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Molecular and Phenotypic Characteristics of Patients with Pseudohypoparathyroidism: Single Center's Experience (가성부갑상선기능저하증 환자의 분자유전학적 및 임상적 특징: 단일기관의 경험)

  • Kim, Min-ji;Yoon, Ju Young;Yoo, Sukdong;Lee, Jun;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.7-14
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    • 2021
  • Purpose: Pseudohypoparathyroidism (PHP) is caused by genetic and epigenetic alteration in the GNAS locus, and characterized by the resistance to multiple hormones and the Albright's hereditary osteodystrophy (AHO) phenotype. This study investigated the phenotypic characteristics and molecular features of PHP. Methods: Eight patients who diagnosed as PHP were enrolled at Pusan National University Children's hospital and clinical features, biochemical and genetic findings were retrospectively reviewed. Results: Of a total of 8 patients, 5 were diagnosed with PHP1a, and 3 were diagnosed with PHP1b. Patients with PHP1a had three different mutations in the GNAS gene, and patients with PHPIb had imprinting defect in differentially methylated regions (DMRs) of the GNAS locus. Two novel GNAS variants were identified in patients with PHP1a, including c.313-2A>T and c.1094G>A. All patients with PHP1a displayed AHO features; short stature (80%), brachydactyly (80%), a round face (80%), obesity (40%), heterotopic ossification (60%), and intellectual disability (60%), whereas only one patient (33.3%) with PHP1b showed AHO feature such as a round face. When phenotypic features between PHP1a and PHP1b patients were compared, patients with PHP1b showed a tendency of higher current height standard deviation scores (SDS) compared to patients with PHP1a, (-3.2±2.1 vs.-1.1±0.8; P=0.06) Conclusions: This study summarizes the phenotypic and genetic features of the PHP patients. Although we found considerable clinical overlap between PHP1a and PHP1b, further long-term follow-up is needed to evaluate the growth and development of children with PHP, as well as the effects of end-organ resistances to endocrine hormones.

The Air-stripping Process Conjugated with the Ultrasonic Treatment to Remove TOC in Groundwater around the LPG Underground Storage Cavern (탈기법과 초음파 처리법을 연계한 LPG 지하공동저장소 주변 오염지하수 내 TOC 제거)

  • Han, Yikyeong;Jun, Seongchun;Kim, Danu;Jeon, Soyoung;Lee, Minhee
    • Economic and Environmental Geology
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    • v.55 no.5
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    • pp.511-519
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    • 2022
  • In order to develop an air-stripping based remediation process to remove the TOC (Total Organic Carbon) in groundwater around the underground LPG storage cavern, the laboratory scale experiments at various conditions (change of air injection volume and temperature, the application of ultrasonic treatment, etc.) for two types of groundwater (initial TOC concentration of 608 mg/L and 153 mg/L, respectively). From results of experiment, as the air injection rate for stripping into groundwater increased from 2 L/min to 11 L/min and as the air-stripping time increased from 1 hour to 24 hour, the TOC removal efficiency of air-stripping increased. However, the TOC concentration of treated groundwater was higher than the discharge tolerance limit (100 mg/L) even after 24 hour stripping at the maximum air injection rate of 11 L/min. The main compounds of the TOC in groundwater were identified as methanol and propane and the long stripping time (more than 24 hour) was needed to separate the methanol from groundwater because of the affinity between water and methanol. At 20℃ and 4 L/min of air injection, the TOC removal efficiency increased to 59.1% after 24 hour air-stripping. When the temperature of groundwater increased to 30℃ and 40℃, the TOC removal efficiency increased up to 80.0% and 82.8%, suggesting that more than 24 hour air-stripping at 40℃ is needed to lower the TOC concentration to below 100 mg/L and the additional TOC removal process as well as the air-stripping is necessary. When the temperature increased to 60℃ and the ultrasonic treatment was conjugated with the air-stripping, the TOC removal efficiency increased to 87.8% within 5 hour stripping and the final TOC concentration (72.4 mg/L) was satisfied with the TOC discharge tolerance limit. The TOC removal efficiency for groundwater having low TOC concentration (153 mg/L) also showed similar removal efficiency of 89.7% (the final TOC concentration: 18.9 mg/L). Results in this study supported that the air-stripping conjugated with the ultrasonic treatment could remove successfully the TOC in groundwater around the underground LPG strorage cavern.

A Comparative Study on the Characteristics of Cultural Heritage in China and Vietnam (중국과 베트남의 문화유산 특성 비교 연구)

  • Shin, Hyun-Sil;Jun, Da-Seul
    • Journal of the Korean Institute of Traditional Landscape Architecture
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    • v.40 no.2
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    • pp.34-43
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    • 2022
  • This study compared the characteristics of cultural heritage in China and Vietnam, which have developed in the relationship of mutual geopolitical and cultural influence in history, and the following conclusions were made. First, the definition of cultural heritage in China and Vietnam has similar meanings in both countries. In the case of cultural heritage classification, both countries introduced the legal concept of intangible cultural heritage through UNESCO, and have similarities in terms of intangible cultural heritage. Second, while China has separate laws for managing tangible and intangible cultural heritages, Vietnam integrally manages the two types of cultural heritages under a single law. Vietnam has a slower introduction of the concept of cultural heritage than China, but it shows high integration in terms of system. Third, cultural heritages in both China and Vietnam are graded, which is applied differently depending on the type of heritage. The designation method has a similarity in which the two countries have a vertical structure and pass through steps. By restoring the value of heritage and complementing integrity through such a step-by-step review, balanced development across the country is being sought through tourism to enjoy heritage and create economic effects. Fourth, it was confirmed that the cultural heritage management organization has a central government management agency in both countries, but in China, the authority of local governments is higher than that of Vietnam. In addition, unlike Vietnam, where tangible and intangible cultural heritage are managed by an integrated institution, China had a separate institution in charge of intangible cultural heritage. Fifth, China is establishing a conservation management policy focusing on sustainability that harmonizes the protection and utilization of heritage. Vietnam is making efforts to integrate the contents and spirit of the agreement into laws, programs, and projects related to cultural heritage, especially intangible heritage and economic and social as a whole. However, it is still dependent on the influence of international organizations. Sixth, China and Vietnam are now paying attention to intangible heritage recently introduced, breaking away from the cultural heritage protection policy centered on tangible heritage. In addition, they aim to unite the people through cultural heritage and achieve the nation's unified policy goals. The two countries need to use intangible heritage as an efficient means of preserving local communities or regions. A cultural heritage preservation network should be established for each subject that can integrate the components of intangible heritage into one unit to lay the foundation for the enjoyment of the people. This study has limitations as a research stage comparing the cultural heritage system and preservation management status in China and Vietnam, and the characteristic comparison of cultural heritage policies by type remains a future research task.

Ecological Characteristics of Benthic Macroinvertebrates according to Stream Order and Habitat - Focused on the Ecological Landscape Conservation Area - (하천 규모와 서식지에 따른 저서성 대형무척추동물의 생태특성 - 생태·경관보전 지역을 중심으로 -)

  • Hwang, In Chul;Kwon, Soon Jik;Park, Young Jun;Park, Jin Young
    • Journal of Wetlands Research
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    • v.24 no.3
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    • pp.185-195
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    • 2022
  • This study conducted a survey over spring and autumn from 2014 to 2020 to confirm the ecological characteristics of the size of streams and habitats, centering on the ecological landscape conservation area, and a total 256 species of benthic macroinvertebrates in 105 families, 25 orders, 8 classes, and 5 phyla appeared. In terms of appearance species, by region, the rate of appearance of Ephemeroptera and Trichoptera was high in regions consisting of lotic area and the rate of appearance of Coleoptera and Odonata was high in regions consisting of lentic areas. When comparing the population of Ephemeroptera-Plecoptera-Trichoptera (EPT) groups by region, they were classified into three groups: upstream area, mainstream area, and lentic areas, and it was confirmed that the population ratio of EPT changed as it moved from upstream to downstream. As the stream order increased, the number of species and populations increased. The Shredder group (SH) tended to decrease as the size of stream increased(r=0.9925), and the Collector-Filtering (CF) tended to increase as the size of stream increased(r=0.9319). It was confirmed that the Scraper (SC) replaced each other between species with the same ecological status as it went downstream from upstream, and it is thought that the SC did not differ significantly by stream order. In order to maintain a healthy ecosystem in the designation and management of ecological landscape conservation areas, it is necessary to consider ecological factors such as competition and physico-chemistry factors such as water quality and substrate conditions. Therefore, if the competent authority designated survey areas including buffer areas that include streams and physical habitats of various sizes, it will be advantageous to the conservative area and securing more biological resources.

Analysis of Determinants of Carbon Emissions Considering the Electricity Trade Situation of Connected Countries and the Introduction of the Carbon Emission Trading System in Europe (유럽 내 탄소배출권거래제 도입에 따른 연결계통국가들의 전력교역 상황을 고려한 탄소배출량 결정요인분석)

  • Yoon, Kyungsoo;Hong, Won Jun
    • Environmental and Resource Economics Review
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    • v.31 no.2
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    • pp.165-204
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    • 2022
  • This study organized data from 2000 to 2014 for 20 grid-connected countries in Europe and analyzed the determinants of carbon emissions through the panel GLS method considering the problem of heteroscedasticity and autocorrelation. At the same time, the effect of introducing ETS was considered by dividing the sample period as of 2005 when the European emission trading system was introduced. Carbon emissions from individual countries were used as dependent variables, and proportion of generation by each source, power self-sufficiency ratio of neighboring countries, power production from resource-holding countries, concentration of power sources, total energy consumption per capita in the industrial sector, tax of electricity, net electricity export per capita, and size of national territory per capita. According to the estimation results, the proportion of nuclear power and renewable energy generation, concentration of power sources, and size of the national territory area per capita had a negative (-) effect on carbon emissions both before and after 2005. On the other hand, the proportion of coal power generation, the power supply and demand rate of neighboring countries, the power production of resource-holding countries, and the total energy consumption per capita in the industrial sector were found to have a positive (+) effect on carbon emissions. In addition, the proportion of gas generation had a negative (-) effect on carbon emissions, and tax of electricity were found to have a positive (+) effect. However, all of these were only significant before 2005. It was found that net electricity export per capita had a negative (-) effect on carbon emissions only after 2005. The results of this study suggest macroscopic strategies to reduce carbon emissions to green growth, suggesting mid- to long-term power mix optimization measures considering the electricity trade market and their role.

Association between physical activity measured using an accelerometer and arterial stiffness based on pulse wave velocity and ankle-brachial index in healthy adults (건강한 성인에서 가속도계로 측정한 신체활동과 맥파전달속도 및 상완-발목 간 혈압비에 기반한 동맥경화지표와의 관계)

  • Lee, Hyunju;Park, Kye Wol;Jun, Ha Yeon;Gwak, Ji Yeon;Kim, Eun Kyung
    • Journal of Nutrition and Health
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    • v.55 no.4
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    • pp.506-520
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    • 2022
  • Purpose: Physical activity (PA) has a beneficial effect on the prevention of arteriosclerosis in healthy adults. The purpose of this study was to analyze the relationship between PA measured using an accelerometer and arterial stiffness in healthy Korean adults. Methods: This study involved 87 subjects (36.8% women) aged 20-64 years. PA was evaluated using an accelerometer (wGT3X-BT, ActiGraph, Florida, USA) for 7 days. Based on the results of the accelerometer measurement, subjects were classified into active and inactive groups according to the World Health Organization (WHO) PA guidelines. The brachial-ankle pulse wave velocity (baPWV) and ankle-brachial index (ABI) to assess arterial stiffness were measured by a non-invasive vascular screening device (VP-1000 Plus, Omron). Results: The average age of the study subjects was 47.7 ± 11.3 years and the WHO PA guideline achievement rate was 29.9%. There was no significant difference in arterial stiffness (baPWV and ABI) between the active and inactive groups. In females, the time spent in light PA were positively correlated with ABI (r = 0.396; p < 0.05) and the number of sedentary bouts over 50 minutes was inversely correlated with ABI (r = -0.402; p < 0.05). However, there was no significant correlation between PA and arterial stiffness in males. Conclusions: The results of this study suggest that light PA and sedentary behavior have a positive correlation with arterial stiffness in females.

Effects of Nitrogen Fertilization Rate at Different Nursery Soils on Seedling Characters and Endosperm Consumption in Rice Seedling (상토 종류별 질소시비량이 벼 어린모 묘소질 및 배아양분 소모에 미치는 영향)

  • Kim, Sang-Su;Choi, Min-Gue;Lee, Seong-Yong;Yoo, Chul-Hyun;Cho, Soo-Yeon;Jun, Byung-Tae
    • KOREAN JOURNAL OF CROP SCIENCE
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    • v.41 no.5
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    • pp.514-520
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    • 1996
  • To clarify the proper nitrogen application level for rasing rice infant seedling under different nursery soil, Dongjinbyeo was raised at seedling box with different basal nitrogen level. The results are as follows. The higher the nitrogen level was, the lower emergence rate and the lower establishment rate. The establishment rate was less than 90%, when the nitrogen was more than 2g/box in hill soil and more than 1g/box in paddy soil. The more the nitrogen level was, the higher the seedling height in hill soil, but was higher in the order of N-2, 3, 1 and 0g /box. Leaf number wasn't significantly different between nusery soils and among nitrogen levels when seedling was raised more than 6 days. The endosperm survival rate was decreased as the increased nitrogen level, but wasn't different between the nursery soils. Amount of root was decreased as the increased nitrogen level in paddy soil, but was heavier in the order of N-1, 0, 2 and 3g /box in hill soil. Mat formation was better as the nitrogen level was decreased in all nursery soils. Considering the emergence rate, seedling charactors and mat formation, the proper nitrogen levels seemed to be 2g /box for hill soil and 1g /box for paddy soil.

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Growth, Yield, and Leaf-macronutrient Content of Grafted Cherry Tomatoes as Influenced by Rootstocks in Semi-forcing Hydroponics (반촉성 수경재배시 대목에 따른 방울토마토 접목묘의 생육, 수량 및 엽 내 양분 함량)

  • Hyewon Lee;Hyo Bong Jeong;Jun Gu Lee;Indeok Hwang;Deok Ho Kwon;Yul Kyun Ahn
    • Journal of Bio-Environment Control
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    • v.32 no.1
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    • pp.40-47
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    • 2023
  • There are many different types of cultivation in tomatoes for year-round production. One of them, semi-forcing cultivation is characterized by growing seedlings in winter season. If grafted seedlings are used in winter season that energy cost can be reduced, because they have tolerance to cold stress. This study was conducted to analyze the rootstock performance by measuring the growth, yield, and leaf-macronutrient content of cherry tomatoes grown in semi-forcing hydroponics. Three domestic rootstocks 'HSF4', '21LM', '21A701', and a control cultivar 'B-blocking' were grafted onto jujube-shaped cherry tomato (Lycopersicon esculentum L.) commercial cultivar 'Nonari'. The total yield per plant with grafted cherry tomato '21A701' was 3,387g, which was 11%, 22% and 24% higher than the yield with 'B-blocking', non-grafted one and 'HSF4'. The stem diameter of '21A701' was thick with 8.26mm, whereas non-grafted one was thin with 7.23mm at 160 days after transplanting. The flowering position of '21LM' was 34% and 47% higher than the flowering position of 'B-blocking' and non-grafted one at 153 days after transplanting. The NO3-N concentration in petiole sap of '21LM' was the highest with 1,746mg·L-1 and non-grafted one and 'HSF4' were the lowest with 1,252mg·L-1 and 1,245mg·L-1 at 167 days after transplanting. The results indicated that rootstock/scion combinations in cherry tomatoes can affect the plant growth, yield, and the concentration of different NO3-N in leaves at the late growth stage. Both '21A701' and '21LM' have vigorous root system, which influence the growth and yield increased.

Next Generation Lightweight Structural Composite Materials for Future Mobility Review: Applicability of Self-Reinforced Composites (미래모빌리티를 위한 차세대 경량구조복합재료 검토: 자기강화복합재료의 적용 가능성)

  • Mi Na Kim;Ji-un Jang;Hyeseong Lee;Myung Jun Oh;Seong Yun Kim
    • Composites Research
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    • v.36 no.1
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    • pp.1-15
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    • 2023
  • Demand for energy consumption reduction is increasing according to the development expectations of future mobility. Lightweight structural materials are known as a method to reduce greenhouse gas emissions and improve energy efficiency. In particular, fiber reinforced polymer composite (FRP) is attracting attention as a material that can replace existing metal alloys due to its excellent mechanical properties and light weight. In this paper, industrial applications and research trends of carbon fiber reinforced composites (CFRP, carbon FRP) and self-reinforced composites (SRC) were reviewed based on the reinforcement, polymer matrix, and manufacturing process. In order to overcome the expensive process cost and long manufacturing time of the epoxy resin-based autoclave method, which is mainly used in the aircraft field, mass production of CFRP-applied electric vehicles has been reported using a high-pressure resin transfer molding process including fast-curing epoxy. In addition, thermoplastic resin-based CFRP and interface enhancement methods to solve the recycling issue of carbon fiber composites were reviewed in terms of materials and processes. To form a perfect matrix-reinforcement interface, which is known as the major factor inducing the excellent mechanical properties of FRP, studies on SRC impregnated with the same matrix in polymer fibers have been reported. The physical and mechanical properties of SRC based on various thermoplastic polymers were reviewed in terms of polymer orientation and composite structure. In addition, a copolymer matrix strategy for extending the processing window of highly drawn polypropylene fiber-based SRC was discussed. The application of CFRP and SRC as lightweight structural materials can provide potential options for improving the energy efficiency of future mobility.

Enhancement of Bioactive Compounds in Mugwort Grown under Hydroponic System by Sucrose Supply in a Nutrient Solution (양액 내 자당 처리에 의한 수경재배 쑥의 생리활성물질 증진)

  • Moon-Sun Yeom;Jun-Soo Lee;Myung-Min Oh
    • Journal of Bio-Environment Control
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    • v.32 no.1
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    • pp.23-33
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    • 2023
  • Sucrose (suc) is a disaccharide that consists of glucose (glu) and fructose (fru). It is a carbohydrate source that acts as a nutrient molecule and a molecular signal that regulates gene expression and alters metabolites. This study aimed to evaluate whether suc-specific signaling induces an increase in bioactive compounds by exogenous suc absorption via roots or whether other factors, such as osmotic stress or biotic stress, are involved. To compare the osmotic stress induced by suc treatment, 4-week-old cultured mugwort plants were subjected to Hoagland nutrient solution with 10 mM, 30 mM, and 50 mM of suc or mannitol (man) for 3 days. Shoot fresh weight in suc and man treatments was not significantly different from the control. Both man and suc treatments increased the content of bioactive compounds in mugwort, but they displayed different enhancement patterns compared to the suc treatments. Mugwort extract treated with suc 50 mM effectively protected HepG2 liver cells damaged by ethanol and t-BHP. To compare the biotic stress induced by suc treatment, 3-week-old mugwort plants were subjected to microorganism and/or suc 30 mM with Hoagland nutrient solution. Microorganisms and/or suc 30 mM treatments showed no difference about the shoot fresh weight. However, sugar content in mugwort treated with suc 30 mM and microorganism with suc 30 mM treatment was significantly higher than that of the control. Suc 30 mM and microorganism with suc 30 mM were effective in enhancing bioactive compounds than microorganism treatment. These results suggest that mugwort plants can absorb exogenous suc via roots and the enhancement of bioactive compounds by suc treatment may result not from osmotic stress or biotic stress because of microorganism, but by suc-specific signaling.