• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.16 no.1
• /
• pp.34-41
• /
• 2016

Adult-onset type II citrullinemia (CTLN2) is characterized by episodes of neurologic symptoms associated with hyperammonemia leading to disorientation, irritability, seizures, and coma. CTLN2 is distinct from classical citrullinemia, which is caused by a mutation of the argininosuccinic acid synthetase (ASS) gene. The serum citrulline level is elevated, while the activity of ASS in liver tissue is decreased. CTLN2 is known to have a poor prognosis if the proper treatment is not taken. We reported a female aged 37 years who developed recurrent attacks of altered consciousness, aberrant behavior, and vomiting. We initially suspected the patient had CTLN2 because of the signs of hyperammonemic encephalopathy, such as altered mentality, memory disturbance, and aberrant behaviors provoked by exercise-induced stress and excessive intravenous amino acid administration. Through her peculiar diet preferences and laboratory findings that included hyperammonemia and citrullinemia, we diagnosed the patient as CTLN2, and SLC25A13 sequencing revealed known compound heterozygous mutations (IVS11+1G>A, c.674C> A). Her parents were heterozygous carriers, and we identified that her older sister had the same mutations. The older sister had not experienced any episodes of hyperammonemia, but she had peculiar diet preferences. The patient and her sister have been well with conservative management. When considering the clinical course of CTLN2, it was meaningful that the older sister could be diagnosed early in an asymptomatic period and that preemptive treatment was employed. Through this case, CTLN2 should be considered in adults who present symptoms of hyperammonemic encephalopathy without a definite etiology. Because of its rare incidence and similar clinical features, CTLN2 is frequently misdiagnosed as hepatic encephalopathy, and it shows a poor prognosis due to the lack of early diagnosis and proper treatment. A high-carbohydrate diet, which is usually used to treat other urea cycle defects, can also exaggerate the clinical course of CTLN2, so proper metabolic screening tests and genetic studies should be performed.

• Korean Journal of Social Welfare
• /
• v.57 no.3
• /
• pp.305-335
• /
• 2005

This paper is a result from validation study for SPDA(A Screening Scale For Potential Drug-use Adolescents) created in 2003 and newly developed during 2004. SPDA aims to screen adolescents in their early stage of drug-use and to help practitioners make a preventive approach for the adolescents. 4307 junior and senior high school students were selected as primary research subjects by stratified and quota sampling methods. 305 adolescents on probation were also selected as a comparison group and asked to answer the same questionnaire. Reliability for SPDA recorded 0.914, which proved to be better than previous year's (0.898). Exploratory and confirmatory factor analyses to test construct validity proved that SPDA could be divided into 7 factors and that each factor structure of SPDA could be a proper measurement model with high level of fitness and factor loadings. Discriminant analysis to test predictive validity confirmed that SPDA could classify the adolescents excellently by the frequency of drug-use, with hit ratio of 86.6 percent(78.8% and 87.4% for junior and senior high school students respectively). For concurrent validity test, Hare Home Self-Esteem Scale, Hare School Self-Esteem, Zuckerman-Kuhlman Sensation-seeking Scale were employed to find correlation with SPDA and all the three scales had significant Pearson correlation coefficients with SPDA. Known-groups validity test indicated that SPDA had an adequate power to classify out adolescents on probation from those in schooling, with a hit ratio of 71.8 percent. Cut-off point to detect adolescents with high risk of substance use was 77, which indicated approximately T score, 55 (0.5 SD), satisfying sensitivity, specificity, and efficiency criteria.

• Proceedings of the Korea Water Resources Association Conference
• /
• 2004.05b
• /
• pp.1310-1315
• /
• 2004

용수수요 추정의 기본은 수도계획에 사용할 수 있는 실사용량에 대한 조사로 이에 대한 자료가 거의 없기 때문에 각 수도사업별로 제시되는 추정방법이 조금씩 상이하며, 추정방법의 진위를 가릴 수 없이 수요추정의 악순환을 초래하고 있는 실정이다. 기존 물사용량 예측은 급수량 기준의 도시 전체에 내한 평균 LPCD를 이용함에 따라 물사용 특성을 충분히 고려찬 수 없어 지역별 실제 물사용량과 큰 오차가 유발되었다. 그러므로 수도계획 및 설계에 사용할 수 있는 신뢰성 있는 설계인자를 도출하기가 불가능하여 물수요 관리정책 수립, 수도요금체계 조정 및 누수방지계획 수립 등 경제적인 수도시설의 건설에 애로를 겪고 있다. 본 연구에서는 생활용수 중 가정용수에 대하여 세탁기, 변기, 싱크대 등 수도전에 유량계를 설치 실제 가정에서 사용하고 있는 용도별 사용량을 실측, 파악하였으며, 이로부터 얻은 용도별 사용량에 대한 기간별 소비특성을 분석하였다. 이로부터 생활용수 사용량의 소비패턴 및 시간대별 부하율 산정이 가능하며, 각종 용도별 사용수량의 소비형태를 찾아낼 수 있었다. 또한 가정용수 중 용도별 물 사용비율은 세탁용수, 변기, 주방, 목욕용수의 순으로 나타났으며 주택유형별로는 아파트 연립주택, 다세대 주택, 단독주택의 순으로 단독주택에서의 물사용량이 가장 적게 나타났다. 생활용수 공급량에 내해서는 시간별, 주별, 월별 그리고 계절변동 총량을 파악하였으며 시간대별로는 오후 3시경이 최대 소비량을 보였고, 주별로는 월요일 그리고 월별로는 7월의 용수사용량이 가장 큰 것으로 조사 되었다. 본 연구로부터 도출된 용도별 실측 물사용량 자료 및 분석결과로부터, 지금까지 공급량 기준의 계획수립이 이루어져왔던 파종 수도시설 규모결정시 합리적인 용수수요예측 및 수요관리가 이루어질 수 있을 것으로 판단되며, 용수수요의 과다예측 오해 해소 등 경제적, 과학적 물관리 정책수립을 위한 기초자료를 제공할 수 있을 것으로 기대된다.는 경제적인 방법이 될 수 있다. 하천수 등의 상호 관계 분석을 통해 장기간의 유역 물순환체계 변화를 분석할 수 있었다.골풀과, 닭의장풀과가 각 1종씩으로, 조사지점( I )보다 좀 더 많은 종이 분포하는 것으로 조사되었다. 또한 어류는 조사지점( I )에서 3회에 걸쳐 총 396개체가 채집되어 3목 8과 21종이었다. 이 중 한국 고유종은 11종이었고, 외래 어종은 검정우럭과 2종이 조사되었으며, Zacco platypus(피라미), Zacco temmincki(갈겨니), Acheilongnathus koreanus(칼납자루), Odontobutis platycephala(동사리), Coreoleuciscus splendidus(쉬리) 순으로 분포하고 있었고, Acheilognathus signifer(묵납자루)는 댐 건설 전에는 많이 분포하였으나 현장조사에서 서식을 확인 할 수 언어 개체수의 큰 감소내지 멸종된 것으로 추정되었다.에서 동시에 시행하였다. 수술 후 1년 내 시행한 심초음파에서 모든 환아에서 단지 경등도 이하의 승모판 폐쇄 부전 소견을 보였다. 수술 후 조기 사망은 없었으며, 합병증으로는 유미흉이 한 명에서 있었다. 술 후 10개월째 허혈성 확장성 심근증이 호전되지 않아 Dor 술식을 시행한 후 사망한 예를 제외한 나머지 6명은 특이 증상 없이 정상 생활 중이다 결론: 좌관상동맥 페동맥이상 기시증은 드물기는 하나, 영유아기에 심근경색 및 허혈성 심근증 또는 선천성 승모판 폐쇄 부전등을 초래하는 심각한 선천성 심질환이다. 그러나 진단 즉시 직접 좌관상동맥-대동맥 이식술로 수술적 교정을 해줌으로써 좋은 성적을 기대할 수 있음을 보여주었다.특히 교사들이 중요하게 인식하는 해방적 행동에 대한 목표를 강조하여 적용할 필요가 있음을 시사하고 있다.교하여 유의한 차이가 관찰되지 않았다. 또한 HSP 환자군에서도 $IL1RN^{*}2$ allele 빈도와 carriage rat

• Proceedings of the Korea Water Resources Association Conference
• /
• 2004.05b
• /
• pp.148-152
• /
• 2004

본 연구에서는 물순환의 재생이나 보전에 필수적인 유역 물순환의 정량화와 유역변화의 영향예측을 위해 개발된 WEP (Water and Energy Transfer Process) 모형의 국내 유역에 내한 적용성을 검토하고, 청계천 유역의 물순환 양상을 모의하였다. WEP 모형은 복잡한 토지이용이 이루어지고 있는 도시하천 유역에 내한 물순환의 정량화를 목적으로 일본의 토목연구소 (PWRI; Public Works Research Institute), 과학기술진흥사업단, Jia 박사 등에 의해 공동으로 개발되었으며 지표면 및 비포화 토양층의 물${\cdot}$열 플럭스 계산, 하도흐름의 추적계산 및 지하수 유동계산, 격자내 토지이용의 불균질성 반영 등이 가능한 물리적인 기반의 공간 분포형 모형 (Physically Based Spatially Distributed)이다. 모형을 적용한 청계천 유역 (유로연장 13.75 km, 유역 면적 $50.96km^2$)은 전체 토지이용중 도시지역이 $75.9\%$를 차지하고, 유역내 인구가 120만명에 이르는 도시유역으로 높은 불투수 면적비율, 인공계 물순환 요소의 영향 등의 도시 유역 특성이 물순환의 구조 전반에 미치는 영향에 대한 연구가 부족하였다. WEP 모형 적용 결과, 모의 기간 동안의 하천 유출량은 실측치에 근사한 값을 나타내었으며 유역의 물순환 양상을 모의할 수 있었다. 청계천 유역은 전형적인 도시 유역의 특성을 보여주었는데, 강우시의 직접유출이 크고, 강우의 유출에 대한 반응이 빠르며, 증발산의 경우는 산림지역보다 도시지역이 상대적으로 적은 것으로 분석되었다. 이번 연구를 통하여 WEP 모형이 유역 물순환 해석에 적절한 모형임을 확인할 수 있었으면, 향후 청계천 유역의 물리적 특성에 대한 매개변수와 인공계 물순환 자료의 보완을 통해 보다 향상된 모의가 가능할 것으로 판단된다. 하였던 Cd과 Mg이 Ca 및 Ca과 vitamin D의 동시(同時) 급여(給與)로 감소(減少)하였고 Cu는 전체적(金體的)으로 변화(變化)가 없었으며 Zn은 Cd 급여(給與)로 감소(減少)하였으나 Ca과 vitamin D의 급여(給與)에 의하여 증가(增加)하였고 Ca은 Ca과 viamin D의 급여(給輿)로 유의(有意)하게 증가(增加)하였다. 신장(腎臟)중의 무기질(無機質) 함량(含量)은 Cd급여(給輿)로 Cu, Mg은 감소(滅少)하였으나 Ca, Zn은 변화(變化)가 없었고 Ca 및 Ca과 Vitamin D의 급여(給與)로 Cd, CU, Zn은 증가(增加)하였다.ce area)는 수술 전100.8$\pm$25.6 mm/$m^{2}$에서 79.3$\pm$ 15.8 mm/$m^{2}$로 감소한 소견을 보였다. 승모판 성형술은 전 승모판엽 탈출증이 있는 두 환아에서 동시에 시행하였다. 수술 후 1년 내 시행한 심초음파에서 모든 환아에서 단지 경등도 이하의 승모판 폐쇄 부전 소견을 보였다. 수술 후 조기 사망은 없었으며, 합병증으로는 유미흉이 한 명에서 있었다. 술 후 10개월째 허혈성 확장성 심근증이 호전되지 않아 Dor 술식을 시행한 후 사망한 예를 제외한 나머지 6명은 특이 증상 없이 정상 생활 중이다 결론: 좌관상동맥 페동맥이상 기시증은 드물기는 하나, 영유아기에 심근경색 및 허혈성 심근증 또는 선천성 승모판 폐쇄 부전등을 초래하는 심각한 선천성 심질환이다. 그러나 진단 즉시 직접 좌관상동맥-대동맥 이식술로 수술적 교정을 해줌으로써 좋은 성적을 기대할 수 있음을 보여주었다.특히 교사들이 중요하게 인식하는 해방적 행동에 대한 목표를 강조하여 적용할 필요가 있음을 시사하고 있다.교하여 유의한 차이가 관찰되지 않았다. 또한 HSP 환자군에서도 $IL1RN^{*}2$ allele 빈도와 carriage

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.5 no.1
• /
• pp.33-43
• /
• 2005

Purpose: X-linked adrenoleukodystrophy(ALD) is a rare disorder that shows a great deal of phenotypic variability. We subdivided chidhood X-linked ALD patients into several phenotypes by the age at onset, the sites of most severe clinical involvement and the rate of progression of neurologic symptoms. Methods: Thirteen patients who had been diagnosed as X-linked ALD and followed up for at least one year were enrolled from 1996 to 2003. Results: 1. Ten had childhood cerebral ALD, who showed first neurologic symptoms at 7.02 years and progressed rapidly: interval between first symptoms and vegetative state was 1.35 years, and interval from initial symptoms to death was 3.35 years. Treatment with Lorenzo's oil did not prevent neurologic progression. Two patients who underwent umbilical cord blood transplantation died. 2. Two had adolescent cerebral ALD. They had first symptoms at 11.5 years, and showed tendency to progress less rapidly than childhood cerebral form patients. 3. One "Addison only" patient who had adrenal insufficiency without nervous system involvement remained asymptomatic during Lorenzo's oil treatment. 4. All cerebral form patients except one showed the lesions in both parieto-ocipital white matter in brain magnetic resonance imaging. Conclusion: The cerebral ALD was the most common form in childhood and was asoociated with a serious prognosis.

• Journal of Music and Human Behavior
• /
• v.3 no.1
• /
• pp.1-11
• /
• 2006

It has been passed more than 50 years of the history of music therapy on school aged children since there had been enforced the 'Education for All Handicapped Children Ace' in United states 1975, which had made an important role of music therapist augmented. In the case of South Korea, through reforming the law on March 2005 that therapist educator has to be allocated on the special class in general school, at least, patrol teacher is charged on that, which made possible to start music therapy as initial stage was realized in realistic way on the spot of school. This research has primary concerns as following. The one thing is intended that figuring the opinions of special educators and music therapists involved with the music therapy in elementary school. And the other thing is intended that making a investigation of actual condition on the spot of existing school, moreover, pinpointing at issue and suggesting the change for the better than now. In order to undertake theses intentions, I examined preceding researches and existing data, distributed the questionnaire involved the music therapy of elementary school to the people who are composed of special educators-65 persons and music therapists-30 persons from 18th October to second of November, and analyzed collected by descriptive statistics. According to the analyzed results, it says that special educator and music therapist know well about the necessity of music therapy in elementary school and its effectiveness is manifested in positive way. Moreover, in order to systemize and promote a program of music therapy, what is most urgent thing is the completion of instrument and music therapy room and there are needed strong supports at the level of nation and institution. It has become pervasive on the demand of therapy education by parents in all of the places involved with disabled children such as special school, special class, local welfare community, and early education. This research shows that how therapy and education was connected as music therapy on the spot of the elementary school, and the necessity of the education of music therapy.

• The Korean Journal of Food And Nutrition
• /
• v.20 no.1
• /
• pp.88-95
• /
• 2007

The purpose of this study was to investigate the effects of a 12 week weight control program, which included nutrition education and regular physical fitness, on body weight, degree of obesity, BMI, and lowering of blood lipids in 16 obese male elementary school students. Changes in waist and hip circumferences and their ratios were measured at the conclusion of the 12 week program conducted from September 9 to November 25, 2005. Body weight after 12 weeks on the program slightly reduced from 55.4${\pm}$10.9 kg to 54.4${\pm}$9.7 kg. Degree of obesity and BMI decreased from 121.3${\pm}$8.1%, and 25.9${\pm}$3.3 to 120.0${\pm}$8.2% and, 25.6${\pm}$2.7, respectively. Body fat percentage decreased slightly from 38.3${\pm}$4.7% to 37.9${\pm}$5.2%. Waist to hip ratio did not change after 12 weeks. There were slight changes in weight of body fat and lean body mass from 22.12${\pm}$6.53 kg and 34.56${\pm}$4.75 kg to 21.68${\pm}$6.05 kg and 34.70${\pm}$4.54 kg, respectively. Also, after 12 weeks on the weight control program, body, waist, and hip circumferences decreased. However, there were no significant differences among the blood lipid components, including triglycerides, total cholesterol, and HDL-cholesterol. Finally, body weight, degree of obesity, BMI, and body fat showed more significant correlations to waist, hip circumference and their ratio than to blood lipid levels.

• Korean Journal of Environment and Ecology
• /
• v.10 no.1
• /
• pp.1-13
• /
• 1996

This study was conducted to investigate the avifauna and to suggest the management discipline for the protection of bird community in Odaesan National Park. Field survey was carried over 2 main trails by line transect method from mid June to early December in 1996. The 1st section included the area from Woljongas to Sangwonsa 7km distance. The 2nd section survey started from Sangwonsa via Bukdaesa and the summit of Odaesan to Sangwonsa 9.8 km distance. The study results were as follows ; The observed birds belonged to 9 orders 22families 52species, they also had Black Woodpecker(Dryocopus martius)designated as natural monument No. 242, Chinese Sparrow Hawk(Accipiter soloensis) and Kestrel(Falco tinnunculus) as natural munumet No. 323, Scops Owl(Out scops)and Korean Wood Owl(Strix aluco) as No. 324. These birds also were classified into 25 species for residents, 16 species for summer visitors, 8 species for passage migrants, 3 species for winter visitors, respectionely. The 2nd section showed a high species richness and individuals in every season, however, had a difference in species composition with 1st section. Nesting guild of breeding bird community used highly in order of bush, hole, and canopy as a nest resources. It is suggested that high bush-nesting guild had a deep relationship with bush layer located in the ecotone of 1st section and that located in the high elevated zone in 2nd section. Hole-nesting guild such as Black Woodpecker(Dryocopus martius), Gray-headed Woodpecker(Picus canus) and Great Spotted Woodpecker(Dendrocopos major) were surveyed only in 2nd section, so it could be attribute to the small fragmentation and the growing of high diameter at breast height(D.B.H) tree in 2nd section. It is urgent that the management of camping ground and people for the conservation of brook in 1nd section, trail protection for the prevention from trail enlargement in 2nd section for the bird protection. Artificial food in snowy winter will provide the good breeding condition with the residents and migrants. And, the endeavor to lessen the habitat fragmentation will be beneficial to the birds who have a large home range such as Black Woodpecker(Dryocopus martius) and Korean Wood Owl(Strix aluco). For the control of Domestic Dove(Columba livia) populations, it could be recommended that the elimination of their nesting resources by net.

• The Journal of Korean Philosophical History
• /
• no.28
• /
• pp.183-212
• /
• 2010

The purpose of this study is to provide a theoretical base for making a character education program and to develop a school violence prevention program and analysis the effects of the program in elementary schools. The prevention program was designed to target students, teachers, parents and community residents and utilized the above groups as participants. This study is developed on the basis of Social Emotional Learning as well as Emotional Intelligence which put on the importance on the role of emotion in the problem solving. In concrete, school violence prevention program based on the social and emotional learning, development of integrative programs articulating three key domains directly and indirectly influencing students' character formation, maximum use of the educational institutes' moral education curriculums and potential curriculums in the surrounding environment. To do so, bibliographical study and analysis of the research materials are carried out, and also the professionals' advice is received. The study object is 113 elementary school students of 2 elementary schools in Seoul. For them, the preparatory program is carried out from September 2009 to November 2009. And then, the analysis for the satisfaction degree of participation in the program and for the descriptive results of the school violence prevention education is carried out.

• Journal of Korean Society of Disaster and Security
• /
• v.6 no.1
• /
• pp.65-72
• /
• 2013

In this study, the early suicide prevention program was applied to the elementary school students and compared the prior & post effect of the program, and verified the status of psychology change like emotional status, or temptation to take a suicide, and presented the possibility as a suicide prevention program. The period of adolescence is the very unstable period in the process of growth being cognitively immature, emotionally impulsive period. It is the period emotionally unstable and unpredictable possible to select the method of suicide as an extreme method to escape the reality, or impulsive problem solving against small conflict or dispute situation. Many stress of the student such as recent nuclear family, expectation of parents to their children, education problem, socio-environmental elements, individual psychological factor lead students to the extreme activity of suicide in recent days. In this study, the scope of stress experienced in the elementary school as well as idea and degree of temptation regarding suicide by the suicide prevention program were identified, and through prevention program such as meditation training, breath training and through experience of anger control, emotion-expression, self overcome and establish positive self-identity and make understanding Self-control, Self-esteem & preciousness of life based on which the effect to suicide prevention was analyzed. The study was made targeting 51 students of 2 classes of 6th grade of elementary school of Goyang-si and processed 30 minutes every morning focused on through experience & activity of the principle & method of brain science. The data was collected for 20 times before starting morning class by using Suicide Probability Scale(herein SPS-A) designed to predict effectively suicide Probability, suicide risk prediction scale, surveyed by 7 areas such as Positive outlook, Within the family closeness, Impulsivity, Interpersonal hostility, Hopelessness, Hopelessness syndrome, suicide accident. Analytical methods and validation was used the Wilcoxon's signed rank test using SPSS Program. Though the process of program in short period, but there was a effective and positive results in the 7 areas in the average comparison. But in the t-test result, there was a different outcome. It indicated changes in the 3 questionnaires (No.7, No.14, No.19) out of 31 SPS-A questionnaires, and there was a no change to the rest item. It also indicated more changes of the students in the class A than class B. And in case of the class A students, psychological changes were verified in the areas of Hopelessness syndrome, suicide accident among 7 areas after the program was processed. Through this study, it could be verified that different results could be derived depending on the Student tendency, program professional(teacher in charge, processing lecturer). The suicide prevention program presented in this article can be a help in learning and suicide prevention with consistent systematization, activation through emotion and impulse control based on emotional stress relief and positive self-identity recovery, stabilization of brain waves, and let the short period program not to be died out but to be continued connecting from childhood to adolescence capable to make surrounding environment for spiritual, physical healthy growth for which this could be an effective program for suicide prevention of the social problem.