• Clinical and Experimental Pediatrics
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• v.48 no.10
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• pp.1132-1138
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• 2005

Purpose : The mucopolysaccharidoses (MPSs) are a heterogeneous group of lysosomal storage disorders. They are caused by a deficiency of the enzymes involved in the degradation of glycosaminoglycans. Early recognition is important because recombinant enzyme replacement therapy is now available for MPS. We studied the clinical characteristics of 80 MPS children with the object of determining the epidemiological, clinical and radiological features in Korean MPS children. Methods : Diagnosis of MPS was confirmed by skin fibroblast enzyme analysis in 80 patients between February 1995 and December 2004. Charts were retrospectively reviewed for clinical and radiological findings, as well as for intelligence and speech evaluations. Results : Hunter syndrome (MPS type II) was the most prevalent type, appearing in 51/80 cases (64 %), followed by Sanfilippo syndrome (MPS III-18%), Hurler syndrome (MPS I-15%), and Morquio syndrome (MPS IV-4%). The average age at diagnosis was 5.5 years (range 1 to 20), and the male-to-female ratio was 4.7 : 1. Typical radiographic changes were observed in 45/54 cases (83%). Mitral regurgitation was the most common cardiac defect. Moderate to profound mental retardation and hearing loss were present in 14/35 cases (56%) and 33/38 cases (82%), respectively. Four MPS II patients had bone marrow transplantation, with mixed outcomes. Five MPS I patients are currently on enzyme replacement therapy. Conclusion : Our study showed a high proportion of MPS II cases (64%), which may represent population variability. By studying the clinical features of these patients, we hope to alert pediatricians of the warning signs of MPS.

• Journal of the Korea institute for structural maintenance and inspection
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• v.20 no.6
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• pp.106-112
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• 2016

In this study, circular concrete column specimens confined by carbon sheet tube with different winding angles and different number of carbon sheet plies(3T, 5T and 7T) were tested to propose design equations and a strength reduction factor. Specimens were designed by 300 mm diameter and 600 mm height with $90^{\circ}{\pm}0^{\circ}$, $90^{\circ}{\pm}30^{\circ}$, $90^{\circ}{\pm}45^{\circ}$, $90^{\circ}{\pm}60^{\circ}$, $90^{\circ}{\pm}75^{\circ}$ and $90^{\circ}{\pm}90^{\circ}$ carbon fiber angles. A 10,000 kN UTM was used for compressive strength test of specimens by displacement control method with 0.01 mm/sec velocity. Estimation equations of compressive strength and ultimate strain of circular concrete column specimens confined by carbon sheet tube using a regression analysis and a strength reduction factor to apply ultimate strength design method of concrete were proposed. The strength reduction factor(${\phi}$) of circular concrete columns confined by carbon sheet tube was estimated as 0.64 by the Monte Carlo Analysis Method. Manufacture and construction process have to be perfectly managed by construction managers because the structural capacities of carbon tubes were depends on construction abilities of manufacturing operators.

• Journal of Distribution Science
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• v.12 no.8
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• pp.5-15
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• 2014

Purpose - The method of TV home shopping is a kind of retail method that provides the viewer with information about products and, further, sells the products to consumers through the media of television. The domestic home-shopping industry has been expanding since 1995, and there are six companies in this arena as of 2012. In this study, we evaluate the management efficiency of TV home-shopping companies and provide suggestions for improving efficiency, using the DEA (data envelopment analysis) model. Hence, we expect to contribute to the progress of the companies' efficiency and the development of the TV home-shopping industry, where deepening competition is inevitable because it is experiencing the maturing market stage in its life cycle. Research design, data, and methodology - Efficiency is the ratio of the quantity of input to the quantity of output of a product or service. It is necessary to estimate aggregate inputs and aggregate outputs, which are calculated by applying a weighting to a number of input and output factors, to measure the efficiency. The DEA model is divided into the CCR model and the BCC model. The CCR model is a basic model that assumed constant returns to scale (CRS), and the BCC model extends the CCR model to accommodate technologies exhibiting variable returns to scale (VRS), and concerns only the technical efficiency without considering the efficiency of returns to scale. In this study, we consider six companies each year from 2008 to 2012 as a DMU (Decision Making Unit) and analyze the differences in efficiency for each company in each year. Furthermore, we evaluate the operating characteristics of TV home-shopping companies, using three models, in accordance with the overall performance, profitability, and marketability of the business. Results - The result of the analysis, using DEA models, shows that Hyundai Home Shopping (2009, 2010, 2011), GS Home Shopping (2011), NS Home Shopping (2011) and CJ O Shopping (2012) possess MPSS (most productive scale size), with a score 1.0 in CCR, BCC, and scale efficiency. Particularly, Hyundai Home Shopping is shown to be the most efficient in terms of overall business performance, marketability, and profitability. The overall efficiency of the home shopping industry has displayed an increasing trend since 2008, even though it decreased marginally in 2012; further, we can observe that home shopping companies operate with increasing efficiency with the passage of time. Conclusions - Home shopping companies have focused on market expansion rather than profits, as they displayed better efficiency in marketability than increase in profitability during the period 2008-2012. In addition, the main reason for the increased efficiency in the home shopping industry is the market expansion through the revenue increase of each home shopping company. This study can be used as a reference when home shopping companies attempt to devise future strategies, as it suggests efficiency benchmarks and development levels for each home shopping company.

• Journal of the Korea Society for Simulation
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• v.31 no.3
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• pp.1-10
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• 2022

Defense fields widely operate unmanned systems to lower vulnerability and enhance combat effectiveness. In the navy, swarm unmanned surface vehicles(USVs) form a cluster within communication range, share situational awareness information among the USVs, and cooperate with them to conduct military missions. This paper proposes an interface system, i.e., Interface Adapter System(IAS), to achieve inter-USV and intra-USV interoperability. We focus on the mission planning subsystem(MPS) for interoperability, which is the core subsystem of the USV to decide courses of action such as automatic path generation and weapon assignments. The central role of the proposed system is to exchange interface data between MPSs and other subsystems in real-time. To this end, we analyzed the operational requirements of the MPS and identified interface messages. Then we developed the IAS using the distributed real-time middleware. As experiments, we conducted several integration tests at swarm USVs simulation environment and measured delay time and loss ratio of interface messages. We expect that the proposed IAS successfully provides bridge roles between the mission planning system and other subsystems.