• Title/Summary/Keyword: Survival Analysis

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The Study on the Effects of Technology Orientation and Market Orientation on Managerial Performance in Innopolis Start-ups: Focusing on the Moderating Effects of Marketing and R&D Expenses (연구소기업의 기술지향성과 시장지향성이 경영성과에 미치는영향: 마케팅 및 연구개발 비용의 조절효과를 중심으로)

  • Kwon, Haram;Yang, Young Seok
    • Asia-Pacific Journal of Business Venturing and Entrepreneurship
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    • v.19 no.1
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    • pp.119-133
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    • 2024
  • As a result of significant investments by the government in promoting public technology commercialization and fostering a venture startup ecosystem, there have been quantitative achievements, such as the registration of over 1,600 Innopolis Start-ups since 2006, generating a total revenue of 1.1 trillion won as of 2021. However, these achievements have been overshadowed by critical qualitative challenges, including a continuous decline in average revenue per Innopolis Start-up. This led to a focus on whether managers' technological and market orientations affect business performance. This study aims to provide insights into improving the qualitative growth of Innopolis Start-ups by analyzing the effects of technological and market orientations on business performance, as well as the moderating effects of adjusting marketing and research and development (R&D) costs on this relationship. Through prior research and empirical analysis, this study derives three main findings. First, technological excellence and innovation significantly influence the business performance of Innopolis Start-ups, while technological intensity does not. Second, customer orientation and competitive orientation significantly impact business performance, whereas entry barriers as a single factor do not. Third, adjusting marketing and R&D costs, as controlled variables obtained through general situations, has no direct impact on other variables. However, it interacts with entry barriers, influencing financial business performance, with R&D costs exhibiting a negative buffering effect and marketing costs showing a positive enhancing effect. This study confirms that both technological and market orientations directly influence the business performance of Innopolis Start-ups, thus being crucial factors affecting their growth. Moreover, it establishes that investments in marketing and R&D play significant roles in alleviating initial entry barriers and enhancing financial performance. Consequently, it underscores the importance of reinforcing technological and market orientations tailored to the characteristics of Innopolis Start-ups. Additionally, it proposes five theoretical contributions: strengthening institutional support systems for technology commercialization and innovation, improving qualitative evaluation criteria during the selection process of Innopolis Start-ups, conducting comprehensive analyses of technological and market aspects during startup selection, enhancing support for marketing education and consulting for smooth market entry, and supporting expenditure strategies and milestone setting tailored to the industrial characteristics of individual Innopolis Start-ups.

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A Study on Market Expansion Strategy via Two-Stage Customer Pre-segmentation Based on Customer Innovativeness and Value Orientation (고객혁신성과 가치지향성 기반의 2단계 사전 고객세분화를 통한 시장 확산 전략)

  • Heo, Tae-Young;Yoo, Young-Sang;Kim, Young-Myoung
    • Journal of Korea Technology Innovation Society
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    • v.10 no.1
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    • pp.73-97
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    • 2007
  • R&D into future technologies should be conducted in conjunction with technological innovation strategies that are linked to corporate survival within a framework of information and knowledge-based competitiveness. As such, future technology strategies should be ensured through open R&D organizations. The development of future technologies should not be conducted simply on the basis of future forecasts, but should take into account customer needs in advance and reflect them in the development of the future technologies or services. This research aims to select as segmentation variables the customers' attitude towards accepting future telecommunication technologies and their value orientation in their everyday life, as these factors wilt have the greatest effect on the demand for future telecommunication services and thus segment the future telecom service market. Likewise, such research seeks to segment the market from the stage of technology R&D activities and employ the results to formulate technology development strategies. Based on the customer attitude towards accepting new technologies, two groups were induced, and a hierarchical customer segmentation model was provided to conduct secondary segmentation of the two groups on the basis of their respective customer value orientation. A survey was conducted in June 2006 on 800 consumers aged 15 to 69, residing in Seoul and five other major South Korean cities, through one-on-one interviews. The samples were divided into two sub-groups according to their level of acceptance of new technology; a sub-group demonstrating a high level of technology acceptance (39.4%) and another sub-group with a comparatively lower level of technology acceptance (60.6%). These two sub-groups were further divided each into 5 smaller sub-groups (10 total smaller sub-groups) through two rounds of segmentation. The ten sub-groups were then analyzed in their detailed characteristics, including general demographic characteristics, usage patterns in existing telecom services such as mobile service, broadband internet and wireless internet and the status of ownership of a computing or information device and the desire or intention to purchase one. Through these steps, we were able to statistically prove that each of these 10 sub-groups responded to telecom services as independent markets. We found that each segmented group responds as an independent individual market. Through correspondence analysis, the target segmentation groups were positioned in such a way as to facilitate the entry of future telecommunication services into the market, as well as their diffusion and transferability.

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Primary Orbital Lymphoma : A Retrospective Analysis of Results of Radiation Therapy (원발성 안와 림프종의 방사선치료 성적에 관한 후향적 분석)

  • Kim Sussan;Ahn Seung Do;Chang Hyesook;Kim Kyoung Ju;Lee Sang-wook;Choi Eun Kyung;Kim Jong Hoon;Huh Jooryung;Suh Cheol Won;Kim Sung Bae
    • Radiation Oncology Journal
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    • v.20 no.2
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    • pp.139-146
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    • 2002
  • Purpose : This study evaluated the treatment outcomes, patterns of failure, and treatment related complications of primary lymphoma patients who received definitive radiation therapy. Materials and Methods : A retrospective analysis was undertaken for 31 patients with primary orbital lymphoma at the Asan Medical Center between February 1991 and April 2001. There were 18 males and 13 females with ages ranging from 3 to 73 years (median, 44 years). The involved sites were 9 conjunctivae, 12 eyelids and 10 other orbits. The histological types were 28 MALT lymphomas (low-grade B-cell lymphoma of mucosa-associated lymphoid tissue type), 1 diffuse large B-cell lymphoma, 1 anaplastic large cell lymphoma and 1 lymphoblastic lymphoma. The Ann Arbor stages were all IE $(100\%)$. Ann Arbor stage III or IV patients were excluded from this study, Bilateral orbital involvement occurred in 6 cases. Radiation therapy was given with one anterior port of high energy electrons $(6\~16\;MeV)$ for the lesions located at the anterior structures like the conjunctivae or eyelids. Lesions with a posterior extension or other orbital lesions were treated with 4 or 6 MeV photons with appropriately arranged portals. In particular, lens blocks composed of lead alloy were used in conjunctival or eyelid lesions. Twelve patients received chemotherapy. The median follow-up period was 53 months. Results : The 5-year overall, cause-specific, and disease-free survival was $91\%,\;96\%,\;and\;80\%$, respectively. The complete response rate 6 months after radiation therapy was $100\%$. Local recurrences were observed in 2 patients at 16 and 18 months after completion of radiation treatment. They were salvaged with additional radiation therapy. Two patients developed distant metastases. A MALT lymphoma patient with a lung relapse was successfully salvaged with radiotherapy, but the other lymphoblastic lymphoma patient with bone marrow relapse expired. There were no severe complications but 5 patients developed radiation-induced cataracts and 2 patients developed dry eye. Conclusion : Most primary orbital lymphomas consisted of MALT lymphomas. Radiation therapy was a successful treatment modality for orbital lymphoma without any severe complications. In cases of local relapses, radiation therapy is also a very successful salvage treatment modality.

A New Exploratory Research on Franchisor's Provision of Exclusive Territories (가맹본부의 배타적 영업지역보호에 대한 탐색적 연구)

  • Lim, Young-Kyun;Lee, Su-Dong;Kim, Ju-Young
    • Journal of Distribution Research
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    • v.17 no.1
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    • pp.37-63
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    • 2012
  • In franchise business, exclusive sales territory (sometimes EST in table) protection is a very important issue from an economic, social and political point of view. It affects the growth and survival of both franchisor and franchisee and often raises issues of social and political conflicts. When franchisee is not familiar with related laws and regulations, franchisor has high chance to utilize it. Exclusive sales territory protection by the manufacturer and distributors (wholesalers or retailers) means sales area restriction by which only certain distributors have right to sell products or services. The distributor, who has been granted exclusive sales territories, can protect its own territory, whereas he may be prohibited from entering in other regions. Even though exclusive sales territory is a quite critical problem in franchise business, there is not much rigorous research about the reason, results, evaluation, and future direction based on empirical data. This paper tries to address this problem not only from logical and nomological validity, but from empirical validation. While we purse an empirical analysis, we take into account the difficulties of real data collection and statistical analysis techniques. We use a set of disclosure document data collected by Korea Fair Trade Commission, instead of conventional survey method which is usually criticized for its measurement error. Existing theories about exclusive sales territory can be summarized into two groups as shown in the table below. The first one is about the effectiveness of exclusive sales territory from both franchisor and franchisee point of view. In fact, output of exclusive sales territory can be positive for franchisors but negative for franchisees. Also, it can be positive in terms of sales but negative in terms of profit. Therefore, variables and viewpoints should be set properly. The other one is about the motive or reason why exclusive sales territory is protected. The reasons can be classified into four groups - industry characteristics, franchise systems characteristics, capability to maintain exclusive sales territory, and strategic decision. Within four groups of reasons, there are more specific variables and theories as below. Based on these theories, we develop nine hypotheses which are briefly shown in the last table below with the results. In order to validate the hypothesis, data is collected from government (FTC) homepage which is open source. The sample consists of 1,896 franchisors and it contains about three year operation data, from 2006 to 2008. Within the samples, 627 have exclusive sales territory protection policy and the one with exclusive sales territory policy is not evenly distributed over 19 representative industries. Additional data are also collected from another government agency homepage, like Statistics Korea. Also, we combine data from various secondary sources to create meaningful variables as shown in the table below. All variables are dichotomized by mean or median split if they are not inherently dichotomized by its definition, since each hypothesis is composed by multiple variables and there is no solid statistical technique to incorporate all these conditions to test the hypotheses. This paper uses a simple chi-square test because hypotheses and theories are built upon quite specific conditions such as industry type, economic condition, company history and various strategic purposes. It is almost impossible to find all those samples to satisfy them and it can't be manipulated in experimental settings. However, more advanced statistical techniques are very good on clean data without exogenous variables, but not good with real complex data. The chi-square test is applied in a way that samples are grouped into four with two criteria, whether they use exclusive sales territory protection or not, and whether they satisfy conditions of each hypothesis. So the proportion of sample franchisors which satisfy conditions and protect exclusive sales territory, does significantly exceed the proportion of samples that satisfy condition and do not protect. In fact, chi-square test is equivalent with the Poisson regression which allows more flexible application. As results, only three hypotheses are accepted. When attitude toward the risk is high so loyalty fee is determined according to sales performance, EST protection makes poor results as expected. And when franchisor protects EST in order to recruit franchisee easily, EST protection makes better results. Also, when EST protection is to improve the efficiency of franchise system as a whole, it shows better performances. High efficiency is achieved as EST prohibits the free riding of franchisee who exploits other's marketing efforts, and it encourages proper investments and distributes franchisee into multiple regions evenly. Other hypotheses are not supported in the results of significance testing. Exclusive sales territory should be protected from proper motives and administered for mutual benefits. Legal restrictions driven by the government agency like FTC could be misused and cause mis-understandings. So there need more careful monitoring on real practices and more rigorous studies by both academicians and practitioners.

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A Study on BRCA1/2 Mutations, Hormone Status and HER-2 Status in Korean Women with Early-onset Breast Cancer (젊은 한국인 유방암 환자에서 BRCA1/2 돌연변이와 호르몬 수용체, HER-2 상태에 관한 연구)

  • Choi, Doo-Ho;Jin, So-Young;Lee, Dong-Wha;Kim, Eun-Seog;Kim, Yong-Ho
    • Radiation Oncology Journal
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    • v.26 no.1
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    • pp.65-73
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    • 2008
  • Purpose: Women with breast cancer diagnosed at an age of 40 years or younger have a greater prevalence of germline BRCA1 and BRCA2 mutations than the prevalence of women with breast cancer diagnosed at older ages. Several immunohistochemical characteristics have been identified in breast cancers from studies of Caucasian women with BRCA1/2 mutations having familial or early-onset breast cancers. The aim of this study is to determine whether early-onset breast cancer in BRCA1 or BRCA2 mutation carriers, who were not selected from a family history, could be distinguished by the use of immunohistochemical methods and could be distinguished from breast cancer in women of a similar age without a germline BRCA1 or BRCA2 mutation. We also analyzed the prognostic difference between BRCA1/2 related and BRCA1/2 non-related patients by the use of univariate and multivariate analysis. Materials and Methods: Breast cancer tissue specimens from Korean women with early-onset breast cancers were studied using a tumor tissue microarray. Immunohistochemical staining of estrogen receptor(ER), progesterone receptor(PR) and HER-2, as well as the histology and grade of these specimens, were compared. The prognostic impact of immunohistochemical and histological factors as well as the BRCA1/2 mutation status was investigated separately. Results: There were 14 cases and 16 deleterious BRCA1/2 mutations among 101 patients tested. A family history(4/14) and bilateral breast cancers(3/9) were high risk factors for BRCA1/2 mutations. BRCA1/2-associated cancers demonstrated more expression of ER-negative(19.4% versus 5.1%, p=0.038) and HER-2 negative than BRCA1/2 negative tumors, especially for tumors with BRCA1 tumors The BRCA1/2 mutation rate for patients with triple negative tumors(negative expression of ER, PR and HER-2) was 24.2%. Tumor size, nodal status, and HER-2 expression status were significantly associated with disease free survival, as determined by univariate and multivariate analysis, but the BRCA1/2 status was not a prognostic factor. Conclusion: Breast cancer that occurs in women with a germline BRCA1 or BRCA2 mutations have recognizable immunohistochemical features, which may be useful in identifying individuals that are more likely to carry germline mutations. Although the BRCA1/2 mutation status was not a prognostic factor in Korean women with early-onset breast cancer, more cases with a longer follow-up period are needed for further study.

Correlation Between the Parameters of Radiosensitivity in Human Cancer Cell Lines (인체 암세포주에서 방사선감수성의 지표간의 상호관계)

  • Park, Woo-Yoon;Kim, Won-Dong;Min, Kyung-Soo
    • Radiation Oncology Journal
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    • v.16 no.2
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    • pp.99-106
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    • 1998
  • Purpose : We conducted clonogenic assay using human cancer cell lines (MKN-45, PC-14, Y-79, HeLa) to investigate a correlation between the parameters of radiosensitivity. Materials and Methods : Human cancer cell lines were irradiated with single doses of 1, 2, 3, 5, 7 and 10Gy for the study of radiosensitivity and subrethal damage repair capacity was assessed with two fractions of 5Gy separated with a time interval of 0, 1, 2, 3, 4, 6 and 24 hours. Surviving fraction was assessed with clonogenic assay using $Sperman-H\"{a}rbor$ method and mathematical analysis of survival curves was done with linear-quadratic (LQ) , multitarget-single hit(MS) model and mean inactivation dose$(\v{D})$. Results : Surviving fractions at 2Gy(SF2) were variable among the cell lines, ranged from 0.174 to 0.85 The SF2 of Y-79 was lowest and that of PC-14 was highest(p<0.05, t-test). LQ model analysis showed that the values of $\alpha$ for Y-79, MKN-45, HeLa and PC-14 were 0.603, 0.356, 0.275 and 0.102 respectively, and those of $\beta$ were 0.005, 0.016, 0.025 and 0.027 respectively. Fitting to MS model showed that the values of Do for Y-79. MKN-45, HeLa and PC-14 were 1.59. 1.84. 1.88 and 2.52 respectively, and those of n were 0.97, 1.46, 1.52 and 1 69 respectively. The $\v{D}s$ calculated by Gauss-Laguerre method were 1.62, 2.37, 2,01 and 3.95 respectively So the SF2 was significantly correlated with $\alpha$, Do and $\v{D}$. Their Pearson correlation coefficiencics were -0.953 and 0,993. 0.999 respectively(p<0.05). Sublethal damage repair was saturated around 4 hours and recovery ratios (RR) at plateau phase ranged from 2 to 3.79. But RR was not correlated with SF2, ${\alpha}$, ${\beta}$, Do, $\v{D}$. Conclusion : The intrinsic radiosensitivity was very different among the tested human cell lines. Y-79 was the most sensitive and PC-l4 was the least sensitive. SF2 was well correlated with ${\alpha}$, Do, and $\v{D}$. RR was high for MKN-45 and HeLa but had nothing to do with radiosensitivity parameters. These basic parameters can be used as baseline data for various in vitro radiobiological experiments.

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Factors Related with the Compliance and Treatment in Patients with Pulmonary Tuberculosis in Urban and Suburb Area (도시와 농촌지역의 폐결핵 환자 순응도 및 치료에 관련된 요인)

  • Kim, Sang-Soon;Kim, Yoon-Ock
    • Research in Community and Public Health Nursing
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    • v.7 no.1
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    • pp.69-79
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    • 1996
  • To investigate the factors related with the compliance and the treatment of the patients with pulmonary tuberculosis in urban and suburb area, we followed up all the 755 registered patients(at urban Public Health Office 544, at suburb 210) as follow from January 1,1992 to December 31, 1993. We describe the general characteristics and the characteristics related with the disease of the patients according to the area as follow. 150 patients(27.5%) were at their age of 20 to 29 years in the urban area, whereas 45 patients (21.4%) were 60 to 69 years and another 45 patients(21.4%) were 70 to 79 years in the suburb area. According to the first chest X -ray examination, 54.5% of all cases were proved to be mild in the urban area. But in the suburb area, moderate cases (44.3%) were more than mild cases(p<0.01). Follow-up X-ray's were performed more properly(p<0.05) in the urban area(94.3%) than in the suburb area(90.0%). Most cases were found in the chest X -ray examination performed by Public Health Office (p<0.01) : payable chest X-ray in the urban area (56.7%) and free chest X-ray in the suburb area(35.2%). More patients were cured in the urban area(90.8%) than in the suburb area(87.1%). The presence of supporting family member were significantly higher(p<0.05) in the urban area(79.1%) than in the suburb area(88.1%). In the analysis of the treatment efficacy, more cure ate were found in the patients cytologically confirmed to be culture (+). In the urban area, 201 culture (+) patients (93.5%) 294 culture (-) patients (89.1%) were cured. In the suburb area, 99 culture (+) patients(91.7%) and 84 culture (-) patients(82.4%) were cured. Age, the presence of supporting family member, and the socioeconomic status of the patient had significant association with the prescription compliance related with the general characteristics of the patients. Whereas, X-ray finding and AFB culture finding were the significant factors associated with the prescription compliance related with pulmonary tuberculosis (p<0.05). The cumulative compliance in the survival analysis was 92.5% in the urban area and 88.1% in suburb area, at sixth month of follow-up. Failure rate for regular drug receipt was highest at second month in the urban area(3.75%) and at fourth month in the suburb area(4.15%). In logistic regression of the factors related with the tratment result, first X-ray examination and prescription compliance were significantly associated in the urban area(p<0.05). However, there is no factor significantly associated with the treatment result in the suburb area. It could be explained by too small size of the sample. In logistic regression of the factors related with the prescription compliance, first chest X-ray, sputum culture outcome and the presence of supporting family member were significant variables in the urban area(p<0.05). Most patients with family member were proved to be compliant with the prescription. This shows that it is important for the patients with long-lasting ilnesses to have supporting family member. Therefore, to improve prescription compliance we should strengthen the health education before the initiation of treatment and take special interest in the patients without supporting family member.

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The Characteristics and Performances of Manufacturing SMEs that Utilize Public Information Support Infrastructure (공공 정보지원 인프라 활용한 제조 중소기업의 특징과 성과에 관한 연구)

  • Kim, Keun-Hwan;Kwon, Taehoon;Jun, Seung-pyo
    • Journal of Intelligence and Information Systems
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    • v.25 no.4
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    • pp.1-33
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    • 2019
  • The small and medium sized enterprises (hereinafter SMEs) are already at a competitive disadvantaged when compared to large companies with more abundant resources. Manufacturing SMEs not only need a lot of information needed for new product development for sustainable growth and survival, but also seek networking to overcome the limitations of resources, but they are faced with limitations due to their size limitations. In a new era in which connectivity increases the complexity and uncertainty of the business environment, SMEs are increasingly urged to find information and solve networking problems. In order to solve these problems, the government funded research institutes plays an important role and duty to solve the information asymmetry problem of SMEs. The purpose of this study is to identify the differentiating characteristics of SMEs that utilize the public information support infrastructure provided by SMEs to enhance the innovation capacity of SMEs, and how they contribute to corporate performance. We argue that we need an infrastructure for providing information support to SMEs as part of this effort to strengthen of the role of government funded institutions; in this study, we specifically identify the target of such a policy and furthermore empirically demonstrate the effects of such policy-based efforts. Our goal is to help establish the strategies for building the information supporting infrastructure. To achieve this purpose, we first classified the characteristics of SMEs that have been found to utilize the information supporting infrastructure provided by government funded institutions. This allows us to verify whether selection bias appears in the analyzed group, which helps us clarify the interpretative limits of our study results. Next, we performed mediator and moderator effect analysis for multiple variables to analyze the process through which the use of information supporting infrastructure led to an improvement in external networking capabilities and resulted in enhancing product competitiveness. This analysis helps identify the key factors we should focus on when offering indirect support to SMEs through the information supporting infrastructure, which in turn helps us more efficiently manage research related to SME supporting policies implemented by government funded institutions. The results of this study showed the following. First, SMEs that used the information supporting infrastructure were found to have a significant difference in size in comparison to domestic R&D SMEs, but on the other hand, there was no significant difference in the cluster analysis that considered various variables. Based on these findings, we confirmed that SMEs that use the information supporting infrastructure are superior in size, and had a relatively higher distribution of companies that transact to a greater degree with large companies, when compared to the SMEs composing the general group of SMEs. Also, we found that companies that already receive support from the information infrastructure have a high concentration of companies that need collaboration with government funded institution. Secondly, among the SMEs that use the information supporting infrastructure, we found that increasing external networking capabilities contributed to enhancing product competitiveness, and while this was no the effect of direct assistance, we also found that indirect contributions were made by increasing the open marketing capabilities: in other words, this was the result of an indirect-only mediator effect. Also, the number of times the company received additional support in this process through mentoring related to information utilization was found to have a mediated moderator effect on improving external networking capabilities and in turn strengthening product competitiveness. The results of this study provide several insights that will help establish policies. KISTI's information support infrastructure may lead to the conclusion that marketing is already well underway, but it intentionally supports groups that enable to achieve good performance. As a result, the government should provide clear priorities whether to support the companies in the underdevelopment or to aid better performance. Through our research, we have identified how public information infrastructure contributes to product competitiveness. Here, we can draw some policy implications. First, the public information support infrastructure should have the capability to enhance the ability to interact with or to find the expert that provides required information. Second, if the utilization of public information support (online) infrastructure is effective, it is not necessary to continuously provide informational mentoring, which is a parallel offline support. Rather, offline support such as mentoring should be used as an appropriate device for abnormal symptom monitoring. Third, it is required that SMEs should improve their ability to utilize, because the effect of enhancing networking capacity through public information support infrastructure and enhancing product competitiveness through such infrastructure appears in most types of companies rather than in specific SMEs.

Clinical Characteristics of Arthrogryposis, Renal Tubular Dysfunction, Cholestasis(ARC) Syndrome in Korea (ARC(Arthrogryposis, Renal Tubular Dysfunction, Cholestasis) 증후군의 발병양상에 관한 연구)

  • Lee Soon Min;Kim Ji Hong;Lee Jae Seung;Han Suk Joo
    • Childhood Kidney Diseases
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    • v.9 no.2
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    • pp.222-230
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    • 2005
  • Purpose : ARC syndrome refers to an association of arthrogryposis, renal tubular dysfunction, and cholestasis. The VPS33B gene was recently identified as the causative gene. So far, 41 cases of ARC syndrome have been reported worldwide, and it has rarely been reported in Korea. This study was conducted to report the clinical findings of seven ARC syndrome cases in Korean children, focusing especially on renal tubular dysfunction. Methods : The hospital records of 7 cases diagnosed as ARC syndrome at Severance Hospital between Mar. 1995 and Aug. 2005 were reviewed and analyzed. Results : Of the 7 cases, 4 were boys and 3 were girls. Six patients(85$\%$) were born with normal birth weight at term, and one was born at preterm. All cases presented with cholestasis and severe jaundice. According to the type of arthrogryposis described by Brown et al, type 3 and 4 were found in 2 patients and type,6, 7, and the undistributed type in one patient respectively. Other associated clinical findings were as follows : failure to thrive in 6(85$\%$), lax skin in 5(71$\%$), and gray platelet syndrome in 4(57$\%$). Urine analysis revealed 6 cases(85$\%$) with proteinuria, 3(43$\%$) with hematuria, 5(71$\%$) with glycosuria, 2(29$\%$) with phosphaturia and 2(29$\%$) with calciuria. Serum electrolytes showed 4 cases(57$\%$) with hyponatremia, 3(43$\%$) with hypokalemia, and 1(14$\%$) with creatinine elevation. Renal tubular dysfunctions were diagnosed as renal tubular acidosis in 6 cases(85$\%$), nephrogenic diabetes insipidus in 2(29$\%$), and Fanconi syndrome in 2(29$\%$). During the follow-up period, 2(29$\%$) had no treatment, 5(85$\%$) had continuous supplementation to correct the electrolyte imbalance and acidosis, and 1(14$\%$) had dialysis. Only one patient had a family history of ARE syndrome in a sibling. Four cases(57$\%$) were diagnosed at the mean age of 8.2 months, and one case was lost during follow-up. Ages of the survived 2 cases were 13 and 25 months, respectively. Conclusion : The rare disease of ARC syndrome Is associated with severe renal dysfunction. However, this study revealed that the renal manifestation of ARC syndrome in Korean children is relatively mild and survival rate is higher than that of previous studies. Contrary to previous reports, this study showed that familial cases are rare and sporadic occurence is possible in Korea. Thus, the diagnosis of this syndrome requires a careful evaluation of the venal function in cases of congenital arthrogryposis, and a mandatory genetic counseling of affected family for prevention of familial occurance. (J Korean Soc Pediatr Nephrol 2005;9:222-230)

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Analysis of Childhood Rapidly Progressive Glomerulonephritis (소아 급속 진행성 사구체신염의 임상적 고찰)

  • Uhm Ji Hyun;Kim Mi Jin;Lee Young-Mock;Kim Ji Hong;Lee Jae Seung;Kim Pyung-Kil;Hong Soon Won;Jeung Hyeun Joo
    • Childhood Kidney Diseases
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    • v.5 no.2
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    • pp.78-86
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    • 2001
  • Purpose: Rapidly progressive glomerulonephritis (RPGN) is characterized by the rapid increase in serum creatitnin and crescents formation involving more than $50\%$ of glomeruli. 10 patients who had been treated for RPGN were studied retrospectively for thier underlying diseases and clinical features Method: Cilinical review was performed on 10 children who were diagnosed with RPGN by clinical features and renal biopsy and followed up at department of pediatrics during tile last 10 years, from May 1990 to May 2000. Result: There were 6 males and 4 females between the ages of 2.1 and 14.3 years (mean $10.9{\pm}3.8$). 3 had Henoch-$Sch{\ddot{o}}nlein$ purpura nephritis; 2, idiopathic rapidly progressive glomerulonephritis; 2, lupus nephritis; 1, hemolytic uremic syndrome; 1, membranous glomerulonephritis and 1, microscopic polyangiitis. The most common chief complaints were gross hematuria and oliguria. Initial clinical features included proteinuria, edema, hypertension, nausea and arthralgia. Mean serum BUN was $74.2{\pm}39.1\;mg/dL$ mean serum creatinin, $3.2{\pm}1.8\;mg/dL$ and mean creatinin clearance, $26.5{\pm}13.2\;mL/min/1.73m^2$. Antineutrophil cytoplasmic antibody was positive only in microscopic polyangiitis. ANA and Anti-DNA antibody were positive in two lupus nephritis patients. Serum complements were decreased in 4 patients. All patients except Hemolytic uremic syndrome received steroid pulse therapy and immunosupressive agents. 3 patients were performed acute peritoneal dialysis and 2 patients were given plasmapheresis. At the last follow up, 1 patient was dead, 4 patients had elevated serum creatinin, 2 of these 4 patients were on chronic ambulatory peritoneal dialysis and 6 patients had normal renal function. Conclusion: Rapidly progressive glomerulonephritis is a medical emergency that requires very rapid diagnosis, classification, and therapy. Appropriate therapy selected on the basis of underlying disease mechanism can substantially improve renal survival. (J. Korean Soc Pediatr Nephrol 2001 ; 5 : 78-86)

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