• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.3
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• pp.99-106
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• 2018

Mucolipidosis type III (pseudo-Hurler polydystrophy) is a mucolipids degrading disorder caused by a mutation in the GNPTAB gene and is inherited by autosomal recessive. It is diagnosed by examining highly concentrated mucolipids in blood and the diagnosis can be confirmed by genetic testing. Mucolipidosis type III is a rare and progressive metabolic disorder. Its initial signs and symptoms usually occur around 3 years of age. Clinical manifestations of the disease include slow growth, joint stiffness, arthralgia, skeletal abnormalities, heart valve abnormalities, recurrent respiratory infection, distinctive facial features, and mild intellectual disability. Here, we are presenting two siblings of mucolipidosis type III, a 4-year-old female and a 2 years and 7 months old male with features of delayed growth and coarse face. The diagnosis was confirmed by [c.2715+1G>A(p.Glu906Leufs*4), c.2544del(p.Glu849Lysfs*22)] mutation in targeted gene panel sequencing. In this case, c.2544del is a heterozygote newly identified mutation in mucolipidosis type III and was not found in the control group including the genome aggregation database. And it is interpreted as a pathogenic variant considering the association with phenotype. Here, we report a Korean mucolipidosis type III patients with novel mutations in GNPTAB gene who have been treated since early childhood. Owing to recent development of molecular genetic techniques, it was possible to make early diagnosis and treatment with pamidronate was initiated appropriately in case 1. In addition to these supportive therapies, efforts must be made to develop fundamental treatment for patients with early diagnosis of mucolipidosis.

• The Korean Journal of Archival Studies
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• no.28
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• pp.35-81
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• 2011

Manhwa is a mass media (to expose all faces of an era such as politics, society, cultures, etc with the methodology of irony, parody, etc). Since the Manhwa records is primary culture infrastructure, it can create the high value-added industry by connecting with fancy, character, game, movie, drama, theme park, advertising business. However, due to lack of active and systematic aquisition system, as precious Manhwa manuscript is being lost every year and the contents hard to preserve such as Manhwa content in the form of electronic records are increasing, the countermeasure of Manhwa contents management is needed desperately. In this study, based on these perceptions, the need of Manhwa records management is examined, and the characteristics and the components of Manhwa records were analyzed. And at the same time, the functions of record management process reflecting the characteristics of Manhwa records were extracted by analyzing various cases of overseas Cartoon Archives. And then, the framework of record-keeping regime was segmented into each of acquisition management service areas and the general Manhwa records archiving strategy, which manages the Manhwa contents records, was established and suggested. The acquired Manhwa content records will secure the context among records and warrant the preservation of records and provide diverse access points by reflecting multi classification and multi-level descriptive element. The Manhwa records completed the intellectual arrangement will be preserved after the conservation in an environment equipped with preservation facilities or preserved using digital format in case of electronic records or when there is potential risk of damaging the records. Since the purpose of the Manhwa records is to use them, the information may be provided to diverse classes of users through the exhibition, the distribution, and the development of archival information content. Since the term of "Manhwa records" is unfamiliar yet and almost no study has been conducted in the perspective of records management, it will be the limit of this study only presenting acquisition strategy, management and service strategy of Manhwa contents and suggesting simple examples. However, if Manhwa records management strategy are possibly introduced practically to Manhwa manuscript repositories through archival approach, it will allow systematic acquisition, preservation, arrangement of Manhwa records and will contribute greatly to form a foundation for future Korean culture contents management.

• 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.

• Journal of Practical Agriculture & Fisheries Research
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• v.4 no.1
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• pp.128-137
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• 2002

This study was conducted to develop the heat exchanger by utilizing the heat energy of underground water(15℃), which might be used for cooling and heating system of the agricultural facilities. We developed the heat exchanger by using the parallel type plat fin tube made of Aluminum(Al 6063), which was named Aloo-Heat(No. 0247164, offered by Korean Intellectual property Office). The trial manufactures were made from Aloo-heat which was 600mm, 700mm length respectively, and It were welded to the end "U" type in order to direct flow of the underground water. The performance test was carried out under the condition of open space and room temperature with the change of flow rate of the underground water and air. The results are as follows. 1. The trial manufactures had convection heat value from 33 to 156 W/m²℃, and It was coincided with design assumption. 2. The amount of energy transfer was increased with the increment of the area of heat transfer, the air flow, the gap of temperature inlet & outlet the underground water and the air. 3. The heat value was 6,825W when the air flow was 6,000m³/h and the gap of temperature between inlet and outlet of the underground water was 6℃, and It dropped from 25.8℃ to 23.2℃(-2.6℃ difference). The convection heat value was 88.5W/m²℃. 4. The heat value was 2.625W when the air flow was 4,000m³/h and the gap of temperature between inlet and outlet the underground water was 2℃, and It dropped from 27℃ to 22.5℃(-4.5℃ difference). The convection heat value was 33.6W/m²℃. 5. Correlation values(R²) of the testing heat values of the trial manufacture type I, II, and III were 0.9141, 0.8935, and 0.9323 respectively, and correlation values(R²) of the amount of the air flow 6,000m³/h, 5,000m³/h, 4,000m³/h were 0.9513, 0.9414, and 0.9003 respectively.

• Journal of Practical Agriculture & Fisheries Research
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• v.4 no.1
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• pp.119-127
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• 2002

This study was conducted to develop the heat exchanger by utilizing the heat energy of underground water(15℃), which might be used for cooling and heating system of the agricultural facilities. We developed the heat exchanger, parallel type plat fin tube made of Aluminum(Al 6063), which was named Aloo-Heat(No. of The registration design : 0247164, by Korean Intellectual property Office). The fin of exchanger was design of the granulated surface for minimizing fouling factor and dew forms, and also placed parallel to the tube in order to minimized the resistance of flows. 1. Aloo-heat was designed to have 0.03m for inside diameter, 0.036m for outside diameter of tube, 0.0012m for thickness of fin and 0.032m for length of plat fin. 2. t was also designed to have 1.5248m²/m for outside area of heat transfer, 0.0942m²/m for inside area contacting hot liquid, and the ratio (Ra) was 16.1869. 3. Efficiency of the fin was 93 percentage when fin length was 0.032m, and the fin thickness satisfied equation $\frac{h{\rho}}{k}$< 0.2 when it was 0.0012m. 4. According to the performance test of Aloo-heat, as the temperature and rate increased, the heating value also increased, heating value was 504kJ/h·m and 6,048kJ/h·m when it was 60℃, 10 𝑙/min and 80℃, 40 𝑙/min respectively. 5. The test of heating value was confident, because correlation value(R²) was 0.9898 for the temperature and 0.9721 for flow rate of hot liquid, respectively.

• Journal of Service Research and Studies
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• v.13 no.1
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• pp.108-126
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• 2023

This study explored the sustainability of a blockchain-based cultural art performance video platform through the construction of Gyeonggi Art On, a new media art broadcasting station in Gyeonggi-do. In addition, the technical limitations of video content transaction using block chain, legal and institutional issues, and the protection of personal information and intellectual property rights were reviewed. As for the research method, participatory observation methods such as in-depth interviews with developers and operators and participation in meetings were conducted. The researcher participated in and observed the entire development process, including designing and developing blockchain nodes, smart contracts, APIs, UI/UX, and testing interworking between blockchain and content distribution services. Research Question 1: The results of the study on 'Which technology model is suitable for a blockchain-based performance video content distribution public platform?' are as follows. 1) The blockchain type suitable for the public platform for distribution of art performance video contents based on the blockchain is the private type that can be intervened only when the blockchain manager directly invites it. 2) In public platforms such as Gyeonggi ArtOn, among the copyright management model, which is an art based on NFT issuance, and the BC token and cloud-based content distribution model, the model that provides content to external demand organizations through API and uses K-token for fee settlement is suitable. 3) For public platform initial services such as Gyeonggi ArtOn, a closed blockchain that provides services only to users who have been granted the right to use content is suitable. Research question 2: What legal and institutional problems should be reviewed when operating a blockchain-based performance video distribution public platform? The results of the study are as follows. 1) Blockchain-based smart contracts have a party eligibility problem due to the nature of blockchain technology in which the identities of transaction parties may not be revealed. 2) When a security incident occurs in the block chain, it is difficult to recover the loss because it is unclear how to compensate or remedy the user's loss. 3) The concept of default cannot be applied to smart contracts, and even if the obligations under the smart contract have already been fulfilled, the possibility of incomplete performance must be reviewed.

• Information Systems Review
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• v.19 no.2
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• pp.1-19
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• 2017

Original scripts of e-learning lectures for the CEOs of corporation S were analyzed using topic analysis, which is a text mining method. Twenty-two topics were extracted based on the keywords chosen from five-year records that ranged from 2011 to 2015. Research analysis was then conducted on various issues. Promising topics were selected through evaluation and element analysis of the members of each topic. In management and economics, members demonstrated high satisfaction and interest toward topics in marketing strategy, human resource management, and communication. Philosophy, history of war, and history demonstrated high interest and satisfaction in the field of humanities, whereas mind health showed high interest and satisfaction in the field of in lifestyle. Studies were also conducted to identify topics on the proportion of content, but these studies failed to increase member satisfaction. In the field of IT, educational content responds sensitively to change of the times, but it may not increase the interest and satisfaction of members. The present study found that content production for CEOs should draw out deep implications for value innovation through technology application instead of simply ending the technical aspect of information delivery. Previous studies classified contents superficially based on the name of content program when analyzing the status of content operation. However, text mining can derive deep content and subject classification based on the contents of unstructured data script. This approach can examine current shortages and necessary fields if the service contents of the themes are displayed by year. This study was based on data obtained from influential e-learning companies in Korea. Obtaining practical results was difficult because data were not acquired from portal sites or social networking service. The content of e-learning trends of CEOs were analyzed. Data analysis was also conducted on the intellectual interests of CEOs in each field.

• Journal of Intelligence and Information Systems
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• v.24 no.1
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• pp.227-252
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• 2018

The evolution of instant communication has mirrored the development of the Internet and messenger applications are among the most representative manifestations of instant communication technologies. In messenger applications, senders use emoticons to supplement the emotions conveyed in the text of their messages. The fact that communication via messenger applications is not face-to-face makes it difficult for senders to communicate their emotions to message recipients. Emoticons have long been used as symbols that indicate the moods of speakers. However, at present, emoticon-use is evolving into a means of conveying the psychological states of consumers who want to express individual characteristics and personality quirks while communicating their emotions to others. The fact that companies like KakaoTalk, Line, Apple, etc. have begun conducting emoticon business and sales of related content are expected to gradually increase testifies to the significance of this phenomenon. Nevertheless, despite the development of emoticons themselves and the growth of the emoticon market, no suitable emoticon recommendation system has yet been developed. Even KakaoTalk, a messenger application that commands more than 90% of domestic market share in South Korea, just grouped in to popularity, most recent, or brief category. This means consumers face the inconvenience of constantly scrolling around to locate the emoticons they want. The creation of an emoticon recommendation system would improve consumer convenience and satisfaction and increase the sales revenue of companies the sell emoticons. To recommend appropriate emoticons, it is necessary to quantify the emotions that the consumer sees and emotions. Such quantification will enable us to analyze the characteristics and emotions felt by consumers who used similar emoticons, which, in turn, will facilitate our emoticon recommendations for consumers. One way to quantify emoticons use is metadata-ization. Metadata-ization is a means of structuring or organizing unstructured and semi-structured data to extract meaning. By structuring unstructured emoticon data through metadata-ization, we can easily classify emoticons based on the emotions consumers want to express. To determine emoticons' precise emotions, we had to consider sub-detail expressions-not only the seven common emotional adjectives but also the metaphorical expressions that appear only in South Korean proved by previous studies related to emotion focusing on the emoticon's characteristics. We therefore collected the sub-detail expressions of emotion based on the "Shape", "Color" and "Adumbration". Moreover, to design a highly accurate recommendation system, we considered both emotion-technical indexes and emoticon-emotional indexes. We then identified 14 features of emoticon-technical indexes and selected 36 emotional adjectives. The 36 emotional adjectives consisted of contrasting adjectives, which we reduced to 18, and we measured the 18 emotional adjectives using 40 emoticon sets randomly selected from the top-ranked emoticons in the KakaoTalk shop. We surveyed 277 consumers in their mid-twenties who had experience purchasing emoticons; we recruited them online and asked them to evaluate five different emoticon sets. After data acquisition, we conducted a factor analysis of emoticon-emotional factors. We extracted four factors that we named "Comic", Softness", "Modernity" and "Transparency". We analyzed both the relationship between indexes and consumer attitude and the relationship between emoticon-technical indexes and emoticon-emotional factors. Through this process, we confirmed that the emoticon-technical indexes did not directly affect consumer attitudes but had a mediating effect on consumer attitudes through emoticon-emotional factors. The results of the analysis revealed the mechanism consumers use to evaluate emoticons; the results also showed that consumers' emoticon-technical indexes affected emoticon-emotional factors and that the emoticon-emotional factors affected consumer satisfaction. We therefore designed the emoticon recommendation system using only four emoticon-emotional factors; we created a recommendation method to calculate the Euclidean distance from each factors' emotion. In an attempt to increase the accuracy of the emoticon recommendation system, we compared the emotional patterns of selected emoticons with the recommended emoticons. The emotional patterns corresponded in principle. We verified the emoticon recommendation system by testing prediction accuracy; the predictions were 81.02% accurate in the first result, 76.64% accurate in the second, and 81.63% accurate in the third. This study developed a methodology that can be used in various fields academically and practically. We expect that the novel emoticon recommendation system we designed will increase emoticon sales for companies who conduct business in this domain and make consumer experiences more convenient. In addition, this study served as an important first step in the development of an intelligent emoticon recommendation system. The emotional factors proposed in this study could be collected in an emotional library that could serve as an emotion index for evaluation when new emoticons are released. Moreover, by combining the accumulated emotional library with company sales data, sales information, and consumer data, companies could develop hybrid recommendation systems that would bolster convenience for consumers and serve as intellectual assets that companies could strategically deploy.

• (The)Study of the Eastern Classic
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• no.62
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• pp.145-176
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• 2016

This paper aims to define the leadership of Gunja (君子, translated into prince, gentleman, or ideal man) based on Confucian Classics which offer the general values and norms of individuals' virtue and social virtuous acts. Thus, humanitarianism is regarded as true value, and the values of a virtuous person who properly practices social human relationships are discussed. The real worth of Gunja image is discussed as a true human image of "self-completion and completion of all things" (成己成物) which involves the convergence of truth, good and beauty where there is a sense of harmony and balance, where there is stern self discipline and self cultivation and where win-win values of human relationships are created. Confucian saint (聖人), wise man (賢人), great man (大人), and gentleman (君子) mean social leaders. They practice human morals, enlighten and beautify society with teachings, and are indicated as equipped with mental and material harmony, good character and competence, and economic power and morality. People today pursue their own personal growth according to their material preferences rather than pure intellectual cultural values, and are engrossed in visually beautiful external unlimited competition. In this digital age, we are supposed to demonstrate our individuality, but many people are obsessed with appearance, go on severe diet, and lose their health beauty, and consequently suffer mental stress. This trend fuels obsession with appearance and the sick practice of valuing appearance. As an alternative method to overcome this phenomenon, we need a leader image with the convergence of truth, good and beauty, which is characterized by internal self cultivation, external professionalism, and handsome and solid character. Confucian thoughts consist in practicing the Way of disciplining oneself for governing others (修己治人). Self discipline involves developing personal virtuous ability for cultivating a virtuous character, and governing others involves interacting to work together in society and to have right human relationships. Thus, leaders should impress not only themselves but also others. Self discipline for governing others means cultivating virtue for oneself and leading others. A true leader has self introspection and establishes himself through self discipline so that he can govern others or reach the realm of settling others where people live together. As all things have a value and a virtue, humans endeavor to cultivate character and virtue by learning and studying for securing their professionalism, reliability, character and ability, so as to create their own brand value. Personal character does not come from a high position, wealth and power. Character is a personal virtue, and is cultivated as immaculate and fresh through self discipline. As such, it well matches with a clean and clear spirit. This offers the ideal leader as the Guja image who has an extremely humane character, as well as being equipped with inherent virtues of intellect, benevolence and courage. Self development can foster virtue and self management through self leadership and self discipline. The leader in the relationship area can practice his virtue through virtuous acts, in other words, even think from another person's perspective. Such leader is mentioned as the principle of measuring square in the Great Learning. In our viewpoint, the beauty of character can breed the seed of virtue through intellect, benevolence and courage, the beauty of win-win can realize the right virtue by showing exemplary acts to others through considerateness, and the beauty of harmony can love and care for others like me through the principle of measuring square, thereby realizing the universal principle of virtue and harmony, which is like my mind. As such, the ideal leader, when his virtue and mind of being considerate of others all blending well, can exercise his ability to the full, can live together and coexist with many people, and can grow again into a triumphant relationship.

• Korea Science and Art Forum
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• v.32
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• pp.319-334
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• 2018

This research is a case study to make sure the enhancement of students' imagination and creativity through developing and applying the Literature-Art-Science Integrated Education Program. Its research object was totally 25 persons of 29 students of the 1st to the 4 th Grades from Gunsan Sulsan Elementary School. Its research period lasted for 4 months from September to December, 2017, and I, as the research place, used the art room at Gunsan Sulsan Elementary School. The programs were totally 10 sessions with a unit of 1 session per each grade for 2 hours from 1:00 to 3:00 in the afternoon from Monday through Friday. I fixed ten themes of this program-eight plane modeling, and two solid modeling, and finished the work of storytelling during summer vacation. And I arranged their levels as low:middle:high(3:5:2) ones. The former was 'A Film of Monster Gorilla'(L), 'Learning the Spirit of Gyeongju Choi's Family'(M), 'A Tale of My Friend Made of Natural Materials'(L), 'The Reading of My Dream'(M), 'Gathering the Objects in My Mobile'(M), 'A Mock Trial of Marrying Off'(M), 'Painting My Favorite Children's Poem'(H), and 'Painting My Favorite Children's Song'(H), and the latter was 'Seeking for a Bluebird in My Mind'(L), and 'Making My Cherished Object' (M). Then I used the unique art expression technique per each theme, which were in sequence marbling, Korean paper art, combine painting, collage, imaginary painting, imaginary painting, play dough art, imaginary painting techniques. And I delivered to the students the scientific knowledge in terms of growing or manufacturing processes of materials used for making artworks. Prior to and after the processing this program, I surveyed about the students' ability of integrated thinking and emotional experience by 'Figure B Type' and 'Figure A Type' of The Torrance Tests of Creative Thinking, and took statistics with the resultant data. And I executed a paired t-test in order to verify the significance of mean difference in the result of investigation with those data. From the analyzed result according to the elements of creativity and the mean quotients of creativity, there showed a significant difference (t=3.47, p<.01) in 'fluency', and also a significant difference(t=3.59, p<.01) in 'creativity.' Judging from the statistic values of two fields such as the student's ability of integrated thinking and emotional experience, I estimate that over the majority of the students showed the enhancement in self-confident creative expression as well as higher interest and concern through this program. The result that I arranged and analyzed the making process of artworks, the photos of the resultant, etc. as such is as follows : Firstly, from this program being proceeded as art-centered STEAM class, the student's systematic problem-solving ability was improved in his ability of integrated thinking to transform the literary contents into artistic one. Secondly, the student obtained the emotional experience such as interest in the class, self-confidence, intellectual satisfaction, self-fulfillment, etc. through art-centered STEAM class using ten art expression techniques. Thirdly, the student's mind willing to cooperate, communicate with his friends, and care for them was ripened in the process of problem-solving. Fourth, the student's self-confidence was further instilled when presenting famous artists and their artworks in the introduction and finale of ten art expression techniques. Likewise, the statistic values on the fields of student's ability of integrated thinking and emotional experience illustrate that over the majority of the students showed improvement in the ability of creative expression with confidence as well as higher interest and concern upon this program.