bluuebunny/crossref_metadata_2025_split huggingface dataset:
Impaired Oligodendrocyte Maturation Is an Early Feature in SCA3 Disease Pathogenesis
Authors: Schuster Kristen H. , Zalon Annie J. , Zhang Hongjiu , DiFranco Danielle M. , Stec Nicholas R. , Haque Zaid , Blumenstein Kate G. , Pierce Amanda M. , Guan Yuanfang , Paulson Henry L. , McLoughlin Hayley S. 
Published: January 2022

Spinocerebellar ataxia Type 3 (SCA3), the most common dominantly inherited ataxia, is a polyglutamine neurodegenerative disease for which there is no disease-modifying therapy. The polyglutamine-encoding CAG repeat expansion in theATXN3gene results in expression of a mutant form of the ATXN3 protein, a deubiquitinase that causes selective neurodegeneration despite being widely expressed. The mechanisms driving neurodegeneration in SCA3 are unclear. Research to date, however, has focused almost exclusively on neurons. Here, using equal male and female age-matched transgenic mice expressing full-length human mutantATXN3, we identified early and robust transcriptional changes in selectively vulnerable brain regions that implicate oligodendrocytes in disease pathogenesis. We mapped transcriptional changes across early, mid, and late stages of disease in two selectively vulnerable brain regions: the cerebellum and brainstem. The most significant disease-associated module through weighted gene coexpression network analysis revealed dysfunction in SCA3 oligodendrocyte maturation. These results reflect a toxic gain-of-function mechanism, asATXN3KO mice do not exhibit any impairments in oligodendrocyte maturation. Genetic crosses to reporter mice revealed a marked reduction in mature oligodendrocytes in SCA3-disease vulnerable brain regions, and ultrastructural microscopy confirmed abnormalities in axonal myelination. Further study of isolated oligodendrocyte precursor cells from SCA3 mice established that this impairment in oligodendrocyte maturation is a cell-autonomous process. We conclude that SCA3 is not simply a disease of neurons, and the search for therapeutic strategies and disease biomarkers will need to account for non-neuronal involvement in SCA3 pathogenesis.SIGNIFICANCE STATEMENTDespite advances in spinocerebellar ataxia Type 3 (SCA3) disease understanding, much remains unknown about how the disease gene causes brain dysfunction ultimately leading to cell death. We completed a longitudinal transcriptomic analysis of vulnerable brain regions in SCA3 mice to define the earliest and most robust changes across disease progression. Through gene network analyses followed up with biochemical and histologic studies in SCA3 mice, we provide evidence for severe dysfunction in oligodendrocyte maturation early in SCA3 pathogenesis. Our results advance understanding of SCA3 disease mechanisms, identify additional routes for therapeutic intervention, and may provide broader insight into polyglutamine diseases beyond SCA3.
On Using ANT for Studying Information Systems: A (Somewhat) Socratic Dialogue
Authors: Latour Bruno 
Published: January 2022

Tai Bruno Latouro straipsnio, kuris parašytas kaip dialogas, „On Using ANT for Studying Information Systems: A (Some­what) Socratic Dialogue“ vertimas. Profesoriaus ir doktoranto dialogo metu atskleidžiamas veikėjo-tinklo-teorijos turinys, išdėstomas B. Latouro požiūris į refeksyvumą, struktūralistinį aiškinimą ir socialinių mokslų paskirtį. Pirmą kartą šį dialogą B. Latouras paskelbė 2004 m. C. Avgerou, C. Ciborra, F. F. Land sudarytoje knygoje The Social Study of Information and Communication Technology. Innovation, Actors, and Contexts(Oxford University Press).
Alterations in oxytocin and vasopressin in men with problematic pornography use: The role of empathy
Authors: Kor Ariel , Djalovski Amir , Potenza Marc N. , Zagoory-Sharon Orna , Feldman Ruth 
Published: January 2022

Abstract

Background
Addictive behaviors share clinical, genetic, neurobiological and phenomenological parallels with substance addictions. Despite the prevalence of compulsive sexual behaviors, particularly problematic pornography use (PPU), how neuroendocrine systems relate to PPU is not well understood. Preclinical studies demonstrate alterations in oxytocin and arginine vasopressin (AVP) function in animal models of addiction, but no human study has tested their involvement in PPU.

Method
Participants included 122 males; 69 reported PPU, and 53 were demographically-matched participants without PPU. Plasma oxytocin and AVP levels and oxytocin-to-AVP balance were measured at baseline. Salivary oxytocin was assessed at baseline and in response to four videos depicting neutral/positive social encounters. Participants reported on empathy and psychiatric symptoms.

Results
Baseline plasma AVP levels were elevated in men with PPU, and the ratio of oxytocin-to-vasopressin suggested AVP dominance. Men with PPU reacted with greater oxytocin increases to presentation of neutral/positive social stimuli. Decreased empathic tendencies were found in men with PPU, and this reduced empathy mediated links between oxytocin and pornography-related hypersexuality. Structural equation modeling revealed three independent paths to pornography-related hypersexuality; two direct paths via increased AVP and higher psychiatric symptoms and one indirect path from oxytocin to pornography-related hypersexuality mediated by diminished empathy.

Conclusions
Findings are among the first to implicate neuropeptides sustaining mammalian attachment in the pathophysiology of pornography-related hypersexuality and describe a neurobiological mechanism by which oxytocin-AVP systems and psychiatric symptomatology may operate to reduce empathy and lead to pornography-related hypersexuality.
Transorbitomaxillaris percutan endoszkópos gastrostomia
Authors: Pap István , Jakab-Péter Kinga , Uzsaly János , Tóth István , Barabás Márta-Andrea , Fábián György , Lujber László 
Published: January 2022

Összefoglaló. Fej-nyaki daganattal diagnosztizált és kezelt
betegeknél a szájon keresztüli táplálás gyakran nem lehetséges vagy nehezítetté
válik a kezelés valamelyik fázisában. A beteg enteralis táplálásának
biztosítására tartós és hatékony megoldást kell nyújtanunk, erre megfelelő
választás lehet a percutan endoszkópos gastrostomia. A fej-nyaki régióban a
daganat elhelyezkedése, kiterjedtsége és a műtétek miatt megváltozott anatómiai
viszonyok sokszor gátat szabnak a gasztroszkóp hagyományos, szájon át történő
sikeres levezetésének, és így különleges megoldásokra lehet szükség.
Közleményünkben egy, a szakirodalomban is ritkán alkalmazott percutan
endoszkópos gastrostomakészítésről számolunk be. A bemutatott esetben egy
kiterjedt, rosszindulatú fej-nyaki daganat miatt totális maxillectomián és
orbitaexenteratión átesett betegben a műtét következtében kialakult
arcdefektuson át történt a gastroscopia és a tápszonda levezetése, ugyanis
komplett szájzár következtében a beteg a táplálkozásra képtelen volt. A fent
említett módszerrel sikerült a beteg hosszú távú enteralis táplálását megoldani
minimálinvazív módon. Az összetett kóros esetek gyakran állítják kihívások elé a
gyakorló klinikust. Esetbemutatásunkkal szeretnénk felhívni a figyelmet a
hagyományos, megszokott módszerek helyett sokszor nagyobb sikerrel alkalmazható,
személyre szabott terápiás lehetőségekre. Orv Hetil. 2022; 163(3): 116–119.
Summary. Peroral, enteral feeding is often impossible in patients
with head and neck cancer. Percutaneous endoscopic gastrostomy is a
well-established, quick, minimally invasive, and safe procedure for providing
long-term enteral feeding. Space-occupying tumour mass and altered anatomy due
to surgery inhibit the gastroscope’s peroral introduction and the feeding tube’s
placement in some instances. Various access routes and modified insertion
techniques are recommended to overcome the feeding tube insertion challenges. We
present a rare case of a 64-year-old head and neck cancer patient who was unable
to eat orally due to trismus and had a facial soft tissue defect following total
maxillectomy and orbital exenteration. A complete oesophago-gastro-duodenoscopy
and feeding tube insertion were performed transfacially. Percutaneous endoscopic
gastrostomy was successful and uneventful via the maxillary and
orbital soft tissue facial defect. No complication was noted, and long-term
enteral feeding of the patient was provided in a minimally invasive way.
Surgeons often face challenging cases when treating patients with head and neck
cancer. The modification of standard procedures is sometimes required to adapt
surgical techniques to the patient’s specific case. Orv Hetil. 2022; 163(3):
116–119.
Arthrogryposis–renalis diszfunkció–cholestasis szindróma
Authors: Mikó Ágnes , Lóth Szendile , Müller Judit , Lotz Bence , Rossitto Patrizio , Szabolcs Andrea , Benyó Gábor , Jávorszky Eszter , Tory Kálmán , Dezsőfi Antal 
Published: January 2022

Összefoglaló. Az arthrogryposis–renalis diszfunkció–cholestasis
(ARC) szindróma igen rossz prognózisú autoszomális recesszív kórkép. A három
vezető tünethez társulhat központi idegrendszeri érintettség, siketség,
cardiovascularis anomália (pitvari és kamrai sövényhiány),
thrombocytafunkció-zavar, rekurrens szepszisek, ichthyosis, valamint
súlyfejlődésben való elmaradás. A háromnapos újszülöttet neuromuscularis
betegség gyanúja miatt vettük át a szülészeti intézményből. Fizikális vizsgálat
során pes equinovarust és hypotrophiás küllemet tapasztaltunk. Kéthetes korában
súlyos tubulopathia, valamint cholestasis igazolódott normális
gamma-glutamil-transzferáz-szint mellett. A perifériás vérkenet vizsgálata során
abnormális morfológiájú thrombocyták ábrázolódtak. Súlygyarapodást komplex
felépített enteralis és parenteralis táplálás segítségével sem sikerült elérni.
Három hónapos korára a gyermek súlya 15%-kal a születési súlya alatt volt. A
kórkép szövődményeként ismétlődő bakteriális véráramfertőzés súlyosbította az
állapotát. Az újszülött klinikai képe az ARC-szindrómának felelt meg. A kóroki
gének szekvenálása során a VPS33B-génben homozigóta
c.498+1G>T variáns igazolódott, mely igazolja a betegség fennállását. Orv
Hetil. 2022; 163(2): 74–78.
Summary. Arthrogryposis–renal dysfunction–cholestasis (ARC) syndrome
is an autosomal recessive multisystem disorder that typically presents with
arthrogryposis, renal tubular leak and neonatal cholestatic jaundice. It can be
accompanied by nervous system abnormalities, deafness, structural cardiac
defects, abnormal platelet morphology, recurrent sepsis, ichthyosis and failure
to thrive. The three-day-old neonate was admitted for a suspected neuromuscular
disorder. On examination, clubfoot, jaundice and hypotonia were found.
Laboratory evaluation revealed tubulopathy and cholestasis with normal
gamma-glutamyl transferase level. Peripheral blood smear evaluation revealed
abnormally giant platelets. Despite the combined enteral and parenteral
nutrition, the infant experienced severe failure to thrive. The phenotype of the
presented neonate is consistent with ARC syndrome. Sequencing of the causal
genes revealed a homozygous consensus splice site VPS33B
mutation (c.498+1G>T), confirming the clinical diagnosis. Orv Hetil. 2022;
163(2): 74–78.
Short-term and long-term changes in seizure frequency and neurocognitive status in pediatric patients after surgery for mixed neuronal-glial brain tumors
Authors: Gorlova L. A. , Shmeleva O. O. , Fedorov E. V. , Kim A. V. , Dinikina Yu. V. , Khachatryan W. A. , Samochernykh K. A. 
Published: January 2022

Background. Neuronal and mixed neuronal-glial tumors are rare brain tumors that are most commonly observed in pediatric patients. The main clinical manifestations are presented by epilepsy substantially affecting child’s physical and neurocognitive development.Objective: to assess short-term and long-term changes in seizure frequency and neurocognitive status in pediatric patients who underwent surgery for mixed neuronal-glial tumors.Material and methods. Thirty-eight pediatric patients underwent surgery for mixed neuronal-glial tumors at the Almazov Research Medical Centre within the period from 2008 to 2018. All patients had comprehensive diagnostic examination including clinical neurological examination, neuroimaging tests, electroencephalography and electrocorticography before and after surgery. The postoperative changes in the seizures rate were assessed by using the 1993, 1996 Engel classification. Neurocognitive status was evaluated using The Child Behavior Checklist (a component of the Achenbach System of Empirically Based Assessment).Results. During the postoperative period, 84% of patients were found to be seizure-free; 42.6% demonstrated no clinically relevant behavioral disturbances or social adaption issues after the surgery. However, 76% of children experienced poor concentration. In a long-term perspective, seizures reoccured in 25% of patients and were not associated with any magnetic resonance signs of tumor progression.Conclusion. A gross total resection of the brain tumor was the main factor that affected changes over time in seizure rate. Assessment of long-term changes over time has shown that the patients tended to retain concentration difficulties, which should be taken into account while planning school education programs.
Discrimination between Earthquake P Waves and Microtremors via a Generative Adversarial Network
Authors: Liu Heyi , Li Shanyou , Song Jindong 
Published: January 2022

ABSTRACT
The accurate and reliable discrimination of earthquakes from background noise is a primary task of earthquake early warning (EEW); however, ubiquitous and complex microtremor signals substantially complicate this task. To mitigate this problem, a generative adversarial network (GAN) is adopted to distinguish between earthquakes and microtremors in this study. We train a GAN based on 52,537 K-NET and KiK-net strong ground motion records from Japan, and use the well-trained discriminator to identify 5373 P waves and 5373 microtremors in the testing set. The results indicate that this algorithm can correctly identify 99.89% of P waves and 99.24% of microtremors with high confidence. In addition, a verification of the proposed algorithm on data from the Great East Japan earthquake confirms that this model can achieve robust results for local records of large events and ultimately discriminate earthquakes from microtremors. This algorithm is an exploratory test of a GAN for identifying earthquake P waves. Though the GAN uses only P waves for training (There are no microtremors in the input data.), it has extensive potential in seismological and EEW applications.
Emerging of Virtual Reality (VR) Technology in Education and Training
Authors: Chimakurthi Venkata Naga Satya Surendra 
Published: January 2022

This study comprehensively explores the incorporation of virtual reality (VR) technology into the teaching and training realm. In general, the research investigates and presents the effectiveness of this modern technology in education in relation to the learning process. Besides presenting the multiple benefits of VR in education, the study also highlights the issues that the education department is experiencing while implementing VR as a modern tool of learning. This study will surely help the scholars to get a better insight into different VR aspects in relation to education and will provide them with a future-thinking to resolve the issues to make it a better tool for the education and training sphere.
Fatores de risco associados à síndrome metabólica em mulheres com tireoidite crônica autoimune
Authors: Prezotto Marina Destefano , Da Silva Adriana Paula , Fonseca Elvi Cristina Rojas , Franciscon Priscila de Melo , Borges Maria de Fátima 
Published: January 2022

Este é um estudo de corte transversal com delineamento descritivo, com dois grupos populacionais, tipo antes e depois, com abordagem quantitativa do tipo analítica com correlação entre variáveis, realizado entre 2018 e 2019, com objetivo de analisar fatores de risco cardiometabólicos, clínicos e laboratoriais associados à síndrome metabólica em mulheres com tireoidite crônica autoimune eutireoideas, na pré e pós-menopausa, acompanhadas em serviço terciário. Participaram 109 mulheres, sendo 56 com Tireoidite Crônica Autoimune em status eutireoideo e 53 sem disfunção tireoidiana. Das 56 mulheres, 25 foram incluídas no grupo com Tireoidite Crônica Autoimune na pré-menopausa e 31 foram incluídas na pós-menopausa e, das 53 mulheres com função tireoidiana normal, 25 foram incluídas no grupo comparativo na pré-menopausa e 28 no grupo comparativo na pós-menopausa. O perfil metabólico sugeriu resistência insulínica, com taxas mais elevadas no grupo com tireoidite pré e pós-menopausa e grupo controle pós, bem como perfil lipídico desfavorável. Apresentaram síndrome metabólica 36% do grupo afetado pré-menopausa e 51,6% do grupo afetado pós, contra nenhuma e 14,3% dos grupos comparativos, respectivamente. Se resistência insulínica ou obesidade decorreram da tireoidite, o estudo não permitiu concluir. Pacientes com tireoidite devem receber atenção com relação ao perfil lipídico.
Komnas HAM, Investigating Serious Human Rights Violations
Authors: Amiruddin Amiruddin 
Published: January 2022

This paper reviews the dynamics and challenges of the investigation conducted by Komnas HAM in accordance with Law No. 26/2000 of the Human Rights Court. In addition, this paper also explains the dynamics between Komnas HAM and the Attorney General as an investigator in Law No.26/2000. A critical related issue is the provision of the authority to investigate the National Human Rights Commission and the Attorney General's Office, which have been a serious obstacle to resolving gross human rights violations in Indonesia. This article also provides an analysis of the likely challenges faced by Komnas HAM in future investigations.
Acute Compartment Syndrome Modeling with Sequential Infusion Shows the Deep Posterior Compartment Is Not Functionally Discrete
Authors: Schupbach Drew , Honjol Yazan , Bouklouch Yasser , Merle Geraldine , Harvey Edward J. 
Published: January 2022

Background:
Clinical case series have indicated that 1 or 2-compartment decompression of the anterior or lateral leg may be sufficient for release, but, currently, no cadaveric model has verified that approach. The objective of this study was to investigate the functional relationship between compartments by alternating sequences of infusion and fasciotomy release.

Methods:
This study utilized multicompartment sequential pressurization with simultaneous monitoring by continuous pressure sensors to model compartment syndrome in a human cadaver leg. Subsequent sequential release of compartments and continuous streaming of pressure readings permitted unique insights.

Results:
A leg model allowed the examination of pressure changes in all 4 compartments as treated with sequential fasciotomies. The successful modeling of lower-leg pressures consistent with compartment syndrome showed that discrepancies relative to accepted concepts were seen when the deep posterior compartment was pressurized in isolation. Also, release of 1 of the 2 of either the anterior or lateral compartments seems to be sufficient for decompression to acceptable pressure levels.

Conclusions:
The deep posterior compartment does not appear to be completely discrete and instead follows the pressurization curve of the posterior muscle group. This indicates that release of the deep posterior compartment may not be needed in all acute compartment syndrome scenarios.

Clinical Relevance:
Surgical techniques can be modified for treatment of acute compartment syndrome to avoid large scar lengths, deep dissection, and multiple exposures that could improve patient outcomes.
Rate of Return to Work After Periacetabular Osteotomy and Its Influencing Factors
Authors: Fujita Jun , Doi Nobunao , Kinoshita Koichi , Sakamoto Tetsuya , Seo Hajime , Yamamoto Takuaki 
Published: January 2022

Background:
Periacetabular osteotomy (PAO) has been reported as a treatment for patients with symptomatic developmental dysplasia of the hip (DDH). Several studies have investigated the rate of return to sports activities after PAO, but few studies have evaluated the rate of return to work. In the present study, we aimed to identify the rate of return to work at 1 year after PAO and its affecting factors.

Methods:
We retrospectively evaluated 83 patients (85 hips) with symptomatic DDH who had undergone PAO between December 2015 and June 2020. Patients who had returned to work at 1 year after PAO were classified into the returnee group, and those who had not were classified into the non-returnee group. The returnee group included patients who could return to their original job (original) or to a different job (non-original). The non-returnee group included patients who could not return to work because of hip symptoms (hip) and those who did not return for reasons other than hip symptoms (non-hip). We analyzed clinical parameters, including the Harris hip score, Japanese Orthopaedic Association Hip Disease Evaluation Questionnaire, 36-Item Short Form Survey, and radiographic parameters, as well as the type of work.

Results:
Sixty-eight patients (70 hips; 82.4%) returned to work at 1 year after PAO (returnee group), and 15 patients (15 hips; 17.6%) were in the non-returnee group. Among the 15 patients in the non-returnee group, 7 were classified into the non-hip subgroup and 8 were classified into the hip subgroup. No significant differences were observed between the returnee group and the hip subgroup in terms of clinical parameters or type of work.

Conclusions:
One year after PAO, 8 patients (8 hips; 9.4%) could not return to work because of hip symptoms; both clinical parameters and the type of work showed no direct relationship with postoperative working status.

Level of Evidence:
Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.
THE EFFECTS OF AGE ON BIOCHEMISTRY PROFILES OF ACEH CATTLE BLOOD
Authors: Khairita Lia , Esfandiari Anita , Widhyari Sus Derthi , Winarsih Wiwin 
Published: January 2022

The purpose of this study was to analyze the effects of age on the biochemistry profiles of aceh cattle, which included examinations of: total protein (TP), albumin, globulin, albumin/globulin ratio (A/G), aspartate aminotransferase (AST), gamma-glutamyltransferase (GGT), alkaline phosphatase (ALP), creatine kinase (CK), blood urea nitrogen (BUN), calcium (Ca), phosphorus (P) and magnesium (Mg) . Sixteen clinically healthy aceh cattle aged 2-4 years old, and 16 aceh cattle aged 4-6 years old were purposively selected for the study. Blood samples were drawn from the jugular vein and subjected to blood biochemistry measurements using commercial kits. The results showed that serum concentrations of TP, globulin, AST and Ca of aceh cattle were significantly (P0.05) higher in age group 4-6 years old, while ALP activity was significantly (P0.05) higher in age group 2-4 years old. In conclusion, age must be considered as a factor when interpreting the blood biochemistry profiles of aceh cattle.
COMPARISON OF DIFFERENT COMMERCIAL SEROLOGY KITS FOR THE DETERMINATION OF SEROPOSITIVE TOXOPLASMOSIS IN CATTLE IN INDONESIA
Authors: Subekti Didik T , Valinata Sisca , Fong Sulinawati 
Published: January 2022

This research aims to explain the evaluation of the differences in four commercial kits for the detection of serological toxoplasmosis used in Indonesia. The results of the study found that the toxoplasmosis seropositivity determined by the four commercial kits showed a significant difference (P0.05). Seropositive toxoplasmosis obtained using Pastorex, Toxotest, IDScreen, and Toxo Ab were 35.12%, 60.12%, 26.19%, and 10.12% respectively. IDScreen had a good agreement with Toxo Ab (Gwet's AC1= 0.623) and a moderate agreement with Pastorex (Gwet's AC1= 0.494-0.511). Toxotest had a low agreement with three commercial kits (Gwet's AC1= 0.2) but had a moderate agreement with western blotting (WB) and modified agglutination test (MAT) (Gwet's AC1= 0.458-0.557).