Park, Seong-Won;Son, Yeong-Bae;Kim, Se-Hwa;Jo, Seong-Yun;Ji, Seon-Tae;Jin, Dong-Gyu
Journal of The Korean Society of Inherited Metabolic disease
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v.10
no.1
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pp.59-66
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2010
Enzyme replacement of therapy (ERT) is one of the most promising therapeutic strategies for the treatment of lysosomal storage disorders. ERT is available in three types of Mucopolysaccharidosis (MPS): for MPS I (Aludrazyme$^{(R)}$), MPS II (Elaprase$^{(R)}$) and MPS VI (Naglazyme$^{(R)}$) patients who are over 5 years old. But recently, early diagnosis can be done by expert clinicians and even in prenatal case. We describe the case of ERT under 5 years old MPS patients. Up to June, 2010 in Samsung Medical Center, there are 6patients who were diagnosed as MPS and started ERT under 5 years old. 3 patients were MPS I, 3 patients were MPS II. 2 patient who was diagnosed as MPS I was female and others were male. Their age at diagnosis were 4 to 37month-old (4, 13, 16, 25, 27, 37 month-old) and they are now 9 to 60 month-old (9, 39, 32, 81, 60 month-old). The youngest patient was started ERT at 4 month-old and others were started at their 13 to 49 month-old (13, 29, 27, 28, 49 month-old). First manifested symptoms of patients were macrocephaly, kyphosis and coarse face appearance. Especially, in 2 of them, one was MPS I and the other was MPS II had elder brother with same disease. And the youngest one was diagnosed by the iduronate-2-sulfatase (IDS) gene analysis from chorionic villi sampling. His mother knew that she was a heterozygous carrier of IDS gene mutation because her younger brother died from MPS II. All of them confirmed as MPS by the enzyme assay in leukocytes and fibroblast skin culture. We started ERT with ${\alpha}$-L-iduronidase(Aldurazyme$^{(R)}$) to MPS I and did recombinant human iduronate-2-sulfatase (Elaprase$^{(R)}$) to MPS II patients as recommended dose as over 5 years old. But for MPS II patient who was 4 month old, we started ERT by recombinant human IDS (Elaprase$^{(R)}$) with reduced dose 0.1 mg/kg and increased dose every 2 weeks by 0.1mg/kg up to 0.5mg/kg IV infusion. During ERT, all patients had no adverse effects and the excretion of GAGs were decreased. We have evaluated other clinical symptoms such as liver/ spleen volume, heart function and neurologic evaluation. We describe a successful ERT to MPS I and MPS II patient under 5 years old without any adverse event. It indicates that ERT in young children are well tolerated and that it has several effects which may confer clinical benefits with long-term therapy.
Meningococcal infections can be associated with abnormalities of the complement system, which contains 5 terminal complement proteins. Furthermore, deficiencies in 1 of these 5, complement component 7 (C7), leads to the loss of complement lytic function, and affected patients show increased susceptibility to recurrent meningococcal meningitis and systemic Neisseria gonorrhoeae infection. In September 2003, an 11-year-old female patient presented at our outpatient department with high fever, lower leg pain, headache, and petechiaes. She rapidly progressed to coma but later achieved full recovery due to prompt treatment. Her final diagnosis was meningococcal sepsis and arthritis. Her elder brother also had a similar bacterial meningoencephalitis history, which encouraged us to perform analyses for complement component and gene mutations. Resultantly, both the brother and sister were found to have the same mutation in the C7 gene. Subsequently, vaccinations of the meningococcal vaccine meningococcal vaccine ($Menomune^{(R)}$) were administered. However, in September 2006, the brother expired due to acute micrococcus meningoencephalitis. At present, the 16-year-old female patient is healthy. Here, we report a Korean family with a hereditary C7 deficiency with susceptibility to meningococcal infections due to C7 gene mutation.
Kim, Mijin;Han, Jinsook;Kwon, Myoungjin;Kim, Younghee
Journal of Digital Convergence
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v.11
no.2
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pp.341-346
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2013
Objectives: This study was designed to provide basic data that would be helpful in planning mental health programs designed to help elderly people have a satisfying life as an elder by identifying the factors that affect cognition of urban and rural elderly people. Methods: Subjects included 160 elderly people on Daejeon and Geumsam. Structured questionnaires were used for data collection from march 2, 2011 to July 30, and descriptive statistics, t-test, ANOVA, Pearson correlation coefficients, and hierarchical regression analysis of SPSS 18.0 program were performed for data analysis. Results: A significant difference in cognition was observed between two groups(t=-2.232, p=.029). Cognition & depression didn't have correlation(urban area(r=.021, p=.860), rural area(r=.-131, p=.271)). Significant factors influencing cognition included education(t=4.069, p<.001) and age(t=-2.812, p=.001) in urban area and sex(t=-3.011, p<.001), age(t=-4.866, p<.001), education(t=3.525, p<.001) in rural area. These factors explained 26.1% and 57.2% of the variance. Conclusion: Findings of this study suggest the need for development of appropriate nursing strategies depending on the difference of the environment to increase cognitive function and to decrease the incidence of depression in elderly people.
Kim, Byeong-Cheol;Lee, Won-Young;Eom, Su-Hong;Jang, Mun-Seok;Kim, Pyeong-Su;Lee, Eung-Hyuk
Journal of rehabilitation welfare engineering & assistive technology
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v.10
no.4
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pp.321-328
/
2016
In this paper, we propose a walk status method which can be fused with conventional walk intention method to improve convenience of an electric assistive walking system for elder people with restricted walking capabilities. The system uses a handlebar as a trigger and regards grabbing a handlebar as expressing will to walk. And the system uses a user's linear velocity and angular velocity as linear velocity and angular velocity of a system, checked by laser range finder. To achieve this, we propose a method to find a virtual central point of a human body by estimating a central point between two legs. The experiments are carried out by comparing user's linear velocity and angular velocity, and system's linear velocity and angular velocity. The results show that the error of linear velocity and angular velocity between a user and a system are 1% and 2.77%, which means the linear velocity and angular velocity of a user can be applied to a system. And it is confirmed that the proposed fusion method can prevent a user from being dragged by an assistive walking system or a malfunction caused by lack of experience
This study examines the impacts of outside directors' characteristics and compensation on stock performances of KOSDAQ and NASDAQ IPO firms. The results of this study indicated the following interesting results. First, there is no significant relation between outside directors' age and CARs on KOSDAQ firms. while significant positive relation between outside directors' age and CARs on NASDAQ firms. And the elder age group shows a more positive impact on performances compared with the younger age group. Second, there is no relation between outside directors' academic background and CARs for KOSDAQ firms. But We find a significantly positive one for NASDAQ firms. Third, In Relation to outside directors' careers, their professional or CEO careers group have more positive impact on stock performances than gray directors' careers group. Lastly, there is an insignificant negative relation between the outside directors' compensation and CARs for KOSDAQ firms. while there is a positive relative for NASDAQ firms. In particular, there is a significant positive relative between value of stock options and CARs for NASDAQ firms. from the result, I could find out the stock option for outside' directors have a positive influence on firm value.
Journal of the Korea Academia-Industrial cooperation Society
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v.12
no.10
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pp.4502-4512
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2011
This study was performed to determine the job satisfaction and its association with sociodemographic and health related characteristics, job-related factors, perception on socioeconomic status, professional job perception, job stress and social support among nursing officers. The survey was administered to 542 nursing officers working at military hospitals of the ROK Armed Forces, from Oct. 1 to Nov. 30. The structured self-administered questionnaires were delivered and they were collected without respondents' personal information. As a results, The job satisfaction of respondents turned out to be significantly higher in the following groups: an elder, married, subjective sleep quality is good, feels they are healthy, higher positional status, a longer job career, satisfied with their work, without consider quitting the job, their physical burden of work is adequate. The survey results showed that respondents' job satisfaction is positively correlated with the professional job perception and social support. while it is negatively correlated with job stress. When analyzed by Covariance Structure, it turned out that the professional job perception is more influential on job satisfaction than job stress and social support. The study found the professional job perception, high social support, and low job stress tends to improve job satisfaction. Meanwhile, the study also found that the professional job perception and high social support reduces job stress.
While the number of crashes and fatalities has decreased for the last few decades, the proportion of elderly-involved crashes has continuously increased from 19.5% in 2001 to 29.6% in 2008. This paper aims to evaluate the effectiveness of traffic safety education to seniors, which has been conducted for the purpose of providing more systematic knowledge such as safety regulations and making the elderly more aware of the risks of crashes, and eventually to reduce elderly-involved crashes. First, we investigated the levels of traffic safety knowledge of the elder people who attend senior community centers and social welfare centers in Seoul and provided traffic safety education. Then, the effectiveness of traffic safety education was evaluated by using the before-and-after analysis. We set up two different groups to improve the reliability of evaluation: one for control group and the other for comparison group. The results showed that the average scores of the control group after education were significantly improved at the 95% significance level compared to the average scores before education, whereas the comparison group did not show a significant differences in the average scores before and after education except for two questions. This indicates that the levels of traffic safety knowledge of the control group have been improved through education and therefore, the safety education seem to be effective to some extent.
Objectives : This research identified the attitude towards senior citizens targeting the workers of the facility for senior citizens to establish Long-Term Care Insurance System early on, and to identify the degree of perception on the importance of senior citizens' oral health and on the need for dental sanitation specialists. Methods : This research targeted 233 workers of the facility for the senior citizens located in some parts of the Gyeongnam area. Survey was conducted from December 1, 2009 to January 31, 2010 to identify the facility workers' attitude towards senior citizens and on the perception towards Long-Term Care Insurance System. Results : 1. As for the awareness towards senior citizens, 50.2% said that they are comfortable around senior citizens while degree of kindness towards senior citizens was 48.5%. 21.0% had experienced training for senior citizens' oral health management. As for the question on who should serve as the trainer, dental sanitation specialists were citied the most with 47.6%. 29.2% was very interested in the senior citizens' oral health while 83.7% said that oral cavity managers are needed in the facility for the senior citizens. As for the question on who should be in charge, if necessary, dental sanitation specialist was cited the most with 57.9%. 2. Awareness on the Long-Term Care Insurance System, treatment for the oral cavity sanitation by visiting homes and information on the activity of long-term care specialists was high following the experience of training for the senior citizens' oral health management in case of the workers at the senior citizens' facility. This manifested statistically significant difference(p<0.05)(p<0.01). Moreover, treatment for oral cavity sanitation manifested statistically significant difference was manifested in the treatment for oral cavity sanitation following the need for oral health managers and who should be in charge, if necessary(p<0.01). Conclusions : These results advocate the need to develop diverse senior citizens' oral health management methods and to continue to carry out actions for senior citizens' oral health improvement that can be carried out by the long-term caretakers for the management of senior citizens' oral cavity health.
The definition of digital poverty has not been changed since it was used at the first digital divide survey in 2000s. The objective of this study is to reexamine and redefine digital poverty targets of digital divide survey. This study examines the present state for the target of the digital divide survey focusing on Disabilities, Low-Income and Elders and then redefines digital poverty for them. The results suggest to reexamine 15 types of disabilities by the revised the Disabled Welfare Law as digital divide targets, rather than limiting to physical, brain, hearing and visual disabilities. For the low-income target, people on the second-to-the bottom income bracket as well as those on basic welfare should be included as the target of low-income people for digital poverty. The age of the elder target for digital poverty should be adjusted to sixty-five or more in the era of aging population.
The purpose of this study was to identify the effects influencing path behaviors of pre-service childcare teachers to present the educational and social welfare measures for promoting children's character development and preventing the alienation of the elderly. The subjects for this study are 176 students who completed the childcare teacher course in four universities located in G city and J province. The scale of this study utilized modified, supplemented scale used in previous studies[1][2] that was constituted for a newly transformed model based on planned behavior theories. The contents of research design include salience beliefs such as attitude beliefs, normative beliefs, control beliefs, etc. as well as a model of planned behavior theories such as attitudes, subjective norms, perceived behavior control, behavior intension, behavior, etc. This study conducted frequency analysis and regression analysis for the data collected by using the SPSS 18.0 for Windows. From the results of verification, all hypothesis was supported. Thus, usefulness of this theory was reconfirmed, and the results of this study are to be utilized as basic materials for professionals who make every endeavor in a perspective of children's character development and in a perspective of reducing alienation phenomenon of the elderly.
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