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Long-term Clinical Consequences in Patients with Urea Cycle Disorders in Korea: A Single-center Experience (요소회로대사 질환 환자들의 장기적인 임상 경과에 대한 단일 기관 경험)

  • Lee, Jun;Kim, Min-ji;Yoo, Sukdong;Yoon, Ju Young;Kim, Yoo-Mi;Cheon, Chong Kun
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.21 no.1
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    • pp.15-21
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    • 2021
  • Purpose: Urea cycle disorder (UCD) is an inherited inborn error of metabolism, acting on each step of urea cycle that cause various phenotypes. The purpose of the study was to investigate the long-term clinical consequences in different groups of UCD to characterize it. Methods: Twenty-two patients with UCD genetically confirmed were enrolled at Pusan National University Children's hospital and reviewed clinical features, biochemical and genetic features retrospectively. Results: UCD diagnosed in the present study included ornithine transcarbamylase deficiency (OTCD) (n=10, 45.5%), argininosuccinate synthase 1 deficiency (ASSD) (n=6, 27.3%), carbamoyl-phosphate synthetase 1 deficiency (CPS1D) (n=3, 13.6%), hyperornithinemia-hyperammonemia-homocitrullinuria syndrome (HHHS) (n=2, 9.1%), and arginase-1 deficiency (ARG1D) (n=1, 4.5%). The age at the diagnosis was 32.7±66.2 months old (range 0.1 to 228.0 months). Eight (36.4%) patients with UCD displayed short stature. Neurologic sequelae were observed in eleven (50%) patients with UCD. Molecular analysis identified 37 different mutation types (14 missense, 6 nonsense, 6 deletion, 6 splicing, 3 delins, 1 insertion, and 1 duplication) including 14 novel variants. Progressive growth impairment and poor neurological outcomes were associated with plasma isoleucine and leucine concentrations, respectively. Conclusion: Although combinations of treatments such as nutritional restriction of proteins and use of alternative pathways for discarding excessive nitrogen are extensively employed, the prognosis of UCD remains unsatisfactory. Prospective clinical trials are necessary to evaluate whether supplementation with BCAAs might improve growth or neurological outcomes and decrease metabolic crisis episodes in patients with UCD.

Biological Toxicity Assessment of Sediment at an Ocean Dumping Site in Korea (폐기물 배출해역 퇴적물의 생물학적 독성평가 연구)

  • Seok, Hyeong Ju;Kim, Young Ryun;Kim, Tae Won;Hwang, Choul-Hee;Son, Min Ho;Choi, Ki-young;Kim, Chang-joon
    • Journal of the Korean Society of Marine Environment & Safety
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    • v.28 no.1
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    • pp.1-9
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    • 2022
  • The effect of sediments in a waste dumping area on marine organisms was evaluated using sediment toxicity tests with a benthic amphipod (Monocorophium acherusicum) and bioluminescent bacterium (Vibrio fischeri) in accordance with the Korean Standard Method for Marine Wastes (KSMMW). Nine sites in the East Sea-Byeong, East Sea-Jeong, and Yellow Sea-Byeong areas were sampled from 2016 to 2019. The test results showed that the relative average survival rate (benthic amphipods) and relative luminescence inhibition rate (luminescent bacteria) were below 30%, which were judged to be "non-toxic." However, in the t-test, a total of 12 benthic amphipod samples (6, 1, 1, and 4 in 2016, 2017, 2018, and 2019, respectively) were significantly different (p<0.05) from the control samples. To identify the source of toxicity on benthic amphipods, a simple linear regression analysis was performed between the levels of eight heavy metals (Cr, As, Ni, Cd, Cu, Pb, Zn, and Hg) in sediments and the relative average survival rate. The results indicated that Cr had the highest contribution to the toxicity of benthic amphipods (p = 0.000, R2 = 0.355). In addition, Cr was detected at the highest concentration at the DB-85 station and exceeded the Marine Environment Standards every year. Although the sediments were determined as "not toxic" according to the ecotoxicity criteria of the KSMMW, the results of the statistical significance tests and toxicity identification evaluation indicated that the toxic effect was not acceptable. Therefore, revising the criteria for determining the toxic effect by deriving a reference value through quantitative risk assessment using species sensitivity distribution curves is necessary in the future.

Comparison of Anti-inflammatory, Skin Barrier Improvement, and Anti-aging Efficacy of Eleutherococcus divaricatus var. chiisanensis and various Eleutherococcus Genus Extract (지리산오갈피, 가시오갈피, 오갈피나무, 오가나무 추출물의 항염증, 피부장벽개선, 항노화 효능 비교)

  • Jiwon, Han;Bomi, Nam;Beom seok, Lee;Jin-A, Ko;Jiyoung, Hwang
    • Journal of the Society of Cosmetic Scientists of Korea
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    • v.48 no.4
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    • pp.373-383
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    • 2022
  • Inflammation caused by active oxygen and the resulting barrier damage have been consistently pointed out as the cause of wrinkle formation. In this study, effective index ingredient search and efficacy analysis were performed to verify the value of use as a functional cosmetic material related to antioxidant, anti-inflammatory and skin barrier improvement, and anti-aging for extracts of four types of Eleutherococcus divaricatus var. chiisanensis (ED), Eleutherococcus senticosus (EN), Eleutherococcus sessiliflorus (ES), and Eleutherococcus sieboldianus (EI) belonging to the Eleutherococcus genus. To identify the effective index composition, the content of the ingredients was measured by high-performance liquid chromatography. The content of eleutheroside E and chlorogenic acid was the highest in ED among the Eleutherococcus genus. As for anti-oxidant activity, DPPH radical scavenging activity was the highest in ED. In anti-inflammatory effects, ED extracts inhibited nitric oxide generation in inflammatory macrophage cells due to lipopolysaccharide by 40% at 100 ㎍/mL. In the case of IL-6 inhibition, which is known as a pro-inflammatory cytokine, ED showed 41% inhibition at 100 ㎍/mL. In addition, filaggrin and involucrin, which are skin barrier-related factors, were increased by 2.5 times and 1.6 times, respectively, in 100 ㎍/mL of ED extracts, and as for the collagenase, which is a wrinkle-related factor, ED extract showed 29% efficacy at 100 ㎍/mL. Thus, these result suggested that ED extract, among the four Eleutherococcus genus, can be used as a cosmetic ingredient for suppressing inflammation in the skin, reinforcing the skin barrier, and reducing wrinkles.

Effect of Chlorine Dioxide, Cold Plasma Gas Sterilization and MAP Treatment on the Quality and Microbiological Changes of Paprika During Storage (이산화염소 및 저온 플라즈마 가스 살균 및 MAP 처리가 파프리카의 저장 중 품질과 미생물학적 변화에 미치는 영향)

  • In-Lee, Choi;Joo Hwan, Lee;Yong Beom, Kwon;Yoo Han, Roh;Ho-Min, Kang
    • KOREAN JOURNAL OF PACKAGING SCIENCE & TECHNOLOGY
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    • v.28 no.3
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    • pp.223-229
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    • 2022
  • This study was conducted to investigate the effect of packaging methods and sterilization treatment on storability and microbial control in paprika fruits. When treated with chlorine dioxide gas for 3, 6, and 12 hours and cold plasma gas for 1, 3, and 6 hours, and then packed in a carton box and stored in a 8 ± 1℃ chamber for 7 days, chlorine dioxide treated 12 hours and plasma treated 6 hours was prevented the development of E·coli and YM(yeast and mold). Accordingly, the control was treated with chlorine dioxide for 12 hours and plasma for 6 hours, packed using a carton box and 40,000 cc·m-2·day-1·atm-1 OTR film (MAP), and stored in a 8 ± 1℃ chamber for 20 days. Fresh weight loss rate during storage was less than 1% in the MAP treatments, and the visual quality of the MAP treatments was above the marketability limit until the end of storage. There was no difference in the contents of oxygen, carbon dioxide, and ethylene in the film. In the case of firmness, the chlorine dioxide treatments was low, and the Hunter a* value, which showed chromaticity, was highest in the Plasma 6h MAP treatment. Off-odor was investigated in the MAP treatments, but it was very low. The rate of mold growth on the fruit stalk of paprika was the fastest and highest in the chlorine dioxide treated box packaging treatments, and the lowest in the chlorine dioxide treated MAP treatments at the end of storage. The aerobic count in the pulp on the storage end date was the lowest in the plasma treated box packaging treatments, the lowest number of E·coli in the chlorine dioxide treated MAP treatments, and the lowest yeast & mold in the chlorine dioxide treated box packaging treatments. As a result, for the inhibition of microorganisms during paprika storage, it is considered appropriate to treat plasma for 6 hours before storage regardless of the packaging method.

Application of Plant Flavonoids as Natural Antioxidants in Poultry Production (가금 생산에서 천연 항산화제로서 식물성 Flavonoids의적용)

  • Kang-Min, Seomoon;In-Surk, Jang
    • Korean Journal of Poultry Science
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    • v.49 no.4
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    • pp.211-220
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    • 2022
  • Poultry are exposed to extremely high levels of oxidative stress as a consequence of the excessive production of reactive oxygen species (ROS) induced by endogenous and exogenous stressors, such as high-stocking densities, thermal stress, environmental and feed contamination, along with factors associated with intensive breeding systems. Oxidative stress promotes lipid peroxidation, DNA damage, and inflammation, which can have detrimental effects on the health of birds. During the course of evolution, birds have developed antioxidant defense mechanisms that contribute to maintaining homeostasis when exposed to endogenous and exogenous stressors. The primary antioxidant defense systems are enzymatic and non-enzymatic in nature and play roles in protecting cells from ROS attack. Recently, plant flavonoids, which have been established to reduce oxidative stress, have been attracting considerable attention as potential feed additives. Flavonoids are a group of polyphenolic compounds that can be stabilized by binding structural compounds with ROS, and can promote the elimination of ROS by inducing the expression of antioxidant enzymes. However, although flavonoids can contribute to reducing lipid peroxidation and thereby enhance the antioxidant capacity of birds, they have low solubility in the gastrointestinal tract, and consequently, it is necessary to develop a delivery technology that can facilitate the effect intestinal absorption of these compounds. Furthermore, it is important to determine the dietary levels of flavonoids by assessing the exact antioxidant effects in the gastrointestinal tract wherein the concentrations of dietary flavonoids are highest. It is also necessary to examine the expression of transcriptional factors and vitagenes associated with the efficient antioxidant effects induced by flavonoids. It is anticipated that the application of flavonoids as natural antioxidants will become a particularly important field in the poultry industry.

Effects of Shading Rate, Rooting Media and Plant Growth Regulators on Rooting of Veronoca L. by Cuttings (삽목 번식 시 차광정도, 삽목용토 및 생장조절제 처리가 꼬리풀(Veronica L.)의 발근에 미치는 영향)

  • Cheon Young, Song;Ja Young, Moon;Jung Won, Sung;Byeong Seon, Park;Jae Ik, Nam;Jeong Min, Kim
    • Journal of Practical Agriculture & Fisheries Research
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    • v.24 no.4
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    • pp.35-44
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    • 2022
  • This study was conducted to develop a mass production for a commercial use by cutting of 4 kinds of V. glabrifolia Kitag., V. pusanensis Y.N.Lee, V. glabrifolia Kitag. × V. Spicata 'Alba' and V. spicata 'Ulster Blue Dwarf' × V. longifolia. Veronica L. Effects of shade, media and concentrations of plant growth regulators on the rooting of Veronica L. were examined. The rooting rate of V. glabrifolia Kitag. was higher as a 60% than the V. pusanensis Y.N.Lee as a 20% in the control of commercial media. In shading treatment, the rooting rate was highest at 30% or 60% shading, and the 30% shading was the best in number of root and root length, but the 90% shading was lower than no shading treatment. For cutting media, the rock wool and 100% perlite increased the rooting rate by more than 10% compared to commercial media, and increased the number of roots by 2 or 3 times. However the Cocopeat of media was lower of rooting rate, root number, and root length compared to another treatment. In the plant growth regulator treatment, the rate of rooting increased in all treatment compared to control, and was highest at IBA 1,000mg·L-1 as a 82.2% and NAA 200 or 400mg·L-1 as a 82.2% or 84.4% respectively, in the V. glabrifolia Kitag. × V. Spicata 'Alba'. However, the root number and root length was decreased as the concentration of growth regulators increased.

Study on nutrition, dietary and health status of middle-aged Korean men according to sedentary hours: based on the 2019 Korea National Health and Nutrition Examination Survey (중장년 한국 남성의 좌식 시간에 따른 영양, 식이 및 건강행태 연구: 국민건강영양조사 제8기 1차년도(2019년) 자료를 이용하여)

  • Jeong, Dajeong;Lee, Jeehyun;Yoon, Eunju
    • Journal of Nutrition and Health
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    • v.55 no.3
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    • pp.359-375
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    • 2022
  • Purpose: Adult Korean men belonging to the main economically active population are known to have long sedentary hours. This study was undertaken to determine the difference and relevance of sedentary hours on the nutrition, diet, and health status of adult men, and to suggest how to prevent health risk factors. Methods: Subjects (n = 1,068) were classified into 4 groups based on their sedentary hours, ranging from the first quartile (Q1) having the least hours spent sitting, to the fourth quartile (Q4) spending the longest hours. Results: Subjects belonging to Q4 had the lowest average age, the largest waist circumference, and the highest level of education. Among those engaged in economic activities, the ratio of white-collar workers was significantly higher in Q4. Accordingly, the rate of not doing high-intensity or moderate-intensity physical activity while working was also the highest in Q4. A significant difference was obtained in the drinking frequency between groups, but this was found to be associated with the average working hours rather than sedentary hours. The proportion of not doing aerobic exercise was higher with longer sitting hours. The highest diagnosis of diabetes (8.8%) was obtained in the Q4 group. Among the factors related to cardiovascular disease, only low density lipoprotein-cholesterol showed a significant difference, with Q4 being significantly higher than Q1. Considering energy and nutrient intake, vitamin B1 and calcium intake were the lowest in the group with the longest sitting hours, as well as the least consumption of vitamin C than the recommended estimated average requirement. Conclusion: The results of this study suggest that the health and nutritional status of Korean adult men are affected by sedentary hours. This should be recognized as a health risk factor and guidelines need to be developed for sedentary lifestyle management.

Fertilizer Effect of Waste Nutrient Solution in Greenhouses for Young Radish Cultivation (열무 재배를 위한 시설하우스 폐양액의 비료 효과)

  • Hong, Youngsin;Moon, Jongpil;Park, Minjung;Son, Jinkwan;Yun, Sungwook
    • Journal of Bio-Environment Control
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    • v.31 no.4
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    • pp.460-467
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    • 2022
  • The purpose of this study is to enhance utilization of the waste nutrient solution (WNS) disposed at the hydroponic greenhouse. Several sets of testing were conducted to examine the effects of WNS: (a) a fertilizer effect, (b) soil column leaching, and (c) crop cultivation. The fertilizer effect test was applied in young radish cultivation by examining the growth characteristics of young radish and soil based on inorganic nitrogen according to the soil treatment of the nitrogen fertilizer (NF) and the WNS. The fertilizer effects and crop cultivation test were conducted with five treatments (A-E): A, non-treatment (water); B, 100% of NF; C, 70% of NF + 30% of WNS; D, 50% of NF + 50% of WNS; and E, 30% of NF + 70% of WNS. The soil column leaching test was conducted with three treatments: non-treatment (water), 100% of NF, 50% of WNS + 50% of NF. As a result, the chemical properties of the WNS were pH 6.0, EC 2.4dS·m-1, total phosphorus (T-P) 28mg·L-1, ammonium nitrogen (NH4-N) 5.0mg·L-1, and nitrate nitrogen (NO3-N) 301mg·L-1. The chemical properties of the soil were pH 5.51, EC 0.31dS/m, organic matter 2.08g·kg-1, NO3-N 9.64mg·kg-1, and NH4-N 3.20mg·kg-1. The results of fertilizer effects showed that the ratio of 50% or less of NF and 50% or more of WNS was high in young radish growth. There was no statistically significant difference between the soil chemistry in the C-E treatments where WNS was mixed with NF and the B treatment where only NF was applied. As a result of the soil column leaching test, there was no significant difference in the concentrations of NO3 and NH4 in the treatment of 100% of NF and 50% of NF + 50% of WNS. The study indicates, if the mixed fertilizer of WNS and NF is applied in the soil cultivation of young radish, it will reduce the use of NF and environmental pollution. This also helps reduce production costs on farmers and increase the yield of young radish.

Leaching Behavior of Vanadium and Possibility of Recovery of Valuable Metals from VTM Concentrate by Sulfuric Acid Leaching (바나듐함유 티탄철석 정광으로부터 황산 침출법에 의한 바나듐의 침출거동 및 유가금속의 회수가능성)

  • Joo, Sung-Ho;Shin, Dong Ju;Lee, Dongseok;Park, Jin-Tae;Jeon, Hoseok;Shin, Shun Myung
    • Resources Recycling
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    • v.31 no.1
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    • pp.65-77
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    • 2022
  • A study was conducted in Korea on the leaching behavior and possibility of recovery of vanadium and other valuable metals from domestic vanadium titanomagnetite (VTM) by direct acid leaching. In this study, a VTM concentrate containing 0.8% V2O5 was used, and the ratio of magnetite to ilmenite was calculated as 1.9:1 by using the HSC program. The leaching behavior of vanadium from the VTM was similar to that of iron, and it was affected by the concentration of sulfuric acid and temperature. Further, titanium could be leached in the form of TiOSO4 at a temperature higher than 75℃. To improve the leaching efficiency of V, Fe, and Ti in VTM, reductive sulfuric acid and oxidative sulfuric acid leaching were performed. When Na2SO3 was used as a reducing agent, the leaching rate of vanadium was 80% of that in that case of leaching by sulfuric acid. Similarly, the leaching rate of titanium increased from 20% to 50%. When Na2S2O8 was used as an oxidation agent, most of the vanadium was leached, and the main residue found by XRD analysis was ilmenite. In studies on the possibility of recovering valuable metals, the selective extraction of metals is hardly achieved by solvent extraction from oxidation leaching solutions; however, in this study, Cyanex 923, a solvation extractant from reductive leaching solutions, could selectively extract Ti.

A Review on Ocean Acidification and Factors Affecting It in Korean Waters (우리나라 주변 바다의 산성화 현황과 영향 요인 분석)

  • Kim, Tae-Wook;Kim, Dongseon;Park, Geun-Ha;Ko, Young Ho;Mo, Ahra
    • Journal of the Korean earth science society
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    • v.43 no.1
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    • pp.91-109
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    • 2022
  • The ocean is a significant sink for atmospheric anthropogenic CO2, absorbing one-third of the total CO2 emitted by human activities. In return, oceans have experienced significant declines in seawater pH and the aragonite saturation state also called ocean acidification. This study evaluates the distribution of aragonite saturation state, an indicator to assess the potential threat from ocean acidification, by combining newly obtained data from the west coast of South Korea with previous datasets covering the Yellow Sea, East Sea, northern South China Sea, and southeast coast of South Korea. In general, offshore waters absorb atmospheric CO2; however, most of the collected water samples show aragonite oversaturation. On the southeast coast, the aragonite saturation state was significantly affected by river discharge and associated variables, such as freshwater input with nutrients, seasonal stratification, biological carbon fixation, and bacterial remineralization. In summer, hypoxia and mixing with relatively acidic freshwater made the Jinhae and Gwangyang Bays undersaturated with respect to aragonite, possibly threatening marine organisms with CaCO3 shells. However, widespread aragonite undersaturation was not observed on the west coast, which receives considerable river water discharge. In addition, occasional upwelling events may have worsened the ocean acidification in the southwestern part of the East Sea. These results highlight the importance of investigating site-specific ocean acidification processes in coastal waters. Along with the above-mentioned seasonal factors, the dissolution of atmospheric CO2 and the deposition of atmospheric acidic substances will continue to reduce the aragonite saturation state in Korean waters. To protect marine ecosystems and resources, an ocean acidification monitoring program should be established for Korean waters.