canadian national case definition was used for generic tetanus and definitions from the World Health Organization were referenced for MNT. R version 4.0.2 was used for analyses.

Results
From 1995 to 2019, 155 non-MNT, 6 neonatal, and 0 maternal tetanus cases were retrieved from CIHI. However, all 6 neonatal cases were excluded after validating with provincial/territorial public health officials. In the same time period, there were 91 national notifications of tetanus. Cases were distributed relatively equally across the country, with the exception of the territories, where zero cases were reported. Adults 75 and over had significantly higher incidence rates compared to younger age groups (p<0.001). Ten deaths were reported during the timeframe.

Conclusion
Tetanus incidence remains low and hospitalization data reveal that Canada has met its reduction target of maintaining 5 cases or fewer annually in recent years. For MNT, Canada has successfully met the elimination target of zero cases. Continued vaccination efforts must be practiced for all age groups, including those aged 75 years and older, to sustain targets moving forward.
Bayesian statistics applied to the location of the source of explosions at Stromboli Volcano, Italy
Authors: Saccorotti Gilberto , Chouet Bernard , Martini Marcello , Scarpa Roberto 
Published: January 2023

Abstract
We present a method for determining the location and spatial extent of the source of explosions at Stromboli Volcano, Italy, based on a Bayesian inversion of the slowness vector derived from frequency-slowness analyses of array data. The method searches for source locations that minimize the error between the expected and observed slowness vectors. For a given set of model parameters, the conditional probability density function of slowness vectors is approximated by a Gaussian distribution of expected errors. The method is tested with synthetics using a five-layer velocity model derived for the north flank of Stromboli and a smoothed velocity model derived from a power-law approximation of the layered structure. Application to data from Stromboli allows for a detailed examination of uncertainties in source location due to experimental errors and incomplete knowledge of the Earth model. Although the solutions are not constrained in the radial direction, excellent resolution is achieved in both transverse and depth directions. Under the assumption that the horizontal extent of the source does not exceed the crater dimension, the 90% confidence region in the estimate of the explosive source location corresponds to a small volume extending from a depth of about 100 m to a maximum depth of about 300 m beneath the active vents, with a maximum likelihood source region located in the 120- to 180-m-depth interval.
A further note on earthquake size distributions
Authors: Scholz Christopher H. 
Published: January 2023

AbstractAt the scale range of 1 to 10 km, faults are not continuous surfaces but are tabular bodies composed of a mesh of subparallel unconnected strands, or subfaults. The cumulative length distribution of these subfaults is observed to be a power law with an approximate exponent of −2 and an upper fractal limit at W*, the seismogenic width. If it is assumed that small earthquakes, with lengths L ≤ W*, represent the rupture of these subfaults, this offers a physical explanation for small earthquakes having their observed power-law distribution with exponent −2/3. This further implies that any earthquake will be composed of a population of subevents involving the rupture of this population of subfaults: precisely what was proposed by Frankel (1991) to explain the ω−2 high-frequency falloff of earthquake displacement spectra.
Clinical, Laboratory, Cytometry and Cytogenetic Characteristics of a Cohort of Patients Diagnosed with Multiple Myeloma for the First Time in a Third-Level Hospital in Medellín, Colombia, Survival after 8 Years of Follow-Up
Authors: Atencia-Flórez Carlos , Quintero-Valencia Catalina , Mondragón-Arismendy María , Cardona-Arias Andrés , Regino-Agamez Carlos , Vélez-Urrego Julián 
Published: January 2023

Background: Multiple myeloma is the second most common hematologic malignancy after lymphomas. Few studies have characterized significant and full variables at the time of diagnosis of multiple myeloma in Colombia, and there is no data evaluating patients for follow-up.
Materials and Methods: A retrospective cohort study is presented, describing the clinical, laboratory, cytometric, and cytogenetic characteristics of patients with a de novo diagnosis of multiple myeloma evaluated in a reference hematology laboratory attached to a highly complex hospital in Medellín, Colombia. We follow them until death as a main outcome.
Results: A total of 170 patients with a de novo diagnosis of multiple myeloma were collected from a database of 421 patients with different monoclonal gammopathies. Mainly, it was found that 50.8% of the patients were men; the median age was 62 years; 65.4% had secretion of the IgG kappa; half of the patients presented International Staging System (ISS) Stage III. The β2 macroglobulin >4 mg/L and creatinine >2 mg/dl were the main variables significantly associated with survival (Hazard Ratio (HR) 2.4 and 2, respectively). Eighty-five percent of patients presented with bone lytic lesion involvement and less than 3% with extramedullary involvement. Conventional Banding Karyotype (CBK) genetic risk assessment yield was poor, compared with although scarce data regarding Cytogenetic risk assessment based on Fluorescence in-situ Hybridization (FISH).
Conclusion: The clinical profile of the patients with a de novo diagnosis of multiple myeloma in our cohort is similar to that described in international studies. The diagnosis of multiple myeloma was documented at younger ages, with more advanced stages, anemia, and a high percentage of bone disease. ISS provides an excellent tool for prognosis purposes. Cytogenetic risk assessment based on FISH should be done for all MM patients from therapeutic implications. We need standardized protocols for bone marrow sample manipulation and processing in order to guarantee good correlation for plasma cells count methods.
Legal aspects of fiscal enforcement in the Second Polish Republic
Authors: Nieścior Bartosz 
Published: January 2023

Purpose of the study: The research goal of the article was to analyze the fiscal enforcement model in the Second Polish Republic's legal system. The research problems are: whether the enforcement activities carried out had a clear legal basis; were the enforcement actions carried out without charge at the time they were carried out?
Methodology: The article was prepared based on the literature on the legal aspects of fiscal enforcement in the Second Polish Republic. "Desk research" is the method that was used in the analysis.
Main findings: The fiscal enforcement institution in the Second Polish Republic fulfilled its role, as well as the normative acts issued by the legislator regulating its scope, also through numerous amendments, accurately adapted the enforcement provisions to the then dynamically changing legal reality.
Application of the study: The article refers to the legal aspects related to the institution of fiscal enforcement in the Second Polish Republic. The article's content may be helpful for historians and lawyers dealing with aspects of fiscal enforcement in the Second Polish Republic.
Originality/Novelty of the study: The article concerns the legal aspects of fiscal enforcement in the interwar period in Poland. The study indicates the legal basis for enforcement, the public authorities responsible for it, and its types. It should be recognized that fiscal enforcement significantly evolved in the early years of the independent Polish state. The legal regulations governing execution until the first half of the Second Polish Republic were modelled on those from the period of partitions, and numerous amendments were introduced only later.