bluuebunny/crossref_metadata_2025_split huggingface dataset:
Genetic and Clinical Features in 24 Chinese Distal Hereditary Motor Neuropathy Families
Authors: Xie Yongzhi , Lin Zhiqiang , Pakhrin Pukar Singh , Li Xiaobo , Wang Binghao , Liu Lei , Huang Shunxiang , Zhao Huadong , Cao Wanqian , Hu Zhengmao , Guo Jifeng , Shen Lu , Tang Beisha , Zhang Ruxu 
Published: December 2020

Background and Objectives: Distal hereditary motor neuropathy (dHMN) is a clinically and genetically heterogeneous group of inherited neuropathies. The objectives of this study were to report the clinical and genetic features of dHMN patients in a Chinese cohort.Aims and Methods: We performed clinical assessments and whole-exome sequencing in 24 dHMN families from Mainland China. We conducted a retrospective analysis of the data and investigated the frequency and clinical features of patients with a confirmed mutation.Results: Two novel heterozygous mutations in GARS, c.373G&gt;C (p.E125Q) and c.1015G&gt;A (p.G339R), were identified and corresponded to the typical dHMN-V phenotype. Together with families with WARS, SORD, SIGMAR1, and HSPB1 mutations, 29.2% of families (7/24) acquired a definite genetic diagnosis. One novel heterozygous variant of uncertain significance, c.1834G&gt;A (p.G612S) in LRSAM1, was identified in a patient with mild dHMN phenotype.Conclusion: Our study expanded the mutation spectrum of GARS mutations and added evidence that GARS mutations are associated with both axonal Charcot-Marie-Tooth and dHMN phenotypes. Mutations in genes encoding aminoamide tRNA synthetase (ARS) might be a frequent cause of autosomal dominant-dHMN, and SORD mutation might account for a majority of autosomal recessive-dHMN cases. The relatively low genetic diagnosis yield indicated more causative dHMN genes need to be discovered.
A Novel Predictive Tool for Determining the Risk of Early Death From Stage IV Endometrial Carcinoma: A Large Cohort Study
Authors: Song Zixuan , Wang Yizi , Zhou Yangzi , Zhang Dandan 
Published: December 2020

BackgroundEndometrial carcinoma is a common gynecological malignancy. Stage IV endometrial carcinoma is associated with a high risk of early death; however, there is currently no effective prognostic tool to predict early death in stage IV endometrial cancer.MethodsSurveillance, Epidemiology, and End Results (SEER) data from patients with stage IV endometrial cancer registered between 2004 and 2015 were used in this study. Important independent prognostic factors were identified by univariate and multivariate logistic regression analyses. A nomogram of all-cause and cancer-specific early deaths was constructed using relevant risk factors such as tumor size, histological grade, histological classification, and treatment (surgery, radiotherapy, chemotherapy).ResultsA total of 2,040 patients with stage IV endometrial carcinoma were included in this study. Of these, 299 patients experienced early death (≤3 months) and 282 died from cancer-specific causes. The nomogram of all-cause and cancer-specific early deaths showed good predictive power and clinical practicality with respect to the area under the receiver operating characteristic curve and decision curve analysis. The internal validation of the nomogram revealed a good agreement between predicted early death and actual early death.ConclusionsWe developed a clinically useful nomogram to predict early mortality from stage IV endometrial carcinoma using data from a large cohort. This tool can help clinicians screen high-risk patients and implement individualized treatment regimens.
Among Persons With Multiple Sclerosis (MS), Objective Sleep, Psychological Functioning, and Higher Physical Activity Scores Remained Stable Over 2 Years—Results From a Small Study Under Naturalistic Conditions
Authors: Sadeghi Bahmani Dena , Gonzenbach Roman , Kesselring Jürg , Bansi Jens , Motl Robert W. , Cordier Dominik , Rothen Oliver , Niedermoser Daryl , Gerber Markus , Brand Serge 
Published: December 2020

Background: Persons with multiple sclerosis (PwMS) are at increased risk to report poor sleep patterns and lower physical activity indices. To date, data on longitudinal objectively sleep assessment is missing. In the present study, we investigated the pattern of objective sleep and subjective physical activity indices over a period of 13.5 months, under naturalistic conditions.Method: 13.5 months after their first assessment, a total of 16 PwMS (mean age = 49.13 median EDSS score: 5; 11 females) were reassessed on their objective sleep via portable sleep-electroencephalogram (EEG-) devices, along with their subjective sleep patterns (symptoms of insomnia, restless legs syndrome (RLS), and sleep-disordered breathing), physical activity indices, psychological functioning (symptoms of depression, fatigue, daytime sleepiness), and MS-related information (fatigue, EDSS; disease-modifying treatments). While the baseline assessment was performed in a rehabilitation center, the follow-up assessment took place at participants' naturalistic and familiar setting.Results: Statistically, symptoms of depression and fatigue, subjective sleep, and physical activity levels did neither increase, nor decrease over time, although descriptively, both moderate and vigorous physical activity levels decreased, and fatigue and subjective insomnia increased. Time awake after sleep onset statistically significantly decreased, while light sleep duration increased by trend.Conclusions: Among a smaller sample of PwMS, objective sleep in their naturalistic setting remained fairly stable over a mean time lapse of 13.5 months after clinic discharge. Physical activity levels descriptively decreased. The present results are of clinical and practical importance for treatment counseling: PwMS can be reassured that their sleep quality does not deteriorate, once they have left a rehabilitation center. Further, they should be encouraged to keeping their physical activity levels as stable as possible.
A Comparison of Radiation Exposure During Endoscopic Retrograde Cholangiopancreatography (ERCP) by Different Fluoroscope Techniques
Authors: Alghsoon Saleh A. , Shaban Khaled S. , Khan Altaf H. , Almeshal Fares M. , Balogun Sulaimon O. , Memon Muhammad Y. , Swied Abdulmonem M. 
Published: December 2020

ABSTRACT

Introduction
Endoscopic retrograde cholangiopancreatography (ERCP) is a relatively new endoscopic procedure combined with fluoroscopy that is performed for multiple diagnostic and therapeutic indications. It carries a known risk of radiation exposure to patients and staff. We aimed to examine radiation administration techniques and to measure the radiation dose delivered by these techniques.

Methods
This was a retrospective analysis of 437 ERCP procedures performed at a tertiary care hospital between April 2015 and April 2017.

Results
A total of 437 ERCP procedural charts were reviewed: fluoroscopy administration was endoscopist controlled (EC, n = 187, 42.79%) or technician controlled (TC, n = 250, 57.21%). The mean (and SD) fluoroscopy time (FT) was 2.107 ± 2.0 minutes. The mean (and SD) dose–area product (DAP) was 15,227.371 ± 16,784.738 Gy·cm2. The degree of ERCP difficulty was evaluated as recommended by the American Society for Gastrointestinal Endoscopy, and graded 1–4. Level I TC procedures had a mean FT and DAP of 1.600 minutes and 12,644.72 Gy·cm2, respectively. The FT and DAP values for level I EC procedures were 1.514 minutes and 12,966.71 Gy·cm2, respectively, as compared with level IV TC procedures (mean FT, 2.539 minutes; mean DAP, 19,469.94 Gy·cm2) and level IV EC procedures (mean FT, 4.890 minutes; mean DAP, 37,921.00 Gy·cm2).

Conclusion
DAP and FT are increased significantly in EC ERCP in American Society for Gastrointestinal Endoscopy 4 procedures. Comparison of the different degrees of difficulty indicated that there is a linear correlation between the degree of difficulty and both FT and DAP.
Problema Pendidikan Agama Hindu pada keluarga dengan latar belakang perkawinan lintas agama
Authors: Anta I made Nuhari 
Published: December 2020

ABSTRACT
Problem formulation: (1) Why does Hindu religious education in families with interfaith marital backgrounds have problems? This study aims: to obtain an overview of education in Hindu families who marry interfaith. To dissect the problem in this study using qualitative methods with the Phenomenological Theory, Theory of Constructionvism, and Beavioristic Theory. Data collection techniques in this study were observation, interviews, document studies, documentation and literature. Data analysis techniques in this study refer to the analytical steps proposed by Kaelan consisting of data reduction, data classification, and data display, providing interpretation and giving conclusions.
The results showed that the problem of Hindu religious education in families with interfaith marital backgrounds consisted of: (a) Problems of Ceremonies and Upakara; (b) Difference between the concept of God and the previous religion; (c) Problems in Using Local Languages ​​(Balinese); (d) Lack of Attention from Husband to Teach Hinduism; (e) Economic and Traditional Issues

ABSTRAK
Rumusan masalah: (1) Mengapa pendidikan agama Hindu pada keluarga dengan latar belakang perkawinan lintas agama memiliki problema? Penelitian ini bertujuan: untuk memperoleh gambaran pendidikan dalam keluarga Hindu yang menikah lintas agama. Untuk membedah permasalahan dalam penelitian ini menggunakan metode kualitatif dengan teori Fenomenologis, Teori Konstruksivisme, dan Teori beavioristik. Teknik pengumpulan data dalam penelitian ini adalah observasi, wawancara, studi dokumen, dokumentasi dan kepustakaan. Teknik analisis data dalam penelitian ini merujuk pada langkah-langkah analisis yang dikemukakan oleh Kaelan yang terdiri dari reduksi data, klasifikasi data, dan display data, memberikan interprestasi dan memberi kesimpulan.
Hasil penelitian menunjukkan bahwa Problem pendidikan agama Hindu pada keluarga dengan latar belakang perkawinan lintas agama terdiri dari: (a) Permasalahan Upacara dan Upakara; (b) Perbedaan Konsep Ketuhanan dengan Agama sebelumnya; (c) Masalah Penggunaan Bahasa Lokal (Bahasa Bali); (d) Kurang Perhatian dari Suami untuk Mengajarkan Agama Hindu; (e) Permasalahan Ekonomi dan Tradisi
CLOUD TECHNOLOGIES AND THEIR ROLE IN DIGITALIZATION OF ELECTRIC POWER FACILITIES
Authors: Konovalov Yuriy , Vaygachev Anton , Tihonova Angelina , Zhil'nikov Dmitriy 
Published: December 2020

The model of cloud computing and the principle of its operation in relation to the
energy service of the enterprise are considered. The classification of cloud technology categories is
presented. The main features of using cloud technologies in enterprises, as well as information
security in their work, are studied. The assessment of existing projects in the energy sector, as well as
options for implementation in the Irkutsk region.
STUDY OF OXAZOLIDIN-2-ONES AS INHIBITORS ACID CORROSION OF STEEL
Authors: Kovalyuk Elena , Vasyutina Anastasiya , Matofonov Vladimir 
Published: December 2020

The article presents the results of a study of individual representatives of
oxazolidin-2-ones as inhibitors of acid corrosion of steel. The influence of temperature and inhibitor concentration on the degree of protection was evaluated by a complete two-factor experiment.
The results of electrochemical tests of steel are presented. Oxazolidin-2-ones can be attributed to a
group of promising inhibitors of acid corrosion of steel and continue research.
DEVELOPMENT OF BIFUNCTIONAL ADDITIVES TO DIESEL FUELS ON THE BASIS OF PETROCHEMICAL PRODUCTS
Authors: Lobanov Igor' 
Published: December 2020

The developed method of determining the theoretical calculated hydraulic resistance and heat transfer for round pipes with rough walls based on multilayer models of turbulent
boundary layer, mainly different from existing theories by taking into account the share volume of
cavities in the sublayer, and the rough and smooth surfaces of the channel that can lead to a reduction of heat transfer of about 15%, it is necessary to consider the mode of developed roughness.
Ferroefremovite, (NH4)2Fe2+2(SO4)3, a new mineral from Solfatara di Pozzuoli, Campania, Italy
Authors: Kasatkin Anatoly V. , Plášil Jakub , Škoda Radek , Campostrini Italo , Chukanov Nikita V. , Agakhanov Atali A. , Karpenko Vladimir Yu. , Belakovskiy Dmitriy I. 
Published: December 2020

ABSTRACT
The new sulfate mineral ferroefremovite, ideally (NH4)2Fe2+2(SO4)3, was discovered at the “Bocca Grande” fumarole, Solfatara di Pozzuoli, Flegrean Volcanic Complex, Naples Province, Campania, Italy. Associated minerals are adranosite, adranosite-(Fe), godovikovite, huizingite-(Al), mascagnite, and opal. Ferroefremovite forms cubic crystals up to 0.1 mm across. The new mineral is colorless and has a vitreous luster and white streak. It is brittle and has an uneven fracture. No cleavage or parting are observed. Mohs hardness is 2. Dmeas. = 2.69(1) g/cm3. Dcalc. = 2.700 g/cm3. Optically, ferroefremovite is isotropic, n = 1.574(3) (λ = 589 nm). It is non-pleochroic. The presence of the NH4+ cation is confirmed by the Raman spectrum. The chemical composition of ferroefremovite is (wt.%; electron microprobe, N determined by CNH analysis) (NH4)2O 11.51, Na2O 0.11, K2O 1.35, MgO 8.38, MnO 0.98, FeO 18.94, SO3 57.08, total 98.35. The empirical formula based on 12 O apfu is [(NH4)1.85K0.12Na0.01]Σ1.98(Fe2+1.11Mg0.87Mn0.06)Σ2.04S2.99O12. Ferroefremovite is cubic, space group P213, with a = 10.0484(9) Å, V = 1014.59(16) Å3, and Z = 4. The strongest lines of the powder X-ray diffraction pattern [d, Å (I, %) (hkl)] are 5.80 (40) (111), 4.50 (20) (201, 210), 4.11 (30) (211), 3.17 (100) (310, 301), 3.02 (20) (311), 2.68 (50) (312, 321), 1.86 (18) (502, 432), 1.62 (18) (523, 532, 611).
Pengembangan Model Ulul Ilmi dalam Pembelajaran PAI untuk Pembentukan Karakter Mahasiswa Era Revolusi Industri 4.0
Authors: Abdussalam Aam , Supriadi Udin , Parhan Muhamad , Budiyanti Nurti 
Published: December 2020

This paper aims to develop an Ulul Ilmi model in Islamic Education learning for the character of 21st-century students, which, of course, can help. Realize these national education goals—the research method used using a qualitative approach and literature study techniques in collecting data. Based on the findings, the Ulul Ilmi Model is very supportive in Islamic Religious Education learning because it has extreme divine values to achieve ma'rifatullah goals to shape 21st-century students' character. The method used is the method (1) the uswah hasanah method, an exemplary method that must be applied to an educator and passed on to students, (2) the ibrah mauidzah method, which is a learning method that must be delivered in the classroom, and (3) the targhib tarhib method, a method of strengthening for character building. Thus this pattern is later called the Ulul Ilmi model.
Tulisan ini bertujuan untuk mengembangkan model Ulul Ilmi dalam pembelajaran PAI untuk pencapaian karakter mahasiswa abad 21, yang tentunya dapat membantu merealisasikan tujuan pendidikan Nasional. Metode penelitian yang digunakan dengan menggunakan pendekatan kualitatif dan teknik studi pustaka dalam pengumpulkan data. Berdasarkan hasil temuan, model Ulul Ilmi ini sangat memungkinkan untuk diterapkan dalam pembelajaran Pendidikan Agama Islam, karena memiliki nilai Ilahiyyah yang sangat kuat mencapai tujuan ma’rifatullah dalam rangka membentuk karakter mahasiswa abad 21. Metode yang digunakan ialah (1) metode uswah hasanah, yang merupakan metode keteladanan yang wajib diterapkan bagi seorang pendidik dan diwariskan kepada para peserta didik, (2) metode ibrah mauidzah, merupakan metode pembelajaran yang wajib untuk disampaikan di dalam kelas, dan (3) metode targhib tarhib, yakni metode penguatan untuk penanaman karakter. Dengan demikian pola inilah yang kemudian disebut sebagai model Ulul Ilmi.
Influence of Solar Disturbances on Galactic Cosmic Rays in the Solar Wind, Heliosheath, and Local Interstellar Medium: Advanced Composition Explorer, New Horizons, and Voyager Observations
Authors: Hill M. E. , Allen R. C. , Kollmann P. , Brown L. E. , Decker R. B. , McNutt R. L. , Krimigis S. M. , Andrews G. B. , Bagenal F. , Clark G. , Elliott H. A. , Jaskulek S. E. , Kusterer M. B. , Leske R. A. , Lisse C. M. , Mewaldt R. A. , Nelson K. S. , Richardson J. D. , Romeo G. , Salazar N. A. , Vandegriff J. D. , Bernardoni E. A. , Gladstone G. R. , Horanyi M. , Linscott I. R. , Singer K. N. , Steffl A. J. , Summers M. E. , Throop H. B. , Young L. A. , Olkin C. B. , Parker J. Wm. , Spencer J. R. , Stern S. A. , Verbiscer A. J. , Weaver H. A. 
Published: December 2020

Abstract
We augment the heliospheric network of galactic cosmic ray (GCR) monitors using 2012–2017 penetrating radiation measurements from the New Horizons (NH) Pluto Energetic Particle Spectrometer Science Investigation (PEPSSI), obtaining intensities of ≳75 MeV particles. The new, predominantly GCR observations provide critical links between the Sun and Voyager 2 and Voyager 1 (V2 and V1), in the heliosheath and local interstellar medium (LISM), respectively. We provide NH, Advanced Composition Explorer (ACE), V2, and V1 GCR observations, using them to track solar cycle variations and short-term Forbush decreases from the Sun to the LISM, and to examine the interaction that results in the surprising, previously reported V1 LISM anisotropy episodes. To investigate these episodes and the hitherto unexplained lagging of associated in situ shock features at V1, propagating disturbances seen at ACE, NH, and V2 were compared to V1. We conclude that the region where LISM magnetic field lines drape around the heliopause is likely critical for communicating solar disturbance signals upstream of the heliosheath to V1. We propose that the anisotropy-causing physical process that suppresses intensities at ∼90° pitch angles relies on GCRs escaping from a single compression in the draping region, not on GCRs trapped between two compressions. We also show that NH suprathermal and energetic particle data from PEPSSI are consistent with the interpretation that traveling shocks and corotating interaction region (CIR) remnants can be distinguished by the existence or lack of Forbush decreases, respectively, because turbulent magnetic fields at local shocks inhibit GCR transport while older CIR structures reaching the outer heliosphere do not.
Calibration of the MaGIXS Experiment. I. Calibration of the X-Ray Source at the X-Ray and Cryogenic Facility
Authors: Athiray P. S. , Winebarger Amy R. , Champey Patrick , Kobayashi Ken , Vigil Genevieve D. , Haight Harlan , Johnson Steven , Bethge Christian , Rachmeler Laurel A. , Savage Sabrina , Beabout Brent , Beabout Dyana , Hogue William , Guillory Anthony , Siler Richard , Wright Ernest , Kegley Jeffrey 
Published: December 2020

Abstract
The Marshall Grazing Incidence Spectrometer (MaGIXS) is a sounding rocket experiment that will observe the soft X-ray spectrum of the Sun from 24 to 6.0 Å (0.5–2.0 keV) and is scheduled for launch in 2021. Component- and instrument-level calibrations for the MaGIXS instrument are carried out using the X-ray and Cryogenic Facility (XRCF) at NASA Marshall Space Flight Center. In this paper, we present the calibration of the incident X-ray flux from the electron impact source with different targets at the XRCF using a CCD camera; the photon flux at the CCD was low enough to enable its use as a “photon counter,” i.e., the ability to identify individual photon hits and calculate their energy. The goal of this paper is two-fold: (1) to confirm that the flux measured by the XRCF beam normalization detectors is consistent with the values reported in the literature and therefore reliable for MaGIXS calibration and (2) to develop a method of counting photons in CCD images that best captures their number and energy.
The Importance of Hall Effect in the Self-similar Collapse of a Filamentary Cloud
Authors: Gholipour Mahmoud 
Published: December 2020

Abstract
Thanks to the observational and simulation works, the importance of the nonideal magnetohydrodynamic (MHD) effects, i.e., Hall effect, ohmic resistivity, and ambipolar diffusion, have been well established at various stages of cloud evolution. To get a comparison between the Hall effect with other effects, we aim to model the time evolution of a rotating filamentary molecular cloud during the isothermal/polytropic collapse phase in the presence of the Hall drift. Three components of the velocity vector are investigated when the angular momentum is fully coupled with the magnetic field at large radii of a filament. For this purpose, the nonideal MHD equations in the self-similar formalism are considered at large radii of a molecular cloud where the magnetic field evolution is affected by the Hall drift. Then, the connection between the self-similar approach with the observational data from the filamentary clouds is examined to get a realistic model. Due to the existence of Hall drift, the significant changes on the rotation of the cloud can be seen when the cloud switches from the isothermal collapse phase to the polytropic collapse phase. Also, the results of this model are useful in the study of the multiple star formation process as well as the initial conditions for driving the outflows during the collapse of the filamentary clouds. Finally, we found that there are some conditions for the comparability of the Hall effect with the ambipolar diffusion in the outer regions of the clouds.
Star Formation and Evolution of Blister-type H ii Region Sh2-112
Authors: Panwar Neelam , Sharma Saurabh , Ojha D. K. , Baug T. , Dewangan L. K. , Bhatt B. C. , Pandey Rakesh 
Published: December 2020

Abstract
We report the observational findings of the Sh2-112 H ii region by using the multiwavelength data analysis ranging from optical to radio wavelengths. This region is powered by the massive O8V-type star BD +45 3216. The surface density distribution and minimum spanning tree analyses of the young stellar object (YSO) candidates in the region reveal their groupings toward the western periphery of the H ii region. A GMRT radio continuum emission peak is found toward the northwest boundary of the H ii region and is investigated as a compact/ultracompact H ii region candidate powered by a B0–B0.5-type star. Toward the southwest direction, a prominent curved rim-like structure is found in the Hα image and GMRT radio continuum maps, where the H2 and 13CO emission is also observed. These results suggest the existence of the ionized boundary layer (IBL) on the surface of the molecular cloud. This IBL is found to be overpressured with respect to the internal pressure of the surrounding molecular cloud. This implies that the shocks are propagating/propagated into the molecular cloud, and the young stars identified within it are likely triggered due to the massive star. It is also found that this region is ionization-bounded toward the west and density-bounded toward the east. Based on the distribution of the ionized gas, molecular material, and YSO candidates, we propose that the Sh2-112 H ii region is a good candidate for the blister-type H ii region that has been evolved on the surface of a cylindrical molecular cloud.
MASTER Optical Observation of LIGO/VIRGO S200302c Event
Authors: Lipunov V. , Kornilov V. , Chasovnikov A. , Tiurina N. , Vlasenko D. , Gorbovskoy E. , Gorbunov I. , Balanutsa P. , Cheryasov D. , Pozdnyakov A. , Gabovich A. , Gress O. A. , Buckley D. , Podesta R. , Rebolo R. , Serra M. , Balakin F. , Topolev V. , Zhirkov K. , Kuznetsov A. , Vladimirov V. , Senik V. , Podesta F. , Francile C. , Budnev N. M. , Sergienko Yu. , Tlatov A. , Grinshpun V. , Minkina E. , Ershova O. , Kuvshinov D. , Yurkov V. 
Published: December 2020

Abstract
The results of LIGO/Virgo S200302c (O3) error-box optical inspection by MASTER are presented. We observed 4242 square degrees square degrees inside 3σ error box during 1 month. We present MASTER OT J141401.39–483305.7, MASTER OT J215856.95–392909.6, MASTER OT J061642.05+435617.9, MASTER OT J052817.95+672801.4—optical transients found by the MASTER auto-detection system during this inspection as the result of a survey of large error-fields, indicating the efficiency of MASTER observational strategy.