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박미림,고정민,전종근,김구환,유한욱 대한소아청소년과학회 2008 Clinical and Experimental Pediatrics (CEP) Vol.51 No.11
Smith-Lemli-Opitz syndrome (SLOS) is a rare, autosomal recessive disease caused by an inborn error in cholesterol synthesis. Patients with this disease suffer from multiple malformations due to reduced activity of 7-dehydrocholesterol reductase (DHCR7), which increases 7-dehydrocholesterol (7DHC) and 8-dehydrocholesterol (8DHC) concentrations and decreases cholesterol concentration in body fluids and tissue. The SLOS phenotypic spectrum ranges from a mild disorder with behavioral and learning problems to a lethal disease characterized by multiple malformations. Here, we describe a newborn male with ambiguous genitalia who was diagnosed to have type II SLOS during the neonatal period. A clinical examination revealed low levels of unconjugated estriol in the maternal serum, and a variety of fetal ultrasound anomalies, including prenatal growth retardation. After birth, the infant was diagnosed to have congenital heart disease (Tetralogy of Fallot with severe pulmonary artery stenosis), cleft lip and palate, micrognathia, postaxial polydactyly, ambiguous genitalia, and cataracts. Clinical investigation revealed extremely low plasma cholesterol levels and the presence of mutation (homozygote of p.Arg352Gln) in the DHCR7 gene. The patient underwent palliative heart surgery (to widen the pulmonary artery) and received intravenous lipid supplementation. Cholesterol levels increased slightly, but not to normal values. The patient died from cardiopulmonary failure and sepsis 72 days after birth. This report provides the first description of a Korean patient with SLOS confirmed by verification of DHCR7 gene mutation and illustrates the need for early recognition and appropriate diagnosis of this disease.
리튬이온 전지 기술을 채용한 인공위성용 전력계 개념 설계
신구환(Goo-Hwan Shin),박경화(Kyung-Hwa Park),김형명(Hyung-Myung Kim),임종태(Jong-Tae Lim) 한국항공우주학회 2007 韓國航空宇宙學會誌 Vol.35 No.2
본 논문은 리튬이온 (Li-ion) 셀을 채용한 인공위성용 전력계의 개념 설계에 대하여 기술한다. 기존의 니켈카드뮴 (NiCd) 셀과 비교할 때, 리튬이온 (Li-ion) 셀은 에너지 밀도, 무게 그리고 부피에서 큰 잇점을 갖고 있다. 니켈카드뮴 (NiCd) 셀의 평균 출력전압은 +1.2V이며, 리튬이온 (Li-ion) 셀의 출력전압은 +3.6V이다. 그러나, 리튬이온 (Li-ion) 셀의 충전과 방전에 있어서의 절차는 기존의 니켈카드뮴 (NiCd) 셀 보다는 어렵다. 따라서, 리튬이온 (Li-ion) 셀의 충전과 방전 시에는 각각의 셀에 대하여 충전 전압과 방전 전압을 검침하고 제어를 해주어야 하므로 별도의 제어 회로가 요구된다. 따라서, 본 논문을 통하여 리튬이온 (Li-ion) 셀을 채용한 전력계의 설계 시 고려하여야 할 사항 및 리튬이온 (Li-ion) 셀의 충ㆍ방전 특성에 대한 연구 결과를 제시하고자 한다. This paper presents the conceptual design of the electrical power system using Li-ion cell technology for a satellite application. Compared to a conventional NiCd cell, a Li-ion cell has a lot of advantages such as an energy density, mass and a volume. Normally, a Li-ion cell has three times than conventional NiCd cells in a capacity such as a cell voltage. The normal voltage of a NiCd cell is around +1.2V and a Li-ion cell could be in +3.6V. However, the handling procedure for a Li-ion cell in charge and discharge might be difficult than a conventional NiCd cell, which means that the charge and discharge of each cell should be monitored and controlled by electrical circuits to prevent an over-charge and over-discharge. Therefore, in this paper we propose the design consideration and the characteristics of a Li-ion cell during charging and discharging battery packs in the point of view of electrical power system.