• Journal of mucopolysaccharidosis and rare diseases
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• 제4권2호
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• pp.42-44
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• 2018

Prader-Willi syndrome (PWS) is a multisystemic complex disorder characterized by hyperphagia and impaired satiety which lead to severe and early obesity. In infancy, hypotonia and poor suck are main problems, and a child goes through Failure-to-thrive. During childhood, clinical manifestations change to food seeking as well as excessive weight gain, short stature, developmental delay, cognitive disability and behavioral problems. Also, growth hormone insufficiency is frequent. Most patients receive the recombinant growth hormone (rGH) therapy that provides improvement in growth, body composition, and physical attributes. The clinical care guideline for rGH therapy in PWS had been noticed in 2013. The rGH therapy helps in body fat, lean body mass, height SDS and head circumference. Also, the rGH therapy helps motor function, psychomotor development and cognition and behavioral issues.In Samsung medical center, there are clinical care guidelines for rGH therapy in PWS and an useful application for the patients. 'Living with PWS', the name of an moblie application for PWS patients, was introduced in the lecture. The application revised to version 2. It was made more convenient to users than in version 1. It helps caregivers to schedule the rGH therapy and to monitor height and weight.

• Clinical and Experimental Pediatrics
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• 제62권5호
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• pp.187-192
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• 2019

Purpose: The importance of the neurodevelopmental outcomes of very-low-birth-weight (VLBW) infants has been emphasized as their mortality rate has markedly improved. This study aimed to assess the validity of the Korean Developmental Screening Test (K-DST), a developmental screening tool approved by the Korean Society of Pediatrics, for the timely diagnosis of neurodevelopmental delay in VLBW infants. Methods: Subjects included VLBW infants enrolled in the Korean Neonatal Network database between January 2012 and December 2014. The collected data were analyzed for sensitivity, specificity, positive predictive value, and negative predictive value (NPV) in the K-DST compared to those in the Bayley Scales of Infant Development-II for VLBW infants. Results: A total of 173 patients were enrolled. Their mean gestational age and mean birth weight were $27.5{\pm}2.8weeks$ and $980.5{\pm}272.1g$, respectively. The frequency of failed psychomotor developmental index (PDI) <85 was similar to that in at least one domain of K-DST <1 standard deviation. Failure in more than one K-DST domain compared with a mental developmental index (MDI) <85 showed a sensitivity and NPV of 73.2% and 75.0%, respectively. Failure in more than one K-DST domain compared with PDI <85 showed a sensitivity and NPV of 60.3% and 71.6%, respectively. Each K-DST domain had a stronger correlation with predicting a failing MDI <85 than a failing PDI <85 (P<0.05). Conclusion: K-DST could be a useful screening tool for predicting mental developmental delay in VLBW infants and referring them for neurodevelopmental assessments.

• Neonatal Medicine
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• 제15권2호
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• pp.123-133
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• 2008

Purpose: The purpose of this study was to determine the outcomes of extremely low birth weight infants (ELBWI) who were born at the Asan Medical Center and evaluate the recent status of neonatal intensive care and associated problems. Methods:We retrospectively evaluated 120 inborn ELBWI who were admitted to the NICU of the Asan Medical Center between 2003 and 2006. The survival rate, neurodevelopmental outcomes, maternal and infant factors, and infant mordibities were evaluated and the relationships with survival and catch-up growth were investigated. Results:The survival rate of the ELBWI was 82% at a mean gestational age of 27+2 weeks, and with a mean birth weight of 801.3${\pm}$129.0 g. The duration of hospitalization was 85.7${\pm}$27.2 days, the duration of O2 use was 43.9${\pm}$35.4 days, and the duration of ventilatory support was 20.9${\pm}$20.9 days among the survivors. The incidence of respiratory distress syndrome, chronic lung disease, severe intraventricular hemorrhage, and periventricular leukomalacia were 41.8%, 61.2%, 3%, and 4%, respectively. The mean mental developmental index and psychomotor development index of Bailey Scales of Infant Development (II) at follow-up were 83.4${\pm}$18.2 and 83.3${\pm}$20.3, respectively. Among the infants who had >18 months of follow-up, 50.8% had catch-up growth at 12 months. Conclusion:The survival rate of ELBWI has improved; however, the morbidities remain high, thus indicating further efforts must be implemented to reduce morbidity and improve neurodevelopmental outcomes.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• 제26권3호
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• pp.183-189
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• 2015

Objectives : The aim of this study was to investigate Korean Wechsler Intelligence profiles and specific abilities related to attention problem of children with attention-deficit hyperactivity disorder (ADHD). Methods : The Korean Wechsler Intelligence Scale for Children-fourth edition (K-WISC-IV) and Advanced Test of Attention (ATA) were administered to 91 children and adolescents (age $8.5{\pm}2.6$ years, 73 boys) with ADHD. Pearson correlation and independent t-tests were used. Results : The means of Working Memory Index (WMI) and Processing Speed Index (PSI) showed a score of low average in K-WISC-IV. WMI scores for the K-WISC-IV showed clinically significant correlations with omission errors, commission errors, and response time variability on auditory ATA. PSI scores also showed significant correlations with response time and variability on visual ATA. In addition, significantly lower digit span backward scores were observed in hyperactive-impulsive/combined subtypes compared to inattentive subtype (t=3.60, p<.001). Conclusion : Children with ADHD showed significantly lower scores in WMI and PSI which were clinically correlated with ATA scores, and hyperactive-impulsive/combined subtypes showed poorer working memory functions in WMI. Follow-up studies are proposed.

• 한국응용약물학회:학술대회논문집
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• 한국응용약물학회 2004년도 Annual Meeting of the Korean Society ofApplied Pharmacology
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• pp.18-34
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• 2004

This paper will review data obtained primarily from our preclinical investigations that show that exposure to stress has a significant impact on drug taking. Stress increases reward associated with psychomotor stimulants, possibly through a process similar to sensitization, and a growing clinical literature indicates that there is also a link between substance abuse and stress in human addicts. One explanation for the high concordance between stress-related disorders and drug addiction is the self-medication hypothesis, which suggests that a dually-diagnosed person often uses the abused substance to cope with tension associated with life stressors or to relieve symptoms of anxiety and depression resulting from a traumatic event. However, another characteristic of drug self-administration is that drug delivery and its subsequent effects on the HPA axis are under the direct control of the individual. This controlled activation of the HPA axis may result in the production of an internal state of arousal or stimulation that is actually sought by the individual (i.e., the sensation-seeking hypothesis). During abstinence, however, exposure to stressors or drug-associated cues can stimulate the HP A axis to remind the individual about the effects of the abused substance, thus producing craving and promoting relapse. Stress reduction, either alone or in combination with pharmacotherapies targeting the HPA axis may prove beneficial in reducing cravings and promoting abstinence in individuals seeking treatment for addiction. Of primary importance is to reduce the impact of cocaine-associated environmental stimuli on the HPA axis so that they no longer function as triggers for relapse.

• 한국식생활문화학회지
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• 제8권2호
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• pp.125-137
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• 1993

The purpose of this study was to develop nutrition education program that promotes eating behavior of preschool children. 118 parents of preschool children were surveyed to understand of children's food preferences and prejudices. The preference for vegetable showed the lowest score(2.345) and fruit was the favorite food(4.11). These results imply the consequence of teaching balanced diet with emphasizing vegetables for preschoolers. Thus, nutrition education program including teaching material were developed to provide the preschoolers with cognitive, affective, and psychomotor activities in order them to the familiar with vegetables. The program were consisted of following five parts: 1. Read-A-Story includes six stories with follow-up activities. 2. Grow-A-Plant gives instructions for growing vegetables. 3. Play-A-Game stimulates children's imagination and learning. 4. Take-A-Trip suggests places to visit that grow, sell, and prepare vegetables. 5. Eat-A-Treat presents easy ways to prepare and enjoy vegetables. Twenty one-Standardized recipes for vegetable dishes were also developed for preschooler's luncheon and snack in daycare center.

• Clinical and Experimental Pediatrics
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• 제52권7호
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• pp.772-777
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• 2009

목 적 : 발달 지연이 있는 환아들을 대상으로 한국형 영유아 발달 검사와 K-BSID-II를 병행하여 서로 간의 상관 관계와 일치도를 분석함으로써 유사한 발달 영역에서 동일한 판정 정도를 확인하고자 하였다. 방 법 : 2007년 2월부터 2008년 6월까지 서울 아산 병원 소아청소년과에 발달 지연을 주소로 내원한 월령 42개월 이하의 환자 28명을 대상으로 하여 한국형 영유아 발달 검사 및 K-BSID-II를 시행하였다. 월령별로 12개월 이하와 13-24, 25-42개월 대상자로, 기질적 뇌병변이 두부 자기 공명 단층 촬영에 의해 확인되었던 군과 그렇지 않은 군으로 분류하여 각각의 한국형 영유아 발달 검사 결과와 K-BSID-II 결과를 비교하였다. 통계 분석은 SPSS를 사용하여 상관 관계를 분석하고 SAS를 사용하여 급내 상관 계수(Intraclass correlation coefficient)를 구하여 일치도를 분석하였다. 결 과 : 상관 관계 면에서 K-BSID-II의 인지 척도는 한국형 영유아 발달 검사의 전 영역과, 동작 척도는 조대 운동, 개인-사회성, 언어, 인지-적응 영역과 통계적으로 유의미한 상관을 나타내었다(P<0.01). 일치도 면에서 K-BSID-II의 인지 척도는 한국형 영유아 발달 검사의 조대 운동, 미세운동, 언어 영역과, 동작 척도는 조대 운동과 높은 일치도를 보였다(P<0.05). 결 론 : 발달 지연이 있는 환아에서 한국형 영유아 발달 검사는 조대 운동을 비롯한 여러 하위 영역에서 K-BSID-II와 유의한 상관 관계 및 일치도를 보였으며 한국 영유아의 발달 정도를 파악하는 비교적 정확한 지표로 사용될 수 있음을 시사한다.

• Journal of Genetic Medicine
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• 제6권2호
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• pp.170-174
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• 2009

저자들은 생후 3개월에 전형적인 페닐케톤뇨증으로 진단받고 식사요법을 유지하였으나 지연된 발달 소견 및 지능 저하를 보이고 경련 증상이 있었던 9세 남자 환아에서 효소 검사와 유전자분석으로 dihydropterine reductase (DHPR) 결핍증을 진단하였다. 그리고 $BH_4$, 신경전달물질 전구체 투여 및 엽산 보충으로 DHPR 결핍증을 치료한 1례를 경험하였기에 문헌고찰과 함께 보고한다.

• Clinical and Experimental Pediatrics
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• 제51권4호
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• pp.426-430
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• 2008

환상 염색체의 발생 기전은 염색체 말단 부분이 결손된 후 양 끝이 융합되거나 끝분절 염기서열이 앞뒤역순상동서열로 융합될 경우로 생각되고 있다. 환상 염색체는 세포 분열을 하는 동안 불안정하기 때문에 세포핵이 없는 딸세포의 사망률이 증가하게 되어 생존하는 세포수가 감소하고 성장 장애가 발생하게 된다. 표현형은 염색체 손실의 정도에 따라 다양하다. 저자들은 최초로 심각한 저신장을 주소로 내원한 환아의 염색체 검사상 환상 9번 염색체를 확인하였기에 이를 보고하는 바이다.

• 대한간호학회지
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• 제46권1호
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• pp.149-158
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• 2016

Purpose: This study was done to develop of the Korean intensive care delirium screening tool (KICDST). Methods: The KICDST was developed in 5 steps: Configuration of conceptual frame, development of preliminary tool, pilot study, reliability and validity test, development of final KICDST. Reliability tests were done using degree of agreement between evaluators and internal consistency. For validity tests, CVI (Content Validity Index), ROC (Receiver Operating Characteristics) analysis, known group technique and factor analysis were used. Results: In the reliability test, the degree of agreement between evaluators showed .80~1.00 and the internal consistency was KR-20=.84. The CVI was .83~1.00. In ROC analysis, the AUC (Area Under the ROC Curve) was .98. Assessment score was 4 points. The values for sensitivity, specificity, correct classification rate, positive predictive value, and negative predictive value were found to be 95.0%, 93.7%, 94.4%, 95.0% and 93.7%, respectively. In the known group technique, the average delirium screening tool score of the non-delirium group was $1.25{\pm}0.99$ while that of delirium group was $5.07{\pm}1.89$ (t= - 16.33, p <.001). The factors were classified into 3 factors (cognitive change, symptom fluctuation, psychomotor retardation), which explained 67.4% of total variance. Conclusion: Findings show that the KICDST has high sensitivity and specificity. Therefore, this screening tool is recommended for early identification of delirium in intensive care patients.