• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.2
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• pp.93-101
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• 2016

Purpose: McArdle disease, glycogen storage disease type V (GSD V), is one of the most common adolescent-onset glycogen storage diseases. It is caused by recessive mutations in PYGM encoding myophosphorylase, which is critical to glycogen metabolism. Since only a few korean patients have been reported, we will observe the clinical and genetic features of three korean patients with McArdle disease. Methods: We retrospectively reviewed the medical records of three patients with genetically confirmed McArdle disease, including the results of forearm ischemic exercise test, electromyogram, nerve conduction velocity, muscle biopsy, and PYGM analysis in peripheral leukocytes. Results: All three cases were males and their age of symptom onset was 12, 5, 14 years old, respectively. A high basal level of serum creatine kinase was noted in all three patients. They experienced the recurrent episodes of rhabdomyolysis, but second wind phenomenon was not definite. In muscle biopsy, subsarcolemmal space vacuoles including periodic acid schiff stained materials were found in two patients, while no evidence of glycogen storage disease was found in the other. A total of five different mutations, $p.Arg50^*$, p.Trp798Arg, $p.Arg50^*$, p.Glu779del, $p.Asp511Thrfs^*28$ and p.Phe710del, were found in three patients. Avoidance of isometric exercise, aerobic exercise and glucose intake before each exercise were recommended for all patients. Conclusion: The three Korean patients with McArdle disease showed the typical manifestations of the condition. The most mutations were private. Therefore, identification of more cases with long-term follow-up will be required to understand the clinical and genetic features of this disease among Korean population.

• The Korean Journal of Nuclear Medicine
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• v.34 no.1
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• pp.82-93
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• 2000

Purpose: We characterized the signals obtained from the components of a small gamma camera using Nal(Tl)-position sensitive photomultiplier tube (PSPMT) and optimized the parameters employed in the modules of the system. Materials and Methods: The small gamma camera system consists of a Nal(Tl) crystal ($60{\times}60{\times}6mm^3$) coupled with a Hamamatsu R3941 PSPMT, a resister chain circuit, preamplifiers, nuclear instrument modules (NIMs), an analog to digital converter and a personal computer for control and display. The PSPMT was read out using a resistive charge division circuit which multiplexes the 34 cross wire anode channels into 4 signals (X+, X-, Y+, Y -). Those signals were individually amplified by four preamplifiers and then, shaped and amplified by amplifiers. The signals were discriminated and digitized via triggering signal and used to localize the position of an event by applying the Anger logic. The gamma camera control and image display was performed by a program implemented using a graphic software. Results: The characteristics of signal and the parameters employed in each module of the system were presented. The intrinsic sensitivity of the system was approximately $8{\times}10^3$ counts/sec/${\mu}Ci$. The intrinsic energy resolution of the system was 18% FWHM at 140 keV. The spatial resolution obtained using a line-slit mask and $^{99m}Tc$ point source were, respectively, 2.2 and 2.3 mm FWHM in X and Y directions. Breast phantom containing $2{\sim}7mm$ diameter spheres was successfully imaged with a parallel hole collimator. The image displayed accurate size and activity distribution over the imaging field of view Conclusion: We proposed a simple method for development of a small gamma camera and presented the characteristics of the signals from the system and the optimized parameters used in the modules of the small gamma camera.

• Nuclear Medicine and Molecular Imaging
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• v.42 no.6
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• pp.469-477
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• 2008

Purpose: The goal of this paper is to present the design and performance of a position encoding circuit for $16{\times}16$ array of position sensitive multi-anode photomultiplier tube for small animal PET scanners. This circuit which reduces the number of readout channels from 256 to 4 channels is based on a charge division method utilizing a resistor array. Materials and Methods: The position encoding circuit was simulated with PSpice before fabrication. The position encoding circuit reads out the signals from H9500 flat panel PMTs (Hamamatsu Photonics K.K., Japan) on which $1.5{\times}1.5{\times}7.0\;mm^3$ $L_{0.9}GSO$ ($Lu_{1.8}Gd_{0.2}SiO_{5}:Ce$) crystals were mounted. For coincidence detection, two different PET modules were used. One PET module consisted of a $29{\times}29\;L_{0.9}GSO$ crystal layer, and the other PET module two $28{\times}28$ and $29{\times}29\;L_{0.9}GSO$ crystal layers which have relative offsets by half a crystal pitch in x- and y-directions. The crystal mapping algorithm was also developed to identify crystals. Results: Each crystal was clearly visible in flood images. The crystal identification capability was enhanced further by changing the values of resistors near the edge of the resistor array. Energy resolutions of individual crystal were about 11.6%(SD 1.6). The flood images were segmented well with the proposed crystal mapping algorithm. Conclusion: The position encoding circuit resulted in a clear separation of crystals and sufficient energy resolutions with H9500 flat-panel PMT and $L_{0.9}GSO$ crystals. This circuit is good enough for use in small animal PET scanners.

• The Journal of Korean Society for Radiation Therapy
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• v.8 no.1
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• pp.19-27
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• 1996

I. Project Title A Study of Brachytherapy for intraocular tumor II. Objective and Importance of the project The eye enucleation or external-beam radiation therapy that has been commonly used for the treatment of intraocular tumor have demerits of visual loss and in deficiency of effective tumor dose. Recently, brachytherapy using the plaques containing radioisotope-now treatment method that decrease the demerits of the above mentioned treatment methods and increase the treatment effect-is introduced and performed in the countries, Our purpose of this research is to design suitable shape of plaque for the ophthalmic brachytherapy, and to measure absorbed doses of Ir-192 ophthalmic plaque and thereby calculate the exact radiation dose of tumor and it's adjacent normal tissue. III. Scope and Contents of the project In order to brachytherapy for intraocular tumor, 1. to determine the eye model and selected suitable radioisotope 2. to design the suitable shape of plaque 3. to measure transmission factor and dose distribution for custom made plaques 4. to compare with the these data and results of computer dose calculation models IV. Results and Proposal for Applications The result were as followed. 1. Eye model was determined as a 25mm diameter sphere, Ir-192 was considered the most appropriate as radioisotope for brachytherapy, because of the size, half, energy and availability. 2. Considering the biological response with human tissue and protection of exposed dose, we made the plaques with gold, of which size were 15mm, 17mm and 20mm in diameter, and 1.5mm in thickness. 3. Transmission factor of plaques are all 0.71 with TLD and film dosimetry at the surface of plaques and 0.45, 0.49 at 1.5mm distance of surface, respectively. 4. As compared the measured data for the plaque with Ir-192 seeds to results of computer dose calculation model by Gary Luxton et al. and CAP-PLAN (Radiation Treatment Planning System), absorbed doses are within ${\pm}10\%$ and distance deviations are within 0.4mm Maximum error is $-11.3\%$ and 0.8mm, respectively. As a result of it, we can treat the intraocular tumor more effectively by using custom made gold plaque and Ir-192 seeds.

• Journal of the Korean Institute of Telematics and Electronics
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• v.26 no.2
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• pp.94-101
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• 1989

Transport properties of 2DEG at AlGaAs/GaAs interface such as average electron energy, flight distance, each valley occupancy ratio, average electron velocity for various fields are investigated by MONTE-CARLO method. As the electric field increases, more electrons transit drastically from (000) valley to (000) upper valley. This phenomenon shows the nonstationary effect such as velocity overshoot. The duration of the transient decreases from about 1.4 psec for electric field E = 7KV/cm to about 0.7 psec for 12KV/cm. The average electron velocity during transient transport in 2DEG is about 8 times the steady-state velocity for E = 12KV/cm at room temperature. In comparison with bulk GaAs the peak velocity in the 2DEG is higher than that in even pure bulk GaAs at electric field E = 7 KV/cm. On the basis of the fact that the electrons in the 2DEG have larger peak velocity and shorter transient time of velocity than those in the bulk GaAs, it is suggested that the device with 2DEG may obtain higher mobility than that with bulk GaAs.

• Journal of the Korean Electrochemical Society
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• v.10 no.4
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• pp.283-287
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• 2007

KIER has been developing the anode-supported flat tubular solid oxide fuel cell unit bundle for the intermediate temperature($700{\sim}800^{\circ}C$) operation. Anode-supported flat tubular cells have Ni/YSZ cermet anode support, 8 moi.% $Y_2O_3$ stabilized $ZrO_2(YSZ)$ thin electrolyte, and cathode multi-layer composed of Sr-doped $LaSrMnO_3(LSM)$, LSM-YSZ composite, and $LaSrCoFeO_3(LSCF)$. The prepared anode-supported flat tubular cell was joined with ferritic stainless steel cap by induction brazing process. Current collection for the cathode was achieved by winding Ag wire and $La_{0.6}Sr_{0.4}CoO_3(LSCo)$ paste, while current collection for the anode was achieved by using Ni wire and felt. For making stack, the prepared anode-supported flat tubular cells with effective electrode area of $90\;cm^2$ connected in series with 12 unit bundles, in which unit bundle consists of two cells connected in parallel. The performance of unit bundle in 3% humidified $H_2$ and air at $800^{\circ}C$ shows maximum power density of $0.39\;W/cm^2$ (@ 0.7V). Through these experiments, we obtained basic technology of the anode-supported flat tubular cell and established the proprietary concept of the anode-supported flat tubular cell unit bundle.

• Korean Journal of Heritage: History & Science
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• v.49 no.2
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• pp.86-103
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• 2016

'Sukchunjeahdo' illustration-book, which was left by Han, Pil-gyo(韓弼敎 : 1807~1878)in the late Joseon Dynasty, includes pictorial record paintings containing government offices, Eupchi, and Feng Shui condition drawn by Gyehwa(界畵) method Sabangjeondomyobeop(四方顚倒描法) and is the rare historical material that help to understand spatial structure and landscape characteristics. Youngyuhyun(永柔縣) and Sincheongun(信川郡) town, the case sites of this study, show Feng Shui foundation structure and placement rules of government offices in the Joseon Period are applied such as 3Dan 1Myo(三壇一廟 : Sajikdan, Yeodan, Seonghwangdan, Hyanggyo), 3Mun 3Jo(三門三朝 : Oeah, Dongheon, Naeah) and Jeonjohuchim(前朝後寢) etc. by setting the upper and lower hierarchy of the north south central axis. The circulation system is the pattern that roads are segmented around the marketplace of the entrance of the town and the structure is that heading to the north along the internal way leads to the government office and going out to the main street leads to the major city. Baesanimsu(背山臨水 : Mountain in backward and water in front) foundation, back hill pine forest, intentionally created low mountains and town forest etc. showed landscape aesthetics well suited for the environmental comfort condition such as microclimate control, natural disaster prevention, psychological stability reflecting color constancy principle etc. and tower pavilions were built throughout the scenic spot, reflecting life philosophy and thoughts of contemporaries such as physical and mental discipline, satisfied at the reality of poverty, returning to nature etc. For government office landscape, shielding and buffer planting, landscape planting etc. were considered around Gaeksa(客舍), Dongheon(東軒), Naeah(內衙) backyard and deciduous tree s and flowering trees were cultivated as main species and in case of Gaeksa, tiled pavilions and pavilions topped with poke weed in tetragonal pond were introduced to Dongheon and Naeah and separate pavilions were built for the purpose of physical and mental discipline and military training such as archery. Back hill pine tree forest formed back landscape and zelkova, pear trees, willow trees, old pine trees, lotus, flowering trees etc. were cultivated as gardening trees and Feng-Shui forest with willow trees as its main species was created for landscape and practical purposes. On the other hand, various cultural landscape elements etc. were introduced such as pavilions, pond serving as fire protection water(square and circle), stone pagoda and stone Buddha, fountains and wells, monument houses, flagpoles etc. In case of Sincheongun town forest(邑藪), Manhagwan(挽河觀), Moonmujeong(文武井), Sangjangdae(上場岱) and Hajangdae(下場岱) Market place, Josanshup<(造山藪 : Dongseojanglim(東西長林)>, Namcheon(南川) etc. were combined and community cultural park with the nature of modern urban park was operated. In this context, government office landscape shows the garden management aspect where square pond and pavilions, flowering trees are harmonized around side pavilion and backyard. Also, environmental design technique not biased to aesthetics and ideological moral philosophy and comprehensively considering functionality (shielding and fire prevention, microclimate control, etc.) and environmental soundness etc. is working.