• Journal of The Korean Society of Inherited Metabolic disease
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• v.13 no.2
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• pp.111-119
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• 2013

Purpose: Mucopolysaccharidosis type I (MPS I) is a lysosomal storage disorder caused by deficiency of the enzyme ${\alpha}$-L-iduronidase, which leads to a broad spectrum of multisystemic manifestations. Short stature and decreased growth velocity are prominent features of MPS I. The aim of the present study was to evaluate the effect of enzyme replacement therapy (ERT) on growth of Korean MPS I patients from a single center. Methods: Height data were obtained by retrospective chart review of 10 Korean patients with MPS I who had received ERT for a minimum of 3 years. Height was expressed as standard deviation scores (SDS) based on normative data. Annual growth rates were calculated before and during ERT. A piecewise regression model was used to analyze height z-scores before and after treatment. Individual analysis was performed for impact of phenotype [(severe (Hurler) versus attenuated (Hurler-Scheie, Scheie)] on growth. Results: Annual growth was 3.3 cm (z-score= -0.21) in the year before ERT and 6.2 cm (z-score= 0.17), 5.8 cm (z-score= 0.07), and 3.8 cm (z-score= -0.4) in the first, second, and third years of ERT, respectively. Regression analysis showed improvement in the slope after ERT (difference= 0.04; P=0.022). Estimated slope differences between severe and attenuated phenotypes were statistically significant before (P=0.001) and after treatment (P<0.0001), although no significant difference was noted when stratified by phenotype. Conclusion: ERT with aldurazyme appears to have a positive impact on linear growth in patients with MPS I.

• Journal of the Korean Society of Propulsion Engineers
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• v.19 no.6
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• pp.91-97
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• 2015

The limit values of mechanical properties(MPs) of HTPB/AP/Al Solid Propellant was reviewed according to the rocket motor development procedures and the in-process values of MPs were analyzed by the tool of Process Capability Index. Based on finding the dependency among MPs, the optimization is proposed for reducing the properties defects and for improving the rocket grain safeties.

• Journal of the Society of Naval Architects of Korea
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• v.45 no.1
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• pp.1-9
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• 2008

The free-surface motions interacting with structures are investigated numerically using the Moving Particle Semi-implicit (MPS) method proposed by Koshizuka et al. (1996) for solving incompressible flow. In the method, Lagrangian moving particles are used instead of Eulerian approach using grid system. Therefore the terms of time derivatives in Navier-Stokes equation can be directly calculated without any numerical diffusion or instabilities due to the fully Lagrangian treatment of fluid particles and topological failure never occur. The MPS method is applied to the numerical study on the fluid impact loads for wet-drop tests in a LNG tank, and the results are compared with experimental ones.

• The Optical Journal
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• s.140
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• pp.16-19
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• 2012

개인용 프린터를 중심으로 한 B2C 상품은 빠른 속도와 선명한 컬러 그리고 모바일 연동 정도의 솔루션을 기반으로 저가 경쟁이 더욱 치열해질 전망이다. 또한 흔히 사무기기 시장이라 칭하는 B2B 출력시장은 MPS4.0을 비롯한 다양한 솔루션의 경쟁이 무한히 계속될 것이다. 물론 속도 경쟁과 컬러 경쟁도 계속되겠지만 이미 대기업과 금융권의 큰 손 고객들은 자기 화사에 꼭 맞는 솔루션을 개발한 회사에 매력을 느끼고 있다.

• Journal of mucopolysaccharidosis and rare diseases
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• v.1 no.1
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• pp.31-33
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• 2015

• Journal of the Korean Institute of Electrical and Electronic Material Engineers
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• v.35 no.3
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• pp.241-245
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• 2022

In this study, we measured and comparatively analyzed the characteristics of MPS (Merged Pin Schottky) diodes in 4H-SiC by changing the areal ratio between the Schottky and PN junction region. Increasing the temperature from 298 K to 473 K resulted in the threshold voltage shifting from 0.8 V to 0.5 V. A wider Schottky region indicates a lower on-resistance and a faster turn-on. The effective barrier height was smaller for a wider Schottky region. Additionally, the depletion layer became smaller under the influence of the reduced effective barrier height. The wider Schottky region resulted in the ideality factor being reduced from 1.37 to 1.01, which is closer to an ideal device. The leakage saturation current increased with the widening Schottky region, resulting in a 1.38 times to 2.09 times larger leakage current.

• Korean Journal of Veterinary Research
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• v.42 no.2
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• pp.219-224
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• 2002

We report the seasonal prevalence of the mycoplasmal pneumoniae of swine (MPS) in slaughter pigs from July of 1999 to June of 2000. Gross finding of lung lesion observed and examined by dot-ELISA. In gross finding of lung lesion from 750 pig samples, 465 (62.0%) was MPS, and 129 (17.2%) was single or double infection with actinobacillosis and pasturellosis. However, 156 (20.8%) had no lesion. In seasonal detection, the prevalence was found to be winter (69.5%), autumn (63.5%), summer (60.0%) and spring (54.7%) in orderly frequency. In dot-ELISA, the result was showed the positive reaction (x16>titre) with 58.0% and negative (x4

• Journal of mucopolysaccharidosis and rare diseases
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• v.2 no.1
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• pp.23-26
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• 2016

Mucopolysaccharidosis (MPS) I is a rare, progressive and multisystemic disease with insidious initial signs and symptoms, and making an early diagnosis can be a challenge for the first-line general medical practitioner. We report a 6-month-old girl who was brought to our well baby clinic for regular immunization with the manifestations of lumbar gibbus, hirsutism, large Mongolian spots over back and buttock, and mild bilateral legs spasticity noticed by the general pediatrician, and then newly diagnosed with MPS I after referral to the geneticist in time. Her surgical history included inguinal hernia repair at 1 month old, $CO_2$ laser supraglottoplasty for laryngomalacia and tracheostomy due to chronic respiratory failure with ventilator dependence at 2 months old. Understanding and identification of the early signs and symptoms of this disease have the potential to early diagnosis and timely appropriate treatment, which could contribute to a better clinical outcome.

• Analytical Science and Technology
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• v.32 no.6
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• pp.243-251
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• 2019

Microplastics (MP) are found in large quantities in the oceans, posing a major threat to the ecosystem. In Korea, MPs have been reported to be detected in sea salts. In order to analyze MPs, information on their composition, size, and shape is required. FT-IR microscopy is used frequently to measure sizes larger than 20 ㎛. Recently, however, Raman microscopy, which can analyze ultrafine plastics below 20 ㎛, has been applied extensively. In this study, 10.0 g samples of commercially available salts were dissolved and filtered through a 45 ㎛ mesh filter with a size of 25.4 mm × 25.4 mm. These filtered samples were then analyzed by both FT-IR microscopy and Raman microscopy. A total of four MPs, including three polyethylene (PE) of size 70-100 ㎛ and a polypropylene (PP) of size 170 ㎛, were detected by FT-IR microscopy, while 10 MPs, including nine PE of size 10-120 ㎛ and one polystyrene (PS) of size 40 ㎛, were detected by Raman microscopy. Approximately, 1,000 MPs/kg was estimated, which was almost two times higher than the previous reported levels (~550-681 particles/kg in sea salts); this is because Raman microscopy can detect much smaller MPs than FT-IR microscopy. A total of 113 particles were found using Raman microscopy: Carbon (35, 31.5 %), minerals (28, 25 %), and glass (16, 14.4 %) were dominant, forming around 70% of the total, but MPs (10, 8.8 %) and cellulose (5, 4.5 %) were also found. Raman microscopy has great potential as an accurate method for measuring MPs, as it can measure smaller size MPs than FT-IR microscopy. It also has a reduced sample preparation time.

• Journal of mucopolysaccharidosis and rare diseases
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• v.3 no.1
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• pp.34-34
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• 2017