• Asia-pacific Journal of Multimedia Services Convergent with Art, Humanities, and Sociology
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• v.7 no.9
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• pp.815-823
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• 2017

The purpose of this study is to investigate the need for practical evaluation of special education field based on the analysis of the importance - performance of special education basic curriculum academic achievement evaluation area. The evaluation categories and areas for the analysis of importance and performance are set in 4 categories - 22 areas of life education derived from Delphi analysis and 7 categories- 27 areas in special education basic curriculum. As a result of analyzing the importance and performance of 126 teachers of intellectual disability special schools in P metropolitan city, statistically significant difference was shown between importance and performance in all areas. As a result of analysis of scatter plot with x - axis and significance as y axis based on averaging point, some elements of subject education showed low recognition level both in importance and execution. The results of this study suggest the necessity of restructuring the evaluation area and the evaluation system of the basic education curriculum of the special education and further revise the contents of the curriculum of the national level basic curriculum and the content area, It implies that discussion is needed.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.34 no.4
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• pp.258-267
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• 2023

Objectives: This study aimed to compare the utility of the Psychoeducational Profile-Revised (PEP-R), Korean Wechsler Preschool and Primary Scale of Intelligence, Fourth Edition (K-WPPSI-IV), and Vineland Adaptive Behavior Scale, Second Edition (VABS-II) for evaluating developmental disabilities (DD) in preschool children. Additionally, we examined the correlations between the PEP-R, K-WPPSI-IV, and VABS-II. Methods: A total of 164 children aged 37-84 months were assessed. Children's development was evaluated using the PEP-R, K-WPPSI-IV, VABS-II, Preschool Receptive-Expressive Language Scale, and Korean Childhood Autism Rating Scale, Second Edition. Results: Of the 164 children, 103 had typical development (TD) and 61 had DD. The mean of the PEP-R Developmental Quotient (DQ), K-WPPSI-IV Full-Scale Intelligence Quotient (FSIQ), and VABS-II Adaptive Behavior Composite (ABC) scores were significantly higher in the TD group than in the DD group (p<0.001). The estimated area under the curve of the PEP-R DQ, K-WPPSI-IV FSIQ, and VABS-II ABC scores was 0.953 (95% confidence interval [CI]=0.915-0.992), 0.955 (95% CI=0.914-0.996), and 0.961 (95% CI=0.932-0.991), respectively, which did not indicate a statistically significant difference. The PEP-R DQ scores were positively correlated with the K-WPPSI-IV FSIQ (r=0.90, p<0.001) and VABS-II ABC scores (r=0.84, p<0.001). A strong correlation was observed between the K-WPPSI-IV FSIQ and VABS-II ABC scores (r=0.89, p<0.001). Conclusion: This study found that the PEP-R, K-WPPSI-IV, and VABS-II effectively distinguished DD from TD in preschool children, and no significant differences in utility were observed between them.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.12 no.1
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• pp.125-137
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• 2001

We investigated the self-concept, subjective depression, and state-trait anxiety of the school boys with learning disabilities(abbr. LD, n=86) and compared them with normal boys(n=52) using Piers-Harris Self-Concept Inventory, Child Depression Inventory(abbr. CDI), and State-Trait Anxiety Inventory(abbr. STAI). With regard to Piers-Harris Self-Concept Inventory total scores, there was no significant difference between two groups, but normal boys showed higher scores in intellectual and school status, physical appearance, and happiness-satisfaction subscales than patients with LD. The male patients with LD showed significantly higher ratings in CDI total scores, and CDI subscales - ineffectiveness, anhedonia, negative self-esteem than normal children. The patients with LD reported significantly higher state anxiety, but not trait anxiety. Correlation analyses revealed that self-concept decreased over time, and depression-anxiety increased across grades in the patients with LD, but not in normal children. Especially, negative mood, anhedonia, negative self-esteem subscales of CDI, and state-trait anxiety showed significant positive correlation with grades. In both groups, CDI scores were inversely correlated with Piers-Harris Self-Concept and positively with State-Trait anxiety. In conclusion, self-concept problems which were related with school achievement and self-esteem were more abundant in the patients with LD than normal children, self-image problem, depression and anxiety increased across grades. According to regression analysis, age, behavior subscale, intellectual-school status, anxiety, popularity, happiness-satisfaction, CDI-ineffectiveness, interpersonal problem, negative self-esteem, and state anxiety could explain the self-concept in the patients with LD, not in normal children. So, the self-concept of the patients with LD were found to be related to the school achievement and stress when comparing with peers. In conclusion, elementary school boys with LD showed lower self-concept, higher depression and anxiety, and these differences increased across grades. Since the patients with LD have concomitant depression and anxiety disorders, it is important that comorbidity with emotional problems should be explored and managed properly.

• Journal of Music and Human Behavior
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• v.3 no.1
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• pp.13-28
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• 2006

Autistic children have difficulties in communication. They tend to have more difficulties in general expression than in understanding sentences. This thesis tested the effect of music activities on children who have difficulties in language expression. As a research method, a group of autistic middle school students was selected who were attending normal schools. Three male students were selected for the test of K-ABC and CARS. Music treatments on the selected students were applied fifteen times, twice a week, for thirty minutes every time. The total treatment process could be divided into two stages. The stage one included the first twelve treatments and the stage two included following three treatments. In the stage one, the selected autistic students learned twenty four musics under real-world-like social circumstances. In the stage two, the students repeatedly learned the twenty four musics. A week before beginning the music treatment, the students were tested by PRES and a test developed by the author of this thesis. And a week after all treatments were completed, the students were tested again with the same test method to check if the students' expressive language ability got improved. The results of the research were as the following: First, the music treatment helped improve the autistic student's ability of expressing themselves such as 'requesting', 'rejecting', 'applying social customs' and 'providing information'. Second, the author of this thesis first had a hypothesis that the tested students might show different levels of achievements according to their intellectual ability or expressive language ability in K-ABC test, PRES and CARS. But it was not true. The student's level of achievement by music activities was proved not to have a significant correlation with their intellectual or expressive language abilities in the tests. Third, it was found that, through pre- and post-test of PRES, the music treatments could improve the receptive language ability as well as the expressive language ability. It saw great effect that musical data that utilize in specially this research investigator according to children's ability, autistic children write lyrics setting in social circumstance that is revealed much routinely and composes. To front, expect that suitable a lot of musics are developed in function and ability of subject person for elevation of expressive language ability.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.17 no.10
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• pp.591-599
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• 2016

Among the employer-supported subscribers to the National Health Insurance Service, 6,797 people with mild disabilities with western ages of 20 and up and who received health checkups were investigated. Of these 6,797 people, 3,186 and 3,611 received health checkups in 2009 and 2013, respectively. Those people who were diagnosed with physical handicaps, brain lesions, visual impairment, hearing impairment, intellectual disabilities, mental disorders, kidney disorders or other disorders according to the classification standard for people with disabilities were classified into disability groups of the 3rd through 6th degrees. The purpose of this study was to examine the dangerous influence of obesity of people with mild disabilities on their hyperglycemia, hypertension and high cholesterol. The items measured in this study were abdominal obesity, body mass index, fasting glucose, total cholesterol, systolic blood pressure and diastolic blood pressure. To look for connections between the obesity level and at-risk groups for each disease, cross tabulation and multinomial logistic regression analyses were utilized. Higher levels of abdominal obesity and BMI were found among those who were male, were younger and had higher incomes. The risks of abdominal obesity and BMI were higher in the abnormal groups for each disease. In 2009, the obesity group whose BMI was higher had a 1.51-fold higher risk of hypertension than the normal group. The abdominal obesity group had a 1.59-fold higher risk of high cholesterol, a 1.26-fold higher risk of hypertension and a 1.54-fold higher risk of hyperglycemia than the normal group. In 2013, the obesity group whose BMI was higher had a 1.72-fold higher risk of high cholesterol and a 1.43-fold higher risk of hypertension than the normal group. Those with abdominal obesity had a 1.59-fold higher risk of hyperglycemia than the normal subjects. As the risk of obesity was higher in those with disabilities than in those without disabilities, the former should be encouraged to undergo health checkups on a regular basis, and the coverage of the health checkups should be extended to keep track of their illness. In addition, appropriate education and concern are both required to prevent obesity.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.3
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• pp.99-106
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• 2018

Mucolipidosis type III (pseudo-Hurler polydystrophy) is a mucolipids degrading disorder caused by a mutation in the GNPTAB gene and is inherited by autosomal recessive. It is diagnosed by examining highly concentrated mucolipids in blood and the diagnosis can be confirmed by genetic testing. Mucolipidosis type III is a rare and progressive metabolic disorder. Its initial signs and symptoms usually occur around 3 years of age. Clinical manifestations of the disease include slow growth, joint stiffness, arthralgia, skeletal abnormalities, heart valve abnormalities, recurrent respiratory infection, distinctive facial features, and mild intellectual disability. Here, we are presenting two siblings of mucolipidosis type III, a 4-year-old female and a 2 years and 7 months old male with features of delayed growth and coarse face. The diagnosis was confirmed by [c.2715+1G>A(p.Glu906Leufs*4), c.2544del(p.Glu849Lysfs*22)] mutation in targeted gene panel sequencing. In this case, c.2544del is a heterozygote newly identified mutation in mucolipidosis type III and was not found in the control group including the genome aggregation database. And it is interpreted as a pathogenic variant considering the association with phenotype. Here, we report a Korean mucolipidosis type III patients with novel mutations in GNPTAB gene who have been treated since early childhood. Owing to recent development of molecular genetic techniques, it was possible to make early diagnosis and treatment with pamidronate was initiated appropriately in case 1. In addition to these supportive therapies, efforts must be made to develop fundamental treatment for patients with early diagnosis of mucolipidosis.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.21 no.1
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• pp.7-14
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• 2021

Purpose: Pseudohypoparathyroidism (PHP) is caused by genetic and epigenetic alteration in the GNAS locus, and characterized by the resistance to multiple hormones and the Albright's hereditary osteodystrophy (AHO) phenotype. This study investigated the phenotypic characteristics and molecular features of PHP. Methods: Eight patients who diagnosed as PHP were enrolled at Pusan National University Children's hospital and clinical features, biochemical and genetic findings were retrospectively reviewed. Results: Of a total of 8 patients, 5 were diagnosed with PHP1a, and 3 were diagnosed with PHP1b. Patients with PHP1a had three different mutations in the GNAS gene, and patients with PHPIb had imprinting defect in differentially methylated regions (DMRs) of the GNAS locus. Two novel GNAS variants were identified in patients with PHP1a, including c.313-2A>T and c.1094G>A. All patients with PHP1a displayed AHO features; short stature (80%), brachydactyly (80%), a round face (80%), obesity (40%), heterotopic ossification (60%), and intellectual disability (60%), whereas only one patient (33.3%) with PHP1b showed AHO feature such as a round face. When phenotypic features between PHP1a and PHP1b patients were compared, patients with PHP1b showed a tendency of higher current height standard deviation scores (SDS) compared to patients with PHP1a, (-3.2±2.1 vs.-1.1±0.8; P=0.06) Conclusions: This study summarizes the phenotypic and genetic features of the PHP patients. Although we found considerable clinical overlap between PHP1a and PHP1b, further long-term follow-up is needed to evaluate the growth and development of children with PHP, as well as the effects of end-organ resistances to endocrine hormones.