Sohn, Young Bae;Jang, Ju Young;Lee, Dakeun;Jang, Ja-Hyun
Journal of The Korean Society of Inherited Metabolic disease
/
v.17
no.3
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pp.96-102
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2017
Glycogen storage disease type IX (GSD IX) is caused by deficiency of phosphorylase kinase which plays a role in breakdown of glycogen. Mutations in PHKA2 are the most common cause of GSD IX (GSD IXa). Clinical manifestations of GSD IXa include hepatomegaly, elevation of liver enzyme, growth retardation, fasting hypoglycemia, and fasting ketosis. However, the symptoms overlap with those of other types of GSDs. Here, we report Korean familial cases with GSD IXa whose diagnosis was confirmed by targeted exome sequencing. A 4-year old male patient was presented with hepatomegaly and persistently elevated liver enzyme. Liver biopsy revealed swollen hepatocyte filled with glycogen storage, suggesting GSDs. Targeted exome sequencing was performed for the differential molecular diagnosis of various types of GSDs. A hemizygous mutation in PHKA2 were detected by targeted exome sequencing and confirmed by Sanger sequencing: c.3632C>T (p.Thr121Met), which was previously reported. The familial genetic analysis revealed that his mother was heterozygous carrier of c.3632C>T mutation and his 28-month old brother had hemizygous mutation. His brother also had hepatomegaly and elevated liver enzyme. The hypoglycemia was prevented by frequent meals with complex carbohydrate, as well as cornstarch supplements. Their growth and development is in normal range. We suggest that targeted exome sequencing could be a useful diagnostic tool for the genetically heterogeneous and clinically indistinguishable GSDs. A precise molecular diagnosis of GSD can provide appropriate therapy and genetic counseling for the family.
Jung, Samooel;Kim, Hyun Joo;Lee, Hyun Jung;Seo, Dong Won;Lee, Jun Heon;Park, Hee Bok;Jo, Cheorun;Nam, Ki Chang
Korean Journal of Poultry Science
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v.42
no.2
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pp.101-108
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2015
This study investigated the initial pH measured at 15 min post-mortem, ultimate pH, water holding capacity (WHC), and color of meats obtained from five lines of Korean native chicken (KNC) for the development of breed for high-quality meat. In addition, the effect of sex was examined. In total, 595 $F_1$ progeny (Black [B], Grey-Brown [G], Red-Brown [R], White [W] and Yellow-Brown [Y]) from 70 full-sib families were used. Chickens were slaughtered at 20 wk of age and the measurement traits of all breast and thigh meats from 595 chickens were analyzed. The initial pH at 15 min post-slaughter of the breast and thigh meats was affected by the line and sex of Korean native chicken (P<0.05). However, there was no line and sex effect on ultimate pH and WHC of the breast and thigh meat except that the ultimate pH of thigh meat had line effect. Except for the $L^*$ value of breast meat, the meat color was significantly affected by line and sex (P<0.05). The ultimate pH showed consistently negative correlation with the $L^*$ value and positive correlation with the $a^*$ value in breast and thigh meats. Based on the results, we concluded that the line W in male chickens and the line G and W in female chickens may be good candidates for the selection to develop breed for high-quality meat because these lines showed property of high initial pH or/and ultimate pH.
This study attempts to develop a theory on the evolving process of wealthy major donors' sharing lives in Korea through a grounded theory approach. To conduct this study, the researchers have in-depth interviews with 11 exemplary wealthy major donors who have more than one million US dollars in his or her own asset and donate more than ten thousand US dollars annually. In data analysis, this study identifies 161 concepts on the evolving process of wealthy major donors' sharing lives; and the concepts are categorized with 33 sub-categories and 14 categories. In the paradigm model on the evolving process of wealthy major donors' sharing lives, it is identified that the central phenomenon, 'practicing sharing lives as noblesse oblige', is related with the causal conditions such as 'learning through memories and observation', 'realizing my duties', and 'emphasizing'; and the central phenomenon is related with the contingent conditions such as 'being sensitive to external evaluation', 'having limited information on giving', 'distrusting donation related environments'. The action/interactional sequences such as 'utilizing relationships' and 'strengthening active participation' are accomplished by moderating conditions such as 'having internal and external supports' and 'guiding by firm conviction'. It reveals that as a result, wealthy major donors enjoy the feeling of becoming a ideal and true wealthy person, establish sharing lives as firm and major parts of overall lives, and experience strong desires for better future and society. In this study, 'generous sharing that shares personal heritages and social benefits' is analyzed as a core category; it shows that sharing of wealthy major donors is related to the characteristics of generosity practice based on moral self-benefiting rather than complete altruistic characteristics or self-sacrificial characteristics. The process analysis reveals that it has the following stages: first, initial giving by exposure to causes or requests; second, routine practice of giving; third, evolution of practice of giving with gradual expansion in quantities and qualities; and fourth, living with giving. In the process, the following four types are identified: devoted wealthy donors for sharing, wealthy donors practicing sharing in daily life, wealthy donors practicing sharing with learning on external stimulus, and wealthy donors practicing sharing on empathy. Finally, this study discusses both meanings of identifying and developing a theory on the evolving process of wealthy major donors' sharing lives and implications of the research results in cultivating and developing potential wealthy major donors in Korea.
Increasingly the institutional theory has been an important theoretical view of decision making process and IT adoption in many academic researches. This study used the institutional theory as a lens through which we can understand the factors that enable the effective appropriation of advanced information technology. It posits that mimetic, coercive, and normative pressures existing in an institutionalized environment could influence the participation of top managers or decision makers and the involvement of users toward an effective use of IT in their tasks. Since the introduction of IT, organizational members have been using IT in their daily tasks, creating and recreating rules and resources according to their own methods and needs. That is to say, the adaptation process of the IT and outcomes are different among organizations. The previous studies on a diverse use of IT refer to the appropriation of technology from the social technology view. Users appropriate IT through not only technology itself, but also in terms of how they use it or how they make the social practice in their use of it. In this study, the concepts of institutional pressure, appropriation, participation of decision makers, and involvement of users toward the appropriation are explored in the context of the appropriation of the mobile electronic medical record system (M-EMRS) in particularly a hospital setting. Based on the conceptual definition of institutional pressure, participation and involvement, operational measures are reconstructed. Furthermore, the concept of appropriation is measured in the aspect of three sub-constructs-consensus on appropriation, faithful appropriation, and attitude of use. Grounded in the relevant theories to appropriation of IT, we developed a research framework in which the effects of institutional pressure, participation and involvement on the appropriation of IT are analyzed. Within this theoretical framework, we formulated several hypotheses. We developed a second order institutional pressure and appropriation construct. After establishing its validity and reliability, we tested the hypotheses with empirical data from 101 users in 3 hospitals which had adopted and used the M-EMRS. We examined the mediating effect of the participation of decision makers and the involvement of users on the appropriation and empirically validated their relationships. The results show that the mimetic, coercive, and normative institutional pressure has an effect on the participation of decision makers and the involvement of users in the appropriation of IT while the participation of decision makers and the involvement of users have an effect on the appropriation of IT. The results also suggest that the institutional pressure and the participation of decision makers influence the involvement of users toward an appropriation of IT. Our results emphasize the mediating effect of the institutional pressure on the appropriation of IT. Namely, the higher degree of the participation of decision makers and the involvement of users, the more effective appropriation users will represent. These results provide strong support for institutional-based variables as predictors of appropriation. These findings also indicate that organizations should focus on the role of participation of decision makers and the involvement of users for the purpose of effective appropriation, and these are the practical implications of our study. The theoretical contribution of this study is lies in the integrated model of the effect of institutional pressure on the appropriation of IT. The results are consistent with the institutional theory and support previous studies on adaptive structuration theory.
Open Source Software Development (OSSD) differentiates itself from traditional closed software development in that it reveals its source codes online and allows anyone to participate in projects. Even though its success was in doubt, many of the open collaborative working models produced successful results. Academia started to get interested in how developers are willing to participate even when there are no extrinsic rewards for their efforts. Many studies tried to explain developers' motivations, and the pursuit of ideology, reputation, and altruism are found to be the answers. Those studies, however, focused mostly on how the first contribution is made out of a certain motivation. Nowadays, OSSD reaches at its maturity and 70% of professional developers have used or utilized open source software or code in their works. As the proportion of people experiencing OSS, the accounts from previous studies are expected to be weakened. Also, extant literature fails to explain how the motivation of participating in OSS evolves over time and experiences. Given that changing over time or over experiences is the natural in the perception of motivation, studies in an attempt to understand how the motivation changes or evolves are in need. In this study, we aimed to explain how the perception about OSS from past usage or related experiences leads to the intention to sustain OSS participations. By doing so, we try to bridge the gap between previous studies and the actual phenomenon. We argued that perceived instrumentality about OSS learned from past experiences will first affect the formation of organizational identity towards general OSS community. And once the organizational identity is formed, it will affect the one's following behaviors related to OSS development, most likely to sustain the favoring stance toward OSS community. Our research distinguishes itself from previous one in that it divides the paths from organizational identity formed to the intention to sustain the voluntary helping behaviors, by altruistic and conforming intentions. Drawing on this structural model, we could explain how organizational identity engages in forming the sustaining intention from past experiences, and that the intention to help at individual level and organizational level works at different level in OSS community. We grounded our arguments on Social identity theory and Organizational Citizenship theory. We examined our assumption by constructing a structural equation model (SEM) and had 88 developers to answer our online surveys. The result is analyzed by PLS (partial least square) method. Consequently, all paths but one in our model are supported, the one which assumed the association between perceived instrumentality and altruistic intention. Our results provide directions in designing online collaborative platforms where open access collaboration is meant to occur. Theoretically, our study suggests that organizational citizenship behavior can occur from organizational identity, even in bottom-up organizational settings. More specifically, we also argue to consider both organizational level and individual level of motivation in inducing sustained participations within the platforms. Our result can be interpreted to indicate the importance of forming organizational identity in sustaining the participatory behaviors. It is because there was no direct association between perceived instrumentality from past experiences and altruistic behavior, but the perception of organizational identity bridges the two constructs. This means that people with no organizational identity can sustain their participations through conforming intention from only the perception of instrumentality, but it needs little more than that for the people to feel the intention to directly help someone in the community-first to form the self-identity as a member of the given community.
Purpose: Ultrasonography and $^{99m}-Tc-MIBI$ scintimammography were validated as useful diagnostic tools for primary breast cancer. However, ultrasound has the problem of low specificity. We compared the diagnostic usefulness of ultrasound with $^{99m}-Tc-MIBI$ scintimammography in the diagnosis of breast cancer. Materials and Methods: This study included 174 patients who had ultrasound and $^{99m}-Tc-MIBI$ scintimammography peformed on breast masses from 1999 to 2000. The pathologic results were obtained by surgery or FNAB. Results: Among the 174 patients, malignant breast disease numbered 117 and benign breast disease numbered 57. Ultrasound revealed 88 TP, 9 FN, 8 FP, 34 TN, and 35 indeterminate cases. $^{99m}-Tc-MIBI$ scintimammography revealed 91 TP, 25 FN, 9 FP, and 48 TN. The sensitivity, specificity, positive predictive value, and negative predictive value of Ultrasound were 66.7%, 44.2%, 67.2%, and 43.6% respectively. The sensitivity, specificity, positive predictive value, and negative predictive value of $^{99m}-Tc-MIBI$ scintimammography were 77.8%, 84.2%, 91%, and 64.9% respectively. Among the 35 indeterminate ultrasound cases, $^{99m}-Tc-MIBI$ scintimammography revealed 13 TP, 15 TN, and 7 FP Conclusion: $^{99m}-Tc-MIBI$ Scintimammography was more sensitive and specific than ultrasound for the detection of primary breast cancer and provided more useful information in cases of indeterminate ultrasound findings.
Prior to the introduction of the CRM, we need to analyze the characteristics and the situations of the company, and should establish a clear vision of the CRM. And each company should identify elements and technologies for introducing the most suitable CRM for them, and optimize them, with long-term perspective. In addition, it requires the implementation strategy which integrates the existing company's routine marketing activities with the concept of the CRM. According to the implementation strategy, the company should improve the business process which is the most effective in investment step by step, and the information system strategy, which develops system investment gradually, should harmonize with it. First, we recognized that raising the company value is important by maximizing customer lifetime value (LTV) by understanding customer needs, and achieving the company's goal through customer satisfaction. Second, we understood that adopting the CRM should be accompanied by changes in the structure, business process and customer contact channels, and it can be successfully integrated with business when it gets proper understandings and attentions of the management. Third, the reality is that there are few cases of successful implementation of domestic companies, and some companies that successfully implement the system mean nothing but implement the solution for developing the CRM. Therefore, it needs to be observed for the long haul, and it seems that we need to approach more systematically to implementation cases for each industry about implementation of the CRM. Fourth, the CRM is no longer the preserve of major companies, and it is the time that medium and small sized enterprises also need it. Taking lesson from Switzerland's small size store merchants who successfully adopt right size of the CRM for their business, for domestic medium and small sized enterprises, the necessity to develop business through developing the CRM models which fit their situations and maintaining relationships with customers has been grown. Fifth, for adopting the CRM business processes, changing or converting the CRM system to the model which fits the company's situation is important rather than applying the advanced company's CRM system model. In other words, the CRM solution which can maximize their own strength by developing the CRM program that makes the most of features and characteristics of the company should be adopted.
The mountain village development program in Korea started in the mountain villages, the 45.9% of total land and one of the typical marginal region, from 1995 to achieve the equilibrium development of national land and the sustainable mountain development in Chapter 13 in Agenda 21, and it has been accelerated to increase the happiness and the quality of life of mountain community residents through the expansion by province and the improvement of related laws and regulations. This study has been aimed to analyze the response of main stakeholder's -mountain village residents and local government officials - on mountain villages development, and to provide the future plan as community development. The survey and interview data were collected from the mountain villages which already developed 59 villages and developing 15 villages in 2003. The mountain village development program has achieved the positive aspects as community development plan in the several fields, - the voluntary participation of residents, the establishment of self-support spirit as the democratic civilians, the development of base of income increasement, the creation of comfortable living environment, the equilibrium development with the other regions. Especially the mountain residents and local government officials both highly satisfy with the development of base of income increasement and the creation of comfortable living environment which are the main concerns to both stakeholder. However through the mountain development program, it is not satisfied to increase the maintenance of local community and the strengthening of traditional value of mountain villages. Also to improve the sustainable income improvement effects, it is necessary to develop the income items and technical extension which good for the each region. In the decentralization era, it is necessary for local government should have the more active and multilateral activities for these. With this, the introduction of methods which the mountain community people and the local government officials could co-participate in the mountain villages' development from the initial stages and the renovation of related local government organizations and the cooperatives will be much helpful to the substantiality of mountain development program. Also it is essential for the assistance of central government to establish the complex plan and the mountain villages network for all mountain area and the exchange of information, the education and training of mountain villages leader who are the core factor for the developed mountain villages maintenance, the composition of national mountain villages representatives. In case the development proposals which based on the interests of the main stakeholder's on mountain community could be positively accepted, then the possibility of the mountain village development as one of community development will be successfully improved in future.
Destruction of oral soft and hard tissues and resulting problems seriously affect the life quality of xerostomic patients. Although artificial saliva is the only regimen for xerostomic patients with totally abolished salivary glands, currently available artificial salivas give restricted satisfaction to patients. The purpose of this study was to contribute to the development of ideal artificial saliva through comparing viscosity and wettability between CMC solutions and human saliva. Commercially-available CMC is dissolved in simulated salivary buffer (SSB) and distilled deionized water (DDW). Various properties of human whole saliva, human glandular saliva, and a CMC-based saliva substitutes known as Salivart and Moi-Stir were compared with those of CMC solutions. Viscosity was measured with a cone-and-plate digital viscometer at six different shear rates, while wettability on acrylic resin and Co-Cr alloy was determined by the contact angle. The obtained results were as follows: 1. The viscosity of CMC solutions was proportional to CMC concentration, with 0.5% CMC solution displaying similar viscosity to stimulated whole saliva. Where as a decrease in contact angle was found with increasing CMC concentration. 2. The viscosity of human saliva was found to be inversely proportional to shear rate, a non-Newtonian (pseudoplastic) trait of biological fluids. The mean viscosity values at various shear rates increased as follows: stimulated parotid saliva, stimulated whole saliva, unstimulated whole saliva, stimulated submandibular-sublingual saliva. 3. Contact angles of human saliva on the tested solid phases were inversely correlated with viscosity, namely decreasing in the order stimulated parotid saliva, stimulated whole saliva, unstimulated whole saliva, stimulated submandibular-sublingual saliva. 4. Boiled CMC dissolved in SSB (CMC-SSB) had a lower viscosity than CMC-SSB (P < 0.01 at shear rate of $90s^{-1}$). 5. For human saliva, contact angles on acrylic resin were significantly lower than those on Co-Cr alloy (P < 0.01). 6. Comparing CMC solutions with human saliva, the contact angles between acrylic resin and human saliva solutions were significantly lower than those between acrylic resin and CMC solutions, including Salivart and Moi-Stir (P <0.01). The effectiveness of CMC solutions in terms of their rheological properties was objectively confirmed, indicating a vital role for CMC in the development of effective salivary substitutes.
The 9.11 demonstrated that terrorist attack could be more serious problem than the war in our modern life. No countries in the world have evaded being a target for terrorists today. As well as South Korea, the whole world must share attentions and responsibilities for fighting against the terrorism. Since the international terrorist groups have expanded their targets from Western countries to Koreans, civilian hostages are no longer other's affair; it became a serious threat to public. Increased Korean investment, trade, missionary, and travel overseas also expanded activity regions worldwide. It also result increased terrorist threats and possible abduction. The number of kidnapping crisis has increased since the terrorists use it as an effective method of sending a message. Piracy refers to a broad range of violent acts at sea, and has traditionally been regarded as common enemies. Piracy constitutes a great threat to the security of navigation as well as to the safety of vessels and crews. Lessons from hostage issues such as Korean hostage crisis in Somalia and Afghanistan show that it can cause criticism on moral issues if armed rescue missions fail or hostages are killed, so the governments and related corporations try to solve it by paying ransom. Terrorists and use these advantages in order to put a huge pressure on the governments. In this study we will look at essential characteristics and types of hostage abductions and recognition of national safety, lessons and solutions to previous Korean hostage cases in overseas. At the same time, it provides a guidelines of the direction in the fighting against terrorist groups and Piracy.
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