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The Ornithine Transcarbamylase (OTC) Deficiency Identified by a Novel Mutation (새로운 유전자 돌연변이로 확진된 Ornithine Transcarbamylase (OTC) 결핍증 1례)

  • Song, Ari;Lee, Kiwuk;Yang, Aram;Kim, Jinsup;Park, Hyung-Doo;Cho, Sung Yoon;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.16 no.3
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    • pp.148-154
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    • 2016
  • A urea cycle disorder is a condition caused by a defect of the enzymes in the urea cycle, and deficiency of ornithine transcarbamylase (OTC), which converts carbamoyl phosphate and ornithine into citrulline, is the most common type of the disorder. OTC deficiency induces the accumulation of precursors of urea, ammonia, and glutamine, leading to neurological symptoms including hypotonia, respiratory failure, seizure, lethargy, and coma and sometimes to death. Because OTC deficiency is inherited in an X-linked manner, typical symptoms such as vomiting, poor feeding, and lethargy appear mainly in male neonates. We recently had a case that presented with neonatal onset lethargy, vomiting, and apnea in a 4-day-old boy. He was diagnosed with OTC deficiency by biochemical phenotype, including hyperammonemia and an increased orotic acid level in the urine. Genetic analysis of the OTC gene showed a novel mutation c.780_781insCAGGCAGTGT (p.Ile261Glnfs*35). He was treated for hyperammonemia using continuous venovenous hemofiltration (CVVH) at 118 hours after birth. After 4 days of CVVH, his consciousness and blood ammonia concentration were normalized, and he was discharged at the age of 53 days. At around 12 months of age, bilateral femur fractures and osteomyelitis occurred in this patient. Two months after the fracture, he died of septic shock, insulin-resistant hyperglycemia, and multi-organ failure.

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Manufacture Lenticular Map of Golf Courses Using Digital Orthophoto (수치정사영상을 이용한 렌티큘러 코스맵 제작)

  • Kim, Kam-Lae;Cheong, Hae-Jin;Cho, Won-Woo
    • Journal of the Korean Society of Surveying, Geodesy, Photogrammetry and Cartography
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    • v.25 no.5
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    • pp.475-482
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    • 2007
  • Most golfers believe that knowing yardages will improve their score. Certainly it helps with club selection. But, simple "Graphic" yardage guides being notorious for error and inaccuracies, which a serious golfer will pick immediately, only serve to erode the players enjoyment and ultimately, golf course satisfaction. Someone believes with low-level aerial photographic images, golfer will be impressed with the accuracy of the depiction, helping them play a more confident game. But, there are no mapping products in true 3-D available in the world that allows a golfer to determine shot distances in yards or meters. So, we suggest an lenticular technology for real 3-D display as a viable alternative to conventional image map solution. This technology is an image display method for the generation of multi-image effects like 3D visualization or animation. This methodology is cutting edge stereoscopic image which overcomes the limitation of conventional photo tech by recomposing and producing 3 dimensional images. A significant strength of this methods its versatility concerning display effects. The main use of the hardcopy 3-D lenticular displays is in the fields of science, education, planning, and representation. This paper gives a concise overview of the lenticular foil technology and describes the production of the true 3-D yardage book of golf courses. For this study, 3-D effects are achieved and evaluated with the lenticular display by incorporation multiple synthetic images based on digital topographic terrain model and by using the two images of the actual stereopair.

Comparing Connectivity in Forest Networks of Seven Metropolitan Cities of South Korea (국내 7대 광역시 산림 연결성 비교 분석)

  • Kang, Wanmo;Kim, Jiwon;Park, Chan-Ryul;Sung, Joo Han
    • Korean Journal of Agricultural and Forest Meteorology
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    • v.16 no.2
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    • pp.93-102
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    • 2014
  • This quantitative research aims to examine the connectivity of forest networks in seven metropolitan cities of South Korea using a graph-theoretical approach. We first estimated an overall network connectivity at multi-scales (i.e., dispersal distances), ranging from 100 m to 20 km, and quantified the contribution of small forest patches (less than 10 ha) to the overall network connectivity by comparing networks according to the presence and absence of small ones. As a result, the cities were divided into two groups depending on the network connectivity; one group of cities with high connectivity such as Daegu, Daejeon, and Ulsan and the other group of cities with low connectivity including Gwangju, Busan, Seoul, and Incheon. The result showed that small forest patches, especially in the cities with low connectivity, played a key role as stepping stones that connect large forested patches, thereby contributing to maintaining connectivity. This study also suggests that large and well-connected forest areas may be the key factor to preserve the connectivity in the cities with high connectivity, while the cites with low connectivity are in need of some complementary strategies. Through the study, we suggest that the creation of new forest patches in the areas where a gap in connectivity presents is needed in order to improve connectivity; and that the conservation of the existing small forest patches is essential in order to maintain the current connectivity level.

Serum Fat Soluble Vitamins in Bile Duct Ligated Rats (담도 결찰한 백서에 있어서 담즙산 및 UDCA 투여에 따른 혈중 지용성 비타민의 농도 변화에 관한 연구)

  • Sim, Jay-Geon;O, Myung-Ho;Kim, Kee-Hyuck
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.2 no.1
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    • pp.59-64
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    • 1999
  • Purpose: The aims of this study are to measure the serum levels of fat soluble vitamins (vitamin A and D) from bile duct ligated rats, and to evaluate the effect of oral bile acids administration to facilitate absorption of fat soluble vitamins. Methods: We measured serum ALT, total bilirubin, vitamin A, and vitamin D of Sprague-Dawley rats 1 week before and 4 weeks after experimental bile duct ligation. Rats were consisted with 3 groups. Group 2 had been fed bile acids and group 3 ursodeoxycholic acid after operation for 4 weeks. Multi-vitamin was given to all groups. Results: 1) Base line (mean value before duct ligation): ALT 74.2 IU, total bilirubin 0.26 mg/dL; vitamin D 13.01 ng/mL vitamin A $0.87\;{\mu}g/mL$, total bile acids $25.16\;{\mu}mol/L$. 2) Four weeks after ligation: ALT 100.7 IU, total bilirubin 2.58 mg/dL; vitamin D 7.89 ng/mL vitamin A $1.37{\mu}g/mL$, total bile acids $278.22\;{\mu}mol/L$. 3) 4 weeks after ligation, each group (group 1, group 2 and group 3) showed vitamin D (7.62, 8.10 and 7.99) ng/mL, vitamin A (1.68, 1.06 and 1.33) ${\mu}g/mL$, total bile acids (233.17, 345.80 and 268.57) ${\mu}mol/L$, which were statistically not significant. Conclusion: Serum level of vitamin A is increased after bile duct ligation although vitamin D is decreased. Oral administration of bile acids does not affect the serum levels of vitamin A and D in bile duct ligated rats.

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The Contamination Characteristics of the Nanji Uncontrolled Landfill and its Surrounding Hydrogeologic Environment (난지도 매립지 주변 지하수환경의 오염 특성에 관한 연구)

  • 이철효;한정상
    • Journal of the Korean Society of Groundwater Environment
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    • v.3 no.1
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    • pp.27-36
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    • 1996
  • The Nanji landfill is one of the biggest uncontrolled landfill in terms of its size and scale in the world. Because the landfill was constructed on the very vulnerable alluvial deposit installing no pollution control systems such as bottom liner and leachate collection systems, it has caused a serious adverse effect to near-by groundwater and surface water systems. A through remedial investigation comprising plume detection and site-characterization was performed to design the remedial measure. As a part the investigation, comprehensive water quality study was conducted, using ten existing observation wells and one bundle type monitoring well, to determine the contaminant indicators for the plume delineation and to define the vertical and horizontal variation of specific contaminants via distances from the landfill. The results clearly shows that EC and temperature are a good pollution indicators and the vertical concentrations of specific contaminants measured in the fully screened wells are 20 to 90% more than those measured at the same depth in bundle type well which is located just 2 m apart. This paper presents a cost effective monitoring and sampling method to define the contaminant plume and obtain a basic data for leachate control measures.

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Study on River Management Plan Considering Ecological Preservation and Flood Control of Riverine Wetland (하도습지의 생태보전 및 치수를 고려한 하천관리 방안 연구)

  • Ann, Byoung-Yun;Kim, Taek-Min;Hong, Seung-Jin;Kim, Gil-Ho;Kim, Soo-Jun;Kim, Jae-Geun;Kim, Hung-Soo
    • Journal of Wetlands Research
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    • v.16 no.4
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    • pp.463-476
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    • 2014
  • The riverine wetlands located in the riverside bring about social conflicts through confrontation between flood control value through flood control project and ecological preservation value of riverine wetland. In this study, we identified the importance of both values through analysis of economic feasibility of flood control and ecological values of riverine wetland, and tried to suggest management plans for riverine wetland considering both of flood control safety and ecological preservation through these results. For this, we calculated the expected annual flood damage of Imjin River using the multi-dimensional flood damage analysis(MD-FDA), and calculated the total value of riverine wetland using the contingent valuation method(CVM) to estimate preservation value of riverine wetland. The result of the analysis shows that the Imjin River needs flood control project and the ecological preservation of riverine wetland is also important. Therefore, the establishment of the management plan for protecting riverine wetland is also needed. As a result, the Imjin riverine wetland was classified as the area where sedimentation continues to take place, and the flood water level to rise. On the basis of the analyzed results, it is judged that the Imjin River needs flood control for public safety and ecological consideration for ecosystem preservation in the river improvement project. So, the stepwise river improvement is desirable to protect riverine wetland and minimize ecosystem disturbance. The results is expected to be made good use as the basic study for establishment of institutional river management plans considering flood control project and riverine wetland preservation in the future.

Analysis of River Channel Morphology and Riparian Land Use Changes using Multi-temporal Aerial Photographs and Topographic Maps of the Early 20th Century in Gyeongan-cheon Watershed (시계열 항공사진과 20세기 초 지형도를 이용한 경안천유역의 하천형태 및 하천부지 변화추세 분석)

  • Park, Geun-Ae;Lee, Mi-Seon;Kim, Hyeon-Jun;Kim, Seong-Joon
    • Journal of Korea Water Resources Association
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    • v.38 no.5 s.154
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    • pp.379-390
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    • 2005
  • This study is to trace the change of stream shape using the past series of aortal photographs and topographic maps, and to compare the land use changes of inland along the stream. For the Gyeongan first & second class of local stream, aerial photographs of 1966, 1981 and 2000 were selected and ortho photographs were made with interior orientation and exterior orientation, respectively. In addition, topographic maps of 1914 - 1915 were used to compare with stream of 1966, 1981 and 2000. As apparent changes of the stream, the consolidated reaches of stream with levee construction were straightened and their stream width widened. Especially the stream width of inlet part of Paldang lake was widened almost twice because of the rise of water level by dam construction in 1974. The land use maps (1966, 1981, 2000) of riparian areas were also made, respectively and classified into 6 categories (water, forest, agricultural land, urban area, road, sandbar) by digitizing, The main changes of land use were agricultural land, urban area, road and sandbar.

Evaluating the Performance of APEX-Paddy Model using the Monitoring Data of Paddy Fields in Iksan, South Korea (국내 논필지 모니터링 자료를 이용한 APEX-Paddy 모델 적용성 평가)

  • Kamruzzaman, Mohammad;Cho, Jaepil;Choi, Soon-Kun;Song, Jung-Hun;Song, Inhong;Hwang, Syewoon
    • Journal of The Korean Society of Agricultural Engineers
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    • v.62 no.1
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    • pp.1-16
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    • 2020
  • The APEX model has been developed for assessing agricultural management efforts and their effects on soil and water at the field scale as well as more complex multi-subarea landscapes, whole farms, and watersheds. Recently, a key component of APEX application, named APEX-Paddy, has been modified for simulating water quality by considering paddy rice management practices. In this study, the performance of the APEX-Paddy model was evaluated using field data at Iksan experimental paddy sites in Korea. The discharge and pollutant load data during 2013 and 2014 were used to both manually and automatically calibrate the model. The APEX auto-calibration tool (APEX-CUTE 4.1) was used for model calibration and sensitivity analysis. Results indicate that APEX-Paddy reasonably performs in predicting runoff discharge rate and nitrogen yield. However, sediment and phosphorus yield is not correctly predicted due to the limitation of model schemes. With APEX-Paddy, the performance in reproducing the discharge and nitrogen yield is found to be a satisfactory level after manual calibration. The manually calibrated model performed better than the automatically calibrated model in nearly all comparisons. For runoff, manual calibration reduced PBIAS while R2 and NSE values of the automatically calibrated model were the same as the manual calibration. For T-N, NSE and PBIAS were reduced when using manual calibration, whereas R2 value was the same as manual calibration. The limitation of the APEX-Paddy model for predicting sediment, as well as the phosphorous yield, was discussed in this study.

Study of Parallelization Methods for Software based Real-time HEVC Encoder Implementation (소프트웨어 기반 실시간 HEVC 인코더 구현을 위한 병렬화 기법에 관한 연구)

  • Ahn, Yong-Jo;Hwang, Tae-Jin;Lee, Dongkyu;Kim, Sangmin;Oh, Seoung-Jun;Sim, Dong-Gyu
    • Journal of Broadcast Engineering
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    • v.18 no.6
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    • pp.835-849
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    • 2013
  • Joint Collaborative Team on Video Coding (JCT-VC), which have founded ISO/IEC MPEG and ITU-T VCEG, has standardized High Efficiency Video Coding (HEVC). Standardization of HEVC has started with purpose of twice or more coding performance compared to H.264/AVC. However, flexible and hierarchical coding block and recursive coding structure are problems to overcome of HEVC standard. Many fast encoding algorithms for reducing computational complexity of HEVC encoder have been proposed. However, it is hard to implement a real-time HEVC encoder only with those fast encoding algorithms. In this paper, for implementation of software-based real-time HEVC encoder, data-level parallelism using SIMD instructions and CPU/GPU multi-threading methods are proposed. And we also proposed appropriate operations and functional modules to apply the proposed methods on HM 10.0 software. Evaluation of the proposed methods implemented on HM 10.0 software showed 20-30fps for $832{\times}480$ sequences and 5-10fps for $1920{\times}1080$ sequences, respectively.

A refinement of customer satisfactory factors in multimedia contentware evaluation process - focused on company website design - (멀티미디어 컨텐트웨어 상품에 대한 소비자 감성 평가 요소(문화성 인자)추출에 관한 연구 - 기업 웹사이트를 중심으로 -)

  • 이종호;김명석;이현이;김태균
    • Archives of design research
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    • v.11 no.1
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    • pp.291-302
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    • 1998
  • This paper covers the development process of multimedia evaluation system, especially focused on customer satisfactory factors while customers navigating net-based Interactive multimedia system. Customers usually experience new level of interaction cased by newly developed web-based technology In ordinary multimedia system. However, if it gives customers satisfactory experience is a matter of question. To find out the relationship between customer satisfaction and interactivity factors exposed by multimedia system, a model has been developed which describes the structure of web-based multimedia system and its relation to customer satisfactory factors. Five different experiments, including 'semantic differential', 'focus group interview', and 'expert review', has been conducted and four customer satisfactory factors were identified. Those are 'customery value', 'structural perfectness', 'visual perfectness', and 'contemporaneity'. With these factors and newly delveoped evaluation system, 7 different web-site has been evaluated and analyzed at the end of this report.

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