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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 Study on the Landscape Cognition of Wind Power Plant in Social Media (소셜미디어에 나타난 풍력발전시설의 경관 인식 연구)

  • Woo, Kyung-Sook;Suh, Joo-Hwan
    • Journal of the Korean Institute of Landscape Architecture
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    • v.50 no.5
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    • pp.69-79
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    • 2022
  • This study aims to assess the current understanding of the landscape of wind power facilities as renewable energy sources that supply sightseeing, tourism, and other opportunities. Therefore, social media data related to the landscape of wind power facilities experienced by visitors from different regions was analyzed. The analysis results showed that the common characteristics of the landscape of wind power facilities are based on the scale of wind power facilities, the distance between overlook points of wind power facilities, the visual openness of the wind power facilities from the overlook points, and the terrain where the wind power facilities are located. In addition, the preference for wind power facilities is higher in places where the shape of wind power facilities and the surrounding landscape can be clearly seen- flat ground or the sea are considered better landscapes. Negative keywords about the landscape appear on Gade Mountain in Taibai, Meifeng Mountain in Taibai, Taiqi Mountain, and Gyeongju Wind Power Generation Facilities on Gyeongshang Road in Gangwon. The keyword 'negation' occurs when looking at wind power facilities at close range. Because of the high angle of the view, viewers can feel overwhelmed seeing the size of the facility and the ridge simultaneously, feeling psychological pressure. On the contrary, positive landscape adjectives are obtained from wind power facilities on flat ground or the sea. Visitors think that the visual volume of the landscape is fully ensured on flat ground or the sea, and it is a symbolic element that can represent the site. This study analyzes landscape awareness based on the opinions of visitors who have experienced wind power facilities. However, wind power facilities are built in different areas. Therefore, landscape characteristics are different, and there are many variables, such as viewpoints and observers, so the research results are difficult to popularize and have limitations. In recent years, landscape damage due to the construction of wind power facilities has become a hot issue, and the domestic methods of landscape evaluation of wind power facilities are unsatisfactory. Therefore, when evaluating the landscape of wind power facilities, the scale of wind power facilities, the inherent natural characteristics of the area where wind power facilities are set up, and the distance between wind power facilities and overlook points are important elements to consider. In addition, wind power facilities are set in the natural environment, which needs to be protected. Therefore, from the landscape perspective, it is necessary to study the landscape of wind power facilities and the surrounding environment.

A Study on Status of Landscape Architecture Industry with National Statistics (국가통계자료를 활용한 조경산업 현황 연구)

  • Choi, Ja-Ho;Yoon, Young-Kwan;Koo, Bon-Hak
    • Journal of the Korean Institute of Landscape Architecture
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    • v.50 no.5
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    • pp.40-53
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    • 2022
  • This study carried out to provide the methodology and basic status material of using Korean national statistics needed to find the actual state of the landscape architecture industry. The landscape architecture industry was classified into 'Design', 'Construction Management', 'construction', 'Maintenance & Management', 'Materials', 'Research', 'Education', and 'Administration' areas. In each field, business types were systemized and associated in accordance with Korean standard industrial classification and legislations pertinent to construction. Among them, the business types directly defined in the construction related legislations under the Ministry of Land, Infrastructure and Transport were focused on, and the establishment, association, integration, distribution, duplication, and omission of national statistics were analyzed. As a result, the business types of statistical analysis were selected. In order for commonality of statistical items and minimized error of interpretation, semantic analysis was conducted. Finally, the number of registered business types, the number of workers, and sales were selected. Based on them, the analysis framework applicable to fundamental analysis and evaluation of the actual state of the industry was proposed. Actual national statical data were applied for analysis and evaluation. In 2019, the number of registered business types related to the landscape architecture industry was 12,160, the number of workers by business type was 106,296, and the sales by business type were 8,308.5 billion KRW. The number of registered business types and the number of workers had been on the rise from 2017, whereas the sales had been on the decrease. It is required to come up with a plan for industrial development. This study was conducted with the national statistics established by multiple public institutions, so that there are limitations in securing consistency and reliability. Therefore, it is necessary to establish systematic and consistent national statistics in accordance with 「Landscaping Promotion Act」. In the future, it will planned to research application and development plans of national statistics according to subjects including park and green.

A study on the nutrient intake of the elderly in Korea based on activity limitations: data from the 2019 Korea National Health and Nutrition Examination Survey (활동제한에 따른 한국 노인의 식생활 및 영양섭취 평가: 2019년 국민건강영양조사 자료를 이용하여)

  • Kim, Soyoung;Lee, Youngmi
    • Journal of Nutrition and Health
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    • v.55 no.5
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    • pp.543-557
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    • 2022
  • Purpose: Conditions that limit activity constitute one of the important factors that threaten the health of the elderly. This study sought to analyze the dietary habits and nutritional intake status of the Korean elderly who were faced with activity limitations. Methods: Utilizing data from the 2019 Korea National Health and Nutrition Examination Survey, we classified 1,383 elderly people aged 65 and over into 2 groups: those with activity limitations (n = 222, LG) and those without (n = 1,161, NG). We conducted a comparative analysis of the general characteristics, dietary habits, intake of major food groups, energy, and major nutrients. The nutrient adequacy ratio (NAR), index of nutritional quality (INQ), and healthy eating practices were also analyzed. Complex sampling analysis was utilized for all the analyses with energy intake and socio-demographic variables adjusted. Results: The average age (73.8 years) of members of the LG was higher than those of the NG (72.6 years; p = 0.010). A higher percentage of participants in the LG lived alone (28.1%) compared to those in NG (17.2%; p = 0.004). The LG participants tended to regard their health status as "bad" or "very bad" (62.7%) more often than those in the NG (19.3%; p < 0.001). The LG subjects ate breakfast less frequently (p = 0.054) and ate out significantly less often than the NG subjects (p < 0.001). The LG subjects ate more grain (p = 0.001), fewer seeds (p = 0.014) and vegetables (p = 0.039). Carbohydrate made up a large percentage of their energy intake (p = 0.026). The LG subjects had significantly lower NARs of energy (p = 0.021), protein (p = 0.031), and riboflavin (p = 0.037). The LG participants also had a significantly lower rate of healthy eating practices (22.7%) compared to those in the NG (37.8%; p < 0.001). Conclusion: Activity-limited elderly, despite their need for appropriate dietary management, were poorly nourished. They were likely to live alone, thereby making it hard to provide them with social support. Hence, there is a need to provide nutrition education and policy support for the elderly bearing the burden of limited activity.

Changes in Chemical Components of Stagnant Water, Germination Rate and Seedling Elongation of Rice Varieties under Different Amount of Barley Straw Mulching (보리짚 피복량에 따른 논물의 화학성분 변화와 벼 발아율 및 유묘신장의 품종간 차이)

  • Cheong, Jin-Il;Kim, Bo-Kyeong;Ha, Ki-Yong;Lee, Jae-Kil;Shin, Hyun-Tak
    • KOREAN JOURNAL OF CROP SCIENCE
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    • v.41 no.5
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    • pp.505-513
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    • 1996
  • This study was conducted to investigate the variation of chemical components in stagnant water under different mulching amount of barley straw after combine harvesting and the effect of stagnent water on the germinatidn and seedling elongation at early growth stage in rice. pH was lower in barley straw mulching than non-mulching and decreased with increasing of mulched barley straw but was not different among treatments in stagnant water at 15 days after flooding. Dissolved oxygen decreased with the increasing of mulched barley straw and increased with the passing of time, while saline content increased with the increasing of mulched barley straw, showing significant difference among treatments, was peaked at 15 days after flooding and thereafter, decreased. NH4-N was higher 5 days after flooding with thicker barley straw mulching but was not different at 10 days. N $O_3$-N increased up to 10 to 15 days after flooding and increased with thicker barley straw mulching. Water soluble phosphorus and cation content ( $K^{+}$, $Ca^{2+}$, $Mg^{2+}$)were higher in thicker barley straw mulching and peaked at 10 and 15 days after flooding. Germination rate was decreased by thicker straw mulching and was high in order of medium late, medium and early maturing rice variety. Keumobyeo, Gancheokbyeo, Ilpumbyeo, Dongjinbyeo and Gyehwabyeo showed higher germination rate. Seedling elongation at early growth stage was poorer with increasing of barley. straw mulching. Ilpumbyeo had good seedling elongation in one time amount of barley straw mulching and Shinunbongbyeo, Unjangbyeo, Nonganbyeo and Dongjinbyeo had poorer, in two times.mes.

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Newly Synthesized Phosphodiesterase 4 (PDE4) Inhibitor, DWP205505, Inhibits TNF-$\alpha$ Secretion and mRNA Expression

  • Lee, Suk-Kyeong;Lee, Sun-A;Byun, Hye-Sin;Cho, Mi-La;Kim, Wan-Uk;Park, Sung-Hwan;Cho, Chul-Soo;Joo, Young-Shil;Lee, Shin-Seok;Yoo, Eun-Sook;Son, Ho-Jung;Kim, Ho-Youn
    • Journal of Microbiology and Biotechnology
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    • v.9 no.1
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    • pp.106-112
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    • 1999
  • The therapeutic potential of phosphodiesterase 4(PDE4) inhibitors in inflammatory diseases including some autoimmune diseases has been explored recently with some hopeful results. These PDE4 inhibitors are thought to show their anti-inflammatory effect by down-regulating tumor necrosis factor-a (TNF-$\alpha$) production in lymphocytes and macrophages. A high concentration of TNF-$\alpha$has been found in rheumatoid arthritis (RA) synovium and reducing TNF-$\alpha$using biological agents was proven to be an effective RA treatment. To test the possibility of using PDE4 inhibitors for RA treatment, the effects of a newly synthesized PDE4 inhibitor, DWP205505, on TNF-$\alpha$ and IL-10 production was tested in cells isolated from normal peripheral blood and rheumatoid arthritis synovial fluid. Cytokine production was assayed at the protein level by sandwich enzyme-linked immunosorbent assay (ELISA) and at the mRNA expression level by semi-quantitative RT-PCR. Another PDE4 inhibitor, RP73401, was used for comparison. DWP205505 and RP73401 had no harmful effect on cell viability up to 10 $\mu$M concentration during the 24 h culture period. DWP205505 as well as RP73401 significantly reduced TNF-$\alpha$ secretion from lipopolysaccharide (LPS)-stimulated peripheral blood mononuclear cells (pBMC) and synovial fluid mononuclear cells (SFMC). The effect of DWP205505 or RP73401 treatment on the mRNA expression of TNF-$\alpha$ was also studied in LPS-stimulated PBMC and SFMC. TNF-$\alpha$ mRNA expression was increased by LPS stimulation and both of the PDE4 inhibitors suppressed TNF-$\alpha$ mRNA expression. For interleukin-l0 (IL-l0), a little different results were obtained from PBMC and SFMC; IL-l0 secretion was unaffected by LPS stimulation and only minimally affected by both of the PDE4 inhibitors in PBMC. In unstimulated SFMC, DWP205505 and RP73401 slightly enhanced IL-10 secretion, while they reduced IL-l0 secretion from LPS-stimulated SFMC where IL-l0 secretion was a lot higher than unstimulated SFMC. These results suggest that the newly synthesized PDE4 inhibitor DWP205505 may have anti-rheumatoid arthritis activity.

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The Trend of Cigarette Design and Tobacco Flavor System Development

  • Wu, Jimmy Z.
    • Journal of the Korean Society of Tobacco Science
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    • v.24 no.1
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    • pp.67-73
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    • 2002
  • In light of addressing consumer health concern, coping with anti-tobacco movement, and promoting new product, tobacco industry is actively pursuing to make a new generation of cigarettes with low tar and nicotine deliveries, and less harmful substances. Low tar and low nicotine cigarettes increases their market shares dramatically world wide, especially in KT&G, multinational tobacco companies, EU countries, even in China regulated by CNTC to set up yearly target to lower tar and nicotine deliveries. On the other hand, to design a new cigarette with reduced harmful substances begins to gain speed. The "modified Hoffmann list" publishes thirty plus substances in tobacco leaf and main smoke stream, which is the prime suspect causing health problems. Various ways and means are developed to reduce such components including new tobacco breeds, new curing method, tobacco leaf treatment before processing, selected filtration system, innovated casing system to reduce free radicals, as well as some non conventional cigarette products. In TSRC held this year, the main topic is related to reduce tobacco specific nitrosamines in tobacco leaf. The new generation of cigarette is in the horizon. It still needs a lot help to produce commercial products with satisfied taste and aroma characters. The flavor industry is not regulated by many governments demanding which ingredients might or might not be for tobacco use. However, most of the cigarette companies self impose a list of ingredients to guide flavor suppliers to design flavors. Unfortunately, the number of ingredients in those lists is getting shorter every year. It is understandable that the health is not the only reason. Some cigarette companies are playing safe to protect the company from potential lawsuit, while others are just copying from their competitors. Moreover, it is obvious that it needs more assistance from casings and flavors to design new generation of cigarettes with missing certain flavor components in tobacco leaf and main smoke stream. These flavor components are either non-existed or at lower level at new form of cured tobacco leaf or filtered in the main smoke stream along with reduced harmful substances. The use of carbon filters and other selected filtration system poses another tough task for flavor system design. Specific flavor components are missing from the smoke analysis data, which brings a notion of "carbon taste" and "dryness" of mouth feel. It is ever more demanded by cigarette industry to flavor suppliers to produce flavors as body enhancer, tobacco notes, salivating agents, harshness reducer, and various of aromatic notes provided they are safe to use. Another trend is that water based flavor or flavor with reduced ethanol as solvent is gaining popularity. It is preferred by some cigarette companies that the flavor is compounded with all natural ingredients or all ingredients should he GMO free. The new generation of cigarettes demands many ways of new thinking process. It is also vital for tobacco industry. It reflects the real needs for the consumers that the cigarette product should be safe to use as well as bearing the taste and aroma characters smokers always enjoyed. An effective tobacco flavor system is definitely a part of the equation. The global trend of tobacco industry is like trends of any other industries lead by consumer needs, benefited with new technology availability, affected by the global economy, and subjected for various rules and regulations. Anti-tobacco organizations and media exceptionally scrutinize cigarette, as a legal commercial product. Cigarette is probably the most studied commercial product for its composition, structure, deliveries, effects, as well as its new developmental trend. Therefore, any new trend of cigarette development would be within these boundaries. This paper is trying to point out what it would be like for tobacco industry in the next few yews and what concerns the tobacco industry. It focuses mostly on the efforts to produce safer cigarettes. It is such a vital task for the tobacco industry and its affiliate industries such as cigarette papers, filters, flavors, and other materials. The facts and knowledge presented in this paper might be well known for the public. Some of the comments and predictions are very much personal opinion for a further discussion.

Geophysical Evidence Indicating the Presence of Gas Hydrates in a Mud Volcano(MV420) in the Canadian Beaufort Sea (캐나다 보퍼트해 진흙화산(MV420) 내 가스하이드레이트 부존을 지시하는 지구물리학적 증거)

  • Yeonjin Choi;Young-Gyun Kim;Seung-Goo Kang;Young Keun Jin;Jong Kuk Hong;Wookeen Chung;Sung-Ryul Shin
    • Geophysics and Geophysical Exploration
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    • v.26 no.1
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    • pp.18-30
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    • 2023
  • Submarine mud volcanos are topographic features that resemble volcanoes, and are formed due to eruptions of fluidized or gasified sediment material. They have gained attention as a source of subsurface heat, sediment, or hydrocarbons supplied to the surface. In the continental slope of the Canadian Beaufort Sea, mud volcano exists at various water depths. The MV420, is an active mud volcano erupting at a water depth of 420 meters, and it has been the subject of extensive study. The Korea Polar Research Institute(KOPRI) collected high-resolution seismic data and heat flow data around the caldera of the mud volcano. By analyzing the multi-channel seismic data, we confirmed the reverse-polarity reflector assumed by a gas hydrate-related bottom simulating reflector(BSR). To further elucidate the relationship between the BSR and gas hydrates, as well as the thermal structure of the mud volcano, a numerical geothermal model was developed based on the steady-state heat equation. Using this model, we estimated the base of the gas hydrate stability zone and found that the BSR depth estimated by multi-channel seismic data and the bottom of the gas hydrate stability zone were in good agreement., This suggests the presence of gas hydrates, and it was determined that the depth of the gas hydrate was likely up to 50 m, depending on the distance from the mud conduit. Thus, this depth estimate slightly differs from previous studies.

A Case Study on Service Philosophy : Assetization on Wisdom of the Founding President of the Republic of Korea (서비스철학 사례 연구: 대한민국 건국대통령 지혜의 자산화)

  • Hyunsoo Kim
    • Journal of Service Research and Studies
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    • v.12 no.2
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    • pp.1-22
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    • 2022
  • This study was conducted based on the service philosophy as a study to capitalize on the wisdom of Korea and Koreans. This is a study that capitalizes on the wisdom of the founding president who led civilization changes in Korea. Wisdom as a visionary leader who played a leading role in Korea's development from a continental civilization to a maritime civilization was derived. We analyzed the wisdom of the founding president, who established the Republic of Korea with a new identity as a liberal democracy and protected it, and laid the foundation for Korea to leap into an advanced country through legal system reform and human resource development. First, we analyzed the essence of politics and presented the essential tragic nature that visionary leaders must bear as politicians. We also analyzes his courage and belief to accept a tragic fate, the insights on the spirit of independence, anti-communism, liberal democracy and the future. We analyze the wisdom as a visionary leader that is consistently revealed through countless choices and decisions to give up. It suggests that such wisdom is essential for the founding and development of a nation, and the wisdom need to be assetized. It needs a solid philosophical foundation to become a useful wisdom asset in the long run. In this study, Syngman Rhee's wisdom was assetized on the basis of the service philosophy. This is because it is wisdom based on the fiercely symmetrical balance principle. Human resource development was a choice that entailed great sacrifice, the market economy was a choice that entailed great sacrifice, and the protection of liberal democracy was also a choice that entailed great sacrifice, so it can be said that it is wisdom that fits the essence of the service philosophy. It is wisdom found only in outstanding visionary leaders. We present the wisdom of building a free democratic country, the wisdom of building a free market economy, and the wisdom of building a talent-oriented nation as assets in terms of national management philosophy, national management owner, time frame, and strategic asset. It also suggests that visionary leaders are continuously required for the long-term sustainable development of mankind and Korean society.