• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• 제14권1호
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• pp.123-127
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• 2003

Cornelia de Lange 증후군은 여러 신체 기관의 선천적 기형과 성장지연, 신경 발달학적 이상을 동반하는 질환이다. 어떠한 생화학적 혹은 염색체 표지자도 아직까지 Cornelia de Lange 증후군에 특이적인 소견은 발견되지 못하였고 진단은 임상적인 관찰에 전적으로 의존하게 되어있다. 따라서 임상가의 경험이 본 질환의 발견에 중요한 만큼 저자들은 정신지체와 자폐장애를 보이는 Cornelia de Lange 증후군 사례를 보고하는 바이다. 본 증례의 환아는 6세 4개월의 여환으로 걷기 등의 운동발달이 늦었다고 하며 현재까지도 한단어도 말하지 못하는 심각한 언어지체를 보였다. 또한 눈 맞춤이 빈약하고 타인과 정서적 교류가 되지 않는 등 사회적 상호작용의 저하와 상동행동을 보였으며 따라서 정신지체와 자폐장애로 진단 할 수 있었다. 환아는 저신장을 보였고 눈썹이 짙고 속눈썹도 길고 짙었으며 치아 간격이 넓고 코 끝이 들려 있었으며 입주위에 수염이 나 있었다. 낮은 헤어라인을 보였고 몸통과 사지에 다모증을 보였으며 손가락은 짧은 편이었다. 방광 요관 역류와 잦은 상기도 감염과 폐렴의 과거력이 있었고 4차례 열성경련이 있었다. 본 증례의 경우 특징적인 얼굴 모습과 성장발달지연, 정신지체, 행동양상, 동반된 내과적 질환의 병력 등으로 Cornelia de Lange 증후군으로 진단할 수 있었다.

• 한국세라믹학회지
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• 제36권12호
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• pp.1327-1334
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• 1999

Behaviors of abnormal expansion during calcination process of Ba2Ti9O20 ceramics and its related effects on the sintering characteristics were investigated as a function of precursors. When BaCO3 and TiO2 powders were used as starting materials. BaTi4O9 phase which has relatively large molar volume was formed drastically with abnormal ex-pansion during the calcination at 95$0^{\circ}C$ to 115$0^{\circ}C$ ON the contrary using BaTiO3 and TiO2 powders as starting materials led to retardation of the formation of BaTi4O9 phase and concurrently suppressed the abnormal expansion during cal-cination process. Especially the calcined powder of BaTiO3 and TiO2 had advantages in the densification and formation of Ba2Ti9O20 single phase in the sintering process.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• 제6권1호
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• pp.109-115
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• 1995

2세 10개월된 중증 정신지체아동의 이미증 치료에서 지시따르기 훈련을 통한 장난감 놀이지도(DRO)와 이미증 제재수단으로 얼굴가리개(Facial Screening)가 사용되었다. 이 아동의 이미증은 주로 손가락 빨기, 장난감 입에 넣고 빨기, 주위의 물건에 닥치는 대로 입을 대고 빠는 형태이다 빠는 행위의 발생은 장난감 놀이지도 전에는 15분 당 평균 18.6회였으나, 놀이지도 기간에는 평균 5.4회, 얼굴가리개를 통해 빠는 행위를 제재한 치료기간에는 평균 2.3회로 감소되어, 얼굴가리개의 사용이 이 아동의 이미증 치료에 큰 효과를 보였다. 이 아동은 이미증 이외에 구토행위를 보였는데, 본 연구에서는 구토행위를 치료하지 않았으나, 놀이지도 및 이미증 치료기간 동안 구토행위가 저절로 감소된 현상을 보인 것은 기존 연구에서 밝혀진 바와 같이 한 행동의 치료가 다른 문제행동의 감소에 영향을 줄 수 있음을 증명하고 있다.

• 대한장애인치과학회지
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• 제1권1호
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• pp.18-24
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• 2005

The purposes of this study were 1) to investigate the oral health problem of the disabled according to the handicapped types, 2) to collect the empirical data for developing and establishing the oral health policies for the handicapped, and 3) to find out the major obstacles against the dental services and oral health promotion. The handicapped subjects were collected by designed cluster sampling and interviewed with structured questionnaire in order to measure the oral health knowledge and behaviors. The subjects were attending the special schools and private or public welfare institutions for crippled disorder, encephalopathy disorder, mental retardation, visual disturbance, hearing defect, and developmental disorder. Oral examination was conducted by 13 trained dentists. 1,476 of handicapped people were finally surveyed. The results were as follows; The experiences of visiting dental office during the last one year in 18~64 year-old disabled people were 48.86% in crippled disorder, 52.50% in mental retardation, 58.24% in visual disturbance, 39.29% in hearing defect, respectively. To improve this challenging situation, we should find out the obstacles against the dental service and oral hygiene maintenance by the types of handicap, and develop the oral health policies which could support and advocate the Korean disabled.

• Journal of mucopolysaccharidosis and rare diseases
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• 제5권1호
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• pp.29-33
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• 2021

Prader-Willi Syndrome (PWS) is a neurodevelopmental genomic imprinting disorder involving a lack of gene expression from the paternal chromosome 15q11-q13 region. This is typically due to paternal 15q11-q13 deletions (in approximately 60% of cases), maternal uniparental disomy 15, or when both 15s are from the mother (about 35% of cases). An imprinting center controls the expression of imprinted genes in the chromosome 15q11-q13 region. PWS is a neurodevelopmental disorder characterized by mental retardation and distinct physical, behavioral, and psychiatric features. Characteristic behavioral disturbances in PWS include excessive interest in food, skin picking, difficulty with a change in routine, temper tantrums, obsessive and compulsive behaviors, and mood fluctuations. Individuals with PWS typically have intellectual disabilities (borderline to mild/moderate mental retardation) and exhibit a higher overall level of behavior disturbances compared to individuals with similar intellectual disabilities. This condition severely limits social adaptations and quality of life. Different factors have been linked to the intensity and form of these behavioral disturbances, but there is no consensus regarding the cause. Consequently, there is still controversy surrounding management strategies and there is a need for new data. PWS is a multisystem disorder. Family members, caregivers, physicians, dieticians, and speech-language pathologists all play an important role in the management and treatment of symptoms in an individual with PWS. Here we analyze behavioral problems in children and adults with PWS by age and review appropriate management and treatment strategies for these symptoms.

• 한국공작기계학회논문집
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• 제10권4호
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• pp.7-13
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• 2001

This Study is to investigate the influence of weld residual stresses on the fatigue crack growth behaviors in pressure ves-sel reinforcement. In order to perform this study, the automatically welded specimens are prepared. The material is ASTM A516 grade 60 steel used in pressure vessel mainly. For pad-on-plate of skip welding continuous welding and PWHT specimen, fatigue crack initiation is generally initiat-ed at weld starting and end toe zone, and ruptured at weld starting toe zone, Fatigue life if pad-on-plate continuous speci-men is increased more than that of pad-on-plate skip fillet welding specimene about 85% under low load, about 20% under high load, and decreased than that of two-pad continuous welding specimen about 85%. In da/dN-$\Delta$ Κ curve under low load, pad-on-plate skip fillet welding specimen showed retardation on the initial crack, and the fatigue crack growth rate at the low region of $\Delta$Κ greater specimene E(3.8{\times}10^{-6}$mm/cycle). And the fatigue life of welding specimen was smaller than that of PWHT specimen.

• 한국안전학회지
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• 제16권3호
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• pp.1-6
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• 2001

The study is to investigate the influence on the fatigue crack growth behaviors for the welded configuration in pressure vessel stiffener. In order to perform this goal, the automatic welded specimens were prepared. The material is ASTM A516 grade 60 steel used in pressure vessel mainly. In skip welding of pad-on-plate, continuous fillet welding and PWHT specimen, fatigue crack is generally initiated at the starting and end old toe zone, and ruptured at the starting old toe zone. The fatigue life of pad-on-plate of the continuous fillet welding specimen is larger than that of pad-on-plate skip fillet welding specimen about 85% under low load, about 20% under high load and less than that of two-pad continuous fillet welding specimen about 85%. In da/dN-$\Delta$K curve under low load, skip fillet welding specimen of pad-on-plate showed retardation on the initial crack, and the fatigue crack growth rate at the low region of $\Delta$K greater specimen E($3.8 {\times} 10^{-6}mm/cycle$). And the fatigue life of welding specimen was smaller than that of PWHT specimen.

• 재활간호학회지
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• 제14권2호
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• pp.95-102
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• 2011

Purpose: The purpose of this study was to analyze intervention studies related to Snoezelen (multisensory environment). Methods: Studies related to Snoezelen (multisensory environment) published between 1995 and 2010 in both Korean and International journals were systematically reviewed, and analyzed following guidelines. Based on inclusion criteria, 23 studies including 5 Korean and 18 International articles were selected. Results: Most studies were conducted in various area of research such as medicine, nursing, and occupational therapy. There was no publication related to Snoezelen (multisensory environment) in Korean nursing journals. In terms of target population, more than 65 % of the study subjects were patients with dementia, mental retardation, and learning disability. Intervention was implemented mostly in less than 30 minutes, once a week for 2 to 4 weeks. The effects on behavior, physical, and psychological contexts were assessed as outcome indicators. There was more 'positive' than 'no effect' in self-stimulatory behaviors, problem behaviors, heart rate, pain, mood state, and anxiety, whereas more 'no effect' than 'positive' in blood pressure, respiration, enjoyment, and relaxation. Conclusion: Future studies are needed to develop the protocol and outcome indicators for effective use of this new intervention in Korea.

• International Journal of Precision Engineering and Manufacturing
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• 제2권3호
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• pp.19-26
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• 2001

In order to investigate the effect of single tensile overload on the short crack growth behavior under the out-of-plane cyclic bending moment, crack opening stresses were continuously measured by an elastic compliance method using strain gages. The characteristics of short crack growth after the single tensile overload are analyzed by the effective stress range ratio. Futhermore, the investigation was carried out with respect to various fatigue crack growth behaviors such as the plastic zone size effect on crack retardation, the retarded crack length and the number of cycles.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제49권4호
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• pp.228-232
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• 2023

Lesch-Nyhan syndrome (LNS) is a rare X-linked recessive disorder caused by a mutation in the hypoxanthine phosphoribosyltransferase 1 (HPRT1) gene. This syndrome is characterized by excessive production of uric acid, mental retardation, self-mutilation, choreoathetosis, and spasticity. The most distinctive symptom is compulsive self-mutilation. For patients with LNS, different methods have been tried to reduce self-biting behaviors including restraints, behavioral treatment, medications, deep brain stimulation, tooth extraction and botulinum toxin A injection. In this report, we present a case of LNS undergoing cheiloplasty due to self-mutilation and tooth extraction of the left deciduous maxillary canine.