• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.12 no.1
• /
• pp.42-48
• /
• 2012

Pompe disease is a rare lysosomal glycogen storage disorder caused by a total or partial deficiency of the acid ${\alpha}$-glucosidase (GAA) enzyme due to the GAA gene mutations. The classic infantile form of Pompe disease is a rapidly progressive multi-organ disease with hypotonia, generalized muscle weakness, and hypertrophic cardiomyopathy, usually leading to death in the first 2 years of life. Enzyme replacement therapy with recombinant human GAA has been shown to be effective and subsequently yielded promising results. Here, we present clinical and genetic characteristics of three Korean non-classic infantile Pompe patients, and the short term efficacy of enzyme replacement therapy. Considering that enzyme replacement therapy can change the natural course of infantile Pompe disease, early diagnosis and early initiation of treatment is critical to improving patient outcomes.

• Journal of the Economic Geographical Society of Korea
• /
• v.15 no.2
• /
• pp.228-239
• /
• 2012

Guanxi could be regarded as communities based on blood, regionalism, school ties centering on individual person. It has been used as a means of personal interests security and acquisition. What is more, it has formed a mode of socio-economic order, which is an inter-obligation relations based on mutual benefits. In practice, it is necessary to understand and accept guanxi in China, as it is a key factor to understand consumer patterns, inter-firm relations and extra-firm relations between firm and government. In this context, the main aim of the reserach is to identify the impact of guanxi on the business activities of Korean foreign direct investment firms in China by investigating their networks based on guanxi.

• Journal of The Korean Association of Information Education
• /
• v.22 no.1
• /
• pp.113-122
• /
• 2018

Collaboration and assistance among peer learners are essential factors for successful learning outcomes. However it is important to investigate students' preferences for computer problem solving methods and interrelationships, since students tend to solve problems more and more by themselves. This is because of the importance of giving appropriate instructions to students. In this context, this paper shows the analysis of the preferred methods and interrelationships of studnets' preferences upon encountering difficulties during computer usage by collecting data from 231 students in K national university of education. As a result, the result shows that students tend to solve problems without asking as they have higher abilities in computer usage, which was also shown to increase along with their grade levels. Furthermore, it showed that students who have family members and relatives, and who are using the internet are more satisfied with their problem solving. Lastly, it is possible to grasp the computer problem solving network within the department by using social network analysis, so it can be used as reference data for selecting the peer learners, which will help to operate the customized computer education practice.

• Korean Journal of Agricultural Science
• /
• v.32 no.1
• /
• pp.53-61
• /
• 2005

Many bovine genetic diseases are currently unidentified in Korea because of the relatively low monitoring systems in the livestock farms. The molecular detection system using PCR-RFLP of two genetic diseases, namely Band 3 (Erythrocyte Membrane Protein Band III) and CHS (Chediak-Higashi Syndrome), have been identified in Japan and used for screening large number of cattle whether each individual has the genetic disease or not. Using the 22 unrelated Korean cattle (Hanwoo) individuals, molecular detection system based on PCR-RFLP have been investigated, which can be distinguishable carriers for the genetic diseases. Even though we could not found the causative mutations for two genetic diseases, the PCR-RFLP techniques used in this study are very valuable for the screening the genetics diseases in Korean cattle, especially for the proven or candidate bulls.

• Archives of design research
• /
• v.15 no.3
• /
• pp.191-200
• /
• 2002

In the 1980s, even though Italy faced economic depression, Italy (she), now, internationally became a strong nation with its best design by national unity and the developmental leap of industry through design. In Italy, especially, small and medium enterprises are accumulated by the same business in each area on the structure of a Cluster system. Italy has some characteristics that being the Italians' elastic way of management, family management and development of a consortium of small and medium enterprises, which have flexibility, imagination and decisiveness. We have to bring these intensified factors of design competitiveness to our country so that we should form the union in order to have a monopoly of specific enterprises based on the specialized technology of small and medium enterprises innovating the structure of a small quantity production having developmental flexibility. We also heighten the sense of designer and publics by achieving social opinion and cult! ural revival. Moreover, the mind of managers has to be changed more aggressively. Finally, the actual policy of design will be established and peformed, and design-company (enterprises) and the industrial economy will generally develop when we activate the structure of the industry centered on small and medium enterprises and design-related association that is united and participates in the establishment of design policy.

• Journal of the Architectural Institute of Korea Planning & Design
• /
• v.34 no.10
• /
• pp.33-44
• /
• 2018

The purpose of this research is to identify the characteristics of the positive and negative expressions about residential common life between the elderly and the young and present the direction on Homeshare. In-depth interviews were conducted from Feb. to May 2016 to identify the experiences of residential common life with 10 elderly and 11 young. The result is as follows. First, Homeshare dwelling was classified into five themes of human being, residential environment, life, management and emotion. Homeshare unlike ordinary dwelling centered on blood relatives, is characteristic to involve the management that connects between the elderly and young. Second, both the elderly and young were analyzed to be positive about the residential common life. It was shown that the elderly are most positive about human being, whereas young are positive about management and residential environment. The elderly is positive to mutual exchange with the young and young's independent life but the young is positive to factors that support comfortable personal life. Third, negative expression was shown a generation gap between the elderly and the young. The elderly recognized to be negative that young people has lived freely without consideration for the elderly. In order to solve these factors, it is necessary to make improvements through arranging rules for living. Fourth, three themes, human being, residential environment and life, analyzed to be the most important factors to sustain residential common life of Homeshare. The link between management and 3 themes leads to an expansion of positive emotion, which is structurally stable and is expected to increase the sustainability of the residential common life on Homeshare.

• The Journal of Korean Philosophical History
• /
• no.38
• /
• pp.97-128
• /
• 2013

Originally confucianism is closer to communitarianism than to individualism. The ideal of confucianism is to realize ethical community. Chu Hsi's theory of the Song dynasty reestablishes the ideal of ethical community and dignitaries(士大夫) take the job to realize it as their own vocation. There are many developed 'family clans and kinsfolk societies', 'local covenants and sacred storehouses' and 'private academies' in the Chosun dynasty as in the Song dynasty. These communities are three kinds of axial confucian community, which are both natural ascriptive groups and voluntary contract groups in nature. Communities of Chosun dynasty are to strengthen the solidarity through friendship and mutual help as well as to cultivate good customs in society. Also these traditional communities are managed by democratic procedures, as these are originally voluntary contract groups. On the other hand, traditional neo-confucian self-training theories like that individual personality should be harmony with general order, or one should pursuit his private interest according to fair principles are the process of sublimation private individual into public citizen.

• The Journal of the Convergence on Culture Technology
• /
• v.7 no.3
• /
• pp.445-451
• /
• 2021

Korean film director Lee Joon-ik's two most recently released works, (2018) and (2021), introduce some changes to the world of male bonds, where bromance colors prevailed. It is a father-son or pseudo father-son relationship that has not been dealt with in the exploration of vary kind of intimacy and relationships between men in the 'Lee Joon-ik's films'. If depicts a blood-related father-son relationship, highlights the teacher-disciple relationship, or pseudo father-son relationship. In that respect, the films continue, change, and expand the relationship setting, the sense of problems in the previous film, and inevitably raise the issue of 'generation' and 'inheritance' required today by illuminating the father-son relationship.

• Journal of the Korea Society of Computer and Information
• /
• v.27 no.3
• /
• pp.79-90
• /
• 2022

'Trust' is 'believing and relying on people or things', and at the same time, it is the foundation and essential condition of existence of a specific community or society. How blockchain technology creates trust can be found in the fact that it is a technology shared by all participants. As a shared technology, blockchain itself shows three characteristics: transparency, immutability, and availability. Because it is shared, it is transparent, and because it is shared, it is immutable, which is difficult to modify or delete. In addition, since it is shared, the entire system operates normally even if some failure occurs. The significance of trust created based on this blockchain technology can be summarized in three aspects as follows. The first is to understand the problem of trust in connection with technology. Second, understanding the relationship between information and trust begins to pay attention. Third, based on the expanded information processing capability, it is possible to establish a large-scale, concrete relationship without central mediation.

• The Journal of the Korea Contents Association
• /
• v.22 no.6
• /
• pp.194-207
• /
• 2022

Amid growing social interest in child abuse, and announced the seriousness of child abuse crimes and aroused public interest in related issues. Based on true stories and characters, both films created unique narratives about child abuse cases, but drew on the traditional representation and discursive construction of child abuse news articles. By setting the stepmother as the perpetrator and the father as the neglecter, the gender role of women as primary caregiver was reconfirmed and the stereotypical image of the 'evil stepmother' in popular narratives was exploited. The cinematic reenactment of the evil stepmother not only highlighted the normative family discourse, but also reinforced the maternal myth by emphasizing the binary opposition between the evil stepmother and the lost birth mother.