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Clinical Findings of Phenylketonuria Patients in Korea (페닐케톤뇨증의 임상적 고찰)

  • Shin, Ik Soon;Lee, Dong Hwan
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.12 no.1
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    • pp.14-22
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    • 2012
  • Objectives: This study was performed to review clinical manifestations of hyperphenylalaninemia patients in Korean. Methods: 178 cases of hyperphenyalaninemia were diagnosed at department of pediatrics, Soonchunhyang University Hospital from January, 1983 to August. We reviewed DNA analysis, MR imaging, EEG, radiography of the left hand and wrist, bone densitometry (BMD), IQ test of hyperphenyalaninemia patients. Results: Out of 178 cases, 161 cases were diagnosed classic phenylketonuria and 17 cases were diagnosed BH4 deficiency. 122 cases performed DNA analysis. R243Q (10.3%), Y204C (9.9%), and IVS4-1G>A (8.1%) mutations were predominant. 22 cases underwent MR imaging. Varying degrees of symmetrical high signal intensity were noted on T2-weighted sequences in the periventricular deep white matter of 15 cases. 23 cases were performed EEG. 12 cases (52.3%) showed abnormal pattern. EEG abnormalities showed in 11 cases. On lumbar BMD four of 11 cases (36%) showed reduced bone density of more than 1 S.D. in four of 11 cases, bone age was less than chronological age by at least one year. 18 cases were performed IQ test. Mean IQ scores was $84{\pm}21.6$. Among older than 15 years (9 cases), Mean IQ scores was $72{\pm}21.2$. PTPS deficiency was 14 cases, DHPR deficiency was 2 cases, and GTPCH deficiency was 1 case. Conclusion: We confirmed there were varieties of DNA mutations. And MR imaging and EEG were nonspecific in PKU patients. Older children showed lower IQ score. Low phenylalanine diet prevents brain damage in PKU patient. Not only first few years of life but also lifetime, Keeping low phenylalanine diet is important.

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Geochemistry of the Kwanaksan alkali feldspar granite: A-type granite\ulcorner (관악산 알칼리 장석 화강암의 지구화학 : A-형 화강암\ulcorner)

  • S-T.Kwon;K.B. Shin;H.K. Park;S.A. Mertzman
    • The Journal of the Petrological Society of Korea
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    • v.4 no.1
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    • pp.31-48
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    • 1995
  • The Jurassic Kwanaksan stock, so far known to be composed of biotite granite only, has the mineral assemblage of quartz+K-feldspar+plagioclase+biotite${\pm}$gernet. The lithology of the stock is classified as alkali feldspar granite by their mode and plagioclase compositions (An<5). Subsolvus feldspars, rather early crystallization of biotite, and shallow emplacement depth estimated from Q-Ab-Or diagram suggest hydrous nature of the magma, which contrasts with anhydrous A-type like geochemistry described below. Major and trace element compositions of the Kwanaksan stock are distinct from those of the adjacent Seoul batholith, suggesting a genetic difference between the two, The Kwanaksan stock shows geochemical characteristics similar to A-type granite in contrast to most other Mesozoic granites in Korea, in that it has high $SiO_2$(73~78wt%), $Na_2O+K_2O$, Ga(27~47 ppm). Nb(22~40 ppm), Y(48~95 ppm), Fe/Mg and Ga/Al, and low CaO(<0.51 wt%). Ba (8~75 ppm) and Sr(2~23 ppm). However, it has lower Zr and LREE and higher Rb(384~796 ppm) than typical A-type granite. LREE-depleted rare earth element pattern with strong negative Eu anomaly of previous studies is reinterpreted as representing source magma characteristics. The residual material during partial melting is not compatible with pyroxenes, amphibole or garnet, while significant amount of plagioclase is required. Similarity of geochemistry of the Kwanaksan stock to A-type granite suggests the origin of the stock has a chose relationship with that of A-type granite. These observations lead us to propose that the Kwanaksan stock was formed by partial melting of felsic source rock.

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Study on Development of Embedded Source Depth Assessment Method Using Gamma Spectrum Ratio (감마선 스펙트럼 비율을 이용한 매립 선원의 깊이 평가 방법론 개발 연구)

  • Kim, Jun-Ha;Cheong, Jea-Hak;Hong, Sang-Bum;Seo, Bum-Kyung;Lee, Byung Chae
    • Journal of Nuclear Fuel Cycle and Waste Technology(JNFCWT)
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    • v.18 no.1
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    • pp.51-62
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    • 2020
  • This study was conducted to develop a method for depth assessment of embedded sources using gamma-spectrum ratio and for the evaluation of field applicability. To this end, Peak to Compton and Peak to valley ratio changes were evaluated according to 137Cs, 60Co, 152Eu point source depth using HPGe detector and MCNP simulation. The effects of measurement distance of PTV and PTC methods were evaluated. Using the results, the source depth assessment equation using the PTC and PTV methods was derived based on the detection distance of 50 cm. In addition, the sensitivity of detection distance changes was assessed when using PTV and PTC methods, and error increased by 3 to 4 cm when detection distance decreased by 20 cm based on 50 cm. However, it was confirmed that if the detection distance was increased to 100 cm, the effects of detection distance were small. And PTV and PTC methods were compared with the two distance measurement method which evaluates the depth of source by the change of net peak counting rate according to the detection distance. As a result of source depth assessment, the PTV and PTC showed a maximum error of 1.87 cm and the two distance measurement method showed maximum error of 2.69 cm. The results of the experiment confirmed that the accuracy of the PTV and PTC methods was higher than two distance measurement. In addition, Sensitivity evaluation by horizontal position error of source has maximum error of less than 25.59 cm for the two distance measurement method. On the other hand, PTV and PTC method showed high accuracy with maximum error of less than 8.04 cm. In addition, the PTC method has lowest standard deviation for the same time measurement, which is expected to enable rapid measurement.

Ecological Characteristics and Vegetation Structure Analysis of Eurya Japonica Community -Focusing on Busan Metropolitan City- (사스레피나무 군락의 생태적 특성 및 식생구조 분석 -부산광역시를 중심으로-)

  • Jang, Jung-Eun;Lee, Sang-Cheol;Choi, Song-Hyun
    • Korean Journal of Environment and Ecology
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    • v.34 no.2
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    • pp.157-169
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    • 2020
  • The purpose of this study is to investigate the ecological characteristics and vegetation structures of Eurya japonica in Busan. As a result of the TWINSPAN and DCA analysis, 89 plots of 100㎡ each were divided into 3 communities: Quercus serrata-Pinus densiflora-E. japonica community, Pinus thunbergii-E. japonica community, and P. thunbergii-Camellia japonica community. Community I consisted of the Quercus serrata-Pinus densiflora-E. japonica which was mainly located in the high altitude inland. While Q. serrata and P. densiflora competed in the tree layer, the dominant species of the understory layer was E. japonica. Since Carpinus tschonoskii, one of the climax species, was distributed evenly from shrub to tree layers, it was likely that deciduous oak trees or Carpinus tschonoskii would become dominant species in community I. In community I, E. japonica was found in higher altitude than the other evergreen broad-leaved tree and was expected to maintain their tree vigor even if the vegetation structure is converted into the deciduous forest. Community II, the P. thunbergii-E. japonica community, was predicted to maintain its tree vigor unless there were unexpected disturbance factors. Community III, consisting of P. thunbergii-C. japonica and located in Dongbaek Island, was under artificial management. In community III, P. thunbergii was the only species in the tree layer, while C. japonica was predominant in the understory layer. E. japonica and various evergreen broad-leaved tree species were present in the understory layer and shrub layer, which were unmanaged areas. Therefore, it is expected that unless C. japonica is continuously managed, E. japonica is likely to become the dominant species. There were also various evergreen broad-leaved species, such as Machilus thunbergii and Pittosporum tobira, present in the shrub layer. If the temperature continues to rise, the habitat is expected to become evergreen broad-leaved forests in the future as P. thunbergii community declines. The result of Pearson's correlation coefficient analysis of E. japonica and species appearing in 89 plots showed that 9 species were had a statistically significant relationship (p<0.05). Four species, including P. tobira and Q. dentata, had a positive correlation. Five species had a negative correlation, and C. japonica, which had the same ecological position as E. japonica, showed the most negative correlation at -0.384.

Neuroprotective effects of Extract of Broccoli, Cultivated in Desalinated Magma Seawater, on neuron-like SH-SY5Y cells (제주도 탈염 용암해수 재배로 제조한 브로콜리 추출물의 신경 세포 보호 효과)

  • Rhee, Jin Seol;Jang, Youn Bi;Choi, Ge Sun;Choung, Jai Jun;Kang, Seung Woo
    • Journal of the Korean Applied Science and Technology
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    • v.37 no.3
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    • pp.448-462
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    • 2020
  • Dietary nutrition is a critical lifestyle factor that can reduce the risk of future cognitive impairments caused by dementia. Accumulating evidence suggests that dietary supplementation with Sulforaphane may help the prevention of cognitive impairments and dementia. Thus, Sulforaphane-enriched broccoli extract would hold promise to improve cognitive impairments of dementia patients. Here, we have used broccoli extracts, prepared from broccoli cultivated in Magma Seawater, to test if the broccoli extracts can be dietary supplement to improve cognitive impairments. Magma Seawater originated from Jeju Island, Korea is unique in terms of containing high concentrations of usable minerals (Zinc, Vanadium and Germanium etc.). Broccoli, grown in Magma Seawater, would contain Sulforaphane and the extra amount of usable minerals. The chemical compositions of the broccoli extracts were analyzed using LC-Q-orbitrap to detect Sulforaphane and Glucoraphanin. Analysis method based on HPLC was developed for measurement of sulforaphane levels in the broccoli extracts. We have tested if the broccoli extracts have anti-apoptotic and anti-inflammatory effects on neuron-like SH-SY5Y cells. In addition, we examined if the broccoli extracts are able to upregulate expression of synaptic plasticity-associated proteins (BDNF and phospho-CREB) and to inhibit acetylcholine esterase (AchE) activity. We have shown that the broccoli extracts inhibited the apoptotic pathway and inflammatory responses. Finally, we present evidence showing that AchE activity was inhibited by the broccoli extracts, but expression of BDNF and phospho-CREB was upregulated. Taken together, these findings suggest that the broccoli extracts from Magma Seawater-grown broccoli would be a good source of dietary nutrition to improve cognitive impairments in the future.

The Effect of Chemical Composition and Sintering Temperature on The Improvement of Physical Properties of Mn-Zn Ferrites (Mn-Zn ferrite의 성분 및 소결 온도에 따른 물리적 특성의 향상 연구)

  • 고재귀
    • Journal of the Korean Magnetics Society
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    • v.5 no.4
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    • pp.269-274
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    • 1995
  • The basic composition of Mn-Zn ferrite was $Mn_{0.631}Zn_{0.316}Fe_{2.053}O_{4}$(specimen A), $Mn_{0.584}Zn_{0.312}Fe_{2.104}O_{4}$(specimen B) and $Mn_{0.538}Zn_{0.308}Fe_{2.154}O_{4}$(specimen C) with additional 0.1 mol % $CaCo_{3}$ and 0.04 mol % $V_{2}O_{5}$. For high per¬meability and acceleration of grain growth, $CaCo_{3}$ and $V_{2}O_{5}$. was added. The mixture of the law materials was calcinated at $950^{\circ}C$ for 3 hours and then milled. The compacts of toroidal type were sintered at different temperature($1250^{\circ}C$, $1300^{\circ}C$, $1350^{\circ}C$) for 2 hours in $N_2$ atmosphere. The effects of the various raw material composition and sintered temperature on the physical properties of Mn-Zn ferrite have been investigated. They turned out to be spinel structure by X-ray diffraction and the size of grain from SEM was from $18\;\mu\textrm{m}\;to\;23\;\mu\textrm{m}$. As the sintering temperature was increased from $1250^{\circ}C$ to $1350^{\circ}C$, the initial permeability and magnetic induction has increased and the both of Q factor and coercive force has decreased. The coercive force and curie temperature were almost the same at each specimen Their values were about 0.45 Oe and $200^{\circ}C$. The frequency of specimen will used in the range from 200 kHz to 2 MHz. The basic composition of $Mn_{0.584}Zn_{0.312}Fe_{2.104}O_{4}$(specimen B) sintered at $1300^{\circ}C$ shows the best results at magnetic induction (Br & Bm).

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Arsenic Removal Using Iron-impregnated Ganular Activated Carbon (Fe-GAC) of Groundwater (철침착 입상활성탄(Fe-GAC)을 이용한 지하수 내 비소 제거기술)

  • Yoon, Ji-Young;Ko, Kyung-Seok;Yu, Yong-Jae;Chon, Chul-Min;Kim, Gyoo-Bum
    • Economic and Environmental Geology
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    • v.43 no.6
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    • pp.589-601
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    • 2010
  • Recently it has been frequently reported arsenic contamination of geologic origin in groundwater. The iron-impregnated ranular activated carbon (Fe-GAC) was developed for effective removal of arsenic from groundwater n the study. Fe-GACs were prepared by impregnating iron compounds into a supporting medium (GAC) with 0.05 M iron nitrate solution. The materials were used in arsenic adsorption isotherm tests to know the effect of iron impregnation time, batch kinetic tests to understand the influence of pH, and column tests to evaluate for the preliminary operation of water treatment system. The results showed that the minimum twelve hours of impregnation time were required for making the Fe-GAC with sufficient iron content for arsenic removal, confirmed by a high arsenic adsorption capacity evaluated in the isotherm tests. Most of the impregnated iron compounds were iron hydroxynitrate $Fe_4(OH)_{11}NO_3{\cdot}2H_2O$ but a mall quantity of hematite was also identified in X-ray diffraction(XRD) analysis. The batch isotherms of Fe-GAC for arsenic adsorption were well explained by Langmuir than Freundlich model and the iron contents of Fe-GAC have positive linear correlations on logarithmic plots with Freundlich distribution coefficients ($K_F$ and Langmuir maximum adsorption capacities ($Q_m$. The results of kinetic experiments suggested hat Fe-GAC had he excellent arsenic adsorption capacities regardless of all pH conditions except for pH 11 and could be used a promising adsorbents for groundwater arsenic removal considering the general groundwater pH range of 6-8. The pseudo-second order model, based on the assumption that the ate-limiting step might be chemisorption, provided the best correlation of the kinetic experimental data and explained the arsenic adsorption system f Fe-GAC. The column test was conducted to valuate the feasibility of Fe-GAC use and the operation parameters in arsenic groundwater treatment system. The parameters obtained from the column test were the retardation actor of 482.4 and the distribution coefficient of 581.1 L/mg which were similar values of 511.5-592.5 L/mg acquired from Freundlich batch isotherm model. The results of this study suggested that Fe-GAC could be used as promising adsorbent of arsenic removal in a small groundwater supply system with water treatment facility.

Vascular Plant Diversity and Vegetation of Samusan Mt. in Jecheon-si, Korean Peninsula (사무산(제천시)의 식물다양성과 식생)

  • Kim, Jung-Hyun;Kim, Jin-Seok;Nam, Gi-Heum;Jung, Eun-Hee;Lee, Kyeong-Ui;Hwang, Yo-Seob
    • Korean Journal of Plant Resources
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    • v.31 no.4
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    • pp.396-418
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    • 2018
  • This study was carried out to investigate the flora and the vegetation of Samusan mountain on Jecheon-si, located in the middle part of the Korean peninsula. The vascular plants which were collected in 9 times from June 2014 to October 2015 were identified as 502 taxa in total, including 102 families, 314 genera, 445 species, 6 subspecies, 49 varieties, 1 form and 1 hybrid. The largest families were as follows; Asteraceae (70 taxa), Poaceae (52 taxa), Rosaceae (30 taxa), Fabaceae (28 taxa), and Cyperaceae (20 taxa). Of them, Korean endemic plants numbered 10 taxa, and vascular plants listed in the red data according to the International Union for Conservation of Nature (IUCN) numbered 14 taxa. The floristic regional indicator plants found in this area were 61 taxa in total. Among them, 5 taxa revealed the floristic grade V, 11 for floristic grade IV, 14 for floristic grade III, 10 for floristic grade II, and 21 for floristic grade I. The alien plants were identified as 43 taxa and the percentage of naturalized index (NI) was 8.6%, and urbanization index (UI) was 13.4%, respectively. Samples of the forest vegetation on the Samusan Mt. were mainly classified as Pinus densiflora, Quercus variabilis, Q. acutissima, Q. mongolica, Zelkova serrata and Robinia pseudoacacia forest. The vertical structures of the forest were stable and the DBH-Class analyses showed that the dominant tree species would be maintained. In the surveyed areas, high plant diversity was shown, and a number of endemic, rare, calcicole plants and phytogeographically important plants were found. Nonetheless, numerous and diverse biological resources native have been consistently disturbed or damaged by human activities without some form of protection. Therefore, it is needed to set up strategies for conservation forest vegetation in this study area.

Effects of Humic Acid on the pH-dependent Sorption of Europium (Eu) to Kaolinite (PH 변화에 따른 카올리나이트와 유로퓸(Eu)의 흡착에 대한 휴믹산의 영향)

  • Harn, Yoon-I;Shin, Hyun-Sang;Rhee, Dong-Seok;Lee, Myung-Ho;Chung, Euo-Cang
    • Journal of Soil and Groundwater Environment
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    • v.14 no.4
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    • pp.23-32
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    • 2009
  • The sorption of europium (Eu (III)) onto kaolinite and the influence of humic acids over a range of pH 3 ~ 11 has been studied by batch adsorption experiment (V/m = 250 : 1 mL/g, $C_{Eu(III)}\;=\;1\;{\times}\;10^{-5}\;mol/L$, $C_{HA}\;=\;5{\sim}50\;mg/L$, $P_{CO2}=10^{-3.5}\;atm$). The concentrations of HA and Eu(III) in aqueous phase were measured by UV absorbance at 254nm (e.g., $UV_{254}$) and ICP-MS after microwave digestion for HA removals, respectively. Results showed that the HA sorption onto kaolinite was decreased with increasing pH and their sorption isotherms fit well with the Langmuir adsorption model (except pH 3). Maximum amount ($q_{max}$) for the HA sorption at pH 4 to 11 was ranged from 4.73 to 0.47 mg/g. Europium adsorption onto the kaolinite in the absence of HA was typical, showing an increases with pH and a distinct adsorption edge at pH 3 to 5. However in the presence of HA, Eu adsorption to kaolinite was significantly affected. HA was shown to enhance Eu adsorption in the acidic pH range (pH 3 ~ 4) due to the formation of additional binding sites for Eu coming from HA adsorbed onto kaolinite surface, but reduce Eu adsorption in the intermediate and high pH above 6 due to the formation of aqueous Eu-HA complexes. The results on the ternary interaction of kaolinte-Eu-HA are compared with those on the binary system of kaolinite-HA and kaolinite-Eu and adsorption mechanism with pH was discussed.

Analysis of Plants Social Network for Vegetation Management on Taejongdae in Busan Metropolitan City (부산 태종대 식생관리를 위한 식물사회네트워크 분석)

  • Sang-Cheol Lee;Hyun-Mi Kang;Seok-Gon Park;Jae-Bong Baek;Chan-Yeol Yu;In-Chun Hwang;Song-Hyun Choi
    • Korean Journal of Environment and Ecology
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    • v.36 no.6
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    • pp.651-661
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    • 2022
  • Plants social network analysis, which combines plants society and social network analyses, is a new research method for understanding plants society. This study was conducted to investigate the relationship between species, using plant social network analysis targeting Taejongdae in Busan, and build basic data for management. Taejongdae, located in the warm temperate forest in Korea, is a representative coastal forest of Busan Metropolitan City, and the Pinus thunbergii-Eurya japonicacommunity is widely distributed. This study set up 100 quadrats (size of 100m2each) in Taejongdae to investigate the species that emerged and analyzed the interspecies association focusing on major species. Based on the results, a sociogram was created using the Gephi 0.9.2, and the network centrality and structure were analyzed. The results showed that the frequency of appearance was high in the order of P. thunbergii, E. japonica, Quercus serrata, Sorbus alnifolia, Ligustrum japonicum, and Styrax japonicusand that many evergreen broad-leaved trees appeared due to the environmental characteristics of the site. The plants social network of Taejongdae was composed of a small-scale network with 50 nodes and 172 links and was divided into 4 groups through modularization. The succession sere identified through a sociogram confirmed that the group that include P. thunbergiiand E. japonicawould progress to a deciduous broadleaf community dominated by Q. serrataand Carpinus tschonoskii, using hub nodes such as Prunus serrulataf. spontaneaand Toxicodendron trichocarpum. Another succession sere was highly likely to progress to an evergreen broad-leaved community dominated by Machilus thunbergiiand Neolitsea sericea, using M. thunbergiias a medium. In some areas, a transition to a deciduous broad-leaved community dominated by Celtis sinensis, Q. variabilisand Zelkova serratausing Lindera obtusilobaand C. sinensisas hub nodes was expected.