• Title/Summary/Keyword: 물질적 결핍

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COVID-19 in a 16-Year-Old Adolescent With Mucopolysaccharidosis Type II: Case Report and Review of Literature

  • Park, So Yun;Kim, Heung Sik;Chu, Mi Ae;Chung, Myeong-Hee;Kang, Seokjin
    • Pediatric Infection and Vaccine
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    • v.29 no.2
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    • pp.70-76
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    • 2022
  • Coronavirus disease 2019 (COVID-19) in patients with underlying diseases, is associated with high infection and mortality rates, which may result in acute respiratory distress syndrome and death. Mucopolysaccharidosis (MPS) type II is a progressive metabolic disorder that stems from cellular accumulation of the glycosaminoglycans, heparan, and dermatan sulfate. Upper and lower airway obstruction and restrictive pulmonary diseases are common complaints of patients with MPS, and respiratory infections of bacterial or viral origin could result in fatal outcomes. We report a case of COVID-19 in a 16-year-old adolescent with MPS type II, who had been treated with idursulfase since 5 years of age. Prior to infection, the patient's clinical history included developmental delays, abdominal distension, snoring, and facial dysmorphism. His primary complaints at the time of admission included rhinorrhea, cough, and sputum without fever or increased oxygen demand. His heart rate, respiratory rate, and oxygen saturation were within the normal biological reference intervals, and chest radiography revealed no signs of pneumonia. Consequently, supportive therapy and quarantine were recommended. The patient experienced an uneventful course of COVID-19 despite underlying MPS type II, which may be the result of an unfavorable host cell environment and changes in expression patterns of proteins involved in interactions with viral proteins. Moreover, elevated serum heparan sulfate in patients with MPS may compete with cell surface heparan sulfate, which is essential for successful interaction between the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) spike protein and the host cell surface, thereby protecting against intracellular penetration by SARS-CoV-2.

A Case of Hunter Syndrome Diagnosed at Age of 2.5 Year (2.5세에 진단된 헌터증후군 1례)

  • Choi, Miran;Kwun, Younghee;Jin, Dongkyu;Lee, Jieun
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.14 no.2
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    • pp.178-181
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    • 2014
  • Hunter syndrome (mucopolysaccharidosis type II, MPS II) is a X-linked lysosomal storage disease caused by a deficiency in the lysosomal enzyme, iduronate-2-sulfatase (IDS), leading to accumulation of glycosaminoglycans within lysosomes of many organs and tissues. Since the enzyme replacement therapy was approved and available in the treatment of MPS I, II, VI, early diagnosis and early therapy can bring the better prognosis of disease and the better quality of life in patients. We described a 2.5 year old child presented with frequent otitis media and developmental delay including speech impairment, who was diagnosed as Hunter syndrome with IDS NM_000202.5:c. 263G>A(p.Arg88His) mutation.

Rhabdomyolysis Following SARS-CoV-2 Infection in Children: A Case Report and Systematic Review

  • Na-Won Oh;Si-Hwa Gwag;Kyu Sik Cho;Young June Choe
    • Pediatric Infection and Vaccine
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    • v.31 no.1
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    • pp.136-139
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    • 2024
  • Rhabdomyolysis is a syndrome that causes various complications due to the release of substances from muscle cells, often associated with preceding infectious diseases. We report the case of a 7-year-old Korean boy with recent severe acute respiratory syndrome coronavirus 2 infection, presenting with fever, chills, and generalized body aches, diagnosed as rhabdomyolysis. Additionally, we conducted a systematic review with the aim of delineating the disease spectrum, treatment, and outcomes. We identified seven reports that met the inclusion criteria. Among the cases, 5 had fever, with creatine kinase levels ranging from 3,717 and 274,664 IU/L. Two individuals received treatment in intensive care unit, 2 underwent renal replacement therapy, and 1 case has deceased. For children with coronavirus disease 2019 infection and muscle pain, a thorough examination of urine color and an assessment of muscle enzymes through blood tests can help diagnose and treat rhabdomyolysis, a condition that might otherwise be overlooked.

The Methodology for Extraction of Geochemical Anomalies, Using Regression Formula: an Example from a Granitic Body in Gyeonggi Province (회귀 수식을 이용한 지구화학적 이상분포지역 도출기법: 경기도화강암의 예)

  • 황상기;신성천;염승준;문상원
    • Economic and Environmental Geology
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    • v.35 no.2
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    • pp.137-147
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    • 2002
  • Natural geological and environmental processes reflect to element abundances in geological materials on the surface. This study aims to elucidate a possibility of geostatistical application to differentiate geochemical anomalies affected by anthropogenic and geogenic factors. A regional geochemical map was produced using 'inverse distance weight interpolation' method for analytical results of stream sediments «150 11m) which were collected from 2,290 first- to second-order streams over the whole Gyeonggi Province. The Jurassic granitic batholith in the southeastern province was selected as a target for the geostatistical examination. Factor analysis was conducted using 22 elements for stream sediments from 445 drainage basins over the granitic body. Co, Cr, Sc, MgO, Fe$_{2}$O$_{3}$, V, and Ni were grouped with high correlation coefficients and the depletion of the components may reflect the whole-rock chemistry of the granite. Regression analysis was done using Co, Cr, and Sc as dependent variables and other six components as independent variables, and the results were drawn as maps. The maps acquired generally show quite similar distribution patterns with those of concentrations of each variable. The similarity in the spatial patterns between the two maps indicates that the application of regression statistics can be valid for the interpretation of regional geochemical data. However, some components show local discrepancies which may be influenced by secondary factors regardless of the basement lithology. The regression analysis may be effective in extracting local geochemical anomalies which may reflect rather anthropogenic pollutions than geogenic influences.

Petrochemical Characteristics of the Duibaejae Volcanic Rocks from Goseong, Gangwon-do, Korea (강원도 고성 뒤배재 화산암의 암석화학적 특성)

  • Kim, Hwa Sung;Kil, Youngwoo;Lee, Moon Won
    • Journal of the Korean earth science society
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    • v.34 no.2
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    • pp.109-119
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    • 2013
  • Duibaejae basalts from Goseong, Gangwon-do, are divided into the lower basalt and the upper basalt depending on the properties, such as occurrence, mineral compositions, and major and trace compositions of the basalts. The lower basalts have characteristics of agglomerate rocks as well as contain, crustal and mantle xenoliths, and olivine, pyroxene, and plagioclase xenocrysts. The upper basalts with columnar joints contain relatively more mantle xenolith and olivine xenocryst than the lower basalts. The major and trace element compositions suggest that the composition of the upper basalts is close to primary magma composition. Enrichment and depletion patterns of the trace and the rare-earth elements of the lower basalts are similar to those of the upper basalts, whereas the lower basalts are more LREE enriched than the upper basalts. The source magmas of the lower and upper basalts from Duibaejae volcanic edifice were generated from about 0.8-1.2% and 3.7-4.0% batch melting of garnet peridotite, respectively. The abundance of granite xenolith, and plagioclase and quartz xenocrysts with reaction rim indicates that the lower basalts, compared with upper basalts, might have been assimilated with the crustal materials during ascending to surface.

The Relationship between Regional Material Deprivation and the Standardized Mortality Ratio of the Community Residents Aged 15-64 in Korea (우리나라에서의 지역의 물질적 결핍수준과 15-64세 인구 표준화사망비의 관계)

  • Jeong, Baek-Geun;Jung, Kap-Yeol;Kim, Joon-Youn;Moon, Ok-Ryun;Lee, Yong-Hwan;Hong, Young-Seoub;Yoon, Tae-Ho
    • Journal of Preventive Medicine and Public Health
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    • v.39 no.1
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    • pp.46-52
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    • 2006
  • Objectives: This study was performed to investigate the relationship between regional material deprivation and the standardized mortality ratios(SMRs) of community residents aged 15-64 in Korea. Methods: SMRs were investigated using the registered death data from 1995 to 2000 that was obtained from the Korean National Statistics Office with the denominators being drawn from the 1995 to 2000 census. Material deprivation was measured using the Townsend score that was calculated from the 1995 to 2000 census. The relationship between the regional material deprivation and the SMRs of the community residents aged 15-64 was investigated by using ANOVA, Spearman's rank correlation analysis and Pearson's correlation analysis. The trends in mortality inequality were investigated using the concentration index. Results: On the ANOVA, the SMRs of the men and women residents in the least deprived areas were the smallest and those in the most deprived areas were the largest. Spearman's rank correlation analysis, Pearson's correlation analysis and the concentration index revealed that significant positive relationships exist between the regional material deprivation and the SMRs of the community residents aged 15-64. Conclusions: This study suggests that there are mortality inequalities among the communities in Korea and part of this difference is due to the material deprivation of the community. Strategies aimed at reducing mortality inequalities among the communities will be needed to address economic inequalities. Further studies are needed to explore the mechanisms of how the regional deprivation influences on health and how the other factors of the community influence on the health of the community residents.

Deterioration Diagnosis and Source Area of Rock Properties at the West Stone Pagoda, Gameunsaji Temple Site, Korea (감은사지 서탑의 풍화훼손도 진단 및 석재의 산지추정)

  • Lee Chan Hee;Lee Myeong Seong;Suh Mancheol;Choi Seok-Won;Kim Man Gap
    • Economic and Environmental Geology
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    • v.37 no.5
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    • pp.569-583
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    • 2004
  • The rock properties of the West pagoda in the Gameunsaji temple site are composed mainly of dark grey porphyritic granodiorite with medium grained equigranular texture and developed with small numerous dioritic xenoliths. These xenoliths occurred with small holes due to different weathering processes. As a weathering results, the rock properties of this pagoda occur wholly softened to physical hardness because of a complex result of petrological, meteorological and biological causes. Southeastern part of the pagoda deteriorated seriously that the surface of rock blocks showed partially exfoliations, fractures, open cavities in course of granular decomposition of minerals, sea water spray and crystallization of salt from the eastern coast. The Joint between blocks has small or large fracture cross each other, contaminated and corrupted for inserting with concrete, cement mortar, rock fragments and iron plates, and partially accelerated coloration and fractures. There are serious contamination materials of algae, fungus, lichen and bryophytes on the margin and the surface on the roof stone of the pagoda, so it'll require conservation treatment biochemically for releasing vegetation inhabiting on the surface and the discontinuous plane of the blocks because of adding the weathering activity of stones and growing weeds naturally by soil processing on the fissure zone. Consisting rock for the conservation and restoration of the pagoda would be careful choice of new rock properties and epoxy to reinforce for the deterioration surfaces. For the attenuation of secondary contamination and surface humidity, the possible conservation treatments are needed.

제도 운영과 수학교육과 교육과정

  • Mun, Gwon-Bae
    • Communications of Mathematical Education
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    • v.12
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    • pp.463-477
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    • 2001
  • 국가의 국가경쟁력은 학창시절 학생의 학력만큼 중요하다. 성인의 경우, 학력을 위한 노하우는 체험을 통해 터득할 수 있었다. 그러나 국가경쟁력에 관해서는 우물 안 내부관점을 벗어나기 힘들어 추진 방향과 제도 운영에 자칫 시행착오를 범하기 쉽다. 이는 사안에 대해 본질적인 접근보다 껍데기만을 쫓기 때문이었다. 이 현상을 분석하려면 관점과 보이지 않는 영역의 것들을 다룰 수 있는 수학적 사고법이 필요하며, 이 능력은 현 지식정보화 사회에서 매우 긴요하다. 그러나 현실은 여러 가지 이유로 수학적 사고법을 비롯한 기초학문을 위기로 몰아가고 있고, 안타깝지만 그 중심에 수학교육이 자리잡고 있다. 수학교육의 위기를 유발하는 요인으로 제도 운영에 관한 건이 있다. 제도 운영에서 한 변수의 변화, 예로 대입의 계열교차지원 허용 건, 교원임용고시에서 교과교육학 영역의 출제 건과 복수전공, 부전공 자격소지자에 대한 가산점 부여 건은 수학교육과 교육과정에 직, 간접적으로 영향을 미친다. 이 관계를 사범대학 수학교육과 현장의 사례를 통하여 조명하고, 그 문제점을 지적하고자 한다. 현 사범교육은 졸업이수학점 140학점 체제하에서 제 7차 교육과정에 따른 복수전공, 부전공 우대 정책을 펴고 있다. 수학교육과의 경우, 부전공 열풍이 불어 전공선택 과목이 3학년 1학기부터 폐강될 위기에 처해 있다. 교양교육의 고사 또는 전광교육이 예전보다 반으로 줄어들게 된 사범대학 실상에 비애감을 느끼게 된다. 이는 전문화된 교사 양성, 나아가 미래 국가경쟁력 향상에 심각한 저해 요소로 작용할 것이다. 복잡다단한 세상에서 최적화를 향한 개선 노력이 멈춰서는 안 된다. 현행 교원임용고시 운영상의 문제점을 공론화하고, 수학교육인의 중지를 모아야 할 긴박한 시점이다. 이를 계기로 교원임용고시의 운영개선과 수학교육과 교육과정을 한층 더 견실하게 하는 데에 이바지하고자 한다. 것이라면 후속연구로 이러한 가능성을 실험연구로 검증하고자 한다.toceros resting spores/Chaetoceroe vegetative cells도 80 cm 보다 상층에서는 높게 나타나 규조온도지수 분포와도 일치하는 경향을 보인다. 이상의 규조군집 분석 결과에 의하면, 홀로세의 후빙기동안 본 연구 지역인 동해 북동부에는 대마 난류의 유입이후 현재와 유사한 환경이 우세하게 발달했으나, 난류종 P. doliolus의 변화는 동해내에서 대마난류의 세기가 반복되었음을 지시하고 있다./3 수준으로 높다. 결론적으로 풍부한 화학물질들을 함유한 제주해류는 남해 및 동해의 생지화학적 과정들에 있어 상당히 중요함을 시사한다.다. 수조 상층수 중 Cu, Cd, As 농도는 모든 FW, SW수조에서 시간이 지남에 따라 일관성 있게 감소하였고, 제거속도는 Cu가 다른 원소에 비해 빨랐다. 제거속도는 FW 3개 수조 중 FW5&6에서 세 원소 모두 가장 느렸고, SW 3개 수조 중에서는 SW1&2에서 가장 빨랐다. SW와 FW간 제거속도 차이는 세 원소 모두 명확치 않았다 Cr은 FW에서 전반적으로 감소하는 경향을 보였지만 SW에서는 실험 초기에 감소하다 24시간 이후에는 증가 후 일정한 양상을 보였다. Pb은 FW에서 전반적으로 감소했지만 SW에서는 초기에 급격히 증가 후 다시 급격히 감소하는 양상을 보였다 Pb 또한 Cu, Cd, As와 마찬가지로 SW1&2에서 제거속도가 가장 빠르게 나타났다. FW 상층수 중 Hg는 시간에 따라 급격히 감소했고, 제거속도는 Fw5&6에서 가장 느렸다. 이러한 결과에 근거할 때 벼가 자라고 있고 이분해성 유기물이 풍부한 FW1&2, FW3&4 토양과 상층수에서는 유기물의 분해 활동이 활발하였지만, 벼가 경작되지 않는 FW5&6과 SW 에서는 유기물이 상대적으로 결핍되어 유기물의 분해활동이 적었을 것으로 판단된다

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Impact of Area Characteristics on the Health of Vulnerable Populations in Seoul (지역특성이 취약집단 건강에 미치는 영향 분석)

  • Kim, Youn-Hee;Cho, Young-Tae
    • Korea journal of population studies
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    • v.31 no.1
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    • pp.1-26
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    • 2008
  • This research examines the effect of area level characteristics on individual health, particularly focusing on the vulnerable populations in Seoul. We consider adult individuals whose family income is under 1.5 million won, who are aged 65 and over, or who have neither spouse nor job but aged 40 and over as vulnerable populations. Using the 2005 Seoul Citizens' Health Interview Survey, we conducted multilevel analyses to simultaneously investigate the effect of area and individual level characteristics on health. Between-area variance of self-rated health status was greater for the elderly population than for all populations. Area material deprivation index and happiness index were associated with the self-rated health of economically disadvantaged populations. Vulnerable populations showed greater between-area variances in emotional health than the same for all populations. Area happiness index, material deprivation index, the proportion of households below poverty line and street safety showed statistically significant association with emotional health. The effect of area characteristics were particularly salient for the emotional health of elderly population and its between area variance was also notable.

A Case of Citrullinemia Type 1 in ASS 1 Mutation (ASS 1 유전자 돌연변이로 확진된 시트룰린혈증 1형 1례)

  • Yim, Dae kyoon;Huh, Rimm;Kwun, Younghee;Lee, Jieun;Cho, Sung Yoon;Park, Hyung Doo;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.15 no.1
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    • pp.29-34
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    • 2015
  • Citrullinemia type1 is an autosomal recessive disorder of the urea cycle characterized by neonatal or late onset of hyperammonemia caused by a deficiency of the enzyme argininosuccinate synthetase (ASS). An ASS1 deficiency demonstrates fatal clinical manifestations that are characterized by the neonatal metabolic coma and early death when untreated. It causes a broad spectrum of effects, ranging from a mild disorder to a severe mental retardation, epilepsy, neurologic deficits. An acute neonatal form is the most common. Infants are normal at birth followed by an acute illness characterized by vomiting, lethargy, seizures and coma. These medical problems are life-threatening in many cases. A later onset form is less frequent and may be milder than the neonatal form. This later-onset form is associated with severe headaches, visual dysfunction, motor dysfunction, and lack of energy. Citrullinemia type1 is caused by mutations in the ASS1 gene located on chromosome 9q34.1 that encodes argininosuccinate synthetase, the third enzyme of the urea cycle catalyzing the formation of argininosuccinic acid from citrulline and aspartic acid. The enzyme is distributed in tissues including liver and fibroblasts. This mutation leads to hyperammonemia, arginine deficiency and elevated citrulline level. In the urea cycle, argininosuccinate synthetase catalyses the conversion of citrulline and aspartate to argininosuccinate.. Here, we describe a female newborn patient with lethargy, rigidity and hyperammonemia who was diagnosed as citrullinemia type1 with a c.[421-2A>G], c.[1128-6_1188dup] mutation.