• 제목/요약/키워드: EBF3

검색결과 22건 처리시간 0.012초

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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    • 제17권2호
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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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    • 제89권3호
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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.

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

  • 이사로;오현주
    • 대한원격탐사학회지
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    • 제35권2호
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    • pp.299-316
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    • 2019
  • 본 연구는 지리정보시스템(GIS) 환경에서 확률 모델인 Weight Of Evidence (WOE)와 Evidential Belief Function (EBF), 기계학습 모델인 Artificial Neural Networks (ANN) 모델을 이용하여 평창지역의 산사태 취약성도를 공간적으로 분석하고 예측하였다. 본 연구지역은 2006년 태풍 에위니아에 의한 집중호우로 산사태가 많이 발생하여 많은 재산 및 인명피해가 발생하였다. 산사태 취약성도를 작성하기 위해 항공사진을 이용하여 3,955개의 방대한 산사태 발생 위치를 탐지하였고, 환경공간정보인 지형, 지질, 토양, 산림 및 토지이용 등의 공간 데이터를 수집하여 공간데이터베이스에 구축하였다. 이러한 공간데이터베이스를 이용하여 산사태에 영향을 줄 수 있는 인자 17개를 추출하여 입력 인자와 EBF, WOE, ANN 모델을 이용하여 산사태 취약성도를 작성하고 검증하였다. 작성 및 검증을 위해 산사태 자료는 각각 50%씩 나누어서 훈련 및 검증을 실시하였고, 검증결과 WOE 모델의 경우는 74.73%, EBF 모델의 경우는 75.03%, ANN 모델의 경우는 70.87%의 예측 정확도를 나타내었다. 본 연구에 사용된 모델 중 EBF 모델이 가장 높은 정확도를 나타냈으며, 모든 모델에서 70% 이상의 예측 정확도를 보여 본 연구에서 사용된 기법이 산사태 취약성도 작성에 유효함을 나타내었다. 본 연구에서 제안된 WOE, EBF, ANN 모델과 산사태 취약성도는 이전에 산사태가 발생하지 않은 지역의 산사태를 예측하는 데 사용될 수 있다. 이러한 취약성도는 산사태 위험 감소를 촉진하고, 토지 이용 정책 및 개발을 위한 기초자료 역할을 할 수 있으며, 궁극적으로 산사태 재해 예방을 위한 시간과 비용을 절약할 수 있다. 향후 보다 많은 지역에서 산사태 취약성도 작성 방법을 적용하여 산사태 위험 예측을 위한 일반화된 모델을 이끌어 내야 한다.

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

  • 이정용;;이소영;김이형
    • 한국습지학회지
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    • 제12권3호
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    • pp.165-175
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    • 2010
  • 급속한 산업화, 도시화 및 경제성장으로 다양한 개발사업이 발생하였다. 다양한 개발사업으로 인하여 각종 오염물질이 발생하였으며, 발생된 오염물질은 자연적으로 형성된 물순환 시스템이 아닌 인위적으로 형성된 물순환 시스템으로 인하여 하천 및 호소로 직접 유입되어 수질이 악화되고 있는 실정이다. 강우시 다양한 토지이용에서 발생하는 비점오염원이 큰 원인으로 나타나고 있다. 본 연구는 강우시 발생하는 비점오염물질 처리를 위한 저류 및 침투기능을 포함하는 한국형 비점오염저감시설인 Eco-Bio Filter (EBF)를 개발하고자 한다. EBF 시설은 저류 및 침투기능을 포함하며, EBF Test-bed를 공주대학교 천안 캠퍼스 내에 설치하여 다양한 강우사상에 대하여 모니터링을 수행하였다. 현재 모니터링 결과를 활용하여 기술의 문제점을 분석하고 개선하는 과정이 진행되고 있으며, 본 연구에서는 개선된 EBF 기술을 제시하였다. 오염물질별 저감효율을 분석한 결과, 입자상 물질, 질소, 인, 중금속에 대하여 70% 이상의 높은 저감효율을 나타내었다. 이러한 연구결과는 향후 국내 강우특성에 맞으며 도시의 생태를 확장할 수 있는 새로운 비점오염기술을 개발하는데 기초자료로 활용될 것이며, 저영향개발(LID) 중 하나가 될 것이다.

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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    • 제18권2호
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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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    • 제16권4호
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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.

Frequency Ratio와 Evidential Belief Function을 활용한 산사태 유발에 대한 환경지리적 민감성 분석과 검증 - 2013년 춘천 산사태를 중심으로 - (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 -)

  • 이원영;성효현;안세진;박선기
    • 한국지형학회지
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    • 제27권1호
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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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    • 제8권3호
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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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    • 제55권12호
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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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    • 제55권7호
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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.