A Case of Urologic Manifestation of IARS2-associated Leigh Syndrome (IARS2 유전자 연관 리 증후군(Leigh syndrome) 여아에서 방광기능장애 증례)
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- Journal of The Korean Society of Inherited Metabolic disease
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- v.23 no.1
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- pp.25-30
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- 2023
Leigh syndrome is a rare progressive neurodegenerative mitochondrial disorder with clinical and genetic heterogeneity. Recently, balletic IARS2 variants have been identified in a number of patients presenting broad clinical phenotypes from Leigh and West syndrome to a rare syndrome CAGSSS characterized by cataracts, growth hormone deficiency, sensory neuropathy, sensorineural hearing loss, and skeletal dysplasia syndrome (OMIM#616007). We describe a child with Korean Leigh syndrome with urologic manifestations resulting from a compound heterozygote mutation in IARS2. A 5-year-old girl visited the emergency room with a complaint of abdominal pain accompanied by abdominal distension. Abdominal-pelvic CT showed a markedly distended urinary bladder without definite obstructive lesions. She was diagnosed with neurogenic bladder dysfunction based on a urodynamic study. She had global delayed development due to neurologic regression after 6 months of age and a history of bilateral cataract surgery at the age of 2 years. Her brain magnetic resonance imaging showed symmetrically increased signal intensities in the bilateral putamen and caudate nuclei with diffuse cerebral atrophy. No gene variants were identified through whole-mitochondrial genome analysis. Whole exome sequencing was performed for diagnosis, and compound heterozygous pathogenic variants were identified in IARS2: c.2446C>T (p. Arg816Ter) and c.2450G>A (p. Arg817His). To the best of our knowledge, this is the first case report of bladder dysfunction manifestation in a patient with IARS2-related Leigh syndrome. Thus, it broadens the clinical and genetic spectrum of IARS2-associated diseases.
Korea has developed as an influential country over Asia and all over the world based on remarkable economic development. And the background of this development was possible due to the existence of those who sacrificed precious lives and contributed to the nation's existence in the past crisis. Every year, Korea holds an annual commemorative event with people of national merit, Korean War veterans, and their families, expressing gratitude for sacrifices and contributions at home and abroad, and providing economic support. The tragedy of the Korean War and the pro-democracy movement in Korea over the past half century will one day become a history of the distant past over time. As generations change and the purpose and method of exchange by region change, the tragic situation that occurred earlier and the way people sacrificed for the country are expected to be different from before. In particular, it is true that the number of Korean War veterans and their families is gradually decreasing as they are now old. In addition, due to the outbreak of global infectious diseases such as COVID-19, it is difficult to plan and conduct face to face events as well as before. Currently, Korea's digital technology is introducing various methods. 5G communication networks, smart-phones, tablet PCs, and smart devices that can experience virtual reality are already used in our real lives. Business meetings are held in a metaverse environment, and concerts by famous singers are held in an online environment. Artificial intelligence technology has also been introduced in the field of human resource recruitment and customer response services, improving the work efficiency of companies. And it seems that this technology can be used in the field of veterans. In particular, there is a metaverse technology that can vividly show the situation during the Korean War, and a way to digitalize the voices and facial expressions of currently surviving veterans to convey their memories and lessons to future generations in the long run. If this digital technology method is realized on an online platform to hold a veterans' celebration event, veterans and their families on the other side of the world will be able to participate in the event more conveniently.
The Korean NPL market was formed by the government and foreign capital shortly after the 1997 IMF crisis. However, this market is short-lived, as the bad debt has started to increase after the global financial crisis in 2009 due to the real economic recession. NPL has become a major investment in the market in recent years when the domestic capital market's investment capital began to enter the NPL market in earnest. Although the domestic NPL market has received considerable attention due to the overheating of the NPL market in recent years, research on the NPL market has been abrupt since the history of capital market investment in the domestic NPL market is short. In addition, decision-making through more scientific and systematic analysis is required due to the decline in profitability and the price fluctuation due to the fluctuation of the real estate business. In this study, we propose a prediction model that can determine the achievement of the benchmark yield by using the NPL market related data in accordance with the market demand. In order to build the model, we used Korean NPL data from December 2013 to December 2017 for about 4 years. The total number of things data was 2291. As independent variables, only the variables related to the dependent variable were selected for the 11 variables that indicate the characteristics of the real estate. In order to select the variables, one to one t-test and logistic regression stepwise and decision tree were performed. Seven independent variables (purchase year, SPC (Special Purpose Company), municipality, appraisal value, purchase cost, OPB (Outstanding Principle Balance), HP (Holding Period)). The dependent variable is a bivariate variable that indicates whether the benchmark rate is reached. This is because the accuracy of the model predicting the binomial variables is higher than the model predicting the continuous variables, and the accuracy of these models is directly related to the effectiveness of the model. In addition, in the case of a special purpose company, whether or not to purchase the property is the main concern. Therefore, whether or not to achieve a certain level of return is enough to make a decision. For the dependent variable, we constructed and compared the predictive model by calculating the dependent variable by adjusting the numerical value to ascertain whether 12%, which is the standard rate of return used in the industry, is a meaningful reference value. As a result, it was found that the hit ratio average of the predictive model constructed using the dependent variable calculated by the 12% standard rate of return was the best at 64.60%. In order to propose an optimal prediction model based on the determined dependent variables and 7 independent variables, we construct a prediction model by applying the five methodologies of discriminant analysis, logistic regression analysis, decision tree, artificial neural network, and genetic algorithm linear model we tried to compare them. To do this, 10 sets of training data and testing data were extracted using 10 fold validation method. After building the model using this data, the hit ratio of each set was averaged and the performance was compared. As a result, the hit ratio average of prediction models constructed by using discriminant analysis, logistic regression model, decision tree, artificial neural network, and genetic algorithm linear model were 64.40%, 65.12%, 63.54%, 67.40%, and 60.51%, respectively. It was confirmed that the model using the artificial neural network is the best. Through this study, it is proved that it is effective to utilize 7 independent variables and artificial neural network prediction model in the future NPL market. The proposed model predicts that the 12% return of new things will be achieved beforehand, which will help the special purpose companies make investment decisions. Furthermore, we anticipate that the NPL market will be liquidated as the transaction proceeds at an appropriate price.
Marketing financial services used to be easier. Today, the competition in financial services is fierce. Not only has the competition become more intense, financial services have also changed structurally. In an environment with various customer needs and severe competitions, the marketing in financial services industry is getting more difficult and more important than before. However, there are still not enough studies on financial services marketing in Korea whereas lots of research papers have been published frequently in some international journals. The purpose of this paper is (1)to review the literature on financial services marketing, (2)to investigate current marketing activities based on in-depth interview with financial marketing managers in Korea, and (3)to suggest some implications for future research on the financial services marketing. Financial products are not consumer products. In fact, they are not products at all in the way product marketing is usually described. Nor are they altogether like services. The financial industry operates in a unique way, and its marketing tasks are correspondingly complex. However, the literature review shows that there has been a lack of basic studies which dealt with inherent characteristics of financial services marketing compared to the research on marketing in other industries. Many studies in domestic marketing journals have so far focused only on the general customer behaviors and the special issues in some financial industries. However, for more effective financial services marketing, we have to answer following questions. Is there any difference between financial service marketing and consumer packaged goods marketing? What are the differences between the financial services marketing and other services marketing such as education and health services? Are there different ways of marketing among banks, securities firms, insurance firms, and credit card companies? In other words, we need more detailed research as well as basic studies about the financial services marketing. For example, we need concrete definitions of financial services marketing, bank marketing, securities firm marketing, and etc. It is also required to compare the characteristics of each marketing within the financial services industry. The products sold in each market have different characteristics such as duration and degree of risk-taking. It means that there are sub-categories in financial services marketing. We have to consider them in the future research on the financial services marketing. It is also necessary to study customer decision making process in the financial markets. There have been little research on how customers search and process information, compare alternatives, make final decision, and repeat their choices. Because financial services have some unique characteristics, we need different understandings in the customer behaviors compared to the behaviors in other service markets. And also considering the rapid growth in financial markets and upcoming severe competition between domestic and global financial companies, it is time to start more systematic and detailed research on financial services marketing in Korea. In the second part of this paper, I analyzed the results of in-depth interview with 20 marketing managers of financial services companies in Korea. As a result, I found that the role of marketing departments in Korean financial companies are mainly focused on the short-term activities such as sales support, promotion, and CRM data analysis although the size and history of marketing departments to some extent show a sign of maturity. Most companies established official marketing departments before 2001. Average number of employees in a marketing department is about 58. However, marketing managers in eight companies(40% of the sample) still think that the purpose of marketing is only to support and manage general sales activities. It shows that some companies have sales-oriented concept rather than marketing-oriented concept. I also found three key words which marketing managers think importantly in financial services markets. They are (1)Trust in customer relationship, (2)Brand differentiation, and (3)Rapid response to customer needs. 50% of the sample support that "Trust" is the most important key word in the financial services marketing. It is interesting that 80% of banks and securities companies think that "Trust" is the most important thing, whereas managers in credit card companies consider "Rapid response to customer needs" as the most important key word in their market. In addition, there are different problems recognition of marketing managers depending on the types of financial industries they belong to. For example, in the case of banks and insurance companies, marketing managers consider "a lack of communication with other departments" as the most serious problem. On the other hand, in the case of securities firms, "a lack of utilization of customer data" is the most serious problem. These results imply that there are different important factors for the customer satisfaction depending on the types of financial industries, and managers have to consider them when marketing financial products in more effective ways. For example, It will be necessary for marketing managers to study different important factors which affect customer satisfaction, repeat purchase, degree of risk-taking, and possibility of cross-selling according to the types of financial industries. I also suggested six hypothetical propositions for the future research.
The purpose of this research is to construct research framework for international franchising based on existing literature and to identify research components in the framework. Franchise can be defined as management styles that allow franchisee use various management assets of franchisor in order to make or sell product or service. It can be divided into product distribution franchise that is designed to sell products and business format franchise that is designed for running it as business whatever its form is. International franchising can be defined as a way of internationalization of franchisor to foreign country by providing its business format or package to franchisee of host country. International franchising is growing fast for last four decades but academic research on this is quite limited. Especially in Korea, research about international franchising is carried out on by case study format with single case or empirical study format with survey based on domestic franchise theory. Therefore, this paper tries to review existing literature on international franchising research, providing research framework, and then stimulating new research on this field. International franchising research components include motives and environmental factors for decision of expanding to international franchising, entrance modes and development plan for international franchising, contracts and management strategy of international franchising, and various performance measures from different perspectives. First, motives of international franchising are fee collection from franchisee. Also it provides easier way to expanding to foreign country. The other motives including increase total sales volume, occupying better strategic position, getting quality resources, and improving efficiency. Environmental factors that facilitating international franchising encompasses economic condition, trend, and legal or political factors in host and/or home countries. In addition, control power and risk management capability of franchisor plays critical role in successful franchising contract. Final decision to enter foreign country via franchising is determined by numerous factors like history, size, growth, competitiveness, management system, bonding capability, industry characteristics of franchisor. After deciding to enter into foreign country, franchisor needs to set entrance modes of international franchising. Within contractual mode, there are master franchising and area developing franchising, licensing, direct franchising, and joint venture. Theories about entrance mode selection contain concepts of efficiency, knowledge-based approach, competence-based approach, agent theory, and governance cost. The next step after entrance decision is operation strategy. Operation strategy starts with selecting a target city and a target country for franchising. In order to finding, screening targets, franchisor needs to collect information about candidates. Critical information includes brand patent, commercial laws, regulations, market conditions, country risk, and industry analysis. After selecting a target city in target country, franchisor needs to select franchisee, in other word, partner. The first important criteria for selecting partners are financial credibility and capability, possession of real estate. And cultural similarity and knowledge about franchisor and/or home country are also recognized as critical criteria. The most important element in operating strategy is legal document between franchisor and franchisee with home and host countries. Terms and conditions in legal documents give objective information about characteristics of franchising agreement for academic research. Legal documents have definitions of terminology, territory and exclusivity, agreement of term, initial fee, continuing fees, clearing currency, and rights about sub-franchising. Also, legal documents could have terms about softer elements like training program and operation manual. And harder elements like law competent court and terms of expiration. Next element in operating strategy is about product and service. Especially for business format franchising, product/service deliverable, benefit communicators, system identifiers (architectural features), and format facilitators are listed for product/service strategic elements. Another important decision on product/service is standardization vs. customization. The rationale behind standardization is cost reduction, efficiency, consistency, image congruence, brand awareness, and competitiveness on price. Also standardization enables large scale R&D and innovative change in management style. Another element in operating strategy is control management. The simple way to control franchise contract is relying on legal terms, contractual control system. There are other control systems, administrative control system and ethical control system. Contractual control system is a coercive source of power, but franchisor usually doesn't want to use legal power since it doesn't help to build up positive relationship. Instead, self-regulation is widely used. Administrative control system uses control mechanism from ordinary work relationship. Its main component is supporting activities to franchisee and communication method. For example, franchisor provides advertising, training, manual, and delivery, then franchisee follows franchisor's direction. Another component is building franchisor's brand power. The last research element is performance factor of international franchising. Performance elements can be divided into franchisor's performance and franchisee's performance. The conceptual performance measures of franchisor are simple but not easy to obtain objectively. They are profit, sale, cost, experience, and brand power. The performance measures of franchisee are mostly about benefits of host country. They contain small business development, promotion of employment, introduction of new business model, and level up technology status. There are indirect benefits, like increase of tax, refinement of corporate citizenship, regional economic clustering, and improvement of international balance. In addition to those, host country gets socio-cultural change other than economic effects. It includes demographic change, social trend, customer value change, social communication, and social globalization. Sometimes it is called as westernization or McDonaldization of society. In addition, the paper reviews on theories that have been frequently applied to international franchising research, such as agent theory, resource-based view, transaction cost theory, organizational learning theory, and international expansion theories. Resource based theory is used in strategic decision based on resources, like decision about entrance and cooperation depending on resources of franchisee and franchisor. Transaction cost theory can be applied in determination of mutual trust or satisfaction of franchising players. Agent theory tries to explain strategic decision for reducing problem caused by utilizing agent, for example research on control system in franchising agreements. Organizational Learning theory is relatively new in franchising research. It assumes organization tries to maximize performance and learning of organization. In addition, Internalization theory advocates strategic decision of direct investment for removing inefficiency of market transaction and is applied in research on terms of contract. And oligopolistic competition theory is used to explain various entry modes for international expansion. Competency theory support strategic decision of utilizing key competitive advantage. Furthermore, research methodologies including qualitative and quantitative methodologies are suggested for more rigorous international franchising research. Quantitative research needs more real data other than survey data which is usually respondent's judgment. In order to verify theory more rigorously, research based on real data is essential. However, real quantitative data is quite hard to get. The qualitative research other than single case study is also highly recommended. Since international franchising has limited number of applications, scientific research based on grounded theory and ethnography study can be used. Scientific case study is differentiated with single case study on its data collection method and analysis method. The key concept is triangulation in measurement, logical coding and comparison. Finally, it provides overall research direction for international franchising after summarizing research trend in Korea. International franchising research in Korea has two different types, one is for studying Korean franchisor going overseas and the other is for Korean franchisee of foreign franchisor. Among research on Korean franchisor, two common patterns are observed. First of all, they usually deal with success story of one franchisor. The other common pattern is that they focus on same industry and country. Therefore, international franchise research needs to extend their focus to broader subjects with scientific research methodology as well as development of new theory.