• Journal of Genetic Medicine
• /
• v.19 no.2
• /
• pp.85-93
• /
• 2022

Purpose: Cornelia de Lange syndrome (CdLS) is a rare genetically heterogeneous disorder caused by genetic variants of the cohesin complex. However, the diverse genetic etiologies and their phenotypic correlations in Korean patients with CdLS are still largely unknown. Hence, this study aimed to clarify the clinical characteristics and genetic background of Korean patients with CdLS. Materials and Methods: The medical records of 15 unrelated patients (3 males and 12 females) genetically confirmed to have CdLS were retrospectively reviewed. All individuals were diagnosed with CdLS using target gene analysis, whole-exome sequencing, and/or chromosomal microarray analysis. The clinical score (CS) was calculated to assess disease severity. Results: The median age at diagnosis was 1.7 (range, 0.0-11.8) years, and median follow-up duration was 3.8 (range, 0.4-11.7) years. Eight (53.3%) patients showed classic phenotypes of CdLS, two (13.3%) showed non-classic phenotypes, and five (33.3%) had other phenotypes sharing limited signs of CdLS. Fifteen causative variants were identified: NIPBL in five (33.3%, including 3 males), SMC1A in three (20.0%), SMC3 in three (20.0%), and HDAC8 in four (26.7%) patients. The CS was significantly higher in the NIPBL group than in the non-NIPBL group (14.2±1.3 vs. 8.7±2.9, P<0.001). Conclusion: We identified the clinical and genetic heterogeneity of CdLS in Korean patients. Patients with variants of NIPBL had a more distinctive phenotype than those carrying variants of other cohesin complex genes (SMC1A, SMC3, and HDAC8). However, further studies are warranted to understand the pathogenesis of CdLS as a cohesinopathy and its genotype-phenotype correlations.

• The Korean Journal of Food And Nutrition
• /
• v.30 no.3
• /
• pp.539-551
• /
• 2017

This study investigated the current status and needs for nutrition education to help reduce children's sugars intake at the Center for Children's Foodservice Management (CCFM, n=115), and Child Care Facilities (CCF, n=646) through an online survey conducted from October $5^{th}$ to $30^{th}$ 2015. A total of 14.8% of CCFM respondents and 31.9% of CCF respondents provided nutrition education on sugars intake to young children as a main topic (p<0.001). A higher percentage (CCFM 47.8%: CCF 42.4%) delivered nutrition education on sugars intake to young children as a sub-component (p<0.001). Over 90% of the CCFM and CCF participants agreed on the necessity of providing nutrition education on sugars intake to children. The most common reasons given for delivering nutrition education on children's sugar intake were "there are many more urgent nutrition education topics" for CCFM, and "insufficient nutrition education information and materials" for CCF. The percentage of nutrition education on children's sugar intake provided to the children's parents was low showing about 20% in the both groups. The percentage of CCFM participants providing nutrition, education on children's sugar intake to the teachers in CCF was also low, showing about 14.8%; however, 68.0% of the CCF participants wanted to received teacher's education on guiding children's sugar intake. Regarding ideas about a nutrition education program on children's sugar intake for young children, most respondents in both groups answered "sugar intake and dental cavities or obesity" for appropriate education contents, "story telling or puppet show" for appropriate education methods, and "dietitian from CCFM and class teacher together" for appropriate educator. For appropriate education time, there was a significantl difference between the CCFM responses (average 2.7 times) and the CCF responses (average 4 times). Based on the above results, we found that implementing nutrition education on children's sugar intake at the CCFM and CCF, was low; however, awareness of the need for nutrition education on children's sugar intake and the program development and supply was very high. Also, the opinions of CCFM and CCF participants about a nutrition education program on children's sugar intake for young children can provide foundation data to develop and implement the CCFM-based nutrition education program.

• Korean Journal of Community Nutrition
• /
• v.17 no.1
• /
• pp.91-100
• /
• 2012

This study aimed to evaluate the effectiveness of the foodservice management support program focusing on menu management in community child centers. The support program provided reference menus, staff training, and field consulting to 10 community child centers in the Jeollanam-do province for one month, August in 2010. One month menus were developed, based on children's preference for menu items, foodservice personnel's preference for food materials, and availability of local specialty foods, and offered as reference menus. In addition, staff training and field consulting focusing on menu management were conducted before and during the pilot period, respectively. To evaluate the support program, menus, foodservice personnel's knowledge level and perceived performance in foodservice management, and children's level of satisfaction for foodservice were analyzed before and after the support program. As a result of analysis of 222 and 210 menus of before and after the support program, respectively, the number of dishes per meal increased from five to six on average, and the proportion of meals including five food groups, which were grain, meat, vegetable, fruit, and milk and dairy product, rose from 2% to 24%. Foodservice personnel's knowledge level regarding foodservice management increased significantly (p = 0.007), however, their perceived performance in foodservice management did not show any significant changes. Children were more satisfied with 'food' (p = 0.001), 'sanitation' (p = 0.001), and 'environment' (p < 0.008) of foodservice in community child centers after the support program. In conclusion, the foodservice management support program focusing on menu management in this study was effective for improving menu quality of and children's satisfaction with foodservice in community child centers.

• Fashion & Textile Research Journal
• /
• v.13 no.5
• /
• pp.686-694
• /
• 2011

The purpose of this study was to understand the preferences of the uniform design for the preschool student's parents. 97 parents became subjects for the research and questionaries were analysed by distribution of frequency and percentage using SPSS V. 18.0. The results of this study could be summarized as followings. First, most of the parents preferred to wearing preschool uniform and expected further improvement in function, material and design. Second, the preferred form was semi formal style consisting of shirt, knit vest, knit cardigan, and coat. Third, the preferred colors were the mixing together bright colors and quiet colors in vivid and pastel tone. Fourth, functional materials to comfort in children's physical activities and to serve convenience in cleaning were preferred. In Conclusion, the preferred design characteristics were combined function to enhance comfort and convenience in children's physical activities with esthetics to present loveliness and modesty as preschool students.

• Journal of the Korea Society of Computer and Information
• /
• v.22 no.1
• /
• pp.135-141
• /
• 2017

This thesis progressed research on the improvements of NCS multimedia utilization for preliminary teachers. A national module has not been developed yet in terms of child education, so this thesis suggests a curriculum according to the courses taught for freshmen and sophomores of K University Child Education majors. To lessen the burden of tremendous work and classes, provide motivation and interest in learning and maximize the effect, this thesis provides NCS based curriculum. It expects to improve task performance of teachers and help them with better skills to make class materials using up-to-date multimedia, regardless of the computer literacy of preliminary teachers. This thesis does prior research on the abilities to make use of computers and understand the level of computer literacy. Then it suggests NCS based curriculum goals and its performance standards to utilize task-suitable software. It aims to enable efficient multimedia usage, and optimize the learning efficiency of education linked to Nuri precesses.

• Journal of Korean Elementary Science Education
• /
• v.25 no.spc5
• /
• pp.567-581
• /
• 2007

This study examined patterns of reasoning of both the scientifically-gifted and children of average ability as witnessed in their science problem solving skills. Science problem solving skills are one of the significant characteristics of scientifically gifted children, and by using methods such as individual interviews, inductive reasoning, abductive reasoning, and deductive reasoning, the characteristics of these children can be to be further explored and categorized. The study also compared the findings with those of average children. This study sought to determine efficient guidelines fur teaching the scientifically-gifted, to come up with basic materials for developing relevant programs, and to find suggestions for identifying such students. The results of the study are as follows: Firstly, the creative science problem solving skills of the scientifically-gifted were better than that of the average students. Secondly, all of the three reasoning patterns used revealed in creative science solving processes were different between the gifted and the average, especially in terms of abductive reasoning, which was proved to reveal the greatest distinction between the two groups.

• Korean Institute of Interior Design Journal
• /
• v.17 no.5
• /
• pp.107-114
• /
• 2008

With the exhibition materials and exhibition spaces are provided, it is the reaction of the visitors that completes the function of science museum. To satisfy the visitors, it is necessary to understand their needs, interests, and motives, apart from the physical environment of the museum. The purpose of this research is to verify the effect of children's personal characteristics on the satisfaction in science museum. In doing so, an interview survey was conducted: 210 primary and middle school students who had finished the exhibition tour were randomly selected and interviewed. The data was analysed by a T-test and an one-way ANOVA to find out the correlation. The results of analysis are as follows: First, a meaningful statistical differences are found by the age groups in terms of motivation, expectation, satisfaction, enjoyment, and total staying time at the museum. The lower the grade, students are satisfied with their everyday living; and this is reflected on their intrinsic motivations and higher expectations on visit. Also, their actual satisfaction and enjoyment are higher and they stay at the museum longer than the other group. Second, the group with intrinsic motivation and higher expectation is more satisfied with the contents of the exhibition than the group with extrinsic motivation. Third, the level of higher education of mothers, rather than that of fathers had mere effect on the learning of the children at the museum. Also, the children who are brought up by full-time house wives have bigger achievement of learning from the exhibition than those with working mothers.

• Imaging Science in Dentistry
• /
• v.49 no.2
• /
• pp.153-158
• /
• 2019

Purpose: The purpose of this study was to investigate variations in velar shape according to age, sex, and race using magnetic resonance imaging (MRI). Materials and Methods: The study sample consisted of 170 participants (85 children, 85 adults) between 4 and 34 years of age. Velar morphology was visually classified using midsagittal MRI scans for each participant by 2 independent raters. Inter- and intra-rater reliability was assessed. Statistical analyses were performed to identify correlations of velar shape with sex, age, and race. Results: The most frequent velar shape was "butt" for both adults(41%) and children (58%) in this study. The least common shapes for adults were "leaf" and "S." The children did not exhibit any "leaf" or "straight" velar shapes. A statistically significant difference was noted for age with respect to velar shape (P=0.014). Sex and race were found to have no significant impact on velar shape in this study. Conclusion: When using MRI to evaluate velar morphology, the "butt" shape was most common in both children and adults. Velar shape varied significantly with age, while race and sex did not have a significant impact.

• Journal of the Korean Home Economics Association
• /
• v.42 no.11
• /
• pp.17-30
• /
• 2004

The purpose of this study was to explore the normative expectations of positive emotions such as happiness and pride in Korean culture by analyzing the children's textbooks in the Chosen Dynasty period. The method of this study was document analysis and the materials for analysis were 'Dongmongsensp', 'Gyukmongyogyul', 'Myungimbogam', and 'Sohak', that were the textbooks for children's education and invoked the ethics of individual life based on Confucianism. The analysis was focused on the antecedent events, emotional consequences, and emotional regulation in the emotional process of happiness and pride. According to the analysis, happiness was caused by the accomplishment of cultural tasks such as supporting parents, observing laws, being moderate, improving oneself with reading good books, and modeling good deeds, as well as parents' love, which had desirable consequences. Hence, the emotional regulation was to enhance happiness, but far as immoderate experience of happiness, the regulatory rule was expected to suppress it. Also, the antecedents of pride were those demonstrating to have power or occupying a high social position, and the consequences of pride led to self-destruction, and pride was therefore not allowed to be expressed. Consequently, happiness was enhanced because it motivated individuals to accomplish cultural tasks of interdependence, whereas pride was inhibited because it tended to disengage self from social connectedness.

• Korean Journal of Radiology
• /
• v.22 no.7
• /
• pp.1185-1193
• /
• 2021

Objective: Clear cell sarcoma of the kidney (CCSK) is the second-most common but extremely rare primary renal malignancy in children after Wilms' tumor. The aims of this study were to evaluate the imaging features that could distinguish between CCSK and Wilms' tumor and to assess the features with diagnostic value for identifying CCSK. Materials and Methods: We reviewed the initial contrast-enhanced abdominal-pelvic CT scans of children with CCSK and Wilms' tumor between 2010 to 2019. Fifty-eight children (32 males and 26 females; age, 0.3-10 years), 7 with CCSK, and 51 with Wilms' tumor, were included. The maximum tumor diameter, presence of engorged perinephric vessels, maximum density of the tumor (Tmax) of the enhancing solid portion, paraspinal muscle, contralateral renal vein density, and density ratios (Tmax/muscle and Tmax/vein) were analyzed on the renal parenchymal phase of contrast-enhanced CT. Fisher's exact tests and Mann-Whitney U tests were conducted to analyze the categorical and continuous variables, respectively. Logistic regression and receiver operating characteristic curve analyses were also performed. Results: The age, sex, and tumor diameter did not differ between the two groups. Engorged perinephric vessels were more common in patients in the CCSK group (71% [5/7] vs. 16% [8/51], p = 0.005). Tmax (median, 148.0 vs. 111.0 Hounsfield unit, p = 0.004), Tmax/muscle (median, 2.64 vs. 1.67, p = 0.002), and Tmax/vein (median, 0.94 vs. 0.59, p = 0.002) were higher in the CCSK compared to the Wilms' group. Multiple logistic regression revealed that engorged vessels (odds ratio 13.615; 95% confidence interval [CI], 1.770-104.730) and Tmax/muscle (odds ratio 5.881; 95% CI, 1.337-25.871) were significant predictors of CCSK. The cutoff values of Tmax/muscle (86% sensitivity, 77% specificity) and Tmax/vein (71% sensitivity, 86% specificity) for the diagnosis of CCSK were 1.97 and 0.76, respectively. Conclusion: Perinephric vessel engorgement and greater tumor enhancement (Tmax/muscle > 1.97 or Tmax/vein > 0.76) are helpful for differentiating between CCSK and Wilms' tumor in children aged below 10 years.