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이정호,Lee, Jeongho 대한유전성대사질환학회 2015 대한유전성대사질환학회지 Vol.15 No.3
All infants should be screened for phenylketonuria (PKU) within the three days of life, in order to allow timely dietary intervention to protect children with PKU from neurologic damage in Korea. A commonly used cut-off level for diagnosis of PKU is $240{\mu}mol/L$ (4 mg/dL). Up to 2% of cases of hyperphenylalaninemias (HPA) detected by the screening test will account for a disorder of $BH_4$ metabolism. Therefore, analysis of blood or urinary pterins is essential, backed up with measurement of DHPR activity, as this allows differentiation of $BH_4$ disorders. A $BH_4$ loading test and measurement of neurotransmitters in CSF provide further important information to the severity of $BH_4$ deficiency and $BH_4$ loading test can detect patients with $BH_4$ deficiency and $BH_4$ responsive PKU. Several protocols for $BH_4$ loading test have been described, involving treatment with $BH_4$ for periods ranging from 1 day to 1 month, and using doses of $BH_4$ of 10-20 mg/kg. There is general agreement that a reduction on blood phenylalanine of at least 30% in response to $BH_4$ loading indicates a clinically significant effect, although in some tests a lower cut-off value may be defined for individual patients, or no specific cut-off value is proposed. The frequency of $BH_4$ responsiveness is highest in patients with mild HPA and mild to moderate PKU resulting from PAH mutations with residual activity.
이정호,Lee, Jeongho 대한유전성대사질환학회 2020 대한유전성대사질환학회지 Vol.20 No.2
Phenylketonuria is the most prevalent disorder caused by an inborn error in aminoacid metabolism. It results from mutations in the phenylalanine hydroxylase (PAH) gene. If untreated or late treated, results in profound and irreversible mental disability. Newborn screening test identify patients with phenylketouria. The early initiation of a phenylalanine restricted diet very soon prevents most of the neuropsychiatric complications. However, the diet therapy is difficult to maintain and compliance is poor, especially in adolescents and adulthood. Since 2015, American Medical College of Medical Genetics and Genomics (ACMG) recommended more strong restrictive diet therapy for target blood level of phenylalanine (<360 umol/L). For over four decades the only treatment was a very restrictive low phenylalanine diet. This changed in 2007 with the approval of cofactor therapy (Tetrahydrobiopterin, BH4) which is effective in up to 30% of patients. Data from controlled clinical trials with sapropterin dihydrochloride indicate a similar occurrence of all-cause adverse events with this treatment and placebo. Large neutral aminoacids (LNAA) competes with phenylalanine for transport across the blood-brain-barrier and have a beneficial effect on executive functioning. A new therapy has just been approved that can be effective in most patients with PAH deficiency regardless of their degree of enzyme deficiency or the severity of their phenotype. Phenylalanine ammonia lyase (PAL-PEG) was approved in the USA by FDA in May of 2018 for adult patients with uncontrolled blood phenylalanine concentrations on current treatment. Nucleic acid therapy (therapeutic mRNA or gene therapy) is likely to provide longer term solutions with few side effects.
이정호,Lee, Jeongho 대한원격탐사학회 2017 대한원격탐사학회지 Vol.33 No.5
Several research cases using remote sensing methods to analyze changes of storage and dynamics of groundwater aquifer were reviewed in this paper. The status of groundwater storage, in an area with regional scale, could be qualitatively inferred from geological feature, surface water altimetry and topography, distribution of vegetation, and difference between precipitation and evapotranspiration. These qualitative indicators could be measured by geological lineament analysis, airborne magnetic survey, DEM analysis, LAI and NDVI calculation, and surface energy balance modeling. It is certain that GRACE and InSAR have received remarkable attentions as direct utilization from satellite data for quantification of groundwater storage and dynamics. GRACE, composed of twin satellites having acceleration sensors, could detect global or regional microgravity changes and transform them into mass changes of water on surface and inside of the Earth. Numerous studies in terms of groundwater storage using GRACE sensor data were performed with several merits such that (1) there is no requirement of sensor data, (2) auxiliary data for quantification of groundwater can be entirely obtained from another satellite sensors, and (3) algorithms for processing measured data have continuously progressed from designated data management center. The limitations of GRACE for groundwater storage measurement could be defined as follows: (1) In an area with small scale, mass change quantification of groundwater might be inaccurate due to detection limit of the acceleration sensor, and (2) the results would be overestimated in case of combination between sensor and field survey data. InSAR can quantify the dynamic characteristics of aquifer by measuring vertical micro displacement, using linear proportional relation between groundwater head and vertical surface movement. However, InSAR data might now constrain their application to arid or semi-arid area whose land cover appear to be simple, and are hard to apply to the area with the anticipation of loss of coherence with surface. Development of GRACE and InSAR sensor data preprocessing algorithms optimized to topography, geology, and natural conditions of Korea should be prioritized to regionally quantify the mass change and dynamics of the groundwater resources of Korea.
이정호(Jeongho Lee),이성환(Seongwhan Lee),이정규(JungKyu Lee),이효정(Hyojeong Lee),신재혁(Jehyuck Shin),정선영(Seonyeong Jeong),오영석(YoungSeok Oh),진호(Ho Jin) 한국항공우주학회 2016 한국항공우주학회지 Vol.44 No.4
SIGMA(Scientific cubesat with Instruments for Global Magnetic field and rAdiation) 큐브위성은 근 지구 자기장 세기와 우주 방사선량 측정을 위해 경희대학교에서 개발한 초소형인공위성이다. 우주 임무 수행을 위해 비행 소프트웨어는 위성을 제어하고 데이터를 처리하는 중요한 역할을 담당한다. 본 연구에서는 SIGMA 큐브위성의 임무 수행을 위해 비행소프트웨어를 구현한 것으로서 일반적으로 임베디드 시스템에서 사용하는 실시간 운영체제를 사용하지 않고 단일 프로세스 내에서 모든 태스크를 처리하도록 구현하였다. 이는 SIGMA 큐브위성의 임무 수행 절차와 시스템 제어 방법을 고려한 것으로서 소프트웨어의 오버헤드(overhead)를 낮추고 임무 수행에 집중할 수 있는 효과적인 방법이다. SIGMA(Scientific cubesat with Instruments for Global Magnetic field and rAdiation) CubeSat has been developed for magnetic field measurement of the Earth and space radiation measurement at Kyung Hee university. The flight software plays important roles in controlling the satellite and processing the data in the space mission. In this paper, the Flight Software has been implemented to process all the tasks in the one thread without RTOS(Real Time Operating System). This is an effective mothed not only to concentrate the space mission of CubeSat but also to reduce the overhead of the Flight Software by considering the mission perform procedures and the system control methods.
LPG 직분 연료 공급 시스템에서 연료 리턴에 의한 연료 냉각효과 연구
이정호(Jeongho Lee),이종혁(Jonghyeok Lee),이충근(Chunggeun Lee),이진학(Jinhak Lee),이대엽(Daeyup Lee) 한국자동차공학회 2015 한국자동차공학회 학술대회 및 전시회 Vol.2015 No.11
In order to meet the emissions standards for PM and PN, a development of technology for LPG SIDI(Spark Ignition Direct Injection) fuel supply system and for its application are necessary. Prevention of a vapor lock with use of LPG fuel leads to begin this work. The purpose of this work is to provide optimum design methods for preventing the locking of the fuel vapor from the LPG fuel supply system, and contributes to construction of the designed data. An effect of fuel cooling by returning some fuel from the high pressure pump to the fuel tank is identified using a rig system with Butane/propane= 70%/30% fuel, which enables to estimate the increase of temperature to slow down up to max. 1.4 times. As returning amount of fuel increases, an effect of cooling fuel pump varies and thus an excessive use of low pressure pump leads to the durability and fuel economy problems. Therefore, this work suggests the variable control of returning amount of fuel needs to be reviewed and applied. Measured results of temperature change of high pressure pump show that, after heating to high temperature, it is confirmed that a start of return(opening of return valve) lowers(cools) the temperature of high pressure pump. This concludes that low pressure pump(in-tank motor) enables to cool down the high pressure pump and thus reduce the start time at high temperature re-startability.
이중관 열교환기를 적용한 차량용 냉난방 시스템 성능예측에 관한 연구
이정호(JeongHo Lee),조용석(Yong-Seok Cho),김광연(Kwangyeon Kim),장길상(Kilsnag Jang),지용준(Yongjun Jee) 한국자동차공학회 2012 한국자동차공학회 부문종합 학술대회 Vol.2012 No.5
In recent years, many automotive companies and researchers say that fuel consumption is one of the most important factor to evaluate cars, because of the depletion of fossil fuel like petroleum. From the this viewpoint, we focus on the air conditioning system to reduce the fuel consumption. Also, we apply the double pipe heat exchanger in the air conditioning system to improve the cooling performance and reduce fuel consumption. So we have to improve the cooling performance of the cars cooling system. This study is that to improve cars cooling system performance prediction using 1-D simulation.