• Title/Summary/Keyword: Milestones

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Novel Mutations in the CPT1A Gene Identified in the Patient Presenting Jaundice as the First Manifestation of Carnitine Palmitoyltransferase 1A Deficiency

  • Choi, Jong Sub;Yoo, Hyeoh Won;Lee, Kyung Jae;Ko, Jung Min;Moon, Jin Soo;Ko, Jae Sung
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.19 no.1
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    • pp.76-81
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    • 2016
  • Carnitine palmitoyltransferase 1A (CPT1A) is an enzyme functioning in mitochondrial fatty acid oxidation (FAO) of the liver. Patients with CPT1A deficiency have impaired mitochondrial FAO and display hypoketotic hypoglycemia and hepatic encephalopathy as typical manifestations. In this report, we present a case of CPT1A deficiency presenting jaundice as the first manifestation. A 1.9 years old boy showed jaundice and elevated levels of free and total carnitine were observed. From direct sequencing analysis of CPT1A, two novel mutations, c.1163+1G>A and c.1393G>A (p.Gly465Arg), were identified. At the age of 2.2 years, hypoglycemia, tachycardia, and altered mental status developed just after cranioplasty for craniosynostosis. High glucose infusion rate was required for recovery of his vital signs and mentality. Diet rich in high carbohydrate, low fat and inclusion of medium chain triglyceride oil resulted in improvement in cholestatic hepatitis and since then the boy has shown normal growth velocity and developmental milestones to date.

A Study on ICT Technology Leading Change of Unmanned Store (무인판매점 변화를 리드하는 ICT 기술에 대한 연구)

  • Lee, Seong-Hoon;Lee, Dong-Woo
    • Journal of Convergence for Information Technology
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    • v.8 no.4
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    • pp.109-114
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    • 2018
  • In general, the simple items we need to live in are purchased through retail stores such as supermarkets near our home. In the store, not only the items but also the management personnel and the payment instruments for the store management are located in one space called the store. Such a general store environment is gradually changing into an 'unmanned market' as a result of the development and fusion of information and communication technology (ICT). An unmanned market is an environment in which no one runs a market as the word has. An example of a typical change is Amazon's Unofficial Amazon Store. In addition, the usage and prospects of unmanned market in China are growing very meaningfully. In this study, the present situation of the unmanned market is examined in the US and China markets, and the development prospects are described. It also describes the key milestones necessary for the unmanned market.

A Comparative Study on Strategic Plans of National Libraries (국가도서관의 전략적 계획 비교연구)

  • Lee, Yong-Jae
    • Journal of Digital Convergence
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    • v.16 no.12
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    • pp.71-76
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    • 2018
  • The purpose of this study is to suggest ways to improve development plan of the National Library of Korea, comparing development plan of the Library and strategic plans of national libraries in western countries, China and Japan. For this purpose, plans of national libraries in Korea and Overseas were collected and analyzed. And a rubric evaluation was applied to the case plans. The findings indicate that plan of the National Library of Korea is written for analyzing present problems, setting practical activity-centered detailed plans. On the other hand, plans of national libraries overseas present distinct mission and vision statement, and set up goals, objectives, concrete activities in the framework, which function as milestones for developing libraries. As a conclusion, this study suggests that the National Library of Korea need mission and vision statement, links among mission, vision, goals, objectives, activities, and ongoing library marketing. This study can contribute for the National Library of Korea to develop plan with the concept of strategic plan.

Developing a Competency-based Dental Curriculum in Korea

  • Ji, Young-A;Lee, Jaeil;Baek, Seungho
    • The Journal of the Korean dental association
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    • v.57 no.8
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    • pp.437-447
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    • 2019
  • Purpose: In recent years, efforts to improve the dental curriculum in South Korea have focused on a shift to outcome-based dental education based on core competencies in dentistry. So far, the field has seen various studies on the development of competencies, performance evaluation, and the importance of outcome-based education, but few studies have documented the development of such an education model. Therefore, this study develops an OBE curriculum for dentistry education and describes the development procedures and then finally this study intends to share our experience to other dental schools. Methods: This study introduces the development procedure and details of an outcome-based education model for dental education and presents the five stages of an outcome-based education model. In this study, 3 educational experts and 2 dental professor composed the TFT and developed the research method according to the ADDIE model. Step 1 is to conduct quantitative / qualitative research analysis through some survey and interview, Step 2 is to do a survey to revise competency, Step 3 is to develop a materials through consensus and participation of our professors of the dental school, Step 4 is to do some workshops, Step 5 is to prepare and conduct a outcome evaluation. Results: Step 1 is a required process for developing an educational model: the Job Analysis & Need Analysis stage. Step 2 is the Development of Outcome and Competency stage, which involves revising the competencies that are the basis of the curriculum. Step 3 is developing competency descriptions, competency levels, and evaluation criteria?the Development of Outcomes and Evaluation Standards. Step 4 is the Development of Milestones for Curriculum and Instructional Strategy, which examines the curriculum's problems and analyzes the improvements of each course. Step 5 is the Evaluating Outcomes stage, conducted based on the competencies specified by the target dental school. Conclustion: The model presented here can serve as a foundation for outcome-based education in other dental schools.

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The Effect of Comprehensive Art Therapy on Physical Performance and Activities of Daily Living in Children with Cerebral Palsy

  • Baek, Suejung;Lee, Myeungsu;Yang, Chungyong;Yang, Jisu;Kang, Eunyeong;Chong, Bokhee
    • Journal of The Korean Society of Integrative Medicine
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    • v.7 no.3
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    • pp.51-59
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    • 2019
  • Purpose : To evaluate the effect of comprehensive art therapy on physical function and activities of daily living in children with cerebral palsy (CP). Methods : Ten ambulant children with diplegic (n=8) or hemiplegic (n=2) CP participated in this study. All were randomly assigned to either the art therapy group (n=5) or the control group (n=5). Both groups received physical therapy based on neurodevelopmental techniques for 20 minutes a day, 1 day a week, for a period of 12 weeks. Children in the art therapy group received additional comprehensive art therapy for 70 minutes once a week for 3 months. Tests for various measurements-Motricity Index (MI) for strength, Trunk Control Test (TCT) for trunk ability, Gross Motor Function Measure (GMFM) and Gross Motor Function Classification System (GMFCS) for gross motor function, Denver Developmental Screening Test-II (DDST-II) for developmental milestones, Functional Independence Measure of Children (WeeFIM) for abilities to complete daily activities, Leg and Hand Ability Test (LHAT) for limb function-were performed before and after treatments. Results : The upper extremity and whole extremity strengths of MI, self-care and total scores of WeeFIM, and leg and arm functions of LHAT improved significantly only for individuals in the art therapy group after the art therapy (p<.05). The value of MI after treatment was at the upper extremity and whole extremity strengths the leg function of LHAT was also significantly improved compared to the control group (p<.05). Conclusion : This study revealed that comprehensive art therapy along with physiotherapy was effective in increasing upper extremity strength and leg ability in children with CP. This suggests that comprehensive art therapy may be a useful adjunctive therapy for children with CP.

Development of a Play Toolkit for Supporting Infant and Toddler Development in Kyrgyzstan with a Focus on the Symbolic Functioning and Fine Motor Development of Infants 6 to 36 Months Old (키르기스스탄 영유아 성장발달을 위한 놀이도구 개발 및 사용효과 평가: 6~36개월 영유아의 상징적 기능 사고와 미세운동 발달 중심으로)

  • Chun, Hyojin;Do, Soyeon;Jung, Soyoon;Jin, Su hyun;Shin, Hyunsook
    • Child Health Nursing Research
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    • v.25 no.2
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    • pp.154-164
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    • 2019
  • Purpose: The study aimed to develop a play toolkit to facilitate infants' and toddlers' symbolic thought and fine motor development. Methods: This study used a methodological study design including two phases of development and evaluation. After reviewing the play culture and developmental health issues in Kyrgyzstan through a literature review and interviews with local experts and parents, the toolkit was developed and evaluated using content validity and utilization tests. Results: The toolkit was based on Westby's symbolic play and the fine motor milestones in the Bright Futures Guidelines. The Toolkits were composed of an overall suggested play schedule according to the child's age, four kinds of play props, and a utilization guidebook for parents. The play props were a felt book, sorting and assembling blocks, cup blocks, and a tangram. The guidebook contained age-appropriate operating methods and alternative ways to use the materials. Conclusion: A play toolkit was developed to enhance nurturing practices among parents of infants and toddlers. The findings may help facilitate effective interactions between parents and their children. Considering that nurturing care is critical for achieving better child health outcomes, enhancing parenting resources and parent-child relations could function as an effective strategy for promoting child health.

A case of TBC1D32-related ciliopathy with novel compound heterozygous variants

  • Ahn, Ji Ye;Kim, Soo Yeon;Lim, Byung Chan;Kim, Ki Joong;Chae, Jong Hee
    • Journal of Genetic Medicine
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    • v.18 no.1
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    • pp.64-69
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    • 2021
  • Primary cilium has a signal transduction function that is essential for brain development, and also determines cell polarity and acts as a mediator for important signaling systems, especially the Sonic Hedgehog (SHH) pathway. TBC1D32 is a ciliary protein, implicated in SHH signaling. Biallelic mutations in the TBC1D32 gene causes a kind of ciliopathy, heterogeneous developmental or degenerative disorders that affect multiple organs, including the brain. Here we report a boy who carried compound heterozygous variants in TBC1D32. The patient showed hypotonia, respiratory difficulty, and multiple anomalies at his birth. He was diagnosed with congenital hypopituitarism and treated with T4, hydrocortisone, and growth hormone. Despite the hormonal replacement, the patient needed long-term respiratory support with tracheostomy and nutritional support with a feeding tube. His developmental milestones were severely retarded. Hydrocephalus and strabismus developed and both required surgery, during the outpatient follow-up. Whole-exome sequencing indicated compound heterozygous variants, c.2200C>T (p.Arg734*) and c.156-1G>T, in TBC1D32 gene. This is the first Korean case of TBC1D32-related ciliopathy and we reported detailed and sequential clinical features. This case demonstrated the utility of whole-exome sequencing and provided valuable clinical data on ultra-rare disease.

Patient-Centered Doctor's Competency Framework in Korea (한국의 환자중심 의사 역량 연구)

  • Jeon, Woo-Taek;Jung, Hanna;Kim, Young-Jon;Kim, Chanwoong;Yune, Sojung;Lee, Geon Ho;Im, Sunju;Lee, Sun-Woo
    • Korean Medical Education Review
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    • v.24 no.2
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    • pp.79-92
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    • 2022
  • With increasing demands for medical care by society, the medical system, and general citizens and rapid changes in doctor's awareness, the competencies required of doctors are also changing. The goal of this study was to develop a doctor's competency framework from the patient's perspective, and to make it the basis for the development of milestones and entrustable professional activities for each period of medical student education and resident training. To this end, a big data analysis using topic modeling was performed on domestic and international research papers (2011-2020), domestic newspaper articles (2016-2020), and domestic social networking service data (2016-2020) related to doctor's competencies. Delphi surveys were conducted twice with 28 medical education experts. In addition, a survey was conducted on doctor's competencies among 1,000 citizens, 407 nurses, 237 medical students, 361 majors, and 200 specialists. Through the above process, six core competencies, 16 sub-competencies, and 47 competencies were derived as subject-oriented doctor's competencies. The core competencies were: (1) competency related to disease and health as an expert; (2) competency related to patients as a communicator; (3) competency related to colleagues as a collaborator; (4) competency related to society as a health care leader (5) competency related to oneself as a professional, and (6) competency related to academics as a scholar who contributes to the development of medicine.

Development of Ground Monitoring and Control System for Korea Augmentation Satellite System

  • Daehee Won;Chulhee Choi;Eunsung Lee;Hantae Cho;Dongik Jang;Eunok Jang;Heetaek Lim;Ho Sung Lee;Jungja Kim;Joohap Choi
    • Journal of Positioning, Navigation, and Timing
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    • v.12 no.2
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    • pp.185-200
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    • 2023
  • The Korea Augmentation Satellite System (KASS) is the first satellite navigation enhancement system in Korea developed in compliance with international standards. Technologies accumulated during the development process should be spread to industries such as academia and serve as the basis for developing the domestic satellite navigation field. This paper introduces the development process from design to implementation, testing, and verification of KASS control systems (KCS). First, development standards, milestones, requirements, and interface standards are presented as KCS development methods, and major functional design, physical design, and hardware/software implementation are described based on the allocated requirements. Subsequently, the verification environment, procedures, and results of the development product are covered and the developed operational and maintenance procedures are described. In addition, based on the experience gained through the development, suggestions were made for beneficial technology development and organization when promoting satellite navigation projects in the future. Since this work has important historical value for the development of domestic satellite navigation, it is expected that the development results will be shared with academia and industry in the future and be used as basic data for similar development.

A Study on the Concept of Military Robotic Combat Using the 4th Industrial Revolution Technology (4차 산업혁명 기술을 활용한 군사로봇 전투개념 연구)

  • Sang-Hyuk Park;Seung-Pil Namgung;Sung-Kwon Kim
    • The Journal of the Convergence on Culture Technology
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    • v.9 no.5
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    • pp.397-401
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    • 2023
  • The study presents milestones for the Korean military to win the future battlefield based on the 4th Industrial Revolution. Chapter 1 deals with the necessity of research on how advanced countries operate industrial technology in the defense sector based on the 4th Industrial Revolution. Chapter 2 examines the current technology status of the 4th Industrial Revolution in Korea and the concept of Korean combat. Chapter 3 analyzes the military robotic technology of advanced military countries through examples of unmanned combat robots in the United States, Israel, and Germany. In the end, in future battles, it will be possible to dominate the battlefield only by taking a leap into a super-connected and super-intelligent military based on a high-tech platform. Our military should also research and develop military robotics in accordance with the characteristics of each combat system, and further expand and develop the concept of combat performance to protect our core capabilities and centers from enemy cyber, electronic warfare, and space attacks.