• 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.

• Journal of the Korean Chemical Society
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• v.56 no.1
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• pp.115-127
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• 2012

This is a survey research which investigated perceptions of 85 elementary and secondary school science teachers on inter-disciplinary teaching in general. It is expected that the results of this survey will help to find out the necessity, the appropriate time, the proper approach and the obstacles of inter-disciplinary education. Results indicated that 49.5% of the teachers were aware of inter-disciplinary teaching and 61.2% of the teachers agreed with the necessity of implementing inter-disciplinary strategy. However, considering difficulties in objective assessment and burdens of college entrance exam preparation, they responded that inter-disciplinary teaching could be more appropriate and feasible to be implemented for lower graders at elementary schools than secondary school students. Of the answers to the integrated approaches, 57.6% of teachers chose the theme-based approach, and 16.5% chose the problem-based approach. When they chose the theme-based approach, they stated the reason of educational readiness. On the other hand, when they chose the problem-based approach, they stated the reason of educational obligation which assumes that a problem solving needs inter-disciplinary approach. The teachers also selected 'lack of knowledge on other subjects' and 'lack of time to prepare teaching materials' as major predicaments in implementing inter-disciplinary approach. This suggests that there needs a clear and specific teaching strategy along with a guidance to teaching materials for inter-disciplinary teaching.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.9 no.2
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• pp.122-126
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• 2013

Cerebral palsy is one of the primary handicapping conditions of childhood. The prevalence of malocclusions in patients with cerebral palsy is approximately twice than in general population. Even though these high rates of malocclusions, most clinicians may feel uncomfortable about treating such problems to reduce inclination of anterior teeth because to reduce of protrusion makes to decrease risk of trauma. This is the case report about mitigation of maxillary anterior teeth protrusion in patient with cerebral palsy. A 14 year old boy who had cerebral palsy visited our dental hospital. He had severe protrusive maxillary anterior teeth and narrow arch form. He was experienced at using Castillo morales appliance in early childhood. He had mild mental retardation and was able to learn simple skills. He and his parents had willing to improve his dental problems. A gentle impression taking on maxilla was done. Removable appliance was made including median screw and labial bow. We provide a period of adaption for 3 weeks. After of anterior teeth through activation of labial bow was done once a month by dentist. The treatment carried out for 10 months and we could observe reduced labial inclination of maxillary right central incisor and more wide arch form. Hawley type retainer was set at maxilla for retention. In conclusion, accompanying careful case selection and treatment, patient with cerebral palsy can be treated and should not be ignored their orthodontic needs.

• Journal of the Korean Society of Food Science and Nutrition
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• v.44 no.2
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• pp.173-181
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• 2015

This study investigated the effects of scopoletin (6-methoxy-7-hydroxycoumarin) supplementation on insulin resistance and the antioxidant defense system in chronic alcohol-fed rats. Rats were fed a Lieber-Decarli liquid diet containing 5% ethanol with or without two doses of scopoletin (0.01 and 0.05 g/L) for 8 weeks. Pair-fed rats received an isocaloric carbohydrate liquid diet. Chronic alcohol did not affect fasting serum glucose levels, although it induced glucose intolerance and hyperinsulinemia compared with the pair-fed group and led to insulin resistance. Both doses of scopoletin similarly improved glucose intolerance, serum insulin level, and insulin resistance. Scopoletin supplementation significantly activated phosphatidyl inositol 3-kinase, which was inhibited by chronic alcohol. Two doses of scopoletin up-regulated hepatic mRNA expression and activity of glucokinase as well as down-regulated mRNA expression and activity of glucose-6-phosphatase compared with the alcohol control group. Both doses of scopoletin significantly reduced cytochrome P450 2E1 activity and elevated aldehyde dehydrogenase 2 activity, resulting in a lower serum acetaldehyde level compared with the alcohol control group. Chronic alcohol suppressed hepatic mRNA expression and activities of antioxidant enzymes such as superoxide dismutase, catalase, and glutathione peroxidase; however, they were reversed by scopoletin supplementation, which reduced hydrogen peroxide and lipid peroxide levels in the liver. These results indicate that dietary scopoletin attenuated chronic alcohol-induced insulin resistance and activated the antioxidant defense system through regulation of hepatic gene expression in glucose and antioxidant metabolism.

• Journal of Hospice and Palliative Care
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• v.20 no.3
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• pp.159-166
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• 2017

According to the advance of antiretroviral regimen and the early treatment strategy, people living with human immunodeficiency virus (PLWH) are achieving the goal of virologic suppression and immune restoration. Most of them no more die of acquired immunodeficiency syndrome (AIDS) defining illnesses, and become older with chronic comorbidities such as cardiovascular, metabolic, hepatic, renal and neurological diseases. However some PLWH still visit hospitals as late presenters with very low CD4+ T cell counts, so that they suffer AIDS defining illnesses to die or experience severe neurological complications resulting in disabilities. Early palliative interventions are needed on the various symptoms of PLWH. Thus far chronic pains such as distal symmetric sensory polyneuropathies have been underevaluated. Active pain-relieving interventions are important to them. Recently we define end of life condition of human immunodeficiency virus (HIV) or eligibility to hospice care after adjusting current status of HIV treatment. Hospice teams should pay attention to the specific medical conditions, psychological needs, and social circumstances of PLWH. With just standard precautions as common infection control measures, general hospice cares can be provided to them like to other hospices subjects. For giving PLWH opportunities to have the end of life with value and dignity, hospice multidisciplinary team should intervene them early and aggressively. Now we need more clinical experiences and institutional improvements.

• Korean Journal of Biological Psychiatry
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• v.8 no.1
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• pp.20-36
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• 2001

The debate about whether depressive disorders should be divided into categories or arrayed along a continuum has gone for decade, without resolution. In our review, there is more evidence consistent with the spectrum concept than there is with the idea that depressive disorders constitute discrete clusters marked by relatively discontinuous boundaries. First, "depression spectrum", "is there a common genetic factors in bipolar and unipolar affective disorder", "threshold model of depression" and "bipolar spectrum disorder" are reviewed. And, a new subtype of depression is so called SeCA depression that is a stressor-precipitated, cortisol-induced, serotonin-related, anxiety/aggression-driven depression. SeCA depression is discussed. But, there is with the idea that depressive disorders constitute discrete subtypes marked by relatively discontinuous boundaries. This subtypes of depressive disorder were reviewed from a variety of theoretical frames of reference. The following issues are discussed ; Dexamethasone suppression test(DST), TRH stimulation test, MHPG, Temperament Character Inventory(TCI), and heart rate variability(HRV).

• Proceedings of the Korea Water Resources Association Conference
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• 2005.05b
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• pp.454-458
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• 2005

여름철 집중강우시 유입되는 고탁수층은 저수지의 밀도성층으로 인하여 표수층 하부에 위치하며, 이를 적기에 배제하지 않을 경우에는 수평방향의 확산현상과 연직방향의 전도현상으로 인해 저수지 전역에 분포하게 되어, 탁수현상의 장기화를 유발한다. 일본 사메우라댐의 경우에는 1977년 9월 태풍 17호에 의한 집중호우로 대량의 고탁수가 유입되어 하류하천으로의 댐 방류수의 고탁도현상이 장기간 지속된 바 있다. 이와 같은 탁수장기화에 대한 저수지내 대책의 하나로서 홍수유입후 탁도가 높은 물을 단기간동안 방류하고, 갈수기에는 탁도가 낮은 물을 방류하는 선택취수 개념이 제안된 바 있다. 국내의 임하댐의 경우에도 2002년 태풍 '루사'와 2003년 태풍 '매미'로 인해 발생한 탁수가 전도현상을 거치며 저수지내 전수층에 확산됨으로써 댐방류수의 고탁도현상이 장기화된바 있다. 이로 인하여 댐하류에 위치한 안동시 용상정수장의 동절기 정수처리 장애로 2003년 11월이후 약 160여일동안 댐방류가 중단된 바 있다. 임하댐의 경우에도 탁수장기화에 대한 대책으로서 홍수기 고탁수층을 우선배제한 후, 저수지내에 유입되는 청수을 담수함으로써 이를 방지할 수 있다. 이러한 선택취수 기법은 동일한 수리동역학적 개념을 적용하여 하류하천의 냉해문제를 대비하기 위한 방법으로 표층의 온수를 선택방류하는 데에도 이용될 수 있다. 이와 같은 선택취수를 효율적으로 수행하기 위해서는 취수시설에 접근하는 밀도흐름을 정확하게 이해하고 있어야 한다. 또한 취수탑 인근에서의 온도 및 탁도 등의 연직분포를 실시간으로 파악할 수 있는 자동수질측정시스템이 구축되어 있어야 한다. 현재 임하댐의 경우, 하류하천의 수질을 고려한 효율적인 탁수배제를 위하여 상류 유입부 2개소, 발전취수탑 전면 1개소, 조정지댐 1개소 등 총 4개의 자동수질측정시스템이 설치되어 탁도와 온도가 실시간으로 모니터링되고 있으며, 향후 강우시 저수지에서의 밀도흐름을 보다 정밀하게 모니터링하기 위하여 저수지내 4개의 주요지점에 추가적으로 설치될 예정이다. 이와 같은 자동측정시스템과 더불어 주기적인 세부 수질조사를 실시하면 저수지내의 온도성층 변화와 탁수층 분포를 매우 정확하게 파악할 수 있으며, 호소의 수질모형과 연계하여 강우시 탁수 도달시간과 탁수량을 사전에 예측할 수도 있다. 또한 이와 같은 기초적인 정보를 바탕으로 하류하천의 탁수 피해를 최소화할 수 있는 선택취수탑의 운영방안을 수립할 수 있다 본 연구에서는 이를 위해 선택취수탑 주위의 성층흐름을 기존의 실험자료와 수치해석을 통하여 분석하였고, 온도성층구조나 취수구의 위치변화에 따른 방류수 수질특성을 조사하였다.

• Journal of Wetlands Research
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• v.15 no.4
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• pp.509-518
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• 2013

Understanding of the exchange between the water and energy which is happening between the surface and atmosphere is the basic of studying water resources. To study these, lots of researches using Noah Land Surface Model(LSM) are in progress. Noah LSM is based on energy and water balance equation and simulates various hydrological factors. There are diverse researches with Noah LSM are ongoing in overseas, on the other hand not enough study has been done. Especially there is almost no study using uncoupled Noah LSM in Korea. In this study we used data from Korea Flux Tower in Haenam(HFK) and Gwangneung(GDK) as forcing data to simulate the model and compared its result of net radiation, sensible heat flux and latent heat flux with the observation data to assess the applicability of Noah LSM in Korea. Regression coefficients of the comparison results of Noah LSM and observation show good agreement with the value of 0.83~0.99 at Haenam and 0.64~0.99 at Gwangneung which means Noah LSM can be trusted.

• Korean Journal of Cognitive Science
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• v.28 no.4
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• pp.267-297
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• 2017

The anatomical structure of the brain reflects a great amount of information about an individual's cognitive ability. The present study reviewed research on developmental changes in brain structure in relation to biological maturation and intellectual growth focusing on children and adolescents. The purpose of the present study was to achieve an understanding of how children and adolescents' brain matures with development and also to examine whether individual differences in intelligence influences the development of brain structure. The first section introduces methods of measurement and analysis of brain structure, such as voxel-based morphometry and structural covariance. The second section reviews studies on the biological maturation of the brain and variables that influence brain development such as sex, environmental factors, and mental disorders, etc. The third section introduces the Parieto-Frontal Integration Theory of intelligence and reviews studies on the association between intelligence and developmental changes of the brain, including changes in structural covariance and functional connectivity. We conclude with a discussion on educational/clinical implications of this work and directions for future studies.

• Korean Security Journal
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• no.13
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• pp.207-234
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• 2007

This study examines the roles of government and reasons of defeat on 9.11 terror, iraq war between 2001 and 2003. The administration functions critical role of national security. And punctual, accurate information supply capability helps policymaker's decision-making. Hence, information of punctuality and accuracy should be given to policymakers. And without two above written factors, it will result in failing. Information concoction on policymaker's pressure, biased informant, inaccurate information and lack of assembly means under the extensive organization and technologized spying means, Fail to keep information objectivity, leads to information failure. In the context of a series of facts, we shall cover the position of government and reasons of calamities. Two incidents deem as information failure by national security service, but concoction of Iraqi mass destruction weaponry is believed as bush administration's deception on account of political gains. For fully functional government role, governing body should reinforce all aspects of gathering, analyzing, and making use of information more objectively in the first place. In particular, information concoction involving policymakers post massive stumbling block to organized outcome. The thesis presents a prospective view of government position under the U.S. secret agent over 9.11 terror and Iraq war.