• 한국사회복지학회:학술대회논문집
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• 2002.04a
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• pp.497-531
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• 2002

It is very important for rehabilitation to deal with psychological aspects of persons with disabilities, as well as efforts improving the institutional and environmental conditions. A majority of persons with severe disabilities in the situation of Korea have difficulty in having and maintaining a job. Work should and would be a source of self-respect and material well-being in this modern society. Therefore, Vocational rehabilitation services are measures in restoration of family functions and social participation of persons with disabilities. This study aims at investigating linguistic characteristics and the validity of constructional concepts of picture interest test Inventories that have been utilized for the segregated groups of people such as persons with developmental disabilities. Picture interest test inventories seemed to be valid for measuring psychological traits and characteristics of people with mental retardation, and this finding can be extended to the group of other developmental disabilities, such as learning disabilities and mild/moderate behavioral deficits. The Holland classification system seemed to be best fitted for developing a comprehensive and accurate vocational interest inventory.

• Journal of the korean academy of Pediatric Dentistry
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• v.27 no.2
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• pp.202-207
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• 2000

The purpose of study was to compare the caries experience between the mentally retarded(MR) and the normal(N) children and adolescents and to survey the dental visit frequency. preventive dental care, and tooth-brushing behavior of the mentally retarded. Two hundred and fifty MR and two hundred and ten N subjects were selected for the study. 1. The number of teeth which had experienced caries was 3.28 in MR and 4.66 in N. The number of decayed teeth was 2.24 in MR and 1.50 in N. The number of filled teeth was 0.71 in MR and 3.06 in N Filled teeth rate was 19.78% in MR and 58.89% in N The differences between MR and N were all significant(P<0.01). 2. Dental visit frequency of MR was 1.15 per year, and the 51.2% of MR did not visit the dental clinic for the last one year. 38.0% of MR had received the preventive dental care. 3. Toothbrushing frequency per day was 2.16 and the toothbrushing time for 78.3% of MR was less than two minutes. 51.2% of MR brushed their teeth by themselves, 13.3% did with the help of the carers, and 35.5% totally depended on the carers. In conclusion, MR experienced less dental caries than N, had more untreated caries than N, and had need for regular preventive dental care.

• Journal of the korean academy of Pediatric Dentistry
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• v.39 no.4
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• pp.412-417
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• 2012

Mucopolysaccharidosis (MPS) is a disorder which is caused by the defect of the lysosomal enzyme that is essentially needed for resolution of glycosaminoglycans (GAGs). Metabolite of GAGs will accumulate in the lysosome of cells and will result in the dysfunction of cells, tissues, and organs. Eventually, patients will manifest both mental retardation and physical disorders. In worst cases, mucopolysaccharidosis can cause premature death. The current clinical types have been classified as MPS from type I to type IX according to the defect of certain enzyme. The dental complications have been reported as delay of eruption, enamel hypoplasia, microdontia, malocclusion, condylar defects, gingival hyperplasia and dentigerous cystlike follicle. This clinical report presents the case of a boy with MPS type II, Hunter Syndrome which has various dental complications.

• Journal of Yeungnam Medical Science
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• v.8 no.2
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• pp.24-34
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• 1991

Delayed emergence of speech or language are frequent causes for concern about development during early childhood. Dealy in evaluation and proper management until school entry in more likely to result in frustration, anxiety and school failure. Many language disturbances and their attendant behavior disorders respond to intensive language therapy. Authors analyzed the medical recoreds of 52 children with delayed language development evaluated during 30 months from January 1986 through June 1988. The results were as follows : 1. The majority of cases were evaluated at the age of 2-2.9 year old(16 cases, 30.8%) and 3-3.9 year old(11 cases, 21.2%) 2. Male to female ratio was 3 : 1 3. The most common cause was mental retardation(53.8%), followed by developmental language disorder(23.1%) and autism(13.5%) 4. The most common associated condition was dysarticulation(17.3%), followed by strabismus(9.6%) and seizures(7.7%) 5. Special education was recommended in cases of 23(44.2%), speech therapy in 12 cases(23.1%) and consultation to child psychiatry in 7 cases (13.5%). Making diagnosis of underlying disorders is not simple because assessment of intelligence in young children is difficult and only a few tests are standardized. More detailed study on children with delayed language development and development of psychometric tests for the handicapped children are necessary, especially in Korea.

• The Journal of the Korean life insurance medical association
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• v.29 no.2
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• pp.29-32
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• 2010

Moyamoya disease (MMD) is a progressive occlusive disease of the cerebral vasculature with particular involvement of the circle of Willis and the arteries that feed it. MMD is one of cerebrovacular accident,which is treated with sugical maeuver in pediatic neurosurgery. Moyamoya (ie, Japanese for "puff of smoke") characterizes the appearance on angiography of abnormal vascular collateral networks that develop adjacent to the stenotic vessels. The steno-occlusive areas are usually bilateral, but unilateral involvement does not exclude the diagnosis. The exact etiology of moyamoya disease is unknown. Some genetic predisposition is apparent because it is familial 10% of the time. The disease may be hereditary and multifactorial. It may occur by itself in a previously healthy individual. However, many disease states have been reported in association with moyamoya disease, including the following: 1) Immunological - Graves disease/thyrotoxicosis 2) Infections - Leptospirosis and tuberculosis 3) Hematologic disorders - Aplastic anemia, Fanconi anemia, sickle cell anemia, and lupus 4) Congenital syndromes - Apert syndrome, Down syndrome, Marfan syndrome, tuberous sclerosis, Turner syndrome, von Recklinghausen disease, and Hirschsprung disease 5) Vascular diseases - Atherosclerotic disease, coarctation of the aorta and fibromuscular dysplasia, 6)cranial trauma, radiation injury, parasellar tumors, and hypertension etc. These associations may not necessarily be causative but do warrant consideration due to impact on treatment.(Mainly neurosurgical operation.) The incidence of moyamoya disease is highest in Japan. The prevalence of MMD is 1 person per 100,000 population. The prevalence and incidence of moyamoya disease in Japan has been reported to be 3.16 cases and 0.35 case per 100,000 people, respectively. With regard to sex, the female-to-male ratio is 1.4:1. A bimodal peak of incidence is noted, with symptoms occurring either in the first decade(5-10yr) or in the third and fourth decades (30-40yr)of life. Mortality rates of moyamoya disease are approximately 10% in adults and 4.3% in children. Death is usually from hemorrhage. In aspect of life insurance, MR is 1700%, EDR is 16 per 1000 persons. Children and adults with moyamoya disease (MMD) may have different clinical presentations. The symptoms and clinical course vary widely from asymptomatic to transient events to severe neurologic deficits. Adults experience hemorrhage more commonly; cerebral ischemic events are more common in children. Children may have hemiparesis, monoparesis, sensory impairment, involuntary movements, headaches, dizziness, or seizures. Mental retardation or persistent neurologic deficits may be present. Adults may have symptoms and signs similar to those in children, but intraventricular, subarachnoid, or intracerebral hemorrhage of sudden onset is more common in adults. Recently increasing diagnosis of MMD with MRI, followed by surgical operation is noted. MMD needs to be considered as the "CI" state now in life insurance fields.

• Journal of Korean society of Dental Hygiene
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• v.8 no.3
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• pp.1-11
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• 2008

Oral health projects that cater to the disabled should be more prevailing in order to ensure the maintenance and successful promotion of the oral health of disabled people. 70 public dental clinics that conducted oral health projects geared toward the disabled were examined to get a precise grip on their oral health projects. The findings of the study were as follows: 1. 31 out of 70 public dental clinics investigated(44.3%) were equipped with two or more dental hygienists who were professional human resources in charge of the oral health projects for the disabled. As for the age and disability type of the beneficiaries of the oral health projects, adolescents(74.3%) and people with mental retardation(87.1%) benefited most from the oral health projects. Concerning the most common implementation frequency of the projects, the projects were carried out once to three times a week(62.9%). 2. The most dominant oral disease treatment provided to disabled people was amalgam treatment and resin treatment(68.6%), which were the early dental caries treatment. The most common preventive treatment that was offered to improve their oral health was oral prophylaxis(82.9%). As for reform measures for the oral health projects, education of personnels in charge of the projects and their specialization(58.6%) were most emphasized. 3. Regarding factors related to the preventive oral health projects for the disabled, the implementation of oral prophylaxis and toothbrushing education was linked to the age of the beneficiaries. More oral prophylaxis was offered to teens, and more toothbrushing education was provided to preschoolers and adolescents. The age of the beneficiaries and the number of dental hygienists responsible for the projects had something to do with the application of fluorides. 4. Concerning the relationship of the preventive oral health projects for the disabled to the number of dental hygienists, one of the personnels in charge of the projects, the application of fluorides( 54.4%) and pit & fissure sealing(56.8%) were more prevalent when there were two or more dental hygienists. There was a statistically significant disparity in that regard(p<0.05). The above-mentioned findings illustrated that in order to boost the oral health of the disabled, dental hygienists who are responsible for the oral health projects for the disabled should put ceaseless efforts into fostering their professional knowledge and ability and offering quality service to disabled patients. Every public dental clinic should be equipped with plenty of professional personnels to enlarge the scope of treatment and ensure the efficiency of treatment and the preventive projects.

• Clinical and Experimental Pediatrics
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• v.58 no.7
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• pp.256-262
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• 2015

Purpose: Kabuki syndrome is a multiple congenital malformation syndrome, with characteristic facial features, mental retardation, and skeletal and congenital heart anomalies. However, the cardiac anomalies are not well described in the Korean population. We analyzed the cardiac anomalies and clinical features of Kabuki syndrome in a single tertiary center. Methods: A retrospective analysis was conducted for a total of 13 patients with Kabuki syndrome. Results: The median age at diagnosis of was 5.9 years (range, 9 days to 11 years and 8 months). All patients showed the characteristic facial dysmorphisms and congenital anomalies in multiple organs, and the diagnosis was delayed by 5.9 years (range, 9 days to 11 years and 5 months) after the first visit. Noncardiac anomalies were found in 84% of patients, and congenital heart diseases were found in 9 patients (69%). All 9 patients exhibited left-side heart anomalies, including hypoplastic left heart syndrome in 3, coarctation of the aorta in 4, aortic valve stenosis in 1, and mitral valve stenosis in 1. None had right-side heart disease or isolated septal defects. Genetic testing in 10 patients revealed 9 novel MLL2 mutations. All 11 patients who were available for follow-up exhibited developmental delays during the median 4 years (range, 9 days to 11 years 11 months) of follow-up. The leading cause of death was hypoplastic left heart syndrome. Conclusion: Pediatric cardiologist should recognize Kabuki syndrome and the high prevalence of left heart anomalies with Kabuki syndrome. Genetic testing can be helpful for early diagnosis and counseling.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.3
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• pp.147-154
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• 2015

Purpose: 3HB and AcAc are two ketone bodies that can be used as energy source in brain via succinyl-CoA:3-ketoacid CoA transferase (SCOT) and mitochondrial acetoacetyl-CoA thiolase (beta-ketothiolase, T2), called ketolysis. In case of malfunction of these enzymes, ketolysis cannot occur fluently causing various clinical manifestations. We want to know the numbers of patients and clinical manifestations of ketolytic defects in Korea. Material: For 67 patients of ketolytic defects out of 2794 patients that have done urine organic acid analysis, we analyzed clinical manifestations and age distribution. The study period was from January 2007 to September 2015. Method: To confirm persistency of ketonuria, repeated and loading organic acid analysis were done at least 1 week period interval. SPSS was used for statistical analysis. Result: Thirty patients in infantile period (2 M-2 Y), 31 patients in childhood period (2 Y-12 Y), 5 patients after adolescent period (>12 Y) and 1 in neonatal period were diagnosed during the study period. The most frequent chief complaint was seizure followed by seizure with developmental delay and developmental delay only. Conclusion: Ketolytic defects were not so rare in Korea. Major clinical manifestations are seizure and developmental delay or mental retardation.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.2
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• pp.244-250
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• 2005

Self-injurious behavior is defined as deliberated harm to one's own body without suicidal intent. It usually occurs as head banging or hitting, body hitting, skin cutting, or finger biting and includes ocular, genital, and self-inflicted oral mutilation. Self-injurious behavior can occur with mental retardation, coma, psychotic problem, poisoning, or character disorders. In pediatric patients, self-injurious behavior usually is reported to lip, cheek and tongue biting, and many kinds of dental management methods have been introduced to prevent self-injurious behavior patients from self biting. This report presents two self-inflicted oral mutilation patients who were all treated successfully with appliances such as modified activator without wire for retention, modified tongue-rake appliance and mouth guard.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.2
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• pp.306-311
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• 2005

Lesch-Nyhan syndrome is a rare disorder of purine metabolism, first described in 1964. The incidence is estimated to be 1:100,000 birth. It is an X-linked recessive disorder in which affected males have a virtually complete deficiency of the enzyme hypoxanthine guanine phosphorybosyl transferase(HGPT). This enzyme deficiency gives rise to excessive uric acid production and consequent hyperuricemia. Lesch-Nyhan syndrome is clinically characterized by mental retardation, choreoathetosis, spastic cerebral palsy, and severe self-mutilation behavior. Patient with Lesch-Nyhan syndrome mostly bite their lip, tongue and finger. In severe cases, partial or total amputation of the lip and tongue is common. Self-inflicted bites are often further complicated by secondary infection to injuried site as well as pain. And tissue loss by biting results in esthetic problems. This report presents a Lesch-Nyhan syndrome patient with self-mutilation, who have a destruction of perioral tissue, especially the lower lip. He was treated successfully with soft mouthguard, psychological and pharmacological method.