• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.2
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• pp.182-185
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• 2014

GM1 gangliosidosis is a rare autosomal recessively inherited metabolic disease due to deficiency of ${\beta}$-galactosidase caused by mutations in the GLB1 gene. There have been three clinical subgroups in GM1 gangliosidosis, however it is difficult to differentiate because there is considerable overlap between classical phenotypes and clinical and imaging findings among the subgroups. Here, we report a Korean girl with type 2 GM1 gangliosidosis, who showed dysostosis multiplex and progressive neurological deterioration. Developmental regression was first noted at the age of 9 months, and she was diagnosed as GM1 gangliosidosis by ${\beta}$-galactosidase enzyme analysis and GLB1 mutation analysis at the age of 16 months.

• Journal of the Korea Society of Computer and Information
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• v.24 no.1
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• pp.225-230
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• 2019

The purpose of this study is to provide basic data that mother's smart phone overdependence prevention education are needed by confirming the relationship between mother' s smartphone overdependence and parent role intelligence. The research subjects were 220 mothers of 0-2 years old who are attending 15 day care centers in Cheong-ju, Chung-buk. The research tool used smartphone overdependence scale, and parent role intelligence scale. For the analysis of the study, frequency analysis, Pearson's Ratio correlation and regression analysis were performed. The results showed that mother's smartphone overdependence was negatively correlated with parental role intelligence and negatively correlated with developmental stimulation, parenting, support, and management. The multiple regression analysis showed that there was a significant negative effect on the smartphone overdependency, parent role intelligence, and sub factors. This suggests that smartphone overdependence prevention education for mother should be needed for nurturing of desirable infants.

• The Journal of the Korea Contents Association
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• v.21 no.11
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• pp.711-722
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• 2021

The purpose of this study is to explore the effects of employment for people with developmental disabilities on their social activities. As a research method, cross-analysis, correlation analysis, and logistic regression analysis were performed using questionnaire data on employment status and social activity for 391 persons with developmental disabilities. As a result of the analysis, first, employment for people with developmental disabilities was found to be significant in social activities. In other words, 79.7% of those with developmental disabilities who were employed participated in social activities, and 39.3% of those who were not employed. Second, employment for people with developmental disabilities, whose personal characteristics were carefully verified as a control variable, had a significant effect on social activities and increased by 5.2 times. According to the results of the study, it can be seen that active employment support for people with developmental disabilities and policy efforts for employment are required for participation in social activities of people with developmental disabilities. Finally it was suggested to supported employment, expand standard workplaces for the disabled, introduce a job coach system, and establish a network with related organizations.

• Korean Journal of Social Welfare
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• v.47
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• pp.7-33
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• 2001

The purpose of this study was to the impact of organizational culture on the organizational effectiveness in hospitals were empirically analysed. Data were collected by 151 social workers including general hospitals with one social worker at least and single-department hospital with two social workers or more, were identified nationwide through the registry of Korean Association of Medical Social Workers and Korean Association of Hospitals. The results of this study were summarized as follows; First, most social workers report that characteristic of culture in their hospitals are group culture the first, hierarchial culture the second, rational culture the third, and developmental culture finally in order of social worker's recognition. Second, according to the types of organizational culture, there were significantly different levels of organizational effectiveness. Social worker's perceived group cultures in hospital showed higher job satisfaction, organizational commitment than other culture patterns. And also, social worker's perceived developmental cultures in hospital showed higher organizational commitment quality of social service than other culture patterns. Third, multiple regression analysis was conducted to find the relationship of organizational culture and organizational effectiveness. As a result, developmental culture showed a strong relationship with job satisfaction, organizational commitment and quality of social services Finally, as for the cultural affects on the organizational effectiveness depended on intensive attention to developmental culture in hospital Consolidating the attribute of developmental culture in hospital is also proposed to improve the organizational cultures.

• Journal of Oral Medicine and Pain
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• v.46 no.4
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• pp.125-130
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• 2021

Purpose: The dental age estimation of children is performed using dental maturity. Postmortem missing of the anterior teeth or the distortion of image of the anterior teeth in panoramic radiographs can make it difficult to analyze the development of the anterior teeth. This pilot study was conducted to derive a new age estimation method based only on the developmental stage of mandibular posterior teeth. Methods: This study was conducted using panoramic radiographs of 650 subjects aged 3 to 15 years old. The dental developmental stages of the lower left first premolar, second premolar, first molar and second molar were evaluated according to the Demirjian's criteria. The intra-/inter-observer reliability was evaluated, and multiple linear regression analyses were performed including the developmental stage of each tooth as an independent variable. Results: The intra-/inter-observer reliability was 0.9626 and 0.8877, respectively, and showed very high reproducibility. Multiple linear regression analyses were performed for males and females, and the age calculation table was derived by obtaining the intercept and the coefficient according to the development stage of each tooth. The coefficient of determination (r²) of the age calculation method was 0.9634 for male and 0.9570 for female subjects, and the mean difference between chronological age and estimated dental age was -0.42 and -0.21, respectively. Conclusions: This pilot study evaluated the developmental stages of four lower posterior teeth in the Korean group according to Demirjian's criteria, and derived age estimation method. The accuracy was lower than when more teeth were used, but it will be useful to estimate age of children when the anterior teeth are difficult to accurately analyze.

• Journal of Genetic Medicine
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• v.19 no.2
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• pp.100-104
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• 2022

The gene encoding solute carrier family 9 member 6 (SLC9A6) on Xq26.3 is associated with Christianson syndrome (CS) mimicking Angelman syndrome. In CS, developmental and epileptic encephalopathy (DEE) appears in about 20%, and DEE with spike-and-wave activation in sleep (SWAS) is reported only in several cases. A 10-year-old boy with DEE showed multidrug resistant focal seizures from 6 months of age. He had progressive microcephaly, regression, global developmental delay without speech, hyperkinesia, and truncal ataxia; he had a long thin face, esotropia, and happy demeanor. Brain magnetic resonance imaging demonstrated cerebellar atrophy. Electroencephalogram at 7.5 years of age showed nearly continuous diffuse paroxysms in slow wave sleep. The seizures were responsive to corticosteroids for a while. Trio whole exome sequencing exhibited a likely pathogenic variant of SLC9A6 in the proband and his asymptomatic mother: c.1194dup (p.Leu399AlafsTer12). This is a rare case report of CS with DEE-SWAS in a Korean patient.

• Journal of the Korean Home Economics Association
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• v.29 no.3
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• pp.153-174
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• 1991

The purpose of this study is to examine the leisure activities of housewives, to identify the factors that influence leisure activities, and to explore the factors contribute to their life satisfaction. So, this study analyses the effects of econo-demographic and socio-cultural variables and of leisure constraint factors on the leisure activities of housewives. And it deals with how these variables and the leisure activities influence life satisfaction of housewives. For these puoposes, 508 housewives residing in Seoul & Daegu were selected for interviews. For data analysis such statistical methods as ANOVA, t-test, Pearson's correlation, adn Multiple Regression Analysis can be summarised. The main findings of the research are as follows: 1. Leisure acivities are classified in Self-developmental, Home-oriented, Time-consuming, Social and Children-concerned types by the technique of factor analysis. The average particiation level was high in Time-consuming type, but low in Self-developmental type. 2. The participation level of leisure activities shows significant differences by selected variables: The Self-developmental type shows significant differences by housewife's education level, income, husband's occupation, role orientation, home management type and leisure constraints. And Children-concerned type was differed to number of family nember, number of children, age of housewife and age of housewife and age of the youngst child. 3. The preference level of leisure activities differ by housewife's education level, income, husband's occupation, home management type and leisure constraints in the Self-developmental and the Social type. And the preference level of Home-oriented leisure activities was high in the middle class of income and husband's occupation. 4. The preference and participation level of leisure activities show differences. And the variables affecting the differences were housewife's age, education level, home management type, role orientation, leisure constraint factors in the Self-developmental type, and were demographic variables such as number of family member, housewife's age in the Home-oriented type. 5. The variables which affected the level of life satisfaction independently were leisure space, income, the participation level of the Self-developmental and the Social type and the preference level of the Self-developmental type.

• Journal of Digital Convergence
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• v.20 no.5
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• pp.703-712
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• 2022

The purpose of this study is to present practical implications for employment of people with developmental disabilities by analyzing the moderating effect of family employment support in the relationship between discrimination experience and employment of people with developmental disabilities. The subjects of the study were 394 people with developmental disabilities who participated in the 2nd wave 4th survey of the 2019 Employment Panel Survey for the Disabled, and a hierarchical binary logistic regression analysis was conducted to verify the hypothesis. As a result of the study, it was found that the experience of discrimination among people with developmental disabilities negative effects employment, and family employment support has a significant moderating effect, strengthening the negative effect of discrimination on employment. Therefore, the importance of improving the working environment reflecting the needs of families with developmental disabilities, the necessity of employment support policies, and strengthening trust with the state and local communities were discussed.

• Journal of Korea Entertainment Industry Association
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• v.13 no.3
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• pp.203-211
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• 2019

The purpose of this study was to investigate the relations between sensory processing and sleep disturbances and to investigate the effect of sensory processing on sleep disorder in preschool children with developmental disorder. This study was conducted for 110 children with developmental disorder in developmental clinic and rehabilitation hospital in Gwang Ju from June to August, 2017. The final 109 data were analyzed. Sensory processing and Sleep disturbances were measured using the Shortened sensory profile(SSP) and Korean-the Children's Sleep Habits Questionnaire(K-CSHQ). Statistical analysis was performed using descriptive analysis, Pearson correlation analysis, and multiple regression analysis were performed. Children with developmental disorder had problems with sensory processing and sleep habits. Sensory processing was related to sleep habit and most important factors of sensory processing influencing sleep was taste/olfactory sensitivity, auditory filtering. Conclusion: In order to help children with developmental disorder with sleep problem, it is necessary to consider the sensory processing especially taste/olfactory sensitivity, auditory filtering.

• Journal of Family Relations
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• v.23 no.3
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• pp.153-175
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• 2018

Objectives: The purpose of this study is to examine the effects of care burden among mothers of children with developmental disabilities on depression and the moderating effects of formal and informal social support. Method: The self-report questionnaires were administered to 200 mothers of children with developmental disabilities (aged 6~20 years) in pre-adulthood in Gyeongi-do. A hierarchical regression analysis was conducted. Result: First, There is a need to provide a mental health program that can relieve depression. Second, Individualized intervention strategies for mothers' psychological counseling and support programs for intensive case management and parenting stress management programs are needed. Third, Individual visit case management is required. In addition, it is necessary to expand the eligibility for care services and to maintain long-term programs. Conclusion: The findings suggested that there is a need to provide mothers with not only personal assistance program but also services such as mothers' psychological counseling, peer mentor program, and parenting stress management.