bluuebunny/crossref_metadata_2025_split huggingface dataset:
The distribution of intrafamilial CYP21A2 mutant alleles and investigation of clinical features in Turkish children and their siblings in Southeastern Anatolia
Authors: Karaoglan Murat 
Published: November 2019

AbstractBackgroundThe genotype-phenotype relationship shows regional variability in 21-hydroxylase deficiency (21-OHD) caused by mutations in theCYP21A2gene. This study focuses on the genotype-phenotype compatibility between patients and their siblings in a region where consanguineous marriage is common.MethodsThe most common mutations (I2G-P30L-I172N-V237E-M239K-V281L-Q318X-R356W-F306 + nt) were studied in 60 children with 21-OHD and 40 siblings (12 symptomatic and 28 asymptomatic; mean age 5.89 ± 4.63 and 8.34 ± 2.22 years, respectively). The allele number (patients; 93 siblings; 70 alleles) was counted for each case. Salt wasting (SW; n = 38), simple virilizing (SV; n = 11) and non-classical congenital adrenal hyperplasia (NCCAH; n = 11) types were compared with their genotypes classified into groups Null-AB-C-D-E based on enzyme impairment.ResultsDisease-causing mutations were identified in unrelated alleles: 80 out of 93 alleles (86%) in the patients: SW, 51/56 (91%); SV, 14/16 (87.4%) and NCCAH, 15/21 (71.4%). There were 43 out of 70 alleles (61.4%) in the siblings (asymptomatic, 25/50 [50%]; symptomatic, 18/20 [90%]). The most frequently detected mutations in the patients were: I2G (22%), Q318X-P30L-V281L (13% each). The distribution of the most common mutations by clinical types was: SW: I2G-Q318X (30.2%-19.6%), SV: I172NI2G (37.5%-18.7%), NCCAH: V281L-P30L (33.3%-28.5%). In patients and symptomatic siblings, the concordance percentages by genotype groups were: Null (100%-100%), A (85%-60%), B (100%-Not applicable), C (41.6%-50%). Eleven out of 28 asymptomatic siblings had disease-causing mutations (four, severe; one, moderate; six, mild). The distribution of genotypes by phenotypes were: SW: Null-A (88%), SV: B-A (50%-41.6%), NCCAH: C (100%).ConclusionsThis study showed that the most common alleles were IN2G-Q381X-R356W-P30L-V281L in the children with 21-OHD and asymptomatic siblings, and that the phenotype can be predicted from the genotype except for the P30L-V281L. This result suggests that the most common mutations in 21-OHD are similar to previous reports, but that the genotype-phenotype compatibility is good except for group C showing regional variability, and that genotyping of siblings discovered new patients.
Crystal structure of catena-[(μ2-pyrazine-κ2
N:N′)-bis(O,O′-di-ethyldithiophosphato-κ2
S,S′)cadmium(II)], {C12H24CdN2O4P2S4}n
Authors: Tan Yee Seng , Tiekink Edward R.T. 
Published: November 2019

Abstract
C12H24CdN2O4P2S4, monoclinic, P2/n (no. 13), a = 11.7844(2) Å, b = 7.6063(1) Å, c = 13.1965(2) Å, β = 111.594(2)°, V = 1099.86(3) Å3, Z = 2, R
gt(F) = 0.0171, wR
ref(F
2) = 0.0467, T = 100 K.
Suitability of eight years kadam tree (Neolamarckia cadamba) in chemical pulping
Authors: Misbahuddin M. , Biswas D. , Roy U. 
Published: November 2019

Abstract
Kadam is a fast growing tree species in Bangladesh. The main aim of this study is to observe the influence of tree age of kadam on chemical pulping. In this research 8, 18 and 30 years old kadam wood were pulped under different active alkali of 16, 18 and 19 % maintaining 25 % sulphidity and its effect on chemical composition, kappa number, pulp yield and delignification was investigated. The result shows that the content of extractive, lignin, alpha-cellulose and hemicellulose increase to different content along with the increase of tree age. There are also some differences of pulping properties with different tree age. Pulp yield of 8 years old kadam is higher than other ages of kadam trees. The hand sheets were made and the physical strength properties likely tear, tensile and burst were evaluated at two freeness level. The pulps produced from 8 year kadam tree obsessed reasonable strength properties. These could be used for producing moderate quality writing, printing and wrapping papers. Pulp wood of 8 years kadam is more suitable for pulping, compared to that 18 and 30 years.
Effect of ionic liquid pretreatment on paper physical property and pulp refining performance
Authors: Li Mingfu , Min Douyong , Long Xing , Tu Qiyuan , Zhang Kelei , Wang Shuangfei , Luo Lianxin 
Published: November 2019

AbstractIonic liquids (IL) of 1-butyl-3-methylimidazolium chloride (BmimCl) homogeneously mixed with bleached kraft pulp (BKP) were used to investigate the change of paper properties and refining performance under the general conditions. Fiber morphology and paper strength were determined by fiber analyzer and paper physical property tester. The surface morphology and chemical properties of fiber was also performed by scanning electron microscope (SEM), X-ray Diffraction (XRD), Fourier transform infrared spectroscopy (FTIR) and X-ray photoelectron spectroscopy (XPS). The results indicated the optimized conditions of BmimCl pretreatment were BmimCl dosage of 1.5 mL/g, the time and temperature of 30 min and 80 °C, respectively. The physical properties of paper and internal bond strength of fiber was improved by BmimCl pretreatment. BmimCl pretreatment facilitated swelling and fibrillation of fiber, increased the crystalline index of fiber and decreased the lignin and extractives on the surface of fiber, which leading to improve fiber strength and tensile strength. For the same refining energy input, saving the maximum refining energy consumption was 14.0 % after BmimCl pretreatment, which is beneficial to improve the application of ionic liquid in pulp and papermaking.
Defibration mechanisms and energy consumption in the grinding zone – a lab scale equipment and method to evaluate groundwood pulping tools
Authors: Heldin Magnus , Wiklund Urban 
Published: November 2019

Abstract
Groundwood pulping is a process that employs large machines, making them difficult to use in research. Lab scale grinders exist, but even though they are smaller, the sizes of the grinding stones or segments make them cumbersome to exchange and tailor. This study presents a method and an apparatus for investigating the detailed mechanisms and the energy requirements behind the fibre separation process. A well-defined grinding tool was used at three different temperatures to demonstrate that the equipment can differentiate levels of energy consumption and defibration rates, confirming the well-known fact that a higher temperature facilitates defibration. It is also shown how the equipment can be used to study the influence of grinding parameters, exemplified by the effect of temperature on the way fibres are separated and the character of the produced fibres. A key feature of the equipment is the use and evaluation of small grinding surfaces, more readily designed, produced, evaluated and studied. This reduces both the cost and time necessary for testing and evaluating. At the same time, a technique to produce well defined grinding surfaces was employed, which is necessary for repeatability and robust testing, not achievable with traditional grinding stones.
Aberrant ER Stress Induced Neuronal-IFNβ Elicits White Matter Injury Due to Microglial Activation and T-Cell Infiltration after TBI
Authors: Sen Tanusree , Saha Pampa , Gupta Rajaneesh , Foley Lesley M. , Jiang Tong , Abakumova Olena S. , Hitchens T. Kevin , Sen Nilkantha 
Published: November 2019

Persistent endoplasmic reticulum (ER) stress in neurons is associated with activation of inflammatory cells and subsequent neuroinflammation following traumatic brain injury (TBI); however, the underlying mechanism remains elusive. We found that induction of neuronal-ER stress, which was mostly characterized by an increase in phosphorylation of a protein kinase R-like ER kinase (PERK) leads to release of excess interferon (IFN)β due to atypical activation of the neuronal-STING signaling pathway. IFNβ enforced activation and polarization of the primary microglial cells to inflammatory M1 phenotype with the secretion of a proinflammatory chemokine CXCL10 due to activation of STAT1 signaling. The secreted CXCL10, in turn, stimulated the T-cell infiltration by serving as the ligand and chemoattractant for CXCR3+T-helper 1 (Th1) cells. The activation of microglial cells and infiltration of Th1 cells resulted in white matter injury, characterized by impaired myelin basic protein and neurofilament NF200, the reduced thickness of corpus callosum and external capsule, and decline of mature oligodendrocytes and oligodendrocyte precursor cells. Intranasal delivery of CXCL10 siRNA blocked Th1 infiltration but did not fully rescue microglial activation and white matter injury after TBI. However, impeding PERK-phosphorylation through the administration of GSK2656157 abrogated neuronal induction of IFNβ, switched microglial polarization to M2 phenotype, prevented Th1 infiltration, and increased Th2 and Treg levels. These events ultimately attenuated the white matter injury and improved anxiety and depressive-like behavior following TBI.SIGNIFICANCE STATEMENTA recent clinical study showed that human brain trauma patients had enhanced expression of type-1 IFN; suggests that type-1 IFN signaling may potentially influence clinical outcome in TBI patients. However, it was not understood how TBI leads to an increase in IFNβ and whether induction of IFNβ has any influence on neuroinflammation, which is the primary reason for morbidity and mortality in TBI. Our study suggests that induction of PERK phosphorylation, a characteristic feature of ER stress is responsible for an increase in neuronal IFNβ, which, in turn, activates microglial cells and subsequently manifests the infiltration of T cells to induce neuroinflammation and subsequently white matter injury. Blocking PERK phosphorylation using GSK2656157 (or PERK knockdown) the whole cascade of neuroinflammation was attenuated and improved cognitive function after TBI.
Critical Dynamics and Coupling in Bursts of Cortical Rhythms Indicate Non-Homeostatic Mechanism for Sleep-Stage Transitions and Dual Role of VLPO Neurons in Both Sleep and Wake
Authors: Lombardi Fabrizio , Gómez-Extremera Manuel , Bernaola-Galván Pedro , Vetrivelan Ramalingam , Saper Clifford B. , Scammell Thomas E. , Ivanov Plamen Ch. 
Published: November 2019

Origin and functions of intermittent transitions among sleep stages, including brief awakenings and arousals, constitute a challenge to the current homeostatic framework for sleep regulation, focusing on factors modulating sleep over large time scales. Here we propose that the complex micro-architecture characterizing sleep on scales of seconds and minutes results from intrinsic non-equilibrium critical dynamics. We investigate θ- and δ-wave dynamics in control rats and in rats where the sleep-promoting ventrolateral preoptic nucleus (VLPO) is lesioned (male Sprague-Dawley rats). We demonstrate that bursts in θ and δ cortical rhythms exhibit complex temporal organization, with long-range correlations and robust duality of power-law (θ-bursts, active phase) and exponential-like (δ-bursts, quiescent phase) duration distributions, features typical of non-equilibrium systems self-organizing at criticality. We show that such non-equilibrium behavior relates to anti-correlated coupling between θ- and δ-bursts, persists across a range of time scales, and is independent of the dominant physiologic state; indications of a basic principle in sleep regulation. Further, we find that VLPO lesions lead to a modulation of cortical dynamics resulting in altered dynamical parameters of θ- and δ-bursts and significant reduction in θ–δ coupling. Our empirical findings and model simulations demonstrate that θ–δ coupling is essential for the emerging non-equilibrium critical dynamics observed across the sleep–wake cycle, and indicate that VLPO neurons may have dual role for both sleep and arousal/brief wake activation. The uncovered critical behavior in sleep- and wake-related cortical rhythms indicates a mechanism essential for the micro-architecture of spontaneous sleep-stage and arousal transitions within a novel, non-homeostatic paradigm of sleep regulation.SIGNIFICANCE STATEMENTWe show that the complex micro-architecture of sleep-stage/arousal transitions arises from intrinsic non-equilibrium critical dynamics, connecting the temporal organization of dominant cortical rhythms with empirical observations across scales. We link such behavior to sleep-promoting neuronal population, and demonstrate that VLPO lesion (model of insomnia) alters dynamical features of θ and δ rhythms, and leads to significant reduction in θ–δ coupling. This indicates that VLPO neurons may have dual role for both sleep and arousal/brief wake control. The reported empirical findings and modeling simulations constitute first evidences of a neurophysiological fingerprint of self-organization and criticality in sleep- and wake-related cortical rhythms; a mechanism essential for spontaneous sleep-stage and arousal transitions that lays the bases for a novel, non-homeostatic paradigm of sleep regulation.
Modelo de Capacidad en Tecnologías de Información en Mipymes Colombianas
Authors: Díaz-Pinzón Beatriz Helena , Rodríguez V. María Teresa , Espinosa Moreno Juan Carlos 
Published: November 2019

La capacidad en tecnologías de información (TI) se reconoce como un conjunto de recursos en TI y su sinergia, que podrán potenciar a las organizaciones en el desarrollo de sus capacidades organizacionales y en la consecución de ventajas competitivas sostenibles. Múltiples definiciones de este constructo han sido propuestas; sin embargo, las condiciones particulares de las micros, pequeñas y medianas empresas (mipymes) hacen necesario proponer y validar un mode­lo que permita evaluar la capacidad en TI en este tipo de contextos. A partir de un análisis factorial exploratorio y confirmatorio, sobre una muestra de 517 mipymes, se valida un modelo de capacidad en TI. El modelo resultante describe la capacidad en TI en cuatro categorías principales: capacidad de cohesión organizacional del área de TI (categoría I), capacidad estratégica en TI (categoría II), infraestructura de TI (categoría III) y capacidad de relacionamiento externo (categoría IV), siendo las categorías I y II las que tienen una influencia más significativa sobre la capacidad de TI. La categoría I está representada por el conocimiento organizacional del personal de TI y su capacidad de relacionamiento al interior de la organización. La categoría II está descrita por el conocimiento estratégico de las TI y su capacidad de planeación estratégica en TI. Este modelo es una base para la medición de la capacidad en ti de las mipymes, así como para la generación de políticas públicas.
The development of phonological awareness during the First grade of elementary school in two different teaching contexts
Authors: Basso Fabiane Puntel 
Published: November 2019

L’objectif de cet article est de vérifier le développement de la conscience phonologique des enfants français au long du CP dans deux contextes différents d’enseignement. L’étude a lieu dans deux écoles primaires, localisées sur la région de Grenoble- France. Au total sont 30 élèves, 15 élèves de l’école A et 15 de la B. L’épreuve de conscience phonologique (ECP) a été utilisée pour évaluer les différents niveaux de conscience phonologique en début et fin d’année scolaire. En début d’année les élèves de la classe B ont eu de meilleurs résultats dans toutes les tâches de l’ECP. En fin d’année les différences n’ont pas été significatives. Il est possible que le contexte didactique et pédagogique des deux classes a joué un rôle important dans ce résultat car l’enseignement de la classe A est celui qui renforce le plus l’entrainement de la conscience phonologique et du code phonographique.***O desenvolvimento da consciência fonológica no primeiro ano do Ensino Fundamental em dois contextos diferentes de ensino*** O objetivo deste artigo foi verificar o desenvolvimento da consciência fonológica de crianças francesas ao longo do CP (primeiro ano do Ensino Fundamental) em dois contextos diferentes de ensino. O estudo foi realizado em duas escolas primárias, localizadas na região de Grenoble, na França. Participaram da pesquisa 30 alunos, 15 alunos da escola A e 15 de B. O teste de consciência fonológica (TCF) foi utilizado para avaliar os diferentes níveis de consciência fonológica no início e no final do ano letivo. No início do ano, os alunos da turma B obtiveram melhores resultados em todas as tarefas do TCF. No final do ano, as diferenças já não eram significativas. É possível que o contexto didático e pedagógico das duas classes tenha desempenhado um papel importante nesse resultado, uma vez que o ensino da classe A foi aquele que mais enfatizou a prática da consciência fonológica e do código fonográfico. Palavras-chave: Consciência fonológica. Ensino. Aprendizagem.
Adolescent Moral Development in Families
Authors: Dedih Ujang 
Published: November 2019

This study aimed to investigate parentsâ€™ goals and objectives in fostering the moral values to their adolescents, parentsâ€™ moral values instilled toward their teenagers, parentsâ€™ approach and methods in instilling moral values, and parentsâ€™ guidance development in nurturing moral values toward adolescent. This research used descriptive-analytic method, and qualitative-naturalistic approach. It applied observation, interview and documentation. It investigated three families with a different education background. Findings show that family with undergraduate education background is relatively very good in fostering the values of Akhlak Karimah to their teenagers. Their success was supported by clear goals and objectives, consistent and continuous delivery of materials, the use of appropriate approaches and methods, and good and harmonious family. These factors contributed to excellent moral education for their teenager.Â Family with a high school education background applied moral education using the formulation of objectives, materials, and the arrangement of a family atmosphere but the result was less optimal because they used less precise methods and approaches. Family with a junior high school education background had not achieved a satisfactory result in moral education for their teenagers because they did not formulate well objectives, materials, methods, approaches and the arrangement of a family atmosphereÂ
Brain Metastases from Biliary Tract Cancers: A Case Series and Review of the Literature in the Genomic Era
Authors: D'Andrea Megan R. , Gill Corey M. , Umphlett Melissa , Tsankova Nadejda M. , Fowkes Mary , Bederson Joshua B. , Brastianos Priscilla K. , Shrivastava Raj K. 
Published: November 2019

Abstract

Background
Biliary tract cancers (BTCs) are highly fatal malignancies that make up less than 1% of all cancers. BTC is often diagnosed at an unresectable stage; surgical resection remains the only definitive treatment. Brain metastases (BMs) from BTC are extremely rare, and few studies on patients with BMs from BTC exist. The aim of this study was to identify clinical characteristics associated with poor prognosis for patients with BMs from BTC.

Materials and Methods
We performed a retrospective review of electronic medical records for patients with BMs from BTC managed at Mount Sinai Hospital from 2000 to 2017. Data on patient characteristics, magnetic resonance imaging findings, treatment regimens, and clinical outcomes were analyzed.

Results
We identified 1,910 patients with BTC. Nine patients developed BMs, with an incidence of 0.47%. Of these nine patients, six had intrahepatic cholangiocarcinoma, two had extrahepatic cholangiocarcinoma, and one had gallbladder cancer. Six (66.7%) patients had one BM, one (11.1%) patient had two BMs, and two (22.2%) patients had three or more BMs. Four (44.4%) patients underwent BM resection, and seven (77.8%) received BM radiation. Median overall survival from time of BM diagnosis was 3.8 months (95% confidence interval 0.1–16.9).

Conclusion
Development of BMs from BTC is rare; however, prognosis is less than 4 months. BM diagnosis can occur within 2 years of primary diagnosis. As targeted therapeutics emerge, future studies ought to focus on identifying genomic BM markers associated with BTC subtypes.

Implications for Practice
In the largest retrospective study of biliary tract cancer brain metastases, the clinical presentation and outcomes are reported of nine patients with an extremely rare clinical entity. The genomic literature and potential therapeutic targets for these patients with limited treatment options is comprehensively and exhaustively discussed.
FORAGE YIELD AND PROTEIN CONTENT OF DIFFERENT FIELD PEA CULTIVARS AND OAT MIXTURES GROWN AS WINTER CROPS
Authors: KRGA İvan , SIMIĆ Aleksandar , MANDIĆ Violeta , BIJELIĆ Zorica , DŽELETOVIĆ željko , VASILJEVIĆ Sanja , ADŽIĆ Slađan 
Published: November 2019

Monoculture and intensive use of mechanization and inorganic chemicals are leading to degradation and
erosion of our soils and agro-ecosystems. At the same time, the production of animal feeds that are rich in
protein is constantly decreasing in many European countries. Modest cultivation needs, favorable content of
nutrients, and beneficial effect on the environment, make field pea and oat mixtures promising crops to tackle
these issues. In three growing seasons and without irrigation or fertilisation, we have examined two basic field
pea cultivars (Kosmaj, OS Adam) and one leafless cultivar (OS Letin) intercropped with oat (NS Jadar), with
seeding rates, field pea: oat – 100:10%; 100:20%. Results have shown that basic type cultivars have better
overall development and higher yields compared to leafless cultivar, whether grown as single crops or in the
mixture. Basic cultivars (OS Adam, Kosmaj) achieved higher protein content, compared to cultivar Letin,
163.9 g kg-1
, 153.3 g kg-1
, 136 g kg-1
, respectively. Yields were higher in 100:20% mixtures, compared to
100:10% mixtures and single grown crops, and we conclude that intercropping these species is superior to
cultivating them separately. Protein content wasn’t significantly different between two seeding rates, thus seed
rates shouldn’t be lower than 100:20%.&nbsp;
Automatic Phase‐Picking Method for Detecting Earthquakes Based on the Signal‐to‐Noise‐Ratio Concept
Authors: Fu Jihua , Wang Xu , Li Zhitao , Meng Hao , Wang Jianjun , Wang Wei , Tang Chengpei 
Published: November 2019

AbstractThe automatic phase‐picking detection of earthquakes is a challenge under the background of big data and strong noise circumstances. The short‐term average/long‐term average (STA/LTA) ratio is widely used to detect earthquake due to its simplicity and robustness. However, STA/LTA‐based methods may not perform well with noisy data. Based on the signal‐to‐noise‐ratio (SNR) concept, a short‐term power/long‐term power (STP/LTP) ratio method is proposed. The characteristic function and the detection thresholds of the STP/LTP method are given physical meanings. Through a sample analysis, the STP/LTP detection results of both the P and S phases are better than the results of the STA/LTA by means of mean deviation, standard deviations, distributions of detection results, error rate, and missed rate on different SNR levels. In general, the STP/LTP method inherits the simple characteristics of the STA/LTA method, and it is suitable for phase picking of low‐SNR seismic data.