• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.17 no.3
• /
• pp.92-95
• /
• 2017

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by an ${\alpha}$-galactosidase A (GLA, MIM 300644) enzyme deficiency due to pathogenic variants in the ${\alpha}$-galactosidase A gene (GLA). The disease leads to accumulation of globotriaosylceramide (Gb3) and related glycophospholipids affecting nearly all major organ systems, with the primary sites damaged by Gb3 including renal glomeruli, myocardium, neurons of the dorsal ganglion and autonomic nervous system, and vascular endothelial and smooth muscle. Progressive deposition in these organ systems present with various clinical manifestations including acroparesthesia, renal failure and heart failure. Here, we report a Chinese male diagnosed with Fabry disease in his late $4^{th}$ decades showing improvement of acroparesthesia during enzyme replacement therapy (ERT). A 48-year-old Chinese man who presented with chronic recurrent severe burning pain in his fingers and toes since the age of 10, with worse involvement of the former visited to our clinic for further evaluation. His medical history included a transient ischemic attack aged 40 and diagnosed with stage 4-5 chronic kidney disease aged 47. In the family history, the patient's brother was found to be have Fabry disease 1 month before his visit. Except for his brother, all other members of the family are healthy. Based on his medical history and family history, he was strongly suspicious for Fabry disease. He was found to have a galactose-alpha-1,3-galactose level 4.96 (Reference range, 42.5-67.9) suggestive of Fabry disease. The followed sequencing of GLA coding region in our patient revealed hemizyosity for the mutation c.988C>T (Q330X) in Exon 7. Since ERT start, he showed significant improvement in his symptoms of burning sensation of fingers and toes. On the contrary, due to deteriorating kidney function even with ERT, he is considered for kidney transplantation. Despite of diagnostic delay until late 4th decades, ERT showed a potential improvement of acroparesthesia in our patient. However, late start of ERT can lead to poor outcome in multiorgan function. Therefore, early diagnosis with high index of suspicion followed by continuous ERT with regular monitoring have an impact on quality of life in Fabry disease.

• Clean Technology
• /
• v.22 no.1
• /
• pp.53-61
• /
• 2016

Steam reforming of tar produced from biomass gasification was conducted using several Ni-based catalysts. In labscale, the catalytic steam reforming of toluene which is a major component of biomass tar was studied. A fixed bed reactor was used at various temperatures of 400-800 ℃. Ru (0.6 wt%) and Mn or K (1 wt%) were applied as a promoter in Ni based catalysts. Generally, Ni/Ru-K/Al₂O₃ catalyst shows higher performance on steam reforming of toluene than Ni/Ru-Mn/Al₂O₃ catalyst. Used catalysts were analyzed by XRD and TGA to detect sintering and carbon deposition. Base on the lab-scale studies, the monolith and pellet type catalysts were tested in 1 ton/day scale biomass gasification system. Ni/Ru-K/Al₂O₃ monolith catalyst shows high tar reforming performance at high temperature. In addition, Ni/Ru-Mn/Al₂O₃ monolith catalyst was showed deactivation with operation time. Reforming performance of Ni/Ru-K/Al₂O₃ pellet catalyst which showed 66.7% tar conversion at 587 ℃ was compared to regenerated one. Overall, Ni/Ru-K/Al₂O₃ pellet catalyst shows higher stability and performance than other used catalysts.

• Journal of the Korean earth science society
• /
• v.32 no.3
• /
• pp.276-293
• /
• 2011

In this study a modeling system consisting of Weather Research and Forecasting (WRF), Sparse Matrix Operator Kernel Emissions (SMOKE), the Community Multiscale Air Quality (CMAQ) model, and the CMAQ-Model of Aerosol Dynamics, Reaction, Ionization, and Dissolution (MADRID) model has been applied to estimate enhancements of $PM_{10}$ during Asian dust events in Korea. In particular, 5 experimental formulas were applied to the WRF-SMOKE-CMAQ (MADRID) model to estimate Asian dust emissions from source locations for major Asian dust events in China and Mongolia: the US Environmental Protection Agency (EPA) model, the Goddard Global Ozone Chemistry Aerosol Radiation and Transport (GOCART) model, and the Dust Entrainment and Deposition (DEAD) model, as well as formulas by Park and In (2003), and Wang et al. (2000). According to the weather map, backward trajectory and satellite image analyses, Asian dust is generated by a strong downwind associated with the upper trough from a stagnation wave due to development of the upper jet stream, and transport of Asian dust to Korea shows up behind a surface front related to the cut-off low (known as comma type cloud) in satellite images. In the WRF-SMOKE-CMAQ modeling to estimate the PM10 concentration, Wang et al.'s experimental formula was depicted well in the temporal and spatial distribution of Asian dusts, and the GOCART model was low in mean bias errors and root mean square errors. Also, in the vertical profile analysis of Asian dusts using Wang et al's experimental formula, strong Asian dust with a concentration of more than $800\;{\mu}g/m^3$ for the period of March 31 to April 1, 2007 was transported under the boundary layer (about 1 km high), and weak Asian dust with a concentration of less than $400\;{\mu}g/m^3$ for the period of 16-17 March 2009 was transported above the boundary layer (about 1-3 km high). Furthermore, the difference between the CMAQ model and the CMAQ-MADRID model for the period of March 31 to April 1, 2007, in terms of PM10 concentration, was seen to be large in the East Asia area: the CMAQ-MADRID model showed the concentration to be about $25\;{\mu}g/m^3$ higher than the CMAQ model. In addition, the $PM_{10}$ concentration removed by the cloud liquid phase mechanism within the CMAQ-MADRID model was shown in the maximum $15\;{\mu}g/m^3$ in the Eastern Asia area.

• Journal of Korean Society of Forest Science
• /
• v.84 no.1
• /
• pp.103-113
• /
• 1995

To estimate buffer capacity and sensitivity of forest ecosystem to acid rain in Taejon, ionic components of throughfall, stemflow, soil leachate, and open rain in Pinus rigida and Quercus variabilis forest were analysed. The spatial sensitivity based on parent rock and forest type was given by IDRISI of GIS which created imagery conversion from soil and vegetation map. Parent rocks and soils were classified into acidic, sedimentary, metamorphic rock and then subdivided based on $SiO_2$ content. Average pH of vegetation leachate was higher in throughfall but lower in stemflow than open rain and higher in Quercus variabilis forest than in Pinus rigida forest. The flow of $SO{_4}^{2-}$, $NO_3{^-}$ and $Cl^-$ through vegetation leaching(throughfall plus stemflow) into soil were 7.2, 4.3, and 2.5 times, respectively, higher in Pinus rigida forest and 4.4, 2, and 2.5 times, respectively, higher in Quercus variabilis forest than in open field. But the concentration of exchangeable cations was 4.1 times higher in Pinus rigida forest and 4.6 times higher in Quercus variabilis forest than in open field. Average pH of soil leachate was lower than that of throughfall, but higher than that of stemflow. The concentration of exchangeable canons and $Al^{3+}$ in soil leachate were more in Pinus rigida forest than in Quercus variabilis forest and increase signficantly with the increase of acidic deposits. Pinus forest had more deposition and canopy interception of acidic pollutants and more nutrient loss than Quercus forest, and Quercus forest had more cation exchange and proton consumption and than consequently had less nutrient loss and better buffer capacity than Pinus forest. The 69% of forest soils was distributed on acidic rock, 25% of it on metamorphic rock, and 6% of it on intermediate and basic rock. Acidic rock residuals which had low very canon exchange capacity and high sensitivity to acid rain occupied a half of total forest land in Taejon area. Therefore forests in Taejon showed high vulnerability to acid rain and will receive much more stress with the increase of acid rain precursors.

• Economic and Environmental Geology
• /
• v.36 no.5
• /
• pp.321-328
• /
• 2003

The epithermal gold and base metal deposit of the Tanggeung district of West Java consists of four major veins(Celak, Cigodobras, Cilangkap and Pasirbedil) with NS to N10$^{\circ}$∼20$^{\circ}$E and N75$^{\circ}$W strikes. The veins occur within fractures cutting the crystal and lithic tuff of Jampang Formation(Oligo-Miocene) in and around the Mt. Subang of the western Java, Indonesia. The ore mineralization is characterized by the occurrence of pyrite, sphalerite, galena, chalcopyrite, and small amounts of bornite and Fe-oxides. Hydrothermal alteration, associated with the mineralization, was dominantly silicified and enveloped by the phyllitic(sericitic), argillic and propylitic alteration containing the disseminated pyrite. Gangue minerals consist of interstratified smectite-illite, chlorite, sericite, and minor kaolinite. The presence of vapor-rich fluid inclusions in quartz veins suggests that boiling occurred locally throughout ore deposition. Fluid inclusion studies suggest that the ore fluid evolved from initial high temperatures(〓34$0^{\circ}C$) to later lower temperatures(〓19$0^{\circ}C$). Salinities range from 0.0 to 8.3 wt percent NaCl equiv. The relatively high increase in salinity(up to 8.3 wt percent NaCl equiv) might be explained by a local boiling and by a participation of magmatic fluids, supported by the sulfur isotope results. Evidence of fluid boiling suggests that the pressure decreased from 200 bars to 120 bars. This corresponds to the depths of approximately 750 to 1,200 m in a hydrothermal system that changed from lithostatic to hydrostatic conditions. Using homogenization temperatures and paragenetic constraints, the calculated $\delta$$^{34}$ S values of $H_2S$ in ore fluid are -0.2 to 1.8 permil close to the 0 permil isotopic value of magmatic sulfur.

• Journal of Animal Science and Technology
• /
• v.52 no.5
• /
• pp.383-388
• /
• 2010

Identification of natural compounds that can prevent the development of obesity in vivo is time consuming and expensive. We have used in vitro systems derived from pig adipose tissue to screen simple aqueous or ethanolic extracts of Korean medicinal herbs (KMH) for their anti-adipogenic potential. A total of 183 extracts were tested for their actions in lipogenesis of pig adipose tissue and differentiation of pig preadipocytes. Ethanol extracts were prepared from 72 and aqueous extracts were prepared from 111 medicinal herbs. Both an ethanolic and an aqueous extract were prepared from 65 of these. Thirteen extracts substantially altered rates of lipogenesis in vitro. The effects of KMH on lipogenesis of pig adipose tissue are as follows. Elevens reduced lipogenesis to rates that were more than 40% lower than control and four of these reduced rates of lipogenesis by more than 70%. The most potent anti-lipogenic extracts were those obtained in ethanol from Iridaceae and from Sophora flavescens AIT as well as both the aqueous and ethanolic extracts from Lysimachia vulgaris L. Two extracts, those prepared in water from Caesalpiniae lignum and from Phellodendri cortex, were found to promote rates of lipogenesis in vitro. The effects of KMH on differentiation of pig preadoipocytes are as follows. Twentyeight extracts altered the rates of differentiation of cultured porcine preadipocytes. Sixteen increased and twelve reduced the rates of differentiation of preadipocytes. Extracts prepared in ethanol from Moutan radicis cortex and from Ostericum koreanum and those prepared in water from Angelicae gigantis radix, from Inula henenium L and from Magnolia flos doubled the rate of differentiation of cultured porcine preadipocytes. Ten extracts reduced the in vitro rate of differentiation of porcine preadipocytes by more than 35%. These were the ethanolic extracts from Glycyrrizae radix, Nepetae spica and from Polygala myrtifolia and the aqueous extracts from Amaranthaceae, Asparagus cochinchinesis, Atractylodis rhizoma alba, Citrus junos TANAKA, Cyperus rotundus, Epimedium grandiflorum and from Moutan radicis cortex. Only the ethanolic extract from Polygala myrtifolia was able to both reduce lipogenesis in adipose tissue slices and retard differentiation of cultured preadipocytes. The results of our study will provide meaningful information to identify medicinal herbs which would reduce fat deposition in livestocks and humans.

• Economic and Environmental Geology
• /
• v.47 no.1
• /
• pp.39-48
• /
• 2014

In coastal region, estuaries have complex environments where dissolved and particulate matters are mixed with marine water and substances. Suspended sediment (SS) dynamics in coastal water, in particular, plays a major role in erosion/deposition processes, biomass primary production and the transport of nutrients, micropollutants, heavy metals, etc. Temporal variation in suspended sediment concentration (SSC) can be used to explain erosion/sedimentation patterns within coastal zones. Remotely sensed data can be an efficient tool for mapping SS in coastal waters. In this study, we analyzed the variation in SSC in coastal water using the Geostationary Ocean Color Imager (GOCI) and Landsat Enhanced Thematic Mapper Plus (ETM+) in Gyeonggi-bay. Daily variations in GOCI-derived SSC showed low values during ebb time. Current velocity and water level at 9 and 10 am is 37.6, 28.65 $cm{\cdot}s^{-1}$ and -1.23, -0.61 m respectively. Water level has increased to 1.18 m at flood time. In other words, strong current velocity and increased water level affected high SSC value before flood time but SSC decreased after flood time. Also, we compared seasonal SSC with the river discharge from the Han River and the Imjin River. In summer season, river discharge showed high amount, when SSC had high value near the inland. At this time SSC in open sea had low value. In contrast, river discharge amount from inland showed low value in winter season and, consequently, SSC in the open sea had high value because of northwest monsoon.

• The Journal of Korean Institute of Electromagnetic Engineering and Science
• /
• v.13 no.7
• /
• pp.667-678
• /
• 2002

In this paper, a new radiating structure with a multi-layered two-dimensional metallic disk array was proposed for shaping the flat-topped element pattern. It is an infinite periodic planar array structure with metallic disks finitely stacked above the radiating circular waveguide apertures. The theoretical analysis was in detail performed using rigid full-wave analysis, and was based on modal representations for the fields in the partial regions of the array structure and for the currents on the metallic disks. The final system of linear algebraic equations was derived using the orthogonal property of vector wave functions, mode-matching method, boundary conditions and Galerkin's method, and also their unknown modal coefficients needed for calculation of the array characteristics were determined by Gauss elimination method. The application of the algorithm was demonstrated in an array design for shaping the flat-topped element patterns of $\pm$20$^{\circ}$ beam width in Ka-band. The optimal design parameters normalized by a wavelength for general applications are presented, which are obtained through optimization process on the basis of simulation and design experience. A Ka-band experimental breadboard with symmetric nineteen elements was fabricated to compare simulation results with experimental results. The metallic disks array structure stacked above the radiating circular waveguide apertures was realized using ion-beam deposition method on thin polymer films. It was shown that the calculated and measured element patterns of the breadboard were in very close agreement within the beam scanning range. The result analysis for side lobe and grating lobe was done, and also a blindness phenomenon was discussed, which may cause by multi-layered metallic disk structure at the broadside. Input VSWR of the breadboard was less than 1.14, and its gains measured at 29.0 GHz. 29.5 GHz and 30 GHz were 10.2 dB, 10.0 dB and 10.7 dB, respectively. The experimental and simulation results showed that the proposed multi-layered metallic disk array structure could shape the efficient flat-topped element pattern.

• Journal of the Korean Electrochemical Society
• /
• v.8 no.3
• /
• pp.139-145
• /
• 2005

[ $SnO_x$ ] films on the flexible substrate of PET film were prepared at ambient temperature under a $(CH_3)_4Sn(TMT: tetra-methyl tin)-H_2-O_2$ atmosphere in order to obtain transparent conductive polymer by using ECR-MOCVD(Electro Cyclotron Resonance Metal Organic Chemical Yfpor Deposition) system. The prepared $SnO_x$ thin films show generally over $90\%$ of optical transmittance at wavelength range of 380-780nm and about $1\times10^{-2\~3}ohm{\cdot}cm$ of electrical resistivity. In the present study, effects of $O_2/TMT\;and\;H_2/TMT$ mole ratio on the properties of $SnO_x$ films are investigated and the other process parameters such as microwave power, magnetic current power, substrate distance and working pressure are fixed. Based on our experimental results, the $SnO_x$ film composition ratio of Sn and O directly influences on the electrical and optical properties of the films prepared. The $SnO_x$ film with low electric resistivity and high transmittance could be obtained by controlling the process parameters such as $O_2/TMT\;and\;H_2/TMT$ mole ratio, which play an important role to change the composition ratio between Sn and O. An increase of $O_2/TMT$ mole ratio brought on the increases 0 content in the $SnO_x$ film. On the other hand, an increase of $H_2/TMT$ mole ratio lead to decreases the oxygen content in the film. The optimized composition ratio of oxygen : tin Is determined as 2.4: 1 at $O_2/TMT$ of 80 and $H_2/TMT$ of 40 mole ratio, respectively.

• Journal of the Korean Society of Radiology
• /
• v.8 no.4
• /
• pp.211-216
• /
• 2014

Radium is rock or soil of crust or uranium of building materials after radioactivity collapse process are created colorless and odorless inert gas that accrue well in sealed space like basement. It inflow to lung circulate respiratory organ and caused lung cancer because of deposition of lung or bronchial tubes. In this study, the air in the elementary school classroom nongdoeul tonkatsu place of measured values were compared using the calculated annual internal radiation exposure. La tonkatsu exposure measured in primary school classroom at least five schools when you close the windows in the average floor 0.56mSv 2 floors ground floor windows when opened 0.384mSv 048mSv 3 floors, 2 floor levels of the same three layers 0.31mSv 0.296mSv the human exposure to radon and radiation on the first floor of 3 floors above ground in a lot of exposure was moderate. When you close the window from the first floor up 0.384mSv 056mSv 3 floors with a minimum annual radiation exposure due to natural radiation in the 16 to 23.3 percent minimum 2.4mSv accounted for. When I opened the window to the maximum annual radiation exposure 2.4mSv 0.296mSv 0.31mSv least a minimum of 12.3 to 12.91% accounted for Results suggest that more than five chodeunghakgyoeun La tonkatsu domestic radon measurements conducted below regulatory requirements and internal exposure has also fall within the normal range. People The less the radiation exposure to the human body because it reduces the impact in the classroom in elementary school vent windows often reduced to the maximum radon concentration in the air, if called tonkatsu be able to reduce radiation exposure for the immune system is weak and elementary will be helpful to experiment more in the future for the school authorities called tonkatsu investigation is done to him if the action to establish a more secure school building facilities is thought would be helpful.