• 대한유전성대사질환학회지
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• 제14권2호
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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.

• 한국컴퓨터정보학회논문지
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• 제24권1호
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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.

• 한국콘텐츠학회논문지
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• 제21권11호
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• pp.711-722
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• 2021

본 연구의 목적은 발달장애인의 취업이 사회활동에 미치는 영향을 파악하여 발달장애인의 사회통합 활동 방안을 모색하는 것이다. 연구방법으로 391명의 발달장애인을 대상으로 취업 여부와, 사회활동에 대한 설문조사 자료를 통해 교차분석과 상관분석, 그리고 로지스틱 회귀분석을 실시하였다. 분석결과 첫째, 발달장애인의 취업은 사회활동에 유의한 것으로 나타났다. 즉 취업한 발달장애인의 사회활동 참여는 79.7% 미취업 상태는 39.3%로 나타났다. 둘째, 개인적 특성을 통제변인으로 신중하게 검증한 발달장애인의 취업은 사회활동에 유의한 영향을 미치는 것으로 5.2배 증가하였다. 연구결과로 볼 때 발달장애인의 사회활동 참여를 위해서는 발달장애인의 적극적인 취업지원과 취업에 대한 정책적 노력이 요구됨을 알 수 있다. 마지막으로 지원고용과 장애인 표준사업장 확대, 직무지도원 제도 도입과 관련 기관과의 네트워크 구축을 제언하였다.

• 한국사회복지학
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• 제47권
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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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• 제46권4호
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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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• 제19권2호
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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.

• 대한가정학회지
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• 제29권3호
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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.

• 디지털융복합연구
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• 제20권5호
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• pp.703-712
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• 2022

본 연구의 목적은 발달장애인의 차별경험과 취업의 관계에서 가족취업지지의 조절효과를 분석하여 발달장애인의 취업을 위한 실천적 함의를 제시하는 데 있다. 연구대상은 2019년도 장애인고용패널조사 2차 웨이브 4차 조사에 참여한 발달장애인 394명이고, 가설검증을 위해 위계적 이항 로지스틱 회귀분석을 실시하였다. 연구 결과 발달장애인의 차별경험은 취업에 부적영향을 미치고, 가족취업지지는 유의미한 조절효과를 보여 차별경험이 취업에 미치는 부적영향을 강화하는 것으로 나타났다. 따라서 발달장애인 가족의 요구를 반영한 근무환경개선과 취업지원정책의 필요성, 국가 및 지역사회와의 신뢰감 강화의 중요성을 논의하였다.

• 한국엔터테인먼트산업학회논문지
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• 제13권3호
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• pp.203-211
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• 2019

이 연구는 4-7세의 발달장애 아동을 대상으로 하여 감각처리와 수면장애의 관계를 알아보고 감각처리가 수면장애에 미치는 영향을 파악하고자 하였다. 2017년 6월부터 8월까지 발달 클리닉 및 재활병원에 내원하는 전반적 발달장애 아동 110명을 대상으로 하여 최종 101명의 자료를 분석하였다. 감각처리와 수면장애를 평가하기 위해 단축형 감각 프로파일(SSP)과 한국형 아동 수면장애 척도(K-CSHQ)로 자료 조사하였다. 통계분석은 기술분석과 피어슨 상과분석, 다중회귀분석을 사용하였다. 연구결과, 발달장애 아동은 감각처리와 수면장애에 문제를 보이는 경우가 많았다. 감각처리는 수면장애와 관련이 있었으며 수면장애에 대한 감각처리의 최종 영향 요인은 맛/냄새 민감성, 청각필터링과 전반적인 감각처리인 것으로 나타났다. 발달장애 아동의 질 높은 수면을 돕기 위해서는 아동의 감각처리 능력을 중재에 고려할 필요가 있으며 특히 맛/냄새 민감성, 청각 필터링 영역을 중재하는 것이 도움이 될 것이다.

• 한국가족관계학회지
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• 제23권3호
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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.