• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.1
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• pp.29-36
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• 2014

Mucopolysaccharidoses (MPSs) are a group of rare inherited metabolic diseases caused by deficiency of lysosomal enzymes. MPSs are clinically heterogeneous and characterized by progressive deterioration in visceral, skeletal and neurological functions. The aim of this article is to review the treatment of MPSs, the unmet needs of current treatments and vision for the future including recent clinical trials. Until recently, supportive care was the only option available for the management of MPSs. Hematopoietic stem cell transplantation (HSCT), another potentially curative treatment, is not routinely advocated in clinical practice due to its high risk profile and lack of evidence for efficacy. From the early 2000s, enzyme replacement therapy (ERT) was approved and available for the treatment of MPS I, II and VI. ERT is effective for the treatment of many somatic symptoms, particularly walking ability and respiratory function, and remains the mainstay of MPS treatment. However, no benefit was found in the neurological symptoms because the enzymes do not readily cross the blood-brain barrier (BBB). In recent years, intrathecal (IT) ERT, substrate reduction therapy (SRT) and gene therapy have been rapidly gaining greater recognition as potential therapeutic avenues. Although still under investigation, IT ERT, SRT and gene therapy are promising MPS treatments that may prevent the neurodegeneration not improved by ERT.

• Journal of Preventive Medicine and Public Health
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• v.43 no.5
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• pp.445-450
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• 2010

Objectives: Emotional support and a stress management program should be simultaneously provided to clients as effective preventive services for healthy behavioral change. This study was conducted to review various relaxation and meditation intervention methods and their applicability for a preventive service program. Methods and results: The author of this paper tried to find various relaxation and meditation programs through a literature review and program searching and to introduce them. The 'Relaxation Response' and 'Mindfulness Based Stress Reduction (MBSR)' are the most the widely used meditative programs in mainstream medical systems. Abdominal breathing, Progressive Musclular Relaxation (PMR), Relaxative Imagery, Autogenic Training (AT) and Biofeedback are other well-known techniques for relaxation and stress management. I have developed and implemented some programs using these methods. Relaxation and meditation classes for cancer patients and a meditation based stress coping workshop are examples of this program. Conclusions: Relaxation and meditation seem to be good and effective methods for primary, secondary and tertiary preventive service programs. Program development and standardization and further study are needed for more and wider use of the mindbody approach in the preventive service area of medicine.

• Biomolecules & Therapeutics
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• v.22 no.3
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• pp.232-238
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• 2014

Alzheimer's disease (AD) is the most common neurodegenerative disease without known ways to cure. A key neuropathologic manifestation of the disease is extracellular deposition of beta-amyloid peptide (Ab). Specific mechanisms underlying the development of the disease have not yet been fully understood. In this study, we investigated effects of 4-O-methylhonokiol on memory dysfunction in APP/PS1 double transgenic mice. 4-O-methylhonokiol (1 mg/kg for 3 month) significantly reduced deficit in learning and memory of the transgenic mice, as determined by the Morris water maze test and step-through passive avoidance test. Our biochemical analysis suggested that 4-O-methylhonokiol ameliorated $A{\beta}$ accumulation in the cortex and hippocampus via reduction in beta-site APP-cleaving enzyme 1 expression. In addition, 4-O-methylhonokiol attenuated lipid peroxidation and elevated glutathione peroxidase activity in the double transgenic mice brains. Thus, suppressive effects of 4-O-methylhonokiol on $A{\beta}$ generation and oxidative stress in the brains of transgenic mice may be responsible for the enhancement in cognitive function. These results suggest that the natural compound has potential to intervene memory deficit and progressive neurodegeneration in AD patients.

• Journal of the Korean Society of Manufacturing Technology Engineers
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• v.23 no.5
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• pp.413-420
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• 2014

Although the transfer forming process has various advantages, it is also inefficient for the use of multiple press machines, especially for small part sizes. In this study, a new forming process was proposed to use multiple tandem dies in a single press machine. A rotary transfer forming device was developed to combine the advantages of the progressive and transfer forming process. In this study, a detailed forming process using this device was analyzed, and the device was designed to perform four series of tandem forming processes in a single press. In order to analyze the feasibility of this forming process, simple forming dies were made. As a result, the position accuracy was 4 arcsec, and the forming speed reached up to 20 strokes per minute. It is thought that this rotary transfer forming device can help to save initial setup costs through the more efficient use of space in a press machine.

• Journal of Korean Foot and Ankle Society
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• v.5 no.1
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• pp.23-27
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• 2001

It has been known that the ankle arthrodesis is a common surgical procedure for treating the ankle arthrosis and deformity that do not respond to the non-operative treatment. To date, various surgical techniques for the ankle arthrodesis have been reported. Clinical and biomechanical trials have shown that the rigid internal fixation leads the increased rate of the union. The ankle arthrodesis may be complicated with the nonunion, delayed union, malunion, and infection. In cases of the Charcot joint of the ankle in diabetic patients, however, arthrodesis could reduce the disadvantage of the nonoperative treatment, such as the loss of the reduction, progressive collapse, multiple ulcerations and infection. The object of this case report is to report our experience of a successful ankle arthrodesis using an anterior T plate in treating the unstable ankle of a diabetic patient, associated with the ankle fracture and the neuropathy. The surgical approach of this technique is simple so that it would allow less soft tissue injury, and this procedure would be regarded as one alternative to provide the rigid internal fixation in the ankle arthrodesis.

• Journal of the Korea Fashion and Costume Design Association
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• v.16 no.2
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• pp.211-225
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• 2014

Globalization in the 21st century and development in the field of IT have caused rapid change in the practical site of fashion industry. Therefore, as colleges aim to produce professional technicians and experts in the applicable fields, they are strengthening professionalism and practicality more than ever before that can respond to the practical situation. In addition, owing to the reduction of population of academic students, colleges would search for specialty and structural innovation with the serious desire to establish new structural system to produce talented persons. Purpose of this study is to strengthen competition of colleges voluntarily, specialize themselves and maximize mutual benefit through cooperative relation with industrial businesses. Thus, this study would compare, analyze and investigate the curriculums of fashion designs in colleges for the plan of curriculum applicable for the industrial changes and corresponding business demand. Considering the result of questionnaires of industrial businesses, most of them would prefer the curriculum for 3 years to that for 2 years. Nowadays industrial businesses will require computerization ability much more than ever before besides the professional knowledge and technology in the field of fashion and it is also showed that education of personality in colleges would be considered to be important for the performance of the vocational duty. Especially, specialty and progressive participation in the curriculum would be strengthened during the education process of colleges. This study will be used for the establishment of specialty of colleges and development of curriculum of them.

• The Journal of Korean Institute of Communications and Information Sciences
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• v.17 no.9
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• pp.982-992
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• 1992

We developed the interface system between D-SCAN TV system and the Teletext system to reduce the effect of interline flick. At first, horizontal and vertical signal is separated from the composite signal. The stable vertical sync and horizontal sync is applied to the conventional Teletext system and the its output has R.G.B signal form and the output of the Teletext system. This signal is converted into composite signal and is switched at the video selector system in TV to process the signal by progressive method.

• Journal of the Korean Institute of Educational Facilities
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• v.23 no.4
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• pp.21-30
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• 2016

This study considers the concept of schools and their spatial composition by John Dewey through literature review who argued for a new, children-centered education in the early 19th century, and it also mentions some suggestions for improvement of the elementary school facilities in Korea that the size and number of students in school has decreased sharply. John Dewey claimed that a school is a big household as well as a small society in which one studies the current occupation and experience in connection between the household-school-society. At the same time, he pursued an organic and open spatial composition which can effectively and efficiently enable educational curriculum's application, social occupation and project learning based on children's native impulse and stage in child growth. Korea's education policy should shift from quantity-based education production to quality-based production due to the reduction of school age population among other reasons. Especially this study emphasizes that small size schools need to be changed to local community facilities with environment-friendly, children-centered and open learning spatial composition with basis on John Dewey's educational philosophy.

• Journal of the Korean Institute of Illuminating and Electrical Installation Engineers
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• v.29 no.11
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• pp.53-58
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• 2015

Systemic heating and cooling air conditioning systems are popular in various industrial fields and even home. Recently, the rate of supply of this kind of multi-heat pump has been increased under ESCO financing supporting system. Generally the heat pumping system has a structural simplicity and easy installation benefits. and has good running efficiency under normal designed condition. But under extreme climate condition (over $+30^{\circ}C$, under $-10^{\circ}C$), this system exposes abnormal power consumption. It causes high progressive electric power rates and resultant peak power capacity of power plant. In this paper, a novel system concept of buffering refrigerant accumulator and constant pressure control system to relieve peak power load is proposed and this system's utility is verified with an prototype experimental system.

• Journal of mucopolysaccharidosis and rare diseases
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• v.3 no.2
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• pp.37-40
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• 2017

Mucopolysaccharidosis (MPS) is caused by accumulation of the glycosaminoglycans in all tissues due to decreased activity of the lysosomal enzyme. Patients exhibit multisystemic signs and symptoms in a chronic and progressive manner, especially with changes in the skeleton, cardiopulmonary system, central nervous system, cornea, skin, liver, and spleen. In the past, treatment of MPS was limited to enzyme replacement therapy (ERT). The outcome for affected patients improved with the introduction of new technologies as hematopoietic stem cell transplantation, relegated to specific situations after ERT became available. Intrathecal ERT may be considered in situations of high neurosurgical risk but still it is experimental in humans. New insights on the pathophysiology of MPS disorders are leading to alternative therapeutic approaches, as gene therapy, inflammatory response modulators and substrate reduction therapy. In this paper, we will highlight the recent novel treatment and clinical trials for MPS and discuss with the goal of fostering an understanding of this field.