Journal of The Korean Society of Inherited Metabolic disease
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v.15
no.2
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pp.87-92
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2015
Mucopolysaccharidosis (MPS) is an inherited disease entity associated with lysosomal enzyme deficiencies. MPS type 2, also known as Hunter syndrome, has a characteristic morphology primarily involving x-l inked recessive defects and iduronate-2-sulfatase gene mutation. The purpose of this case report is to provide important clues to help pediatricians identify Hunter syndrome patients earlier (i.e., before the disease progresses). A 30-month-old boy showed developmental delay and decreased speech ability. Physical examinations revealed a flat nose and extensive Mongolian spots. Brain magnetic resonance images (MRIs) showed bilateral multiple patchy T2 hyperintense lesions in the periventricular and deep white matter, several cyst-like lesions in the body of the corpus callosum, and diffuse brain atrophy, which were in keeping with the diagnosis. Based on these findings, the patient was suspected of having MPS. In the laboratory findings, although the genetic analysis of IDS (Iduronate-2-sulfatase) did not show any pathogenic variant, the enzymatic activity of IDS was not detected. We could confirm the diagnosis of MPS, because other sulfatases, such as ${\alpha}$-L-iduronidase, were detected in the normal range. Early enzymatic replacement therapy is essential and has a relatively good prognosis. Therefore, early diagnosis should be made before organ damage becomes irreversible, and brain MRIs can provide additional diagnostic clues to help distinguish the disorder.
With the rapid development of the Internet and information technology, a company that deals with personal information does not have proper action to protect personal privacy and not take measures for the safe handling and management of personal information. It generates the case to abuse of personal information occurring frequently. In order to focus the effort to reduce damage and protect the privacy of personal information entity and enhance privacy laws based on the connection method and the processing of personal information, Korea encourages a company to follow regulation by providing certain criteria. However, in the case of items of measures standard of safety of personal information such as priority applicable criteria in accordance with the importance of itemized characteristics and the company of each individual information processing is not taken into account, and there are some difficulties to execute. Therefore, we derive criteria by law and reviewing existing literature related, the details of the measures standard of safety of personal information in this study and generate a hierarchical structure by using the KJ method for layering and quantification of the evaluation in integration of the reference item similar and the grouping. Accordingly, the weights calculated experts subject using the AHP method hierarchical structures generated in this manner, it is an object of the proposed priority for privacy and efficient more rational enterprise.
Potentials of AR CDM project in North Korea are assessed and feasible land area for AR CDM project is estimated. According to our estimation, There could be 515,000 hectares of forest lands deforested before 1990 in North Korea and 8,854 hectares at the regional level of Gae-sung City, which are eligible for AR CDM project, based on researches of satellite image analyses conducted from 1980's to 1990's. A baseline scenario assumed 44.73 tones of carbon stored in soil per hectare with no vegetation above ground remained during the project period following the default value of IPCC's Good Practice Guidance for LULUCF considering soil structure, climate and land use of the project area. The scenario also assumes that black rocust (Robinia pseudoacacia) is planted and the CDM project is implemented for 20 years. The costs for producing greenhouse gases CER (certified emission reduction) credits include costs of tree planting and forest management, and costs of project negotiation and transactions for issuing the credits. It is estimated that 376 tones of carbon dioxide per hectare can be accumulated and 503 temporary CER credits per hectare and 265 long-term CER credits per hectare could be produced during the project period. It is estimated to cost US$ 4.04 and US$ 7.67 to provide one unit of temporary credit and long-term credit, respectively. These values can be regarded as the cost of conferring emission commitment of a country or a private entity. However, it is not clear which option is better economically because the replacement periods are different in these two cases.
Recently, as the paradigm of regional development has been transformed into characterization, decentralization and cooperation, small and medium scale development is in the spotlight. In particular, as the transfer of planning authority to local governments accelerates, LH is in the process of seeking to transform itself into a system that is in line with local government demand. The purpose of this study is to elaborate the regional pending projects that meet the demand of the region. The Jeju Special Self-Governing Province is the area where land and housing prices have increased more than three times recently, which is the area of interest in recent years due to the various demand for development projects. Another objective is to establish a local government based on LH's system, it is aimed to derive a collaboration method with local government, province corporation and local researchers. The criteria for deriving the cooperation projects between the local government and LH are basically the ones that can be carried out by LH and future-oriented projects. The process of deriving has undergone the process of statutory planning, unscrupulous plan analysis, and consultation of experts' advisory committees. In order to derive the regional cooperation project, four criteria such as local uniqueness, future possibility, business promotion efficiency, and local cooperation project were set. Major projects of the Jeju Special Self-Governing Province are improvement of the surrounding traffic system, construction of the hinterland due to the construction of the second airport, and establishment of Cruise Port(Jeju Port, Seogwipo Port). The role of each entity in the implementation of regional cooperation projects is as follows. Local government should request subsidies for the projects in case of lack of budget support and secure them through competition with other regions. In addition, it should be responsible for the operation and management of the facility once it has been supported and completed smoothly. The Ministry of Land, Infrastructure and Transport affects each region through approval and subsidy of the development plan. After the development project is completed, it evaluates the development project through monitoring and plays a role of continuously improving the system. As a business operator, the provincial corporation will carry out small-scale projects including non-physical projects such as community participation. In the case of LH regional headquarters, the general manager of the region will establish a comprehensive business plan, secure development availability, and carry out large-scale growth promotion projects.
Kon Satoshi is a representative animation film director who explores human's intrinsic self-identity based on ambivalence and delivers a message for self-reflection of entity. The aim of this study is to analyze the persona of character according to the way how a self incorporates with another self in his film; that is to say, how a mask of the personality expresses and realizes itself from the perspectives of the Jung's analytical psychology. The persona, defined as the upmost external personality, involves social behavior playing a specific role to establish constant relationship with others in a society. With increasing demand in a society, individuals get to adapt to outer environment by playing his/her given role in various masks of personality. In this way, although persona acts as a major function in relationship with outer world, it would exert a negative influence on formation of self-identity because of the inconsistency between consciousness and unconsciousness when immersed only in mask of personality, complying excessively with the demand of group. However, persona does not always have a negative effect on relationships. Developed adequately to fulfill its social function, persona is helpful to establish a healthy self-identity with balanced control between inner world of unconsciousness and outer world of consciousness. From these perspectives, this study can identify how the desire of mask hidden inside of each character expresses to function in a positive or negative way by analyzing characters in the film according to integrated pattern and correlation between consciousness and unconsciousness. Additionally, I hope that the psychological research methods in this study will be full of help for interpreting on a character analysis as a theoretical approach.
Journal of the Korean Society of Surveying, Geodesy, Photogrammetry and Cartography
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v.31
no.4
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pp.269-275
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2013
Recently, importance of pedestrian road network is getting emphasized as it is possible to provide mobile device users with both route guidance services and surrounding spatial information. However, it costs a tremendous amount of budget for generating and renovating pedestrian road network nationally, which hinder further advances of these services. Hence, algorithms extracting pedestrian road network automatically based on raster data are needed. On the other hand, road dataset generated from raster data usually has unnecessary vertices which lead to maintenance disutility such as excessive turns and increase in data memory. Therefore, this study proposed a method of selecting a proper threshold automatically for separate road entity using not only Douglas-Peucker algorithm but also road attribute data of digital map in order to remove redundant vertices, which maximizes line simplification efficiency and minimizes distortion of shape of roads simultaneously. As a result of the test, proposed method was suitable for automatic line simplification in terms of reduction ratio of vertices and accuracy of position.
The biological resources research data around the world are not only very critical themselves but should be shared and utilized. Up to now, the biological resources have been compiled and managed individually depending on the purpose and characteristics of the study without any clear standard. So, in this study, the data reference model would be suggested which is applicable in the phase ranging from the start of the construction of the information system and which can be commonly used. For this purpose, the data model of the related information system would be expanded based on the domestic and foreign standards and data control policy so that the data reference model which can be commonly applicable to individual information system would be developed and its application procedure would be suggested. In addition, for the purpose of proving the excellence of the suggested data reference model, the quality level would be verified by applying the Korgstie's data model evaluation model and its level of data sharing with the domestic and foreign standards would be compared. The test results of this model showed that this model is better than the conventional data model in classifying the data into 4 levels of resources, target, activities and performances and that it has higher quality and sharing level of data in the data reference model which defines the derivation and relation of entity.
This paper examines political leaders' image based on the concepts of social identity and distance. These concepts come from the two ideas: (1) Individuals create events to confirm the sentiments that they have about themselves and others in the current situation. (2) During this process structured sets of beliefs (social identity) about attributes of various entities are formed. Statistical analysis of the data from college students in Seoul offers three main findings. First, attributes clustered into four major groups-sociability, responsibility, power and activity. Sociability concerns as sense of approval or disapproval that can elaborated into closeness and intimacy. Responsibility relates to the judgement of morality, public obligation, or other standards. Power refers to social power, physical magnitude and so on. Activity indexes an entity's spontaneity, which can be elaborate into judgements of agency, speed and so on, Second, respondents evaluate negatively political leaders at the dimensions of sociability, responsibility, and activity, They are positively evaluated only at the dimension of power. Third, political leaders who are felt to be closer than others seem to have more political support nowadays.
Journal of the Korean Institute of Traditional Landscape Architecture
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v.33
no.3
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pp.50-57
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2015
A traditional garden in Korea has diverse cultural, historical values, such as the then phases of the times, life phase, culture and art, etc. because it was developed on the basis of the harmony between nature and artificial structures. However, in reality, it's urgent to do efficient, continuous maintenance of traditional gardens which are being damaged and lost due to the problems like an owner's aging, inheritance, and lack of management, etc., especially in case of private property which was designated as a cultural property among such traditional gardens under the Cultural Properties Protection Law. Accordingly, this study conducted this research in a bid to use these research results as basic evidentiary materials for suggesting directivity in introduction of park planning of traditional gardens in folk houses in the near future by implementing the case investigation of transformation into public parks from traditional private gardens in Japan, which is putting the newly introduced park planning to efficient use, together with its systematic management, and the survey on domestic traditional gardens in folk houses status, as well as the hearing-based survey on a traditional gardens in folk houses owner's level of willingness to accept the introduction of parking planning. As a result, this study could confirm that in case of traditional gardens in Japan, they are mobilizing the revenue from admission fees for traditional gardens maintenance by incorporating the main entity of possession, and Japan is promoting transformation of traditional gardens into parks on the basis of use and preservation through the connection with local governments and research institutes. In addition, as a result of surveying domestic traditional gardens in folk houses, it was found that most of the garden owners had hardships in its management, and they were positive about systematic maintenance of gardens through park planning.
This study investigated development method of governance of local residence, central and local government based on fishing ports as a new paradigm for restoration of sustainable space environment on Hyeonpo Harbor in Ulleung Island. According to the result of conducting a questionnaire survey on residents of Ulleung Island in order to carry out the research purposes, vulnerable living environment as poor accessibility and lack of hospital, community health center, and shopping district were pointed out as the biggest problem of residential environment in Ulleung Island of today. Secondly, there was a high recognition that development project on Ulleung Island of the government and local government is not actually contributing to the revitalization of regional economy and improvement in quality of life of Ulleung Island residents, and thirdly, lack of understanding on the importance or usage of various resources in Ulleung Island and insufficient support of the central government and local autonomous entity were pointed out as the bottleneck factors in implementation of the Ulleung Island development project. Fourthly, they perceived the current management level of Hyeonpo Harbor to be unsatisfactory. The results of this study demonstrated the importance of durability of development entities, vision of development of Hyeonpo Harbor, effort for the change of ownership mind, phased implementation considering reality of the area, and development that can provide small-scale profitable contents than a large theme.
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