bluuebunny/crossref_metadata_2025_split huggingface dataset:
Quantitative Study on Morphological Characteristics of Barchan Dunes in Yamarak Desert, China
Authors: Guang-Pu Jia , Guang Yang , Xue-Ying Han , He-Jun Zuo , Yun-Feng Yao 
Published: March 2022

Although scholars have conducted many studies on barchan dunes in deserts and sandy areas around the world, few studies have been conducted on the morphological characteristics and changes in movement patterns of barchan dunes in the Yamarak Desert, China. To assess the changes in and movement patterns of these dunes, As well as the impact on the wind-sand hazards and geohazards around the study area, we selected several typical barchan dunes of different sizes in the Yamarak Desert and measured and calculated their morphologies using a combination of a 3-D laser scanner (RIEGL VZ-2000) and remote sensing image interpretation. The results show that the average coverage of the barchan dunes in the Yamarak Desert was 37.19%. Moreover, 62.81% of this area was covered by interdune sites. The movement velocity of the barchan dunes in this study area was 0–20 m/a, with an average movement velocity of 8.45 m/a. Of these dunes, 44.18% were fast-dominated dunes, 37.20% were particularly fast-dominated dunes, and 18.62% were moderately fast-dominated dunes. The barchan dunes moved in the 95–130° direction, which was basically consistent with the main wind direction of the area. The determination and function fitting of the barchan dune morphology shows that the correlation coefficients between all of the morphological parameters are greater than 0.8, except for the degree of spread and the symmetry of the two wings. We suggest adding the influences of other factors affecting barchan dunes and dune chains in the Yamarak Desert in future studies and paying attention to new wind-sand hazards and geohazards in the downwind corridor.
HLA-DRB1 and –DQB1 Alleles, Haplotypes and Genotypes in Emirati Patients with Type 1 Diabetes Underscores the Benefits of Evaluating Understudied Populations
Authors: Al Yafei Zain , Mack Steven J. , Alvares Marion , Ali Bassam R. , Afandi Bachar , Beshyah Salem A. , Sharma Charu , Osman Wael , Mirghani Rajaa , Nasr Amre , Al Remithi Sareea , Al Jubeh Jamal , Almawi Wasim Y. , AlKaabi Juma , ElGhazali Gehad 
Published: March 2022

Background: HLA class II (DR and DQ) alleles and antigens have historically shown strong genetic predisposition to type 1 diabetes (T1D). This study evaluated the association of DRB1 and DQB1 alleles, genotypes, and haplotypes with T1D in United Arab Emirates.Materials and Methods: Study subjects comprised 149 patients with T1D, and 147 normoglycemic control subjects. Cases and controls were Emiratis and were HLA-DRB1 and -DQB1 genotyped using sequence-based typing. Statistical analysis was performed using Bridging Immunogenomic Data-Analysis Workflow Gaps R package.Results: In total, 15 DRB1 and 9 DQB1 alleles were identified in the study subjects, of which the association of DRB1*03:01, DRB1*04:02, DRB1*11:01, DRB1*16:02, and DQB1*02:01, DQB1*03:02, DQB1*03:01, and DQB1*06:01 with altered risk of T1D persisted after correcting for multiple comparisons. Two-locus haplotype analysis identified DRB1*03:01∼DQB1*02:01 [0.44 vs. 0.18, OR (95% CI) = 3.44 (2.33–5.1), Pc = 3.48 × 10−10]; DRB1*04:02∼DQB1*03:02 [0.077 vs. 0.014, OR = 6.06 (2.03–24.37), Pc = 2.3 × 10−3] and DRB1*04:05∼DQB1*03:02 [0.060 vs. 0.010, OR = 6.24 (1.79–33.34), Pc = 0.011] as positively associated, and DRB1*16:02∼DQB1*05:02 [0.024 vs. 0.075, OR = 0.3 (0.11–0.74), Pc = 0.041] as negatively associated with T1D, after applying Bonferroni correction. Furthermore, the highest T1D risk was observed for DR3/DR4 [0.104 vs. 0.006, OR = 25.03 (8.23–97.2), Pc = 2.6 × 10−10], followed by DR3/DR3 [0.094 vs. 0.010, OR = 8.72 (3.17–25.32), Pc = 3.18 × 10−8] diplotypes.Conclusion: While DRB1 and DQB1 alleles and haplotypes associated with T1D in Emiratis showed similarities to Caucasian and non-Caucasian populations, several alleles and haplotypes associated with T1D in European, African, and Asian populations, were not observed. This underscores the contribution of ethnic diversity and possible diverse associations between DRB1 and DQB1 and T1D across different populations.
Incidence of Developmental Venous Anomalies in Patients With Multiple Sclerosis: A 3 Tesla MRI Study
Authors: Magyar Marton , Gattringer Thomas , Enzinger Christian , Hassler Eva , Partl Richard , Khalil Michael , Reishofer Gernot , Deutschmann Hannes , Fazekas Franz 
Published: March 2022

ObjectivesThere is evidence of involvement of the venous system in multiple sclerosis (MS). If this bears also an association with the frequency and extent of developmental venous anomalies (DVA) still has to be determined. We therefore investigated this in patients with different phenotypes of MS and in comparison, to a control population.MethodsWe analyzed the contrast-enhanced T1-weighted MR scans of 431 patients (clinically isolated syndrome—CIS, n = 108; MS, n = 323) and of 162 control individuals for the presence of a DVA. We also measured the size of the DVA and draining vein and compared the DVA frequency between MS phenotypes.ResultsA DVA was found in 38 (8.8 %) of patients with CIS or MS and in 11 (6.8%) controls (p = 0.4). DVA frequency was highest in CIS (14.8%) and lowest in progressive MS (4.0%). The mean cranio-caudal and axial extension of the DVA was significantly lower in MS patients than controls (p &lt; 0.05).ConclusionsThe frequency of DVA in MS patients is comparable to that in controls. Whether DVA size and appearance may change over time will have to be investigated in a longitudinal manner and with larger sample size.
SCN2A-Related Epilepsy: The Phenotypic Spectrum, Treatment and Prognosis
Authors: Zeng Qi , Yang Ying , Duan Jing , Niu Xueyang , Chen Yi , Wang Dan , Zhang Jing , Chen Jiaoyang , Yang Xiaoling , Li Jinliang , Yang Zhixian , Jiang Yuwu , Liao Jianxiang , Zhang Yuehua 
Published: March 2022

ObjectiveThe aim of this study was to analyze the phenotypic spectrum, treatment, and prognosis of 72 Chinese children with SCN2A variants.MethodsThe SCN2A variants were detected by next-generation sequencing. All patients were followed up at a pediatric neurology clinic in our hospital or by telephone.ResultsIn 72 patients with SCN2A variants, the seizure onset age ranged from the first day of life to 2 years and 6 months. The epilepsy phenotypes included febrile seizures (plus) (n = 2), benign (familial) infantile epilepsy (n = 9), benign familial neonatal-infantile epilepsy (n = 3), benign neonatal epilepsy (n = 1), West syndrome (n = 16), Ohtahara syndrome (n = 15), epilepsy of infancy with migrating focal seizures (n = 2), Dravet syndrome (n = 1), early infantile epileptic encephalopathy (n = 15), and unclassifiable developmental and epileptic encephalopathy (n = 8). Approximately 79.2% (57/72) patients had varying degrees of developmental delay. All patients had abnormal MRI findings with developmental delay. 91.7% (55/60) patients with de novo SCN2A variants had development delay, while only 16.7% (2/12) patients with inherited SCN2A variants had abnormal development. 83.9% (26/31) SCN2A variants that were located in transmembrane regions of the protein were detected in patients with development delay. Approximately 69.2% (9/13) SCN2A variants detected in patients with normal development were located in the non-transmembrane regions. Approximately 54.2% (39/72) patients were seizure-free at a median age of 8 months. Oxcarbazepine has been used by 38 patients, and seizure-free was observed in 11 of them (11/38, 28.9%), while 6 patients had seizure worsening by oxcarbazepine. All 3 patients used oxcarbazepine and with seizure onset age &gt; 1 year presented seizure exacerbation after taking oxcarbazepine. Valproate has been used by 53 patients, seizure-free was observed in 22.6% (12/53) of them.ConclusionThe phenotypic spectrum of SCN2A-related epilepsy was broad, ranging from benign epilepsy in neonate and infancy to severe epileptic encephalopathy. Oxcarbazepine and valproate were the most effective drugs in epilepsy patients with SCN2A variants. Sodium channel blockers often worsen seizures in patients with seizure onset beyond 1 year of age. Abnormal brain MRI findings and de novo variations were often related to poor prognosis. Most SCN2A variants located in transmembrane regions were related to patients with developmental delay.
Lockdown Impact on Stress, Coping Strategies, and Substance Use in Teenagers
Authors: Bourduge Cédrine , Teissedre Frédérique , Morel Florence , Flaudias Valentin , Izaute Marie , Brousse Georges 
Published: March 2022

BackgroundIn response to the COVID-19 pandemic, the French government took many measures, the most notable of which was a national lockdown on 17 March 2020. Its effects have been widely studied, but to our knowledge, no study has sought to determine how adolescents have adapted to cope with this situation. The present study set out to explore teenagers' stress levels, coping strategies, and substance use during this period.MethodsThis paper is a cross-sectional study that rides on an existing prevention program interviewed 348 French middle school students (209 girls and 139 boys) in grade 8 (Mage = 13.45; SDage = 0.54) using an online questionnaire between March 17 and May 11, 2020 (COVID-19 lockdown). The study examined the teenagers' perceived stress, coping strategies they had used, including recent use of tobacco, alcohol and cannabis, during COVID-19 lockdown.ResultsTeenagers reported lower perceived stress during lockdown than usually, with a significant decrease for girls. Those who perceived the least social support reported the highest levels of stress. The strategies of planning, behavioral disengagement, self-distraction, positive reframing, acceptance, and religion were used more than usual, while active coping and self-blame were used less. Acceptance was the most often used strategy and a source of decreased stress during lockdown. A significant decrease in recent tobacco, alcohol and cannabis use was also observed.ConclusionChanges in the use of coping strategies, withdrawal from the stressful school environment, and greater exposure to parents than to peers caused adolescents to be less stressed and to decrease their substance use during the lockdown.
Differences in the Awareness and Knowledge of Radiological and Nuclear Events Among Medical Workers in Japan
Authors: Ochiai Kanae , Oka Tomo , Kato Nagisa , Kondo Yuji , Otomo Yasuhiro , Swienton Raymond E. 
Published: March 2022

BackgroundPrevious research revealed a lack of comfort and knowledge regarding nuclear and radiological events among medical staff. We investigated the awareness and knowledge of radiological and nuclear events among the Japanese medical staff by comparing differences by occupation (doctors, nurses, and other medical specialists).MethodsWe conducted a cross-sectional questionnaire survey among trainees undergoing Japanese disaster medical training courses between July 2014 and February 2016. The differences by occupation were evaluated for all questions on awareness and knowledge concerning disasters or radiological and nuclear events and demographics.ResultsAmong the occupations, there were significant differences in the willingness to work onsite based on the types of disaster, familiarity with the national disaster medical response system, the accuracy rate of some knowledge about medical practice and the risk, and demographic characteristics such as practical experience and educational degree. The accuracy rates of responses to some questions on knowledge were very low in all occupations.ConclusionThere were significant differences in awareness and knowledge of radiological and nuclear events by occupation. We believe that the results can be used to develop and modify the content of training courses on radiological and nuclear events to make such courses beneficial for each healthcare worker.
Lessons Learnt From the Experiences of Primary Care Physicians Facing COVID-19 in Benin: A Mixed-Methods Study
Authors: Bello Kéfilath , De Lepeleire Jan , Agossou Christian , Apers Ludwig , Zannou Djimon Marcel , Criel Bart 
Published: March 2022

IntroductionIn sub-Saharan Africa, there is a need to better understand and guide the practice of primary care physicians (PCPs), especially in a crisis context like the COVID-19 pandemic. This study analyses the experiences of PCPs facing COVID-19 in Benin and draws policy lessons.MethodsThe study followed a fully mixed sequential dominant status design. Data were collected between April and August 2020 from a sample of PCPs in Benin. We performed descriptive analyses on the quantitative data. We also performed bivariate analyses for testing associations between various outcomes and the public/private status of the PCPs, their localization within or outside the cordon sanitaire put in place at the beginning of COVID-19, and their practice' category. A thematic content analysis was done on qualitative data. Results from both analyses were triangulated.ResultsNinety PCPs participated in the quantitative strand, and 14 in the qualitative. The median percentage of the COVID-19 control measures implemented in the health facilities, as reported by the PCPs, was 77.8% (interquartile range = 16.7%), with no difference between the various groups. While 29.4% of the PCPs reported being poorly/not capable of helping the communities to deal with COVID-19, 45.3% felt poorly/not confident in dealing with an actual case. These percentages were bigger in the private sector. The PCP's experiences were marked by anxiety and fear, with 80.2% reporting stress. Many PCPs (74.1%) reported not receiving support from local health authorities, and 75.3% felt their concerns were not adequately addressed. Both percentages were higher in the private sector. The PCPs especially complained of insufficient training, insufficient coordination, and less support to private providers than the public ones. For 72.4 and 79.3% of the PCPs, respectively, the pandemic impacted services utilization and daily work. There were negative impacts (like a decrease in the services utilization or the quality of care), but also positive ones (like improved compliance to hygiene measures and new opportunities).ConclusionOur study highlighted the need for more structured support to PCPs for optimizing their contribution to epidemics control and good primary healthcare in Benin. Efforts in this direction can build on several good practices and opportunities.
EMIL FROESCHEL'S CONTRIBUTION TO THE DEVELOPMENT OF SPEECH THERAPEUTIC SCIENCE AND PRACTICE
Authors: BEREZAN V. 
Published: March 2022

The article presents the figure of the prominent Austrian scientist Emil Froeschels, who introduced the term “logopedics” into clinical practice, is considered the founder of speech pathology (speech therapy) education, founded the world's first International Association of Logopedics and Phoniatrics (IALP) and a number of other national associations and unions. The article focuses on his leadership qualities as a scientist, teacher and public figure. On the basis of the research the directions of researches of the scientist are defined: problems of speech therapy (stuttering, aphasia, dysarthria, etc.), speech and voice therapy, methodical materials and the analysis of comparative researches, researches of physiology and problems of speech and voice and speech organs, philosophical researches, questions speech psychology, problems of education, training and upbringing of children with and without disabilities, phonetic observations, etc. The methods of speech pathology (speech therapy) work with the patient proposed by E. Froeschels, which were introduced into clinical practice, are analyzed.
Myotonic Dystrophy of Rossolimo – Curschmann – Steinert – Batten: Own Clinical Observation and Features of Differential Diagnosis
Authors: Яровая К.А. , Солодовникова Ю.А. , Дрибина С.И. , Сон А.С. 
Published: March 2022

Миотоническая дистрофия (МД) Россолимо – Куршманна – Штейнерта – Баттена, или тип 1, – наследственное аутосомно-доминантное заболевание с сочетанием миотонического, миопатического синдромов и мультисистемных проявлений и феноменом антиципации. МД была описана несколькими авторами: Г.И. Россолимо (1901 г.), F.F. Batten, H. Curschmann, H. Steinert (1909 г.). Заболевание чаще проявляет себя на 2–4-м десятилетиях жизни. В начале болезни миотония превалирует над миодистрофией, но по мере прогрессирования дистрофии миотонический компонент становится менее заметным. Пациенты имеют характерный фенотип из-за лобной алопеции, двустороннего птоза, «губ тапира», амимии, атрофии мимических, височных, жевательных мышц, грудино-ключично-сосцевидных мышц. К другим неврологическим симптомам относятся сомноленция в дневное время, прогрессирующий когнитивный дефицит, дизартрия, отсутствие ахилловых и коленных рефлексов, атаксия; атрофия мышц дистальных отделов ног, со временем распространяющаяся на кисти, степпаж. МР-признаки в головном мозге неспецифичны и включают в себя умеренную корковую атрофию и вентрикуломегалию, диффузные двусторонние повреждения белого вещества в лобно-теменной и передней височной областях. К системным признакам относят раннюю субкапсулярную катаракту, нарушения сердечного ритма и проводимости, расстройства дыхания, гастроэзофагеальную рефлюксную болезнь, эндокринные нарушения, костные изменения.
Myotonic dystrophy (MD) of Rossolimo – Kurshman – Steinert – Batten or type 1 is a hereditary autosomaldominantdiseasewithacombinationofmyotonic, myopathicsyndromesandmultisystem manifestations and the phenomenon of anticipation. MD has been described by several authors:G.I. Rossolimo (1901), F.F. Batten, H. Curschmann, H. Steinert (1909). The disease often manifestsin the 2nd-4th decades of life. At the onset of the disease, myotonia prevails over myodystrophy, but as dystrophy progresses the myotonic component becomes less noticeable. Patients have a characteristic phenotype due to frontal alopecia, bilateral ptosis, tapir mouth, amimia, atrophy of the facial, temporal, and masticatory muscles, sternocleidomastoid muscles. Other neurological symptoms include daytime somnolence, progressive cognitive deficiency, dysarthria, absence of Achilles and knee reflexes, ataxia; distal muscular dystrophy of legs, eventually spreading to the hands, steppage. MR signs in the brain are nonspecific and include moderate cortical atrophy and ventriculomegaly, diffuse bilateral lesions of white matter in the frontoparietal and anterior temporal regions. Systemic signs include early subcapsular cataracts, cardiac arrhythmias and conduction disorders, respiratory disorders, gastroesophageal reflux disease, endocrine diseases, bone changes.
Limb-Girdle Muscular Dystrophy Type 2A (Leiden- Möbius Phenotype) with Compound Heterozygous Mutationin 4 Exon of CAPN3 Gene: Own Observation
Authors: Рушкевич Ю.Н. , Мальгина Е.В. , Куликова С.Л. , Лихачев С.А. , Мирзоян А.Р. 
Published: March 2022

В статье приведено описание собственного наблюдения генетически подтвержденного случая конечностно-поясной мышечной дистрофии (КПМД) 2А типа (фенотип Лейдена – Мебиуса). По клиническим признакам различные типы КПМД крайне трудно дифференцировать между собой. КПМД являются примером, когда мутации разных генов, локализованных в разных хромосомах, кодирующих разные белки, приводят к развитию сходных клинических фенотипов. Кальпаинопатия является наиболее распространенной формой КПМД и характеризуется значительным клиническим полиморфизмом и тяжестью заболевания. Установить точный диагноз позволяет молекулярно-генетическое исследование, которое должно быть рекомендовано в случае наличия типичных клинических проявлений, а также при выявлении высоких цифр сывороточной креатининфосфокиназы. В последнее десятилетие достигнуты значительные успехи в изучении молекулярных механизмов наследственных нервно-мышечных заболеваний. Важным аспектом является вопрос планирования детей и необходимость молекулярно-генетического исследования супругов пациентов для оценки риска рождения больного ребенка.
The article describes own observation of a genetically confirmed limb-girdle muscular dystrophy type 2A (Leiden- Möbius phenotype). According to clinical signs, various types of CPMD are extremely difficult to differentiate between themselves. LGMD’s are an example where mutations of different genes located in different chromosomes encoding different proteins lead to the development of similar clinical phenotypes. Calpainopathy is the most common form of LGMD and is characterized by significant clinical polymorphism and severity of the disease. It is possible to establish an accuratediagnosis of molecular genetic study, which should be recommended in case of typical clinical manifestations, as well as in case of detection of high figures of serum CK. Significant advances have been made over the past decade in the study of the molecular mechanisms of hereditary neuromuscular diseases. An important aspect is child planning issues and the need for molecular genetic research of patient spouses to assess the risk of having a sick child.
Pengaruh Sistem Produksi Dan Peran Penyuluh Pertanian Terhadap Keberhasilan Usahatani Jamur Tiram Putih (Pleurotus ostreatus)
Authors: Sumarga Amar Romidin , Dasipah Euis , KS Karyana 
Published: March 2022

This study aims to find out the relationship between the Production System And Role of Agricultural Extensionists on the Success of White Oyster Mushroom Farming (Pleurotus Ostreatus). The analysis unit is farmers of White Oyster Mushroom Farming. The technique of determining respondents was carried out in a census of 60 units. Analysis techniques are used pearson correlation and path analysis. The results of the study obtained by the production system in the business activities of white oyster mushrooms inCugenangSubdistrict are characterized at the level of application of white oyster mushroom cultivation technology consisting of dimensions: from the process of providing raw materials; production process; harvesting and post-harvest handling; Distribution and warehousing obtained a rate of achievement of 78.87 % good criteria. The role of PPL is related to the business activities of white oyster mushrooms inCugenangSubdistrict which consists of dimensions: Advisors; Technicians; Liaison; Organizer; and innovators obtained a level of achievement of 77.01 % good criteria. The success of the white oyster mushroom farming business inCugenangSubdistrict consisting of dimensions: productivity; product quality; continuity ; and farming efficient . Obtained a level of achievement of 77.50 % good criteria. There is a strong relationship between the role of PPL and the production system in the white oyster mushroom farming business inCugenangSubdistrict, indicated by the correlation coefficient r = 0.93. The better the role of PPL, the better the production system. The production system and the role of PPL have a positive effect on the success of white oyster mushroom farming inCugenangSubdistrict. Production System contribution was obtained 81,23 % and contribution of PPL Role 12.60 %.