• Journal of The Korean Society of Inherited Metabolic disease
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• 제16권1호
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• pp.34-41
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• 2016

Adult-onset type II citrullinemia (CTLN2) is characterized by episodes of neurologic symptoms associated with hyperammonemia leading to disorientation, irritability, seizures, and coma. CTLN2 is distinct from classical citrullinemia, which is caused by a mutation of the argininosuccinic acid synthetase (ASS) gene. The serum citrulline level is elevated, while the activity of ASS in liver tissue is decreased. CTLN2 is known to have a poor prognosis if the proper treatment is not taken. We reported a female aged 37 years who developed recurrent attacks of altered consciousness, aberrant behavior, and vomiting. We initially suspected the patient had CTLN2 because of the signs of hyperammonemic encephalopathy, such as altered mentality, memory disturbance, and aberrant behaviors provoked by exercise-induced stress and excessive intravenous amino acid administration. Through her peculiar diet preferences and laboratory findings that included hyperammonemia and citrullinemia, we diagnosed the patient as CTLN2, and SLC25A13 sequencing revealed known compound heterozygous mutations (IVS11+1G>A, c.674C> A). Her parents were heterozygous carriers, and we identified that her older sister had the same mutations. The older sister had not experienced any episodes of hyperammonemia, but she had peculiar diet preferences. The patient and her sister have been well with conservative management. When considering the clinical course of CTLN2, it was meaningful that the older sister could be diagnosed early in an asymptomatic period and that preemptive treatment was employed. Through this case, CTLN2 should be considered in adults who present symptoms of hyperammonemic encephalopathy without a definite etiology. Because of its rare incidence and similar clinical features, CTLN2 is frequently misdiagnosed as hepatic encephalopathy, and it shows a poor prognosis due to the lack of early diagnosis and proper treatment. A high-carbohydrate diet, which is usually used to treat other urea cycle defects, can also exaggerate the clinical course of CTLN2, so proper metabolic screening tests and genetic studies should be performed.

• Korean Journal of Social Welfare
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• 제59권2호
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• pp.5-38
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• 2007

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.

• Sleep Medicine and Psychophysiology
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• 제12권2호
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• pp.122-132
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• 2005

Objectives: The objective of this study is to assess cognitive functions and their relationship with sleep symptoms in young narcoleptic patients. Methods: Eighteen young narcolepsy patients and 18 normal controls (age: 17-35 years old) were recruited. All narcolepsy patients had HLA $DQB_1$ *0602 allele and cataplexy. Several important areas of cognition were assessed by a battery of neuropsychological tests consisting of 13 tests: executive functions (e.g. cognitive set shifting, inhibition, and selective attention) through Wisconsin card sorting test, Trail Making A/B, Stroop test, Ruff test, Digit Symbol, Controlled Oral Word Association and Boston Naming Test; alertness and sustained attention through paced auditory serial addition test; verbal/nonverbal short-term memory and working memory through Digit Span and Spatial Span; visuospatial memory through Rey-Osterrieth complex figure test; verbal learning and memory through California verbal learning test; and fine motor activity through grooved pegboard test. Sleep symptoms in narcolepsy patients were assessed with Epworth sleepiness scale, Ullanlinna narcolepsy scale, multiple sleep latency test, and nocturnal polysomnography. Relationship between cognitive functions and sleep symptoms in narcolepsy patients was also explored. Results: Compared with normal controls, narcolepsy patients showed poor performance in paced auditory serial addition (2.0 s and 2.4 s), digit symbol tests, and spatial span (forward)(t=3.86, p<0.01; t=-2.47, p=0.02; t=-3.95, p<0.01; t=-2.22, p=0.03, respectively). There were no significant between-group differences in other neuropsychological tests. In addition, results of neuropsychological test in narcolepsy patients were not correlated with Epworth sleepiness scale score, Ullanlinna narcolepsy scale score and sleep variables in multiple sleep latency test or nocturnal polysomnography. Conclusion: The current findings suggest that young narcolepsy patients have impaired attention. In addition, impairment of attention in narcolepsy might not be solely due to sleep symptoms such as excessive daytime sleepiness.

• The Journal of Korea Institute of Information, Electronics, and Communication Technology
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• 제12권6호
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• pp.657-665
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• 2019

In this study, we conducted a study on the transcranial magnetic electrode, a method for the study of dementia and muscle pain, a neurodegenerative disease caused by an aging society, which is becoming a problem worldwide. In particular, transcranial magnetic electrodes have been studied to improve their ability to be deteriorated by dementia symptoms such as speech, cognitive ability, and memory by outputting magnetism deep into the brain using coils on the head epidermis. In this study, simulation was performed using Maxwell 3D program for the design of coil, the core of transcranial magnetic electrode. As a result of the simulation comparison between the coil designed by the previous research and the coil through the research and development, the output was found to be superior to the conventional designed coil. The graphs of the coil outputs of B-Field and H-Field are found to be symmetrical, but the symmetry between each coil is pseudo-symmetrical and not accurate. Based on these results, an experiment was conducted to confirm whether the output of the head epidermis through both coils is possible. In the magnitude field of the reverse-coil 2-coil analysis, the maximum output was 3.3920e + 004 H [A_per_meter], and the vector field showed the strongest magnetic field around 35 to 165 degrees. It was confirmed that the magnetic output canceled due to the magnetic output. In the case of the forward 2-coil, a maximum of 3.2348e + 004H [A_per_meter] similar to the reverse coil was observed, but in the case of the vector field, the magnetic output regarding the forward output and the head skin output was confirmed. However, when the height change in the output coil, the magnetic output was reduced.

• The Korean Journal of Nuclear Medicine
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• 제36권2호
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• pp.91-101
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• 2002

Purpose : The aim of this study was to identify the brain areas in which reductions of regional cerebral blood flow (rCBF) were correlated with decline of general mental function, measured by Mini-Mental State Examination (MMSE). Materials and Methods : Tc-99m HMPAO brain SPECT was peformed in 9 probable AD patients at the initial and follow-up periods of 1.8 years (average) after the first study. MMSE scores were also measured in both occasions. The mean MMSE score of the initial study 16.4 (range: 5 - 24) and the mean MMSE score of the follow-up was 8.1 (range: 0 - 17). Each SPECT image was normalized to the cerebellar activity and a correlation analysis was peformed between the level of rCBF in AD patients and the MMSE scores by voxel-based analysis using SPM99 software. Results : Significant correlation was found between the blood-flow decrease in left inferior prefrontal region (BA 47) and left middle temporal legion (BA 21) and the MMSE score changes. Additional areas such as anterior and posterior cingulate cortices, precuneus, and bilateral superior and middle prefrontal regions showed the similar trends. Conclusions : A relationship was found between reduction of regional cerebral blood flow in left prefrontal and temporal areas and decline of cognitive function in Alzheimer's disease(AD) patients. This voxel-based analysis is useful in evaluating the progress of cognitive function in Alzheimer's disease.

• Journal of the Korean Society of Food Science and Nutrition
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• 제34권4호
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• pp.466-475
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• 2005

Docosahexaenoic acid (22:6n-3, DHA) is highly enriched in membrane of brain and retina, and plays an important role in maintaining an optimal function of the central nervous system. We investigated the effect of n-3 fatty acid deficiency on rat brain, retina and liver fatty acyl composition at two different ages (3 wks and 15 wks) under DHA deficient condition. Rat pups born to dams fed a diet with $3.1\%$ of total fatty acids as $\alpha-linolenic$ acid (LNA) were fed using an artificial rearing system either an n-3 deficient (n-3 Def) or n-3 adequate (n-3 Adq) diet. Both diets contained $17.1\%$ linoleic acid (LA) but the n-3 Adq diet also contained $3.1\%$ LNA. Rats consuming the n-3 Def diet showed a lower brain $(50\%\;in\;13\;wks\;and\;70\%\;in\;15\;wks,\;p<0.05)$ and retinal $(50\%\;in\;13\;wks\;and\;63\%\;in\;15\;wks,\;p<0.05)$ DHA than those on the n-3 Adq diet, which was largely compensated for by an increase in docosapentaenoic acid (22:5n-6, DPAn-6). In the liver of the n-3 Def group, the percentage of DHA decreased by $97\%$ at 3 wks of age with an apparent increase in DPAn-6 relative to the n-3 Adq group (p<0.05), while there was a $65\%$ lower liver DHA in n-3 Def group at 15 wks of age than the n-3 Adq group (p<0.05). Liver arachidonic acid (20:4n-6, AA) was increased at 3 wks of age but decreased at 15 wks of age in the n-3 Def group compared with n-3 Adq group (p<0.05). In conclusion, the replacement of DHA by DPAn-6 in brain and retina fatty acid composition may be related to the suboptimal function in spatial learning, memory and visual acuity. This artificial rearing method presents a first generation model for n-3 deficiency that is similar to the case of human nutrition that commonly employed two generation model.

• Journal of the Korean Society of Food Science and Nutrition
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• 제34권4호
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• pp.544-548
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• 2005

The objective of this study was to determine the effect of die temperature and dimension on extraction pattern, extract yield, and crude saponin content of extruded white ginseng. The extrusion variables were die temperature $(110\;and\;120^{\circ}C)$ and die dimension (3 holes with 1.0 mm, 2 holes with 2.0 mm, and 1 hole with 3.0 mm diameter). The browness and redness were indicator of active components in ginseng extract. Both were used to evaluate the effect of die temperature and die dimension on release pattern and release rate constant. Browness and redness of extract achieved its lowest value at die temperature $110^{\circ}C$ and 2 holes with 2.0 mm diameter, indicating the lowest extraction rate constant. Extract yield highly increased by extrusion treatment. Extract yield and crude saponin content were the highest at die temperature $120^{\circ}C$ and 1 hole with 3.0 mm diameter. In conclusion, extrusion process has contributed significantly in improvement of release rate of its active components.

• The Journal of the Korea Contents Association
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• 제6권12호
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• pp.253-260
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• 2006

As media and product became variety, the propensity of the people is be coming various. From diversity, there we could search for some popularity is called 'the mania.' When Mania takes shape, the product will being longer even masses in these days only have short-term life. Also there are hundreds of animations that has short-term life whom people forgot everyday they watch. However, the animations could lasting its value which has the Mania. This thesis is a studies on the constitution of Mania from animation 'Fullmetal Alchemist's Character The BONES had made. We can learn that the audience were not just like the animation, but get crazy for it by comparing Japanese animation industry in those days; before it has been shrinking and manufacturing various contents from Fullmetal Alchemist means there is enough consumtions. There are many reasons to form Mania group, but specially the character symbols at the works as a whole. From this study is to know a cause of how the animation 'Fullmetal Alchemist' made huge Mania group, and significance value of the work those group left.

• Nuclear Medicine and Molecular Imaging
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• 제41권4호
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• pp.280-290
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• 2007

Purpose: Gender differences in personality are considered to have biological bases. In an attempt to understand the gender differences of personality on neurobiological bases, we conducted correlation analyses between regional brain glucose metabolism and temperament factors of personality in males and females. Materials and Methods: Thirty-six healthy right-handed volunteers (18 males, 33.8$\pm$17.6 y; 18 females, 36.2$\pm$20.4 y) underwent FDG PET at resting state. Three temperament factors of personality (novelty seeking (NS), harm avoidance (HA), reward dependence (RD)) were assessed using Cloninger's 240-item Temperament and Character Inventory (TCD within 10 days of FOG PET scan. Correlation between regional glucose metabolism and each temperament factor was tested using SPM2. Results: In males, a significant negative correlation between NS score and glucose metabolism was observed in the bilateral superior temporal gyri, the hippocampus and the insula, while it was found in the bilateral middle frontal gyri, the right superior temporal gyrus and the left cingulate cortex and the putamen in females. A positive HA correlation was found in the right midbrain and the left cingulate gyrus in males, but in the bilateral basal ganglia in females. A negative RD correlation was observed in the right middle frontal and the left middle temporal gyri in males, while the correlation was found in the bilateral middle frontal gyri and the right basal ganglia and the superior temporal gyrus in females. Conclusion: These data demonstrate different cortical and subcortical metabolic correlates of temperament factors of personality between males and females. These results may help understand biological substrate of gender differences in personality and susceptibility to neuropsychiatric illnesses.

• Proceedings of the Korean Journal of Food and Nutrition Conference
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• 한국식품영양학회 2000년도 정기총회 및 동계학술심포지움
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• pp.29-34
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• 2000

산업 발달에 따라 날로 많은 식품들이 새롭게 개발되어지고 있다. 또한 이와 병행해서 식품으로 인한 알레르기 발생 빈도도 날로 증가하고 있으며 그 증상 또한 점차 심화되고 있는 것이 세계적인 추세이다. 우리나라도 예외는 아니어서 일반 알레르기 환자뿐 아니라 식품으로 인한 알레르기 환자들이 점차 증가됨이 보고되어지고 있다. 농산물 시장의 수입개방이후 우리나라에는 많은 해외 농산물이 수입되어지고 있으며 그 중 작년 한해의 경우 총 수입 농산물의 10%를 넘는 유전자 재조합 농산물이 우리나라에 수입되어진 것으로 통계 보고되어졌다. 이러한 관점에서 알레르기 환자의 증가와 새로운 식품 (특히 유전자 재조합 식품)의 증가에는 서로 관련성이 있을 것으로 추측되어지고 있어 (새로운) 식품에 대한 알레르기성의 예측과 관리가 필요한 실정이다. 이에 몇몇 발표된 유전자 재조합 식품에 관련된 알레르기성 검사 논문들과 실험실에서 이루어진 연구 결과들을 중심으로 유전자 재조합 식품의 알레르기 위험성에 대해 알아보고자 한다. 일반적으로 식품의 단백질이 알레르겐(allergen)으로 작용하기 위해서는 먼저 소화효소에 의해 분해되어지고 장에서 흡수되어져서 immunopotent cell에 의해 process 되어 immune system에 present 되어져야 한다. 따라서 단백질로 인한 알레르기 반응은 그 단백질의 자연적 형태 뿐만이 아니라 소화 효소에 분해된 단편들의 구조 또는 다른 알레르겐 단백질과의 유사 구조로 인한 교차 반응에 의해 발생함을 기억해야 한다. 식품 단백질 중 어떤 단백질이 알레르겐으로 작용하는가에 대한 특이성 조사에 많은 관심이 집중되어지고 있지만 아직까지는 대략 다섯 개 정도의 일반적인 특성으로서 요약되어질 수 있다. 그러나 이러한 대략의 특성에 적용되지 않는 식품 알레르겐도 많음을 잊어서는 안 될 것이다. 알레르겐으로 작용하는 식품 단백질의 일반적 특성 1. 좋은 수용성 2. 식품내에 많은 부분을 차지하는 주 단백질이 주 알레르겐으로 작용 3. 단백질내에 하나 이상의 IgE-binding site 존재 4. 위장액에 대한 저항성 5. 10～70 kDa 크기 유전자 재조합 기술이란 말 그대로 유전자를 인위적으로 새롭게 조합하는 기술로 이전의 기술로는 불가능했던 유전적 변형을 농작물과 동물에 가능하게 했으며 이로 인해 유전적으로 변형된 식용 동식물의 개발이 가능하게 되었다. 새로운 유전인자를 개체에 삽입함으로 새로운 단백질이 발현 될수 있고 그로 인해 1) 해충과 질병에 대한 저항성 증가, 2) 화학 제초제에 대한 새로운 저항성 부여, 3) 식품의 저장성 향상, 4) 식품의 영향적 보충/향상 등의 이점을 얻을 수 있다 (표 1). 세계적으로 유전자 재조합 된 새로운 농산물의 재배는 날로 증가추세에 있으며 그 중에서 가장 많은 부분을 차지하는 농산물로 soybean을 들 수 있으며 (표 2) soybean을 중심으로 그 알레르기성의 변화가 연구 조사된 몇 가지 예를 살펴보고자 한다. (표 3)에 요약된 soybean중 첫 번째 경우는 재초제에 대한 저항성을 높여주기 위해 Agrobacterium에 존재하는 EPSPS라는 단백질을 콩에서 발현하도록 찬 유전자 재조합 된 콩의 경우이다. 이 콩의 경우에는 첫째. 이전된 새로운 단백질 EPSPS가 다른 여러 식물에 이미 존재하고 있는 단백질로서 우리가 이미 이러한 식품을 섭취할 때 이 단백질도 같이 섭취해오고 있었다는 점, 둘째. 이 단백질이 소화액 분해 실험에서 짧은 시간내에 분해가 되었다는 점, 셋째. 재조합 된 콩과 자연 콩이 성분 분석에서 차이를 나타내지 않았다는 점, 네 번째. 쥐를 통한 다양섭취 실험에서 아무런 이상 반응이 없었다는 점등의 결과를 기준으로 알레르기에 대한 개별 검사 없이 안전한 콩으로 결론짓고 있다. 영양성을 높이기 위해 Brazil nut에서 methionine 함량이 풍부한 2s albumine을 콩에서 발현하도록 한 두 번째 유전자 재조합 콩의 경우 이전된 단백질 때문에 Brazil nut에 알레르기 반응을 일으키는 알레르기 환자들을 조사한 결과 역시 재조합 된 콩에도 알레르기 반응을 일으켰다는 보고이다. Brazil nut에서 콩으로 이전된 단백질이 Brazil nut에서의 알레르기성을 그대로 유지한 점을 볼 때 새로운 단백질이 어디에서 유래하는가가 중요함을 잘 보여준 연구이다 세 번째 콩의 경우 역시 영양성을 높여주기 위해 corn에서 10 kDa과 HSZ 단백질을 콩에서 발현하도록 유전자 재조합했는데 이 콩의 경우는 알레르기 환자들이 유전자 재조합 된 콩과 자연 콩에 반응의 차이를 나타내지 않았다는 결과 보고이다. 위의 세 실험 결과들을 종합해 볼 때 무엇보다도 새롭게 발현된 단백질이 원래 어떤 성질을 갖고 있으며 어디에서 유래했는지가 알레르기성 조사에 중요한 역할을 한다 할 수 있겠다. 또한 유전자 재조합된 식품들은 알레르기 환자들을 위해 표기되어져야 할 것인데 이를 위한 알레르기성 검사 실험은 공공단체를 통해 이루어져야 할 것이며 환자들마다 알레르겐으로 작용하는 단백질의 인식부위(epitope)가 다를 수 있기 때문에 적어도 10명 이상의 알레르기 환자들이 조사되어져서 검사가 이루어져야 할 것이다. 환자들의 혈청을 통한 in vitro 실험에서는 ELISA, RAST, immunoblotting과 같은 검사 방법들이 적용될 수 있고, 그 결과가 음성인 경우에 그 다음 단계로 in vivo 실험에서는 직접 환자의 피부반응검사 (skin prick test)나 DBPCFC (double-blind placebo-controlled food challenge) 검사 방법을 통해 확인되어져서 이 모든 경우가 음성인 경우와 하나라도 양성인 경우를 구별하여 식품에 표기함으로 알레르기 환자들의 유전자 재조합 식품에 대한 안전성이 보장되어져야 할 것이다.