• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.37-40
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• 2016

Mucopolysaccharidosis type III (MPS III) is a rare genetic disorder caused by lysosomal storage of heparan sulfate. MPS IIIB results from a deficiency in the enzyme alpha-N-acetyl-D-glucosaminidase (NAGLU). Affected patients begin showing behavioral changes, progressive profound mental retardation, and severe disability from the age of 2 to 6 years. We report a patient with MPS IIIB with a long-term follow-up duration. He showed normal development until 3 years. Subsequently, he presented behavioral changes, sleep disturbance, and progressive motor dysfunction. He had been hospitalized owing to recurrent pneumonia and epilepsy with severe cognitive dysfunction. The patient had compound heterozygous c.1444C>T (p.R482W) and c.1675G>T (p.D559Y) variants of NAGLU. Considering that individuals with MPS IIIB have less prominent facial features and skeletal changes, evaluation of long-term clinical course is important for diagnosis. Although no effective therapies for MPS IIIB have been developed yet, early and accurate diagnosis can provide important information for family planning in families at risk of the disorder.

• Journal of the Korean Society of Physical Medicine
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• v.15 no.4
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• pp.75-85
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• 2020

PURPOSE: Cerebral palsy is a common cause of disability in children, requiring constant rehabilitation. Improving balance in children with cerebral palsy helps to alleviate daily movement and the quality of life. This study examines a program that combines Resistance Exercise and Group Exercise, and investigates the effect on trunk control ability, balance ability, maximum grip strength, and quality of life of children with cerebral palsy. METHODS: Totally, 9 children with cerebral palsy participated in this study. Resistance exercise was performed for 8 weeks, 40 minutes every day. Group Exercise was conducted for 8 weeks, 40 minutes each Sunday. All participants were evaluated by the Korean version of the trunk control measurement scale, pediatric reach test, grip strength test, and KIDSCREEN-52. RESULTS: The trunk control ability was significantly improved in all subcategories (p < .05). In the pediatric reach test, the left and right directions were significantly improved (p < .05). Maximum grip strength was significantly improved in both hands (p < .05). The quality of life significantly improved in total score, physical well-being, general moods, self-perception, autonomy, relationship with parents, and home life (p < .05). CONCLUSION: Considering the encouraging results, we propose to use Resistance Exercise and Group Exercise as programs other than rehabilitation treatment in hospitals, to improve motor function and quality of life of children with cerebral palsy.

• International Journal of Human Ecology
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• v.5 no.2
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• pp.51-59
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• 2004

Parents' marital conflicts are often linked to various emotional and behavioural difficulties of their children but rarely known about later adolescent and adult lives. This study was undertaken to examine if serious parental conflict such as divorce and/or separation in childhood was associated with emotional and social performance during later adolescent and adult lives. The research literature on divorce was briefly reviewed. The data was derived from the National Child Development Study (NCDSS 1974/age 16 - 1991/age 33) which followed the lives of 17,500 individuals born on March 3-9, 1958 in England, Scotland and Wales. Regression analyses indicate strong evidence that the experience of parental divorce or separation in childhood negatively affects the child's emotional and social performance in their subsequent life. The higher likelihood of having emotional problems, being less competent at their job, experiencing health/disability problems, being a lone-parent, unemployed, and homeless were strongly associated with parental divorce or separation experienced in childhood. Intervention is desirable to address the concerns influencing socio-psychological performance amongst children who have experienced parental divorce or separation.

• Journal of Music and Human Behavior
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• v.6 no.2
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• pp.25-41
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• 2009

The purpose of this case study is to examine how group rhythmic playing can help enhance imitation skills for 3 children with developmental disabilities aged between 30-50 months. The program was composed of social musical play, the therapist's imitating the way young children played musical instruments, and the young children's imitating the way the therapist played musical instruments. Every session was videotaped, and the researcher and two music therapists checked the videotaped sessions to ensure the reliability of findings. The case study demonstrated the following results. First, the three young children who participated in the playing of rhythmic musical instruments showed an increase in imitation behaviors. Second, the infants exhibited the greatest change from 1 beat imitation according to the degree of difficulty of group rhythm musical instrument demonstrated. Third, the most radical change in the infants' ability to imitate appeared in Sessions 3-7 where the therapist imitated the operation and a performance of the infant. In conclusion, the activity of playing rhythmic musical instruments in a group generated improvement in the imitation ability of young children with developmental disabilities.

• Journal of Korea Society of Industrial Information Systems
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• v.18 no.4
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• pp.1-8
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• 2013

In the modern society, instructional methods using diverse media have appeared thanks to the development of Information & Communication Technology, and applicability of such instructional methods has been fully corroborated. However, customized contents allowing for disabled children's environment are still insufficient. Hence, this study produced educational contents of play activities for children with developmental disability, through applying N-screen technology, IT technology that can provide the same contents via a variety of digital media. The produced contents allow programs to be set up according to a child's individual characteristics and be carried out anywhere and anytime via an Internet-enabled digital device. Further, the developed contents were produced so that they could be accessed from a child's various environment (home, school, etc.) via a PC, a smart phone, a portable from a child's various environment (home, school, etc.) via a PC, a smart phone, a portable device, etc. and that the same educational program could be conducted in linkage at home, school, etc. Three children with Intellectual disability and autism spectrum disorder were applied to the manufactured content. As a result, Content interaction between interaction between teachers and students in play training could use as a medium.In addition, the children's ability to select the appropriate components and reinforcements, special education professionals have used the content of the interviews are helpful in mediation than the existing content.

• Journal of the Korean Society of Physical Medicine
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• v.13 no.1
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• pp.27-38
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• 2018

PURPOSE: The purpose of this study was to find out the treatment patterns of Children with cerebral palsy, and to analyze the effect of physical function level and treatment intensity on improvement of physical function in children with cerebral palsy for six months. METHODS: Participants were 126 children (boys 83, girls 43) diagnosed cerebral palsy that the mean age was at 33months, ranged from 8 months to 77 months. We collected data related on demography and disable characteristic, treatment pattern using by questionnaire constructed ourselves for six months on caregivers. The treatment pattern includes, type, frequency, and institute of treatment. We performed the evaluation of Gross Motor Function Measurement (GMFM) and Pediatric Evaluation of Disability Inventory (PEDI) between pre and post for six months in order to find out improvement of physical function. We analyzed the effect of physical functional level measured by Gross Motor Functional Classification system, age, treatment intensity on physical function using by repeated measures ANOVA for SPSS PC ver. 22.0. RESULTS: The average of treatment frequency for physical therapy was 5.74 times per week, occupational therapy was 3.96 times, speech therapy was 2.96 times, treatment for accompanying disability was 3.12 times. Physical function level and age was significantly factors affecting improvement of physical function, there was no significant difference according to treatment intensity. CONCLUSION: We suggest that physical function and age might be important factors on improvement of physical function and professional rehabilitation team must consider the appropriate treatment type customized to each children.

• Journal of Genetic Medicine
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• v.10 no.2
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• pp.113-116
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• 2013

Alexander disease is a rare degenerative leukodystrophy caused by dominant mutations in glial fibrillary acidic protein (GFAP). The neonatal form of Alexander disease may manifest as frequent and intractable seizures or obstructive hydrocephalus, with rapid progression leading to severe disability or death within two years. We report a case of a 50-day-old male who presented with intractable seizures and obstructive hydrocephalus. His initial magnetic resonance imaging (MRI) suggested a tumor-like lesion in the tectal area causing obstructive hydrocephalus. Despite endoscopic third ventriculostomy and multiple administrations of antiepileptic drugs, the patient experienced intractable seizures with rapid deterioration of his clinical status. After reviewing serial brain MRI scans, Alexander disease was suspected. Subsequently, we confirmed the de novo missense mutation in GFAP (c.1096T>C, Y366H). Although the onset was slightly delayed from the neonatal period (50 days old), we concluded that the overall clinical features were consistent with the neonatal form of Alexander disease. Furthermore, we also suspected that a Y366 residue might be closely linked to the neonatal form of Alexander disease based on a literature review.

• Journal of the Korean BIBLIA Society for library and Information Science
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• v.19 no.2
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• pp.107-128
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• 2008

The purpose of this study is to portray the phenomena of information poverty of the families of children with developmental disabilities. The focus was especially put on investigating their information needs of everyday lives and information-seeking behaviors. The sample group includes six children with developmental disabilities and their families, mainly mothers. The investigation was conducted by the participant observation and in-depth interviews. The study found out that they sought the formal information sources such as medical specialists and special agencies first with which they were not satisfied. As a result, they rather relied on informal information sources. And the information from 'the group of families of children with developmental disabilities' was thought to be the most reliable information for them. In terms of information related behaviors, the strongest information needs were regarding the children's special education, especially to the school education, and they made effort to seek reliable information. They, however, tended to passively treat their personal concerns that are not related to their disabled children. This study found out that their information poverty is in terms of the information environment, information sources and their attitude to information, and that it comes from the external(social) factors as well as internal(individual) ones.

• 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.

• Proceedings of the Korea Contents Association Conference
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• 2009.05a
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• pp.1076-1082
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• 2009

This study investigated caregivers' communication styles and children's emotional development. Emotion-laden puzzle tasks were used to elicit caregivers' communication styles while interacting with their children. Participants included children with Down syndrome (N=10) and typical children (N=15) and their caregivers. As expected, caregivers of children with Down syndrome (DS) used more behavior and attention directives with their children, and caregivers of typical children used more conversation-eliciting prompts with their children. Parents of children with Down syndrome also used a unique communication style in which they asked a question and immediately answered it themselves. Additionally, caregivers of typical children focused more on emotion concepts in their communications with their children and caregivers of DS used more cognitive concepts such as labeling colors and shapes. The results revealed that caregivers of children with Down syndrome usually tried to educate children by emphasizing cognitive concepts to compensate for their delayed development. Because the children are delayed in their emotional development, parents may need help in intervening on the area of emotional development.