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A Case of Urologic Manifestation of IARS2-associated Leigh Syndrome (IARS2 유전자 연관 리 증후군(Leigh syndrome) 여아에서 방광기능장애 증례)

  • Hyunjoo Lee;Ji-Hoon Na;Young-Mock Lee
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.23 no.1
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    • pp.25-30
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    • 2023
  • Leigh syndrome is a rare progressive neurodegenerative mitochondrial disorder with clinical and genetic heterogeneity. Recently, balletic IARS2 variants have been identified in a number of patients presenting broad clinical phenotypes from Leigh and West syndrome to a rare syndrome CAGSSS characterized by cataracts, growth hormone deficiency, sensory neuropathy, sensorineural hearing loss, and skeletal dysplasia syndrome (OMIM#616007). We describe a child with Korean Leigh syndrome with urologic manifestations resulting from a compound heterozygote mutation in IARS2. A 5-year-old girl visited the emergency room with a complaint of abdominal pain accompanied by abdominal distension. Abdominal-pelvic CT showed a markedly distended urinary bladder without definite obstructive lesions. She was diagnosed with neurogenic bladder dysfunction based on a urodynamic study. She had global delayed development due to neurologic regression after 6 months of age and a history of bilateral cataract surgery at the age of 2 years. Her brain magnetic resonance imaging showed symmetrically increased signal intensities in the bilateral putamen and caudate nuclei with diffuse cerebral atrophy. No gene variants were identified through whole-mitochondrial genome analysis. Whole exome sequencing was performed for diagnosis, and compound heterozygous pathogenic variants were identified in IARS2: c.2446C>T (p. Arg816Ter) and c.2450G>A (p. Arg817His). To the best of our knowledge, this is the first case report of bladder dysfunction manifestation in a patient with IARS2-related Leigh syndrome. Thus, it broadens the clinical and genetic spectrum of IARS2-associated diseases.

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Analysis of the perception degree on dental medical accident and dispute experience, psychological status and preventive education according to clinical career of dental hygienist (일부지역 치과위생사의 임상경력에 따른 치과 의료사고 및 분쟁 경험, 심리상태, 예방교육에 대한 인식정도 분석)

  • Yoon, Na-Na;Lee, Myung-Ju;Seong, Mi-Gyung
    • Journal of Korean Academy of Dental Administration
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    • v.5 no.1
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    • pp.13-21
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    • 2017
  • Objective: To understand the experience of medical accidents and disputes according to clinical career of dental hygienists. Methods: A self-administrated questionnaire survey was conducted from May 2012 to June 2012 targeting 313 dental hygienists in Gyeongsangnam-do, Republic of Korea. Data were analyzed by descriptive analysis, χ2-test, and one-way analysis of variance (ANOVA). Collected data were analyzed using SPSS 18.0. Results: 1) The group who had more than 6 years of clinical experience experienced complaints from patients (70.3%) and dental hygienists duty (30.7%); 2) Complaints were related to diagnosis, dental treatment instruments and materials, scaling, impression preparation, and prosthodontics. Pediatric patients had significantly more complaints compared to other patients. Overall, there were more frequent cases in the group with over 6 years of clinical career; 3) For the psychological state after experiencing patients' complaints, 'the process is difficult, but I forget after thinking it could be happen' had 160 respondents. Among them, 38.1% had clinical experience of more than 6 years, 37.5% had less than 3 years, and 24.4% had clinical career of 3~6 years; 4) About 73.6% of those had 3~6 years of clinical career answered that 'sometimes it is doubtful' on the issue of medical accidents and conflicts on prevention education of dental hygienists that 'there is a doubt about the issue of medical treatment or the occurrence of disputes'. About 60.0% of those who had less than 3 years of clinical experience answered that it was highly 'necessary but not urgent' on the necessity of preventive education, while 87.1% of those who had more than 6 years of clinical career answered that it was highly 'necessary but not urgent' on the necessity of preventive education. The difference between the two was not statistically significant. Conclusions: It is necessary and urgent to provide education related to prevention and countermeasures against malpractice disputes for dental hygienists.

Analysis of Tourism Popularity Using T-map Search andSome Trend Data: Focusing on Chuncheon-city, Gangwon-province (T맵 검색지와 썸트랜드 데이터를 이용한 관광인기도분석: 강원도 춘천을 중심으로)

  • TaeWoo Kim;JaeHee Cho
    • Journal of Service Research and Studies
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    • v.12 no.1
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    • pp.25-35
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    • 2022
  • Covid-19, of which the first patient in Korea occurred in January 2020, has affected various fields. Of these, the tourism sector might havebeen hit the hardest. In particular, since tourism-based industrial structure forms the basis of the region, Gangwon-province, and the tourism industry is the main source of income for small businesses and small enterprises, the damage is great. To check the situation and extent of such damage, targeting the Chuncheon region, where public access is the most convenient among the Gangwon regions, one-day tours are possible using public transportation from Seoul and the metropolitan area, with a general image that low expense tourism is recognized as possible, this study conducted empirical analysis through data analysis. For this, the general status of the region was checked based on the visitor data of Chuncheon city provided by the tourist information system, and to check the levels ofinterest in 2019, before Covid-19, and in 2020, after Covid-19, by comparing keywords collected from the web service sometrend of Vibe Company Inc., a company specializing in keyword collection, with SK Telecom's T-map search site data, which in parallel provides in-vehicle navigation service and communication service, this study analyzed the general regional image of Chuncheon-city. In addition, by comparing data from two years by developing a tourism popularity index applying keywords and T-map search site data, this study examined how much the Covid-19 situation affected the level of interest of visitors to the Chuncheon area leading to actual visits using a data analysis approach. According to the results of big data analysis applying the tourism popularity index after designing the data mart, this study confirmed that the effect of the Covid-19 situation on tourism popularity in Chuncheon-city, Gangwon-provincewas not significant, and confirmed the image of tourist destinations based on the regional characteristics of the region. It is hoped that the results of this research and analysis can be used as useful reference data for tourism economic policy making.

Assessing the Sensitivity of Runoff Projections Under Precipitation and Temperature Variability Using IHACRES and GR4J Lumped Runoff-Rainfall Models (집중형 모형 IHACRES와 GR4J를 이용한 강수 및 기온 변동성에 대한 유출 해석 민감도 평가)

  • Woo, Dong Kook;Jo, Jihyeon;Kang, Boosik;Lee, Songhee;Lee, Garim;Noh, Seong Jin
    • KSCE Journal of Civil and Environmental Engineering Research
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    • v.43 no.1
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    • pp.43-54
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    • 2023
  • Due to climate change, drought and flood occurrences have been increasing. Accurate projections of watershed discharges are imperative to effectively manage natural disasters caused by climate change. However, climate change and hydrological model uncertainty can lead to imprecise analysis. To address this issues, we used two lumped models, IHACRES and GR4J, to compare and analyze the changes in discharges under climate stress scenarios. The Hapcheon and Seomjingang dam basins were the study site, and the Nash-Sutcliffe efficiency (NSE) and the Kling-Gupta efficiency (KGE) were used for parameter optimizations. Twenty years of discharge, precipitation, and temperature (1995-2014) data were used and divided into training and testing data sets with a 70/30 split. The accuracies of the modeled results were relatively high during the training and testing periods (NSE>0.74, KGE>0.75), indicating that both models could reproduce the previously observed discharges. To explore the impacts of climate change on modeled discharges, we developed climate stress scenarios by changing precipitation from -50 % to +50 % by 1 % and temperature from 0 ℃ to 8 ℃ by 0.1 ℃ based on two decades of weather data, which resulted in 8,181 climate stress scenarios. We analyzed the yearly maximum, abundant, and ordinary discharges projected by the two lumped models. We found that the trends of the maximum and abundant discharges modeled by IHACRES and GR4J became pronounced as changes in precipitation and temperature increased. The opposite was true for the case of ordinary water levels. Our study demonstrated that the quantitative evaluations of the model uncertainty were important to reduce the impacts of climate change on water resources.

Analysis of Factors Affecting the Length of Stay in Children(Aged 0 to 12) with Injuries: Centering Around the Data from the Korea National Hospital Discharge In-Depth Injury Surveys (어린이(0-12세) 손상환자의 재원일수에 미치는 요인분석: 퇴원손상심층자료를 중심으로)

  • Lee Chae Kyung
    • The Journal of the Convergence on Culture Technology
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    • v.9 no.3
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    • pp.137-143
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    • 2023
  • This study was conducted to analyze factors affecting the length of stay in children with injuries by determining relationships between length of stay and characteristics of children(aged 0 to 12) with injuries. 7,804 patients aged 0 to 12 who participated in the Korea Nation Hospital Discharge In-Depth Injury Surveys, got a diagnosis of sequelae of injuries and of other consequences of external causes(S00-T98), and were discharged between 1 January 2016 and 31 December 2020 were investigated. A frequency analysis, independent samples t-test, and ANOVA were performed. Also, to identify factors affecting the length of stay, a regression analysis was performed. The average length of stay for the patients investigated in this study was 5.5 days. The length of stay for school-age children(aged 7 to 12) and children who had either public or private coverage was higher than that for preschoolers(aged 0 to 6) and children who didn't have public or private coverage, respectively. The length of stay for children admitted to a hospital in a rural area(Jeolla-do or Gyeongsang-do) was higher than that for children admitted to a hospital in a metropolitan area and the length of stay for children admitted to a hospital that had 100-299 hospital beds was relatively long. However, children who first visited a hospital for outpatient care stayed relatively short in hospital and children who had been burned or injured in traffic crashes stayed relatively long in hospital. Children who got a secondary diagnosis and had a principal procedure or who died after being discharged were in hospital for a long time. The findings of this study shall be useful, as they identified characteristics related to the length of stay for Korean children with injuries and factors that determine the length of stay for those children by analyzing the national dataset, or more specifically, the data from the Korea National Hospital Discharge In-Depth Injury Surveys. The risk of child injuries can be easily reduced by taking actions to prevent them and providing safety education programs. The present study has provided essential baseline data for the provision of aggressive care for child injuries and the establishment of a range of policies for child injury prevention.

A study on the field tests and development of quantitative two-dimensional numerical analysis method for evaluation of effects of umbrella arch method (UAM 효과 평가를 위한 현장실험 및 정량적 2차원 수치해석기법 개발에 관한 연구)

  • Kim, Dae-Young;Lee, Hong-Sung;Chun, Byung-Sik;Jung, Jong-Ju
    • Journal of Korean Tunnelling and Underground Space Association
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    • v.11 no.1
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    • pp.57-70
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    • 2009
  • Considerable advance has been made on research on effect of steel pipe Umbrella Arch Method (UAM) and mechanical reinforcement mechanism through numerical analyses and experiments. Due to long analysis time of three-dimensional analysis and its complexity, un-quantitative two-dimensional analysis is dominantly used in the design and application, where equivalent material properties of UAM reinforced area and ground are used, For this reason, development of reasonable, theoretical, quantitative and easy to use design and analysis method is required. In this study, both field UAM tests and laboratory tests were performed in the residual soil to highly weathered rock; field tests to observe the range of reinforcement, and laboratory tests to investigate the change of material properties between prior to and after UAM reinforcement. It has been observed that the increase in material property of neighboring ground is negligible, and that only stiffness of steel pipe and cement column formed inside the steel pipe and the gap between steel pipe and borehole contributes to ground reinforcement. Based on these results and concept of Convergence Confinement Method (CCM), two dimensional axisymmetric analyses have been performed to obtain the longitudinal displacement profile (LDP) corresponding to arching effect of tunnel face, UAM effect and effect of supports. In addition, modified load distribution method in two dimensional plane-strain analysis has been suggested, in which effect of UAM is transformed to internal pressure and modified load distribution ratios are suggested. Comparison between the modified method and conventional method shows that larger displacement occur in the conventional method than that in the modified method although it may be different depending on ground condition, depth and size of tunnel, types of steel pipe and initial stress state. Consequently, it can be concluded that the effect of UAM as a beam in a longitudinal direction is not considered properly in the conventional method.

Responses of Health Physical Fitness and Arterial Stiffness through Cigarette Smoking (흡연습관이 성인 남성의 건강관련체력 및 동맥경직도에 미치는 영향)

  • Jung, Min-Kyung;Park, Eun-Kyung;Yoo, Jae-Hyun
    • Journal of Korea Entertainment Industry Association
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    • v.13 no.2
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    • pp.197-205
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    • 2019
  • This study was to compare arterial stiffness and hemodynamic responses between male smokers and non-smokers. This study also investigates the influences of smoking before exercise on arterial stiffness and hemodynamic responses. 24 male subjects of age 20-29 without history of cardiorespiratory disease were divided into smokers and non-smokers. Smokers had more than 5 years of smoking experience. In order to evaluate the effects of pre-exercise smoking, smokers were tested twice, once with a cigarette before the exercise and the other once without one. Data was collected from bio-impedance analysis, SphygmoCor XCEL, graded exercise test, and fitness test. Main results of this study are as follows: First, there are differences between smokers and non-smokers in cardiorespiratory and hemodynamic response functions, as shown by maximal oxygen consumption, exercise duration, and heart rate. Second, the although the arterial stiffness between smokers and non-smokers showed statistically significant differences in the speed of the pulse wave velocity and augmentation index, smoker had a faster rate. It shows that smoking behavior has a negative impact on the cardiovascular system. Third, pre-exercise smoking behavior does have an impact on cardiorespiratory and hemodynamic response functions, as shown by exercise duration and heart rate. Lastly, arterial stiffness between smokers and non-smokers showed statistically not significant in the speed of the pulse wave velocity and augmentation index. However, the difference was not statistically significant. Brachial systolic pressure, a component of pulse wave analysis, on the other hand, was significantly dependent on pre-exercise smoking behavior. Subjects who participated in this study are college students in early 20s. Given their relatively short history of smoking, it is possible that their smoking habits are not severe enough to develop into cardiorespiratory or cardiovascular diseases. But Smokers showed lower levels of cardiopulmonary functions, as maximal oxygen consumption and exercise duration than nonsmokers.

Development and Validation of Classroom Problem Behavior Scale - Elementary School Version(CPBS-E) (초등학생 문제행동선별척도: 교사용(CPBS-E)의 개발과 타당화)

  • Song, Wonyoung;Chang, Eun Jin;Choi, Gayoung;Choi, Jae Gwang;ChoBlair, Kwang-Sun;Won, Sung-Doo;Han, Miryeung
    • Korean Journal of School Psychology
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    • v.16 no.3
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    • pp.433-451
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    • 2019
  • This study aimed to develop and validate the Classroom Problem Behavior Scale - Elementary School Version (CPBS-E) measure which is unique to classroom problem behavior exhibited by Korean elementary school students. The focus was on developing a universal screening instrument designed to identify and provide intervention to students who are at-risk for severe social-emotional and behavioral problems. Items were initially drawn from the literature, interviews with elementary school teachers, common office discipline referral measures used in U.S. elementary schools, penalty point systems used in Korean schools, 'Green Mileage', and the Inventory of Emotional and Behavioral Traits. The content validity of the initially developed items was assessed by six classroom and subject teachers, which resulted in the development of a preliminary scale consisting of 63 two-dimensional items (i.e., Within Classroom Problem Behavior and Outside of Classroom Problem Behavior), each of which consisted of 3 to 4 factors. The Within Classroom Problem Behavior dimension consisted of 4 subscales (not being prepared for class, class disruption, aggression, and withdrawn) and the Outside of Classroom Problem Behavior dimension consisted of 3 subscales (rule-violation, aggression, and withdrawn). The CPBS-E was pilot tested on a sample of 154 elementary school students, which resulted in reducing the scale to 23 items. Following the scale revision, the CPBS-E was validated on a sample population of 209 elementary school students. The validation results indicated that the two-dimensional CPBS-E scale of classroom problem behavior was a reliable and valid measure. The test-retest reliability was stable at above .80 in most of the subscales. The CPBS-E measure demonstrated high internal consistency of .76-.94. In examining the criterion validity, the scale's correlation with the Teacher Observation of Classroom Adaptation-Checklist (TOCA-C) was high and the aggression and withdrawn subscales of the CPBS-E demonstrated high correlations with externalization and internalization, respectively, of the Child Behavior Checklist - Teacher Report Form CBCL-TRF). In addition, the factor structure of the CPBS-E scale was examined using the structural equation model and found to be acceptable. The results are discussed in relation to implications, contributions to the field, and limitations.

The Effects of Characteristics of Mobile Coupon Service on Consumers' Intention of Using Mobile Coupons (모바일 쿠폰서비스의 특성이 소비자의 쿠폰이용의도에 미치는 영향과 자기해석의 조절효과에 관한 연구)

  • Jeong, Seong Min;Kim, Sang Hee;Cho, Seong Do
    • Asia Marketing Journal
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    • v.13 no.3
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    • pp.103-134
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    • 2011
  • The recent economic recession and price rise reduces excessive consumption as a whole. So companies take more interest in and use discount coupons as a means of sales promotion to reinforce their competitiveness. The combination of Internet and mobile communication technology leads to an explosive increase in the use of mobile Internet service, which promotes commercialization of mobile coupons. Nevertheless, there are absolutely insufficient researches on mobile coupons than those on paper ones. In this context, this study tries to consider intention of accepting and using mobile coupons. The innovated Technology Acceptance Model (TAM) was used to see factors of using mobile coupons considered important by customers. Through the combination of characteristics of mobile coupon service and values obtained from mobile coupons, effects of variables to enhance intention of using mobile coupons were empirically analyzed. In particular, this study suggested importance of psychological as well as economic values of mobile coupons and emphasized good considerations of the psychological aspect, such as shame, stinginess, and reputation sensitivity, in using mobile coupons as an important factor for intention of using the coupons. Another empirical analysis was made of what moderating roles consumers' self-construalplayed in the effects of mobile coupon values perceived by consumers on intention of using coupons. As a result, immediate connectivity and situational provision among characteristics of mobile coupon service were found to affect ease and usability. It was also shown that perceived ease and usability had significant effects on both economic and psychological values, which then had significant effects on intention of using a mobile system. After testing moderating effects of self-construal, the degree of effects of perceived mobile coupon values on intention of using mobile coupons was greater among inter-dependent self-construal users than among independent ones. This study considered various schemes of improving intention to use mobile coupons and provided a foundation to help companies make a strategy for mobile coupons to be activated in the future.

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Cultural Characteristics and Artificial Cultivation of Edible Mushroom, Clitocybe maxima (흰깔대기버섯의 배양적 특성 및 인공재배에 관한 연구)

  • Kim, M.K.;Kim, D.U.;Kang, H.W.;Seo, G.S.
    • Journal of Practical Agriculture & Fisheries Research
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    • v.20 no.1
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    • pp.35-47
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    • 2018
  • A edible mushroom, Clitocybe maxima (Lentinus giganteusis) commercially cultivated in China and Taiwan. However, the researches of cultivation and cultural characteristics were not reported in Korea. In this study, we conducted on cultural characteristics and artificial cultivation of C. maxima. Six isolates were collected from China(3 isolates, commercial strain), Taiwan(1 isolate, commercial strain) and Korea(2 isolates, wild type). C. maxima and L. giganteus collected in China and Taiwan, respectively, are the same in China and are estimated to be of the same species as cultured characteristics. The mycelial growth of the collected strains was not significantly different in agar medium but it showed the best growth in YPMG in liquid culture. Optimum temperature for mycelial growth and induction of fruit body were 25℃ and 30℃, respectively. In order to artificial cultivation of C. maxima, cultural characteristics and artificial cultivation were carried out using agricultural by-products and forestry by-products materials. Mycelial growth was suitable in rice straw, cottonwood sawdust, corncob and rice seed medium, and it was selected as a cultivation medium. The suitable medium for artificial cultivation of C. maxima was selected to mixed medium 2(compounding ratio(v/v): 55% of hardwood sawdust, 5% of cottonseed pellets, 10% of cottonseed, 15% of beet pulp, 15% of swollen rice husks). It took about 30 days to be able to harvest, it was faster than oyster mushrooms. The cultivation period was about 30days. A isolate, CMA-002 was not initiation to fruit body primordiuma on the used cultivation substrate. Other 5 isolates were initiate and development to fruit body on the substrate used in this study. The strain CMA-003 was initiated to be fruiting body by 8~10 days after induction of fruiting body in all of the substrates. Isolate CMA-003 was generate to a bundle fruit body. Other isolates, however, were form fruit body individually. The CMA-003 strain was likely highly recommendable strains for farming. The optimum conditions for the induction and growth of C. maxima fruit body were 25~30℃, 8 hr illumination per day with white fluorescent lamp, 90~95% relative humidity, and 1,500 ppm of CO2 concentration in a cultivation room.