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진동규 대한소아청소년과학회 2012 Clinical and Experimental Pediatrics (CEP) Vol.55 No.7
Prader-Willi syndrome (PWS) is a complex multisystem genetic disorder characterized by hypothalamic-pituitary dysfunction. The main clinical features include neonatal hypotonia, distinctive facial features, overall developmental delay, and poor growth in infancy, followed by overeating with severe obesity, short stature, and hypogonadism later in development. This paper reviews recent updates regarding the genetic aspects of this disorder. Three mechanisms (paternal deletion, maternal disomy,and deficient imprinting) are recognized. Maternal disomy can arise because of 4 possible mechanisms: trisomy rescue (TR), gamete complementation (GC), monosomy rescue (MR), and postfertilization mitotic nondisjunction (Mit). Recently, TR/GC caused by nondisjunction at maternal meiosis 1 has been identified increasingly, as a result of advanced maternal childbearing age in Korea. We verified that the d3 allele increases the responsiveness of the growth hormone (GH)receptor to endogenous GH. This paper also provides an overview of endocrine dysfunctions in children with PWS, including GH deficiency,obesity, sexual development, hypothyroidism, and adrenal insufficiency, as well as the effects of GH treatment. GH treatment coupled with a strictly controlled diet during early childhood may help to reduce obesity, improve neurodevelopment, and increase muscle mass. A more active approach to correct these hormone deficiencies would benefit patients with PWS.
순도를 달리한 알루미나 세라믹스의 분위기 압력변화에 따른 Tribology 특성
진동규,박흥식,서영백,옥철호,Jin, Dong-Gyu,Park, Heung-Sik,Seo, Yeong-Baek,Ok, Cheol-Ho 대한기계학회 2000 大韓機械學會論文集A Vol.24 No.2
This study was undertaken to investigate tribological characteristics with atmospheric pressure for three alumina ceramics of different purity, such as, 85 %, 95 % and 99.7 %. The wear test was carried out using the wear test device which was designed for this study in air, $10^-2$ Torr and $10^-4$ Torr. The friction coefficient depends entirely on atmospheric pressure and alumina with lower purity than higher purity has been much affected by heat accumulation. The friction surface of ceramics have been protected in the air by the influence of the oxides transferred from STB2. However, the protective layer can not be formed due to the decrease of oxygen in vacuum.
Systematic review of the clinical and genetic aspects of Prader-Willi syndrome
진동규 대한소아청소년과학회 2011 Clinical and Experimental Pediatrics (CEP) Vol.54 No.2
Prader-Willi syndrome (PWS) is a complex multisystem genetic disorder that is caused by the lack of expression of paternally inherited imprinted genes on chromosome 15q11–q13. This syndrome has a characteristic phenotype including severe neonatal hypotonia,early-onset hyperphagia, development of morbid obesity, short stature, hypogonadism, learning disabilities, behavioral problems,and psychiatric problems. PWS is an example of a genetic condition caused by genomic imprinting. It can occur via 3 main mechanisms that lead to the absence of expression of paternally inherited genes in the 15q11.2–q13 region: paternal microdeletion, maternal uniparental disomy, and an imprinting defect. Over 99% of PWS cases can be diagnosed using DNA methylation analysis. Early diagnosis of PWS is important for effective long-term management. Growth hormone (GH) treatment improves the growth, physical phenotype, and body composition of patients with PWS. In recent years, GH treatment in infants has been shown to have beneficial effects on the growth and neurological development of patients diagnosed during infancy. There is a clear need for an integrated multidisciplinary approach to facilitate early diagnosis and optimize management to improve quality of life,prevent complications, and prolong life expectancy in patients with PWS.
진동규 대한교통학회 2017 대한교통학회 학술대회지 Vol.76 No.-
기존의 교통안전교육은 다수 인원을 대상으로 한 강의식 교육이 주를 이루어왔다. 이는 대규모 교육이 가능하다는 효율성 측면에서 이점을 가진다고 하겠지만, 실제 행동변화를 가져오는 효과성 측면에서 다소 부족했던 것이 사실이다. 강의식 교육이 가지는 한계를 극복하기 위해 어린이, 청소년을 대상으로 하는 체험 교육이 이뤄지고 있고 상습음주운전자로 대표되는 취약계층에 대한 심리상담기법을 시도되고 있다. 이에 더하여 최근 체험교육이 가지는 시공간상 취약점을 극복하고 그 장점은 유지하면서 실제 경험하기 힘든 부분까지 체득할 수 있는 가상현실기술이 교육 분야에 접목되고 있다. 도로교통공단은 가상현실영상과 4D시뮬레이터를 결합하여 체험의 현실성을 높인 가상현실체험센터를 구축하였다. ①난폭·보복운전, ②안전띠, 카시트 ③악천후(빗길, 안갯길), ④운전 중 주의분산(스마트폰) 등 4개의 가상현실체험영상을 제작하였으며, 향후 어린이 관련 교통안전영상을 단계적으로 추가 제작하여 전 계층을 아우르는 가상현실체험교육을 마련해갈 계획이다.