Journal of The Korean Society of Inherited Metabolic disease
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v.18
no.3
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pp.78-86
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2018
Purpose: We aimed to delineate clinical spectrum and short-term effects after enzyme replacement therapy (ERT) for 5 mucopolysaccharidosis type II (MPS II). Methods: Five patients were diagnosed with MPS II by clinical findings, enzyme activity, and genetic testing. Idursulfase was administered by intravenous infusion at a dose of 0.5 mg/kg every week. Observational chart analysis of patients, who underwent systematic investigations more than 12 months after initiation of ERT was done retrospectively. Results: Three patients were classified as having the attenuated type, and 2 patients were classified as having the severe type. The median age at the diagnosis was 9.6 years (range 3.4-26 years). Four different mutations in 5 Korean patients (4 families) with MPS II were identified, among which two were novel mutations (1 small insertion mutation: p.Thr409Hisfs*22, and 1 missense mutation: p.Gly134Glu). Two severe type sibling patients with the same mutation had different clinical manifestation. Urinary glycosaminoglycan excretion decreased within the twelve months of ERT (P=0.043). Liver and spleen volumes showed reductions that were maintained in all patients (P=0.043 and P=0.043, respectively). Improvements were also noted in left ventricular mass index (P=0.042), shoulder flexion (P=0.043), shoulder abduction (P=0.039), knee flexion (P=0.043), elbow flexion (P=0.042), and respiratory distress index (P=0.041). Conclusion: This study demonstrates that Korean patients with MPS II are clinically heterogeneous and indicates that idursulfase is relatively effective in several clinical parameters including heart size and respiratory distress index without infusion-related reactions in patients with MPS II.
In this article, we examined the patterns of activities of the Sarmathians though in a humble measure, with a focus on the regions where the Sarmathian sheaths spreaded. One of the main weapons the mounted nomads like the Scythias, the Sarmathians, and the Alans used at war was a spear. Though complementary, a sword was the most convenient and appropriate weapon when fighting at a near distance, fallen from the horse to the ground. The Sarmathian swords continued the tradition of the Akinakes which the Scythias or the Persians used, but those of the Sarmathians showed some advances in terms of the easiness with which a sword was drawn out from a sheath, and the way the sheaths were worn to parts of a human body. It turns out that the Sarmathian sheaths, which were designed for the people to draw swords easily, having the sheaths attached to thighs through 4 bumps, spread extensively from Pazyryk, Altai, to South Siberia, Bactria, Parthia and Rome. The most noteworthy out of all the Sarmathian sheaths were the ones that were excavated from the 4th tomb in Tillatepe, Afghanistan which belonged to the region of Bactria. The owner of the fourth tomb of Tilla-tepe whose region was under the control of Kushan Dynasty at that time, was buried wearing Sarmathian swords, and regarded as a big shot in the region of Bactria which was also under the governance of Kushan Dynasty. The fact that the owner of the tomb wore two swords suggests that there had been active exchange between Bactria and Sarmathia. It seemed that the reason why the Sarmathians could play an important role in the exchange between the East and the West might have something to do with their role of supplying Chinese goods to Silk Road. That's why we are interested in how the copper mirrors of Han Dynasty, decoration beads like melon-type beads, crystal beads and goldring articulated beads, and the artifacts of South China which produced silks were excavated in the northern steppe route where the Sarmathians actively worked. Our study have established that the eye beads discovered in Sarmathian tomb estimated to have been built around the 1st century B.C. were reprocessed in China, and then imported to Sarmathia again. We should note the Huns as a medium between the Sarmathians and the South China which were far apart from each other. Thus gold-ring articulated beads which were spread out mainly across the South China has been discovered in the Huns' remains. On the other hand, between 2nd century B.C. and 2nd century A.D. which were main periods of the Sarmathians, it was considered that the traffic route connecting the steppe route and the South China might be West-South silk road which started from Yunnan, passed through Myanmar, Pakistan, and Afghanistan, and then went into the east of India. The West-south Silk road is presumed to have been used by nomadic tribes who wanted to get the goods from South China before the Oasis route was activated by the Han Dynasty's policy of managing the countries bordering on Western China.
A pattern of tropical cyclone (TC) movement in the western North Pacific area was studied using the empirical orthogonal function (EOF) and the best track data from 1951 to 2007. The independent variable used in this study was defined as the frequency of tropical cyclone passage in 5 by 5 degree grid. The $1^{st}$, $2^{nd}$ and $3^{rd}$ modes were the east-west, north-south and diagonal variation patterns. Based on the time series of each component, the signs of first and second mode changed in 1997 and 1991, respectively, which seems to be related to the fact that the passage frequency was higher in the South China Sea for 20 years before 1990s, and recent 20 years in the East Asian area. When the eigen vectors were negative values in the first and second modes and TC moves into the western North Pacific, TC was formed mainly at the east side relatively compared to the case of the positive eigen vectors. The first mode seems to relate to the pressure pattern at the south of Lake Baikal, the second mode the variation pattern around $30^{\circ}N$, and the third mode the pressure pattern around Japan. The first mode was also closely related to the ENSO and negatively related to the $Ni\tilde{n}o$-3.4 index in the correlation analysis with SST anomalies.
In this Paper, we analyze the application of Interactive Data Broadcasting in DMB(Digital Multimedia Broadcasting) in the accordance with convergence of service and technology. With the acceleration of digital convergence in the Ubiquitous period substantial development of digital media technology and convergence of broadcasting and telecommunication industry are being witnessed. Consequently these results gave rise to newly combined-products such as DMB(Digital Multimedia Broadcasting), WCDMA(Wide-band code division multiple access), Wibro(Wireless Broadband Internet), IP-TV (Internet protocol TV) and HSDPA(High speed downlink packet access). The preparatory stage for the implementation of Interactive Data Broadcasting Service will be reached by the end of December, 2006. DMB is the first result of a successful convergence service between Broadcasting and Telecommunication in new media era. Multimedia technology and services are the core elements of DMB. The Data Broadcasting will not only offer various services of interactive information such News, Weather, Broadcasting Program etc, but also be linked with characteristic function of mobile phone such as calling and SMS(Short Message Service) via Return Channel.
Journal of the Korea Academia-Industrial cooperation Society
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v.13
no.2
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pp.947-953
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2012
In this study we investigated the manufacturing method for the activated carbon using the char from the pyrolysis of waste tire. The physical activation method using the steam in the fixed-bed quartz reactor was used for preparation of activated carbon. The primary experiment parameters are the activation temperature, activation time, heating rate, and the injection quantity of active agent. From the results of pore distribution of activated carbon, the micropore which was made in $850^{\circ}C$ of activation temperature, $5^{\circ}C$/min of heating rate, and 3 hours of activation time was developed in biggest quantity, and mesopore and macropore were developed in the biggest quantity too. The optimum conditions for producing the activated carbon using the pyrolysis residue were $850^{\circ}C$ of activation temperature, 3 hours of activation time, $5^{\circ}C$/min of heating rate, and 3 g $H_2O/char-g{\cdot}hr$ of active agent through this study. The produced activated carbon in these conditions showed that the potentiality of utilization as activated carbon because the BET specific surface area was $517.6m^2/g$ and total pore volume was $0.648cm^3/g$.
Kim, Gi-Hong;Yune, Chan-Young;Lee, Hwan-Gil;Hwang, Jae-Seon
Journal of the Korean Society of Surveying, Geodesy, Photogrammetry and Cartography
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v.29
no.1
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pp.47-53
/
2011
From 12 to 16 July 2006, 4 days' torrential rainfall in Deoksan-ri, Inje-up, Inje-gun, Gangwon-do caused massive landslide and debris flow. Huge losses of both life and property, including two people buried to death in submerged houses, resulted from this disaster. As the affected region is mostly mountainous, it was difficult to approach the region and to estimate the exact extent of damage. But using aerial photographs, we can define the region and assess the damage quickly and accurately. In this study the debris flow region in inje, Gangwon-do was analyzed using aerial photographs. This region was divided into three sections - beginning section, flow section and sedimentation section. Informations for each section were extracted by digitizing the shot images with visual reading. Topographic, forest physiognomic and soil characteristics and debris flow occurrences of this region were analyzed by overlaying topographic map, forest type map and soil map using GIS. Comprehensive analysis shows that landslide begins at slope of about $36^{\circ}$, flows down at $26^{\circ}$ slope, and at $21^{\circ}$ slope it stops flowing and deposits. Among forest physiognomic factors, species of trees showd significant relationship with debris flow. And among soil factors, effective soil depth, soil erosion class, and parent materials showed meaningful relationship with debris flow.
The low-field tunnel-type magnetoresistance (MR) properties of sol-gel derived $La_{0.7}Pb_{0.3}MnO_3(LPMO)$ thin film deposited on different substrate have been investigated. Polycrystalline thin films were fabricated by spin-coating on $SiO_2/Si(100)$ substrate and that with yttria-stabilized zirconia (YSZ) buffer layer, while c-axis-oriented thim film was grown on $LaAlO_3(001)$ (LAO) single crystal substrate. The full width half maximum (FWHM) of the rocking curve scan of LPMO/LAO film is $0.32^{\circ}$. Tunnel-type MR ratio is 0.52 % in $LPMO/SiO_2/Si$(100) film and that of $LPMO/YSZ/SiO_2/Si$(100) film is as high as 0.68 %, whereas that of LPMO/LAO(001) film is less than 0.4 % under the applied field of 500 Oe at 300 K. Well-pronounced MR hysteresis was registered with an MR peak in the vicinity of the coercive field. The low-field tunnel-type MR characteristics of thin films deposited on different substrates originates from the behavior of grain boundary properties.
Park, Chang-Min;Kwon, Ju-Chan;Han, Na-Kyeong;Joung, Min-Seok;Paek, Kee-Yoeup;Choi, Jong-Wan
Journal of the Society of Cosmetic Scientists of Korea
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v.40
no.1
/
pp.29-36
/
2014
Dendrobium candidum is one of the well-known orchid on traditional and rare medicinal herb in China. We artificially cultured protocorm-like body and multiple shoots using the bioreactor culture system from Dendrobium candidum and experimented an efficacy as a cosmetic ingredient for skin care. Several studies previously have reported anti-tumor, wound healing and immunological function of Dendrobium candidum but other efficacies were not well known. In the present study, we investigated the cosmetic efficacy to know applicable value of protocorm-like body and multiple shoots cultured from Dendrobium candidum as a cosmetic ingredient. The biological activities of extracts from protocorm-like body, multiple shoots, aerial part and underground part of Dendrobium candidum were investigated. Results were found that extracts of protocorm-like body are superior to other extracts (underground part, aerial part and multiple shoots extracts) on anti-oxidant effect. Also, protocorm-like body extract contained the phenolic and flavonoid compounds more than aerial part, underground part and multiple shoots extracts. In addition, we investigated skin whitening effect related to whitening of skin. In tyrosinase activity and melanin synthesis assay, multiple shoots extract is superior to other extracts (aerial part, underground part and protocorm-like body), on inhibitory effect of tyrosinase activity and melanin synthesis respectively. These results indicate that the protocorm-like body and multiple shoots extracts cultured from Dendrobium candidum presents a new possibility of being applicable to skin improvement as a cosmetic ingredient.
Uljin cassiterite deposit had been known to be a pegmatitic origin derived from the Wangpiri (Buncheon) granitic gneiss of Precambrian period. Lithium ore also shows the same origin and its lithium bearing mineral was ascertained to be a taeniolite. But the presence of leucocratic granites which played the role of host rocks haven't been clearly designated yet in these provinces. Even though Bonghwa and Youngweol sericite deposits situated in the vicinities of Hambaeg syncline had been known to have their host rocks as Hongjesa Granites of Precambrian period and Pegmatitic migmatite of unknown age respectively. But younger leucocratic granites are characterized by more amounts of albite and sericite (muscovite-3T type) than those of the older granitic rocks which contain plenty of biotite and chlorites. Although the younger granites show rather higher contents of alkalies such as $Na_2O$ (0.13~8.03 wt%) and $K_2O$ (1.71~6.38 wt%), but CaO (0.05~1.21 wt%) is very deficient due to the albitization and greisenization. Manisan granite, which is assumed to be Daebo granite which intruded the Gyunggi Gneiss Complex was again intruded by leucocratic granite whose microclinized part changed into kaolins. Taebaegsan region shows a wide distribution of carbonate rocks which are especially favorable to the ore depositions. And the presence of alkali granites which formed in the later magmatic evolution are well known to be worthwhile to the prospections of various rare metals and REEs resources.
Ipriflavone (IP) stimulates proliferation and differentiation of osteoblast and also enhances calcitonin secretion in the presence of estrogen. Poly(lactide-co-glycolide) (PLCA) due to its controllable biodegradability and relatively good biocompatibility is one of the most significant candidates for the study of drug controlled release system. In this study, IP-loaded PLGA microspheres (MSs) was prepared by using conventional O/W solvent evaporation method. The size of MSs was in the range of 5~200 $mu extrm{m}$. The morphology of MSs was characterized by SEM. And, in vitro release amounts of IP were analyzed by HPLC. The highest encapsulation efficiency were obtained by using gelatin and polyvinyl alcohol (PVA) as emulsifiers. The morphology, size distribution, and in vitro release pattern of MSs were changed by several preparation parameters such as molecular weights (8, 20, 33 and 90 kg/mol), polymer concentrations (2.5, 5, 10 and 20%), emulsifier types (PVA, gelatin and Tween 80), initial drug loading amount (5, 10, 20 and 30%) and stirring speed (250, 500 and 1000 rpm). The release of IP in vitro was more prolonged over 30 days, with close to zero-order pattern by controlling the preparation parameters. The physicochemical properties of IP-loaded PLGA MSs were investigated by XRD and DSC.
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