• Title/Summary/Keyword: EBF3

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Hypotonia, Ataxia, and Delayed Development Syndrome caused by the EBF3 mutation in a Korean boy with muscle hypotonia

  • Kim, Tae-Gyeong;Choi, Yoon-Ha;Lee, Ye-Na;Kang, Min-Ji;Seo, Go Hun;Lee, Beom Hee
    • Journal of Genetic Medicine
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    • v.17 no.2
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    • pp.92-96
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    • 2020
  • Hypotonia, Ataxia, and Delayed Development Syndrome (HADDS) is an autosomal-dominant, extremely rare neurodevelopmental disorder caused by the heterozygous EBF3 gene mutation. EBF3 is located on chromosome 10q26.3 and acts as a transcription factor that regulates neurogenesis and differentiation. This syndrome is characterized by dysmorphism, cerebellar hypoplasia, urogenital anomaly, hypotonia, ataxia, intellectual deficit, and speech delay. The current report describes a 3-year-old Korean male carrying a de novo EBF3 mutation, c.589A>G (p.Asn197Asp), which was identified by whole exome sequencing. He manifested facial dysmorphism, hypotonia, strabismus, vermis hypoplasia, and urogenital anomalies, including vesicoureteral reflux, cryptorchidism, and areflexic bladder. This is the first report of a case of HADDS cause by an EBF3 mutation in the Korean population.

Evaluation of cyclic behavior of lateral load resisting system with eccentric brace and steel plate

  • Reza Khalili Sarbangoli;Ahmad Maleki;Ramin K. Badri
    • Structural Engineering and Mechanics
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    • v.89 no.3
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    • pp.239-252
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    • 2024
  • Steel plate shear walls (SPSWs) are classified as lateral load-resisting systems. The feasibility of openings in the steel plate is a characteristic of SPSWs. The use of openings in SPSWs can lower the load capacity, stiffness, and energy dissipation. This study proposes a novel form of SPSWs that provides convenient access through openings by combining steel plates and eccentrically braced frames (EBFs). The proposed system also avoids a substantial reduction in the strength and stiffness. Hence, various geometric forms were analyzed through two different structural approaches. Groups 1, 2, and 3 included a steel EBF with a steel plate between the column and EBF in order to improve system performance. In Group 4, the proposed system was evaluated within an SPSW with openings and an EBF on the opening edge. To evaluate the performance of the proposed systems, the nonlinear finite element method (NL-FEM) was employed under cyclic loading. The hysteresis (load-drift) curve, stress contour, stiffness, and damping were evaluated as the structural outputs. The numerical models indicated that local buckling within the middle plate-EBF connection prevented a diagonal tension field. Moreover, in group 4, the EBF and stiffeners on the opening edge enhanced the structural response by approximately 7.5% in comparison with the base SPSW system.

Landslide Susceptibility Prediction using Evidential Belief Function, Weight of Evidence and Artificial Neural Network Models (Evidential Belief Function, Weight of Evidence 및 Artificial Neural Network 모델을 이용한 산사태 공간 취약성 예측 연구)

  • Lee, Saro;Oh, Hyun-Joo
    • Korean Journal of Remote Sensing
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    • v.35 no.2
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    • pp.299-316
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    • 2019
  • The purpose of this study was to analyze landslide susceptibility in the Pyeongchang area using Weight of Evidence (WOE) and Evidential Belief Function (EBF) as probability models and Artificial Neural Networks (ANN) as a machine learning model in a geographic information system (GIS). This study examined the widespread shallow landslides triggered by heavy rainfall during Typhoon Ewiniar in 2006, which caused serious property damage and significant loss of life. For the landslide susceptibility mapping, 3,955 landslide occurrences were detected using aerial photographs, and environmental spatial data such as terrain, geology, soil, forest, and land use were collected and constructed in a spatial database. Seventeen factors that could affect landsliding were extracted from the spatial database. All landslides were randomly separated into two datasets, a training set (50%) and validation set (50%), to establish and validate the EBF, WOE, and ANN models. According to the validation results of the area under the curve (AUC) method, the accuracy was 74.73%, 75.03%, and 70.87% for WOE, EBF, and ANN, respectively. The EBF model had the highest accuracy. However, all models had predictive accuracy exceeding 70%, the level that is effective for landslide susceptibility mapping. These models can be applied to predict landslide susceptibility in an area where landslides have not occurred previously based on the relationships between landslide and environmental factors. This susceptibility map can help reduce landslide risk, provide guidance for policy and land use development, and save time and expense for landslide hazard prevention. In the future, more generalized models should be developed by applying landslide susceptibility mapping in various areas.

Development of Infiltration System for Stormwater Management from Paved Area (포장지역 강우유출수 관리를 위한 침투도랑 기술개발)

  • Lee, Jeong-Yong;Maniquiz, Marla C.;Lee, So-Young;Kim, Lee-Hyung
    • Journal of Wetlands Research
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    • v.12 no.3
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    • pp.165-175
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    • 2010
  • Recently, various development projects have occurred due to rapid industrialization and urbanization. Both industry and population grew rapidly which causes a corresponding rise in pollution. The accumulated pollutants are washed-off during rainy days, highly degrading the water quality of the receiving water body. This is because a lot of non point source (NPS) pollutants were accumulated on the surface during dry periods. This research focuses on the development of Eco-Bio Filter (EBF) system that treats NPS pollutants. It is constructed at Kongju National University campus. The EBF is a type of a structured infiltration system that falls under the category of Low Impact Development (LID) hydrologic design practices. The monitoring of rain events was performed. It includes the development of design, the lessons learned from the post-construction testing and the improvements in the renovation made. After a series of monitoring, the MOE will use the results to apply the design to other areas in the country.

Benefits of a Dedicated Breastfeeding Facility and Support Program for Exclusive Breastfeeding among Workers in Indonesia

  • Basrowi, Ray W;Sulistomo, Astrid B;Adi, Nuri Purwito;Vandenplas, Yvan
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.18 no.2
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    • pp.94-99
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    • 2015
  • Purpose: A mother's working environment is believed to be a major determinant of exclusive breastfeeding (EBF) practice. We aimed to define the influence of a facility dedicated to breastfeeding and a breastfeeding support program at the workplace on breastfeeding practice. Methods: A cross-sectional study was performed in five workplaces. The inclusion criteria were female workers whose last child was between 6 and 36 months old. Observational data were obtained and a questionnaire was filled out. The World Health Organization definition for EBF was used. Results: Data from 186 subjects (74 office workers and 112 factory workers) were collected. Just over half (52%) of the mothers were between 20 and 46 years old, 75.3% had graduated from high school and university, 12.9% had more than two children and 36.0% owned a house. The prevalence of EBF during the last 6 months was 32.3%. A proper dedicated breastfeeding facility was available for 21.5% of the mothers, but only 7.5% had been in contact with a breastfeeding support program. The presence of a dedicated breastfeeding facility increased EBF practice almost threefold, by an odds ratio (OR) of 2.74 and a 95% confidence interval (CI) of 1.34-5.64 (p<0.05). Knowledge of the breastfeeding support program increased EBF practice by almost six times (OR, 5.93; 95% CI, 1.78-19.79) (p<0.05). Conclusion: Our findings suggest that Governments should make it obligatory for employers to offer a breastfeeding support program and a dedicated breastfeeding facility at the workplace as these simple measures significantly increase EBF.

On the optimum performance-based design of eccentrically braced frames

  • Mohammadi, Reza Karami;Sharghi, Amir Hossein
    • Steel and Composite Structures
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    • v.16 no.4
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    • pp.357-374
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    • 2014
  • The design basis is being shifted from strength to deformation in modern performance-based design codes. This paper presents a practical method for optimization of eccentrically braced steel frames, based on the concept of uniform deformation theory (UDT). This is done by gradually shifting inefficient material from strong parts of the structure to the weak areas until a state of uniform deformation is achieved. In the first part of this paper, UDT is implemented on 3, 5 and 10 story eccentrically braced frames (EBF) subjected to 12 earthquake records representing the design spectrum of ASCE/SEI 7-10. Subsequently, the optimum strength-distribution patterns corresponding to these excitations are determined, and compared with four other loading patterns. Since the optimized frames have uniform distribution of deformation, they undergo less damage in comparison with code-based designed structures while having minimum structural weight. For further investigation, the 10 story EBF is redesigned using four different loading patterns and subjected to 12 earthquake excitations. Then a comparison is made between link rotations of each model and those belonging to the optimized one which revealed that the optimized EBF behaves generally better than those designed by other loading patterns. Finally, efficiency of each loading pattern is evaluated and the best one is determined.

Analysis and Validation of Geo-environmental Susceptibility for Landslide Occurrences Using Frequency Ratio and Evidential Belief Function - A Case for Landslides in Chuncheon in 2013 - (Frequency Ratio와 Evidential Belief Function을 활용한 산사태 유발에 대한 환경지리적 민감성 분석과 검증 - 2013년 춘천 산사태를 중심으로 -)

  • Lee, Won Young;Sung, Hyo Hyun;Ahn, Sejin;Park, Seon Ki
    • Journal of The Geomorphological Association of Korea
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    • v.27 no.1
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    • pp.61-89
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    • 2020
  • The objective of this study is to characterize landslide susceptibility depending on various geo-environmental variables as well as to compare the Frequency Ratio (FR) and Evidential Belief Function (EBF) methods for landslide susceptibility analysis of rainfall-induced landslides. In 2013, a total of 259 landslides occurred in Chuncheon, Gangwon Province, South Korea, due to heavy rainfall events with a total cumulative rainfall of 296~721mm in 106~231 hours duration. Landslides data were mapped with better accuracy using the geographic information system (ArcGIS 10.6 version) based on the historic landslide records in Chuncheon from the National Disaster Management System (NDMS), the 2013 landslide investigation report, orthographic images, and aerial photographs. Then the landslides were randomly split into a testing dataset (70%; 181 landslides) and validation dataset (30%; 78 landslides). First, geo-environmental variables were analyzed by using FR and EBF functions for the full data. The most significant factors related to landslides were altitude (100~200m), slope (15~25°), concave plan curvature, high SPI, young timber age, loose timber density, small timber diameter, artificial forests, coniferous forests, soil depth (50~100cm), very well-drained area, sandy loam soil and so on. Second, the landslide susceptibility index was calculated by using selected geo-environmental variables. The model fit and prediction performance were evaluated using the Receiver Operating Characteristic (ROC) curve and the Area Under Curve (AUC) methods. The AUC values of both model fit and prediction performance were 80.5% and 76.3% for FR and 76.6% and 74.9% for EBF respectively. However, the landslide susceptibility index, with classes of 'very high' and 'high', was detected by 73.1% of landslides in the EBF model rather than the FR model (66.7%). Therefore, the EBF can be a promising method for spatial prediction of landslide occurrence, while the FR is still a powerful method for the landslide susceptibility mapping.

Block-Ordered Layered Detector for MIMO-STBC Using Joint Eigen-Beamformers and Ad-Hoc Power Discrimination Scheme

  • Lee Won-Cheol
    • Journal of Communications and Networks
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    • v.8 no.3
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    • pp.275-285
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    • 2006
  • Suitable for multi-input multi-output (MIMO) communications, the joint beamforming space-time block coding (JBSTBC) scheme is proposed for high-speed downlink transmission. The major functionality of the scheme entails space-time block encoder and joint transmit and receive eigen-beamformer (EBF) incorporating with block-ordered layered decoder (BOLD), and its operating principle is described in this paper. Within these functionalities, the joint EBFs will be utilized for decorrelating fading channels to cause an enhancement in the spatial diversity gain. Furthermore, to fortify the capability of layered successive interference cancellation (LSIC) in block-ordered layered decoding process, this paper will develop a simple ad-hoc transmit power discrimination scheme (TPDS) based on a particular power discrimination function (PDF). To confirm the superior behavior of the proposed JBSTBC scheme employing ad-hoc TPDS, computer simulations will be conducted under various channel conditions with the provision of detailed mathematical derivations for clarifying its functionality.

Determination of buildup factors for some human tissues using both MCNP5 and Phy-X / PSD

  • Mohammad M. Alda'ajeh;J.M. Sharaf;H.H. Saleh;Mefleh S. Hamideen
    • Nuclear Engineering and Technology
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    • v.55 no.12
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    • pp.4426-4430
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    • 2023
  • In this article, Exposure Buildup Factor(EBF) and the Energy Absorption Buildup Factor(EABF) have been determined for blood, brain, and muscle using the Monte Carlo method which is represented by MCNP5 codes and compared with geometric progression(G-P) fitting method which is represented by Phy-X/PSD online platform. The novelty of the present work is used an energy source of less than 0.1 MeV to determine buildup factors using MCNP5 and using Phy-X/PSD for some human tissues. thus, the energy range used in this case study was 0.06-3 MeV for penetration depths covered 0.5-3 MFP. Results of MCNP5 and Phy-X/PSD are validated against reference values of water that were reported at ANS-6.4.3. present results of EABFs and EBFs for the previously mentioned human tissues appeared good agreement between MCNP5 in comparison with Phy-X/PSD, whereas, the maximum average relative deviation did not exceed 2.37%. results of our article can be used in different medical applications, such as brachytherapy, radiotherapy, and diagnostics.

Gamma ray attenuation behaviors and mechanism of boron rich slag/epoxy resin shielding composites

  • Mengge Dong;Suying Zhou ;He Yang ;Xiangxin Xue
    • Nuclear Engineering and Technology
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    • v.55 no.7
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    • pp.2613-2620
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    • 2023
  • Excellent thermal neutron absorption performance of boron expands the potential use of boron rich slag to prepare epoxy resin matrix nuclear shielding composites. However, shielding attenuation behaviors and mechanism of the composites against gamma rays are unclear. Based on the radiation protection theory, Phy-X/PSD, XCOM, and 60Co gamma ray source were integrated to obtain the shielding parameters of boron rich slag/epoxy resin composites at 0.015-15 MeV, which include mass attenuation coefficient (µt), linear attenuation coefficient (µ), half value thickness layer (HVL), electron density (Neff), effective atomic number (Zeff), exposure buildup factor (EBF) and exposure absorption buildup factor (EABF).µt, µ, HVL, Neff, Zeff, EBF and EABF are 0.02-7 cm2/g, 0.04-17 cm-1, 0.045-20 cm, 5-14, 3 × 1023-8 × 1023 electron/g, 0-2000, and 0-3500. Shielding performance is BS4, BS3, BS3, BS1 in descending order, but worse than ordinary concrete. µ and HVL of BS1-BS4 for 60Co gamma ray is 0.095-0.110 cm-1 and 6.3-7.2 cm. Shielding mechanism is main interactions for attenuation gamma ray by BS1-BS4 are elements with higher content or higher atomic number via Photoelectric Absorption at low energy range, and elements with higher content via Compton Scattering and Pair Production in Nuclear Field at middle and higher energy range.