Song, Ari;Lee, Jin Sung;Im, Minji;Park, Hyung Doo;Cho, Sung Yoon;Jin, Dong-Kyu
Journal of The Korean Society of Inherited Metabolic disease
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v.18
no.2
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pp.62-67
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2018
Hunter syndrome, also known as mucopolysaccharidosis Type II (MPS II), is one of the lysosomal storage diseases caused by a lack of the enzyme iduronate 2-sulfatase (I2S). Lack of the I2S enzyme activity leads to accumulation of the glycosaminoglycans (GAG), causing dysfunction of multiple organs and systems. MPS II is an X-linked recessive disease due to mutation of IDS gene located on long arm of the X chromosome (Xq28). To date, more than 350 mutations of IDS gene have been identified in Hunter syndrome. Phenotypes of MPS II are classified as either severe or attenuated depending on the degree of cognitive impairment. Because the phenotype of MPS II is related to the type of mutation, identifying mutations is useful in predicting prognosis. We recently had a case of MPS II diagnosed by exome sequencing in a 7 month old boy with infantile spasm uncontrolled by AED. He was diagnosed with hearing loss at 2 months of age, and he took vigabatrin and prednisolone to control infantile spasms diagnosed at 3 months of age. At 6 months of age, whole exome sequencing was performed to evaluate the infantile spasm and hearing loss in this patient, and the mutation c.851C>T (p.Pro284Leu) inherited from hemizygous mother was revealed. The results of urine Cetylpyridinium Chloride (CPC) precipitation test, which were negative until 8 months of age, were positive from 9 months of age. We report a case of MPS II diagnosed by exome sequencing and treated through enzyme replacement therapy from 9 months after birth.
Journal of the Korea Organic Resources Recycling Association
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v.27
no.3
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pp.81-89
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2019
The major problem encountered during the storage of pig slurry (PS) is the release of huge amounts of greenhouse gases (GHGs), which are dominated by methane ($CH_4$). To reduce this, concentrated sulfuric acid has been used as an additive to control the pH of pig slurry to 5.0-6.0. However, other low-risk substitutes have been developed due to some limitations to its use, such as corrosiveness, and hazards to animal and human health. In this study, sugar addition was proposed as an eco-friendly approach for limiting $CH_4$ emission from PS during storage. The pH of PS has been reduced from $7.1{\pm}0.1$ (control) to $5.8{\pm}0.1$, $4.6{\pm}0.1$, $4.4{\pm}0.1$, $4.1{\pm}0.1$, and $4.0{\pm}0.1$, by the addition of 10, 20, 30, 40, and 50 g sugar/L, respectively. Lactate, acetate, and propionate were detected as the dominant organic acids and at sugar concentration above 20 g/L, lactate concentration represented 42-72% (COD basis) of total organic acids. For 40 d of storage, $20.6{\pm}2.3kg\;CO_2\;eq./ton\;PS$ was emitted in the control. Such emission, however, was found to be reduced to $8.7{\pm}0.4$ and $0.4{\pm}0.1kg\;CO_2\;eq./ton\;PS$ at 10 and 20 g/L, respectively. Small amount of $CH_4$ from PS at 10 g/L was emitted until 30 d of storage, while for rest of storage period, it has increased to $8.7{\pm}0.4kg\;CO_2\;eq./ton\;PS$ ( 40% of the control) when methanogens have recovered by increasing pH to 7.0. By the end of storage, VS and COD removal in the control reached 24% and 27%, while their ranges reached 15-4% and 12-17% in the sugar added experiments, respectively. It was found that more than 90% of COD removal was done by aerobic biological process.
Lee, Wang Shik;Im, Suk Ju;Kim, Bum Jun;Kim, Young Jin;Kim, Dong Hoon;Hwang, Kyung Jun;Kim, Si Hyun;Woo, Jae Hoon;Park, Nam Geon
Journal of The Korean Society of Grassland and Forage Science
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v.38
no.4
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pp.310-319
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2018
The southern type grasses announced for the test were the Bermudagrass cultivars (Giant, Cheyenne, Mohawk, Panchero Frio, Common and Tifton 85) and the Bahiagrass cultivars (TifQuik, Tifton 9), and the changes in the productivity and nutrient content were surveyed in Jeju area (450m altitude). The different cultivars were sowed by broadcasting or drill seeding method, and Tifton 85 was transplanted from sprigs. The fresh and dry matter yield showed varying significant differences for different cultivars (p<0.05). The fresh yields of Tifton 85, TifQuik and Tifton 9 were excellent, compared to the other cultivars, and for the dry matter yield, Tifton 85 and Tifton 9 were excellent when compared to the other cultivars. Crude protein content showed significant differences among different cultivars (p<0.05). Cheyenne, Mohawk, Panchero Frio, and Common showed differences in the crude protein content by sowing method and harvest time (p<0.05). The different cultivars showed differences in the crude fiber content (p<0.05), and Tifton 9 registered significantly high content and Mohawk and Tifton 85 showed significant crude fiber content by harvest time (p<0.05). According to these results, the southern type grass cultivars showed big differences in the regenerative capacity against damage from frost, productivity, and nutrient content, so they need to be chosen according to the purposes, and to increase their usage, their evaluation needs to be conducted at various altitudes.
Intensive Monitoring Survey of Nearby Galaxies (IMSNG) is a high cadence observation program monitoring nearby galaxies with high probabilities of hosting supernovae (SNe). IMSNG aims to constrain the SN explosion mechanism by inferring sizes of SN progenitor systems through the detection of the shock-heated emission that lasts less than a few days after the SN explosion. To catch the signal, IMSNG utilizes a network of 0.5-m to 1-m class telescopes around the world and monitors the images of 60 nearby galaxies at distances D < 50 Mpc to a cadence as short as a few hours. The target galaxies are bright in near-ultraviolet (NUV) with $M_{NUV}$ < -18.4 AB mag and have high probabilities of hosting SNe ($0.06SN\;yr^{-1}$ per galaxy). With this strategy, we expect to detect the early light curves of 3.4 SNe per year to a depth of R ~ 19.5 mag, enabling us to detect the shock-heated emission from a progenitor star with a radius as small as $0.1R_{\odot}$. The accumulated data will be also useful for studying faint features around the target galaxies and other science projects. So far, 18 SNe have occurred in our target fields (16 in IMSNG galaxies) over 5 years, confirming our SN rate estimate of $0.06SN\;yr^{-1}$ per galaxy.
Kim, Hyun-Ah;Lee, Jong Wook;Kim, Seung-Jun;Oh, Hong-Seok;Im, Woo Young;Kim, Ji-Woong
Korean Journal of Psychosomatic Medicine
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v.26
no.2
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pp.188-193
/
2018
Objectives : Clozapine is a widely prescribed antipsychotic drug for schizophrenia and is known to increase the risk of cardiovascular disease due to its metabolic side effects. However, little is known about the effect of clozapine on the platelet activation, another important factor in the development of cardiovascular disease. In this study, we tried to investigate the effect of clozapine on platelet activity in patients with schizophrenia by comparing the mean platelet component (MPC) values before and after the clozapine administration. Methods : A retrospective review of medical records of patients with schizophrenia, who newly started clozapine treatment from September 1st, 2003 to April 30th, 2007 at the Department of Psychiatry, Konyang University Hospital in Republic of Korea was performed. The final statistical analysis included 14 participants. Bayer ADVIA $120^{(R)}$ system was used to measure MPC. Results : Among the 14 participants, five subjects were males (28.60%), and ten subjects were females (71.40%). The mean age of participants was $37.50{\pm}11.64years$. Average of duration of illness was $91.00{\pm}93.96months$, with the mean dosage of clozapine taken by participants at the time of the last blood test was $337.50{\pm}109.52mg$. The mean MPC measurement before and after receiving clozapine was $26.12{\pm}2.22g/dL$ and $25.14{\pm}2.08g/dL$ respectively. Wilcoxon signed rank test showed that there was a statistically significant decrease in MPC levels after clozapine administration (V=16, p=0.024). Conclusions : Decreased MPC levels after clozapine administration implies that clozapine may increase platelet activation which could have an adverse effect on the occurrence of thromboembolic disease. Our findings also suggest that careful monitoring of the risk factors of cardiovascular diseases, such as platelets activity, is necessary when administering clozapine.
Kim, Seungjin;Im, Gikyo;Yi, Chi-Yeong;Lee, Seehyung;Sa, Jae-Hwan;Jeon, Eui-Chan
Journal of Climate Change Research
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v.4
no.1
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pp.51-61
/
2013
To address the problems associated with climate change and energy shortage, Korea has been making efforts to turn waste materials into usable energy. Due to the ongoing efforts to convert waste materials into energy, waste incineration is expanding to utilize the heat generated, and the subsequent greenhouse gas emissions from these waste material incineration are expected to increase. In this study, a municipal waste incineration plant that generates heat and electricity through heat recovery was selected as a subject facility. Methods for estimating the greenhouse gas emissions in the municipal waste incineration plant that was selected as a subject plant were sought, and the greenhouse gas emissions and emission factor were estimated. The $CO_2$ concentrations in discharge gas from the subject facility were on average 6.99%, and the result from calculating this into greenhouse gas emissions showed that the total amount of emissions was $254.60ton\;CO_2/day$. The net emissions, excluding the amount of greenhouse gas emitted from biomass incineration, was shown to be $110.59ton\;CO_2/day$. In addition, after estimating the emissions by separating the heat and electricity generated in the incineration facility, greenhouse gas emission factors were calculated using the greenhouse gas emissions produced per each unit of output. The estimated emission factor for heat was found to be $0.047ton\;CO_2/GJ$ and the emission factor for electricity was found to be $0.652ton\;CO_2/MWh$. The estimated emission factor was shown to be about 17% lower than the $0.783ton\;CO_2/MWh$ emission factor for thermal power plants that use fossil fuels. Waste material types and fossil carbon contents were evaluated as being the factors that have major effects on the greenhouse gas emissions and emission factor.
This study investigated the physiochemical properties, the anti-oxidant and alcohol metabolism enzyme activities, and the anti-inflammatory effects of three muskmelon vinegars prepared under different fermentation conditions, namely MV-1, MV-2, and MV-3. The total acidity of each vinegar was 4.00%, 4.32%, and 4.35%, respectively. Organic acid analysis showed that malic acid (58.37 mg/ml) was the most prevalent in MV-1 and that acetic acid was most prevalent in both MV-2 (46.95 mg/ml) and MV-3 (66.70 mg/ml). The total phenolic content of the muskmelon vinegars was highest at $129.74{\mu}g$ tannic acid equivalents (TAE)/ml in MV-3. The DPPH radical scavenging activity of the vinegars increased in a dose-dependent manner (p<0.05) and was 89.28% at MV-3 40% concentration. Similarly, SOD activitity increased in a concentration-dependent manner (p<0.05) so that levels for MV-1, MV-2, and MV-3 at 60% concentrations were 40.84%, 52.17% and 72.55%, respectively (p<0.05). Moreover, the ADH and ALDH activities of muskmelon vinegar were seen to increase in a concentration-dependent manner; ADH activity at 60% concentration was highest at 136.58% in MV-1 and ALDH activity at 60% concentration was highest at 100.25% in MV-2. The nitrite scavenging activities of MV-1, MV-2, and MV-3 at pH 1.2 were found to be 81.58%, 94.72%, and 87.75%, respectively. Anti-inflammatory effects were also examined, using LPS-stimulated RAW 264.7 cells, and nitric oxide production was reduced to 25.93%, 10.01%, and 79.75% by addition of MV-1, MV-2, and MV-3 at 1% concentration, respectively (p<0.05). These results suggest that the MV-3 muskmelon vinegar has great potential as an ingredient for high quality functional health beverages.
Despite the utility of solid-state NMR, NMR studies of iron-bearing silicate glasses remain a challenge because the variations in the peak position and width with increasing iron content reflect both paramagnetic effect and iron-induced structural changes. Therefore, it is essential to elucidate the effect of temperature on the NMR signal for iron-bearing silicate glasses. Here, we report the $^{29}Si$ and $^{27}Al$ MAS NMR spectra for $(Mg_{0.95}Fe_{0.05})SiO_3$ and $Fe_2O_3$-bearing $CaAl_2Si_2O_8$ (anorthite) glasses with varying spinning speed to interpret the NMR spectra for iron-bearing silicate glasses. The increase in the spinning speed results in an increase in the sample temperature. The current NMR results allow us to understand the origins of the changes in NMR signal with increasing iron content and to provide information on the dipolar interaction between nuclear spins. The $^{29}Si$ NMR spectra for $(Mg_{0.95}Fe_{0.05})SiO_3$ glass and $^{27}Al$ NMR spectra for $Fe_2O_3$-bearing $CaAl_2Si_2O_8$ glasses show that the peak shape and position of iron-bearing glasses do not change with increasing spinning speed up to 30 kHz. These results suggest that the NMR signal in the Fe-bearing glasses may stem from the 'survived nuclear spins' beyond the cutoff radius from the Fe, not from the paramagnetic shift. Based on the current results, the observed apparent shifts toward lower frequency of Al peak for $Fe_2O_3$-bearing $CaAl_2Si_2O_8$ glasses with increasing $Fe_2O_3$ at all spinning speed (15 kHz to 30 kHz) indicate the increase in the fraction of ${Q^4}_{Al}$(nSi) with lower n (i.e., 1 or 2) with increasing $Fe_2O_3$ and the spatial proximity between Fe and ${Q^4}_{Al}$(nSi) with higher n (i.e., 3 or 4). The present results show that changes in the NMR signal for iron-bearing silicate glasses reflect the actual iron-induced structural changes. Thus, it is clear that the applications of solid-state NMR for iron-bearing silicate glasses hold strong promise for unraveling the atomic structure of natural silicate glasses.
Journal of The Korean Association For Science Education
/
v.39
no.3
/
pp.363-377
/
2019
This study reveals the aspects of subjectivity in the test results in a science-specific aspect when assessing science-related affective characteristic through self-report items. The science-specific response was defined as the response that appear due to student's recognition of nature or characteristics of science when his or her concepts or perceptions about science were attempted to measure. We have searched for cases where science-specific responses especially interfere with the measurement objective or accurate self-reports. The results of the error due to the science-specific factors were derived from the quantitative data of 649 students in the 1st and 2nd grade of high school and the qualitative data of 44 students interviewed. The perspective of science and the characteristics of science that students internalize from everyday life and science learning experiences interact with the items that form the test tool. As a result, it was found that there were obstacles to accurate self-report in three aspects: characteristics of science, personal science experience, and science in tool. In terms of the characteristic of science in relation to the essential aspect of science, students respond to items regardless of the measuring constructs, because of their views and perceived characteristics of science based on subjective recognition. The personal science experience factor representing the learner side consists of student's science motivation, interaction with science experience, and perception of science and life. Finally, from the instrumental point of view, science in tool leads to terminological confusion due to the uncertainty of science concepts and results in a distance from accurate self-report eventually. Implications from the results of the study are as follows: review of inclusion of science-specific factors, precaution to clarify the concept of measurement, check of science specificity factors at the development stage, and efforts to cross the boundaries between everyday science and school science.
Kim, Sangtae;Shin, Ilhyoung;Kim, Kyoungsu;Kim, Daae;Kim, Heungrae;Im, Jahae;Lee, Jajun
Journal of Environmental Impact Assessment
/
v.28
no.4
/
pp.387-399
/
2019
This research aimed to prepare the classification of the damage types and the damage rating system of noise barriers for expressway noise barriers and to develop deterioration evaluation method of noise barriers by reflecting them. The noise barrier consists of soundproof panels, foundations and posts and the soundproof panels with 10 different types of materials are used in a single or mixed form.In this paper, damage of soundproof panel shows a single or composite damage, and thus a evaluation model of deterioration has been developed for noise barriers that can reflect the characteristic of noise barriers. Materials used mainly for soundproof walls were divided into material types for metal, plastic, timber, transparent and concrete. And damage types for noise barrier were classified into corrosion, discoloration, deformation, spalling and dislocation and damage types were subdivided according to the noise barrier's components and materials. Damage rating was divided into good, minor, normal and severe for each major part of noise barrier to assess damage rating of soundproof panel, foundation and post. The deterioration degree of noise barrier was evaluated comprehensively by using the deterioration evaluation method of whole noise barrier using weighted average. Deterioration evaluation method that can be systematically assessed has been developed for noise barrier using single or mixed soundproof panel and noise barrier with single or complex damage types. Through such an evaluation system, it is deemed that the deterioration status of noise barrier installed can be systematically understood and utilized for efficient maintenance planning and implementation for repair and improvement of noise barriers.
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