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박성원,손영배,김세화,조성윤,지선태,진동규,Park, Seong-Won,Son, Yeong-Bae,Kim, Se-Hwa,Jo, Seong-Yun,Ji, Seon-Tae,Jin, Dong-Gyu 대한유전성대사질환학회 2010 대한유전성대사질환학회지 Vol.10 No.1
Enzyme replacement of therapy (ERT) is one of the most promising therapeutic strategies for the treatment of lysosomal storage disorders. ERT is available in three types of Mucopolysaccharidosis (MPS): for MPS I (Aludrazyme$^{(R)}$), MPS II (Elaprase$^{(R)}$) and MPS VI (Naglazyme$^{(R)}$) patients who are over 5 years old. But recently, early diagnosis can be done by expert clinicians and even in prenatal case. We describe the case of ERT under 5 years old MPS patients. Up to June, 2010 in Samsung Medical Center, there are 6patients who were diagnosed as MPS and started ERT under 5 years old. 3 patients were MPS I, 3 patients were MPS II. 2 patient who was diagnosed as MPS I was female and others were male. Their age at diagnosis were 4 to 37month-old (4, 13, 16, 25, 27, 37 month-old) and they are now 9 to 60 month-old (9, 39, 32, 81, 60 month-old). The youngest patient was started ERT at 4 month-old and others were started at their 13 to 49 month-old (13, 29, 27, 28, 49 month-old). First manifested symptoms of patients were macrocephaly, kyphosis and coarse face appearance. Especially, in 2 of them, one was MPS I and the other was MPS II had elder brother with same disease. And the youngest one was diagnosed by the iduronate-2-sulfatase (IDS) gene analysis from chorionic villi sampling. His mother knew that she was a heterozygous carrier of IDS gene mutation because her younger brother died from MPS II. All of them confirmed as MPS by the enzyme assay in leukocytes and fibroblast skin culture. We started ERT with ${\alpha}$-L-iduronidase(Aldurazyme$^{(R)}$) to MPS I and did recombinant human iduronate-2-sulfatase (Elaprase$^{(R)}$) to MPS II patients as recommended dose as over 5 years old. But for MPS II patient who was 4 month old, we started ERT by recombinant human IDS (Elaprase$^{(R)}$) with reduced dose 0.1 mg/kg and increased dose every 2 weeks by 0.1mg/kg up to 0.5mg/kg IV infusion. During ERT, all patients had no adverse effects and the excretion of GAGs were decreased. We have evaluated other clinical symptoms such as liver/ spleen volume, heart function and neurologic evaluation. We describe a successful ERT to MPS I and MPS II patient under 5 years old without any adverse event. It indicates that ERT in young children are well tolerated and that it has several effects which may confer clinical benefits with long-term therapy.
박성원,Park, Seong-Won 한국방위산업진흥회 1994 國防과 技術 Vol.- No.181
항공우주산업과 첨단무기체계의 발달로 전차의 중요성이 간과되어 가는 것이 지금의 현실이다 그러나 지상전력에 있어 전차의 중요성은 더 말할 나위가 없다. 이것은 지난 걸프전에서 소련의 T-72 전차를 모든 면에서 압도한 M1의 위력에서 잘 나타났다 이에 세계 각국에서 추진되는 전차의 발전 추세를 살펴보는 것도 의미있을 것이다. 이 글은 국과연에서 정기적으로 발해하는 "국방기술동향" 제1호에 게재된 내용이다
박성원,서일원,Park. Sung-Won,Seo. Il-Won 한국방재학회 2008 한국방재학회 학술발표대회논문집 Vol.2008 No.1
General behaviors based on hydraulic characteristics of natural streams and channels have been recently analyzed and developed via various numerical models. However in the states of natural hydraulics, an experimental research must be performed simultaneously with the mathematical analysis due to effects of hydraulic properties such as meander, sediment, and so on. In this study based on 2-D advection-dispersion equation, flow and tracer experiments were performed in the S-curved meandering laboratory channel with a rectangular cross-section. The channel was equipped with instrument carriages which was equipped with an auto-traversing system to be used with velocity measuring sensors throughout the depth and breadth of the flow field. To measure concentration distribution of the salt solution was adjusted to that of the flume water by adding methanol and a red dye (KMnO4) was added to aid the visualization of the tracer cloud, the tracer was instantaneously injected into the flow as a full-depth vertical line source by the instantaneous injector and the initial concentration of the tracer was 100,000 mg/l. The secondary current as well as the primary flow pattern was analyzed to investigate the flow distribution in the meandering channels. The velocity distribution of the primary flow for all cases skewed toward the inner bank at the first bend, and was almost symmetric at the crossovers, and then shifted toward the inner bank again at the next alternating bend. Thus, one can clearly notice that the maximum velocity occurs taking the shortest course along the channel, irrespective of the flow conditions. The result of the tracer tests shows that pollutant clouds are spreading following the maximum velocity lines in each cases with various mixing patterns like superposition, separation, and stagnation of pollutant clouds. Flow characteristics in each cases performed in this study can be compared with tracer dispersion characteristics with using evaluation of longitudinal and transverse dispersion coefficients(LDC, TDC). As expected, LDC and TDC in meandering parts have been evaluated with increasing distribution and straight parts have effected to evaluate minimum of LDC and TDC due to symmetric flow patterns and attenuations of secondary flow.
박성원,Park, Seong-Won 한국방위산업진흥회 1994 國防과 技術 Vol.- No.182
제2차 세계대전이후 화력향상, 기동력 증대 방어력 보강 등에 중점을 두어 전차 개발이 이루어졌는데, 이런 흐름의 집대성이 독일의 Leopard, 미국의 M1, 영국의 Challenger의 등장으로 나타났다.