• Title/Summary/Keyword: Mass Storage

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From diagnosis to treatment of mucopolysaccharidosis type VI: A case report with a novel variant, c.1157C>T (p.Ser386Phe), in ARSB gene

  • Yoo, Sukdong;Lee, Jun;Kim, Minji;Yoon, Ju Young;Cheon, Chong Kun
    • Journal of Genetic Medicine
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    • v.19 no.1
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    • pp.32-37
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    • 2022
  • Mucopolysaccharidosis type VI (MPS VI) is an autosomal recessive lysosomal disorder caused by the deficiency of arylsulfatase B due to mutations in the ARSB gene. Here, we report the case of a Korean female with a novel variant of MPS VI. A Korean female aged 5 years and 8 months, who is the only child of a healthy non-consanguineous Korean couple, presented at our hospital for severe short stature. She had a medical history of umbilical hernia and recurrent otitis media. Her symptoms included snoring and mouth breathing. Subtle dysmorphic features, including mild coarse face, joint contracture, hepatomegaly, and limited range of joint motion, were identified. Radiography revealed deformities, suggesting skeletal dysplasia. Growth hormone (GH) provocation tests revealed complete GH deficiency. Targeted exome sequencing revealed compound heterozygous mutations in the ARSB genes c.512G>A (p.Gly171Asp; a pathogenic variant inherited from her father) and c.1157C>T (p.Ser386Phe; a novel variant inherited from her mother in familial genetic testing). Quantitative tests revealed increased urine glycosaminoglycan (GAG) levels and decreased enzyme activity of arylsulfatase B. While on enzyme replacement therapy and GH therapy, her height increased drastically; her coarse face, joint contracture, snoring, and obstructive sleep apnea improved; urine GAG decreased; and left ventricular mass index was remarkably decreased. We report a novel variant-c.1157C>T (p.Ser386Phe)-of the ARSB gene in a patient with MPS VI; these findings will expand our knowledge of its clinical spectrum and molecular mechanisms.

Development of Plastic/Gelatin Bilayer Active Packaging Film with Antibacterial and Water-Absorbing Functions for Lamb Preservation

  • Shijing Wang;Weili Rao;Chengli Hou;Raheel Suleman;Zhisheng Zhang;Xiaoyu Chai;Hanxue Tian
    • Food Science of Animal Resources
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    • v.43 no.6
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    • pp.1128-1149
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    • 2023
  • In order to extend the shelf life of refrigerating raw lamb by inhibiting the growth of microorganisms, preventing the oxidation of fat and protein, and absorbing the juice outflow of lamb during storage, an active packaging system based on plastic/gelatin bilayer film with essential oil was developed in this study. Three kinds of petroleum-derived plastic films, oriented polypropylene (OPP), polyethylene terephthalate, and polyethylene, were coated with gelatin to make bilayer films for lamb preservation. The results showed significant improvement in the mechanical properties, oxygen, moisture, and light barriers of the bilayer films compared to the gelatin film. The OPP/gelatin bilayer film was selected for further experiments because of its highest acceptance by panelists. If the amount of juice outflow was less than 350% of the mass of the gelatin layer, it was difficult for the gelatin film to separate from lamb. With the increase in essential oil concentration, the water absorption capacity decreased. The OPP/gelatin bilayer films with 20% mustard or 10% oregano essential oils inhibited the growth of bacteria in lamb and displayed better mechanical properties. Essential oil decreased the brightness and light transmittance of the bilayer films and made the film yellow. In conclusion, our results suggested that the active packaging system based on OPP/gelatin bilayer film was more suitable for raw lamb preservation than single-layer gelatin film or petroleum-derived plastic film, but need further study, including minimizing the amount of essential oil, enhancing the mechanical strength of the gelatin film after water absorption.

Overview of separate effect and integral system tests on the passive containment cooling system of SMART100

  • Jin-Hwa Yang;Tae-Hwan Ahn;Hong Hyun Son;Jin Su Kwon;Hwang Bae;Hyun-Sik Park;Kyoung-Ho Kang
    • Nuclear Engineering and Technology
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    • v.56 no.3
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    • pp.1066-1080
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    • 2024
  • SMART100 has a containment pressure and radioactivity suppression system (CPRSS) for passive containment cooling system (PCCS). This prevents overheating and over-pressurization of a containment through direct contact condensation in an in-containment refueling water storage tank (IRWST) and wall condensation in a CPRSS heat exchanger (CHX) in an emergency cool-down tank (ECT). The Korea Atomic Energy Research Institute (KAERI) constructed scaled-down test facilities, SISTA1 and SISTA2, for the thermal-hydraulic validation of the SMART100 CPRSS. Three separate effect tests were performed using SISTA1 to confirm the heat removal characteristics of SMART100 CPRSS. When the low mass flux steam with or without non-condensable gas is released into an IRWST, the conditions for mitigation of the chugging phenomenon were identified, and the physical variables were quantified by the 3D reconstruction method. The local behavior of the non-condensable gas was measured after condensation inside heat exchanger using a traverse system. Stratification of non-condensable gas occurred in large tank of the natural circulation loop. SISTA2 was used to simulate a small break loss-of-coolant accident (SBLCOA) transient. Since the test apparatus was a metal tank, compensations of initial heat transfer to the material and effect of heat loss during long-term operation were important for simulating cooling performance of SMART100 CPRSS. The pressure of SMART100 CPRSS was maintained below the design limit for 3 days even under sufficiently conservative conditions of an SBLOCA transient.

Investigations on seismic performance of nuclear power plants equipped with an optimal BIS-TMDI considering FSI effects

  • Shuaijun Zhang;Gangling Hou;Chengyu Yang;Zhihua Yue;Yuzhu Wang;Min He;Lele Sun;Xuesong Cai
    • Nuclear Engineering and Technology
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    • v.56 no.7
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    • pp.2595-2609
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    • 2024
  • This paper introduces a base isolation system-tuned mass damper inerter (BIS-TMDI) hybrid system to the AP1000 nuclear power plant (NPP), which reduces seismic damage potential of the NPP structure. The effects of fluid-structure interaction (FSI) caused by the passive containment cooling system water storage tank (PCCWST) on NPP's seismic performance are investigated. The FSI of water tank theoretical model is considered based on the Housner's model, and a series of time history analyses are performed to prove the rationality of the proposed model. Three single-objective optimization strategies are employed to minimize the relative displacement variance and absolute acceleration variance of the upper structure, as well as the filtered energy index (FEI). Furthermore, a multi-objective optimization strategy considering all these three indexes is proposed to obtain optimal parameters of vibration control. The influence of vibration control strategies on the relative deformation and acceleration of the upper structure is explored with various water level ratios. The analytical results indicate that the proposed BIS-TMDI strategy has significantly reduced the NPP structure's seismic response. The effectiveness of the vibration control strategy is influenced by the water level ratio, emphasizing the significance of designing an appropriate water level ratio to reduce NPP structure's seismic response.

DNA Sequences Compression using Repeat technique and Selective Encryption using modified Huffman's Technique

  • Syed Mahamud Hossein; Debashis De; Pradeep Kumar Das Mohapatra
    • International Journal of Computer Science & Network Security
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    • v.24 no.8
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    • pp.85-104
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    • 2024
  • The DNA (Deoxyribonucleic Acid) database size increases tremendously transmuting from millions to billions in a year. Ergo for storing, probing the DNA database requires efficient lossless compression and encryption algorithm for secure communication. The DNA short pattern repetitions are of paramount characteristics in biological sequences. This algorithm is predicated on probing exact reiterate, substring substitute by corresponding ASCII code and engender a Library file, as a result get cumulating of the data stream. In this technique the data is secured utilizing ASCII value and engendering Library file which acts as a signature. The security of information is the most challenging question with veneration to the communication perspective. The selective encryption method is used for security purpose, this technique is applied on compressed data or in the library file or in both files. The fractional part of a message is encrypted in the selective encryption method keeping the remaining part unchanged, this is very paramount with reference to selective encryption system. The Huffman's algorithm is applied in the output of the first phase reiterate technique, including transmuting the Huffman's tree level position and node position for encryption. The mass demand is the minimum storage requirement and computation cost. Time and space complexity of Repeat algorithm are O(N2) and O(N). Time and space complexity of Huffman algorithm are O(n log n) and O(n log n). The artificial data of equipollent length is additionally tested by this algorithm. This modified Huffman technique reduces the compression rate & ratio. The experimental result shows that only 58% to 100% encryption on actual file is done when above 99% modification is in actual file can be observed and compression rate is 1.97bits/base.

Analysis of a Groundwater Flow System in Fractured Rock Mass Using the Concept of Hydraulic Compartment (수리영역 개념을 적용한 단열암반의 지하수유동체계 해석)

  • Cho Sung-Il;Kim Chun-Soo;Bae Dae-Seok;Kim Kyung-Su;Song Moo-Young
    • The Journal of Engineering Geology
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    • v.16 no.1 s.47
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    • pp.69-83
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    • 2006
  • This study aims to evaluate a complex groundwater flow system around the underground oil storage caverns using the concept of hydraulic compartment. For the hydrogeological analysis, the hydraulic testing data, the evolution of groundwater levels in 28 surface monitoring boreholes and pressure variation of 95 horizontal and 63 vertical water curtain holes in the caverns were utilized. At the cavern level, the Hydraulic Conductor Domains(fracture zones) are characterized one local major fracture zone(NE-1)and two local fracture zones between the FZ-1 and FZ-2 fracture zones. The Hydraulic Rock Domain(rock mass) is divided into four compartments by the above local fracture zones. Two Hydraulic Rock Domains(A, B) around the FZ-2 zone have a relatively high initial groundwater pressures up to $15kg/cm^2$ and the differences between the upper and lower groundwater levels, measured from the monitoring holes equipped with double completion, are in the range of 10 and 40 m throughout the construction stage, indicating relatively good hydraulic connection between the near surface and bedrock groundwater systems. On the other hand, two Hydraulic Rock Domains(C, D) adjacent to the FZ-1, the groundwater levels in the upper and lower zones are shown a great difference in the maximum of 120 m and the high water levels in the upper groundwater system were not varied during the construction stage. This might be resulted from the very low hydraulic conductivity$(7.2X10^{-10}m/sec)$ in the zone, six times lower than that of Domain C, D. Groundwater recharge rates obtained from the numerical modeling are 2% of the annual mean precipitation(1,356mm/year) for 20 years.

The Study on the Confidence Building for Evaluation Methods of a Fracture System and Its Hydraulic Conductivity (단열체계 및 수리전도도의 해석신뢰도 향상을 위한 평가방법 연구)

  • Cho Sung-Il;Kim Chun-Soo;Bae Dae-Seok;Kim Kyung-Su;Song Moo-Young
    • The Journal of Engineering Geology
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    • v.15 no.2 s.42
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    • pp.213-227
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    • 2005
  • This study aims to assess the problems with investigation method and to suggest the complementary solutions by comparing the predicted data from surface investigation with the outcome data from underground cavern. In the study area, one(NE-1) of 6 fracture zones predicted during the surface investigation was only confirmed in underground caverns. Therefore, it is necessary to improve the confidence level for prediction. In this study, the fracture classification criteria was quantitatively suggested on the basis of the BHTV images of NE-1 fracture zone. The major orientation of background fractures in rock mass was changed at the depth of the storage cavern, the length and intensity were decreased. These characteristics result in the deviation of predieted predicted fracture properties and generate the investigation bias depending on the bore hole directions and investigated scales. The evaluation of hydraulic connectivity in the surface investigation stage needs to be analyze by the groundwater pressures and hydrochemical properties from the monitoring bore hole(s) equipped with a double completion or multi-packer system during the test bore hole is pumping or injecting. The hydraulic conductivities in geometric mean measured in the underground caverns are 2-3 times lower than those from the surface and furthermore the horizontal hydraulic conductivity in geometric mean is six times lower than the vertical one. To improve confidence level of the hydraulic conductivity, the orientation of test hole should be considered during the analysis of the hydraulic conductivity and the methodology of hydro-testing and interpretation should be based on the characteristics of rock mass and investigation purposes.

An Analysis of a Porous Film Containing $Chamaecyparis$ $obtusa$ Extract (편백나무 추출물을 함유한 다공성 필름 분석)

  • Kim, Kyeong-Yee;Lee, Eun-Kyung
    • The Korean Journal of Food And Nutrition
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    • v.24 no.4
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    • pp.551-558
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    • 2011
  • This present study was performed to analyze the efficiency and volatility of a porous film containing $Chamaecyparis$ $obtusa$ extract as a method to effectively package food compounds. Phytoncide was contacted the state of gas and showed effective antimicrobial properties. Limonene can be distilled without decomposition as a relatively stable terpene and was one of the extract components. $Chamaecyparis$ $obtusa$ essential oil. The optimal solvent composition was a ratio 5:20:0.3 of T-500:ethanol:hardener to effectively manufacture film containing phytoncide essential oil and the minimum antibacterial concentration was 2%. The films were made under different conditions(A-50LF1, A-25SF2, B-50SF1, C-50LF1, C-25SF2 and D-50SF1) containing phytoncide and the amounts of limonene inside the 1-L reaction chamber depending on storage were measured by gas chromatography-mass selective detention. The results showed that the 25SF2(width, 25 mm; length, 20 cm) revealed more amount of limonene compared with 50LF1(width 50 mm, length 20 cm). We confirmed that the gas emission amount showed a better layer on the film side than on the internal film. An effect of film thickness on phytoncide emissions was observed in that the amounts was less than the expectation for a thicker film at the beginning time, but the emitting amounts increased with increasing storage periods. In the storage testing of various films at $35^{\circ}C$ and 70% humidity for 14 days, 25SF2 showed longer preservation compared with that of 50LF in the case of bread. $C.$ $obtusa$ essential oil is a useful fresh ingredients, hence, analysis of limonene emission kinetics from various film was helpful to develop films with an optimal antimicrobial effect, and will allow application of such films in food packaging systems.

Recent Progress in Air-Conditioning and Refrigeration Research : A Review of Papers Published in the Korean Journal of Air-Conditioning and Refrigeration Engineering in 2016 (설비공학 분야의 최근 연구 동향 : 2016년 학회지 논문에 대한 종합적 고찰)

  • Lee, Dae-Young;Kim, Sa Ryang;Kim, Hyun-Jung;Kim, Dong-Seon;Park, Jun-Seok;Ihm, Pyeong Chan
    • Korean Journal of Air-Conditioning and Refrigeration Engineering
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    • v.29 no.6
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    • pp.327-340
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    • 2017
  • This article reviews the papers published in the Korean Journal of Air-Conditioning and Refrigeration Engineering during 2016. It is intended to understand the status of current research in the areas of heating, cooling, ventilation, sanitation, and indoor environments of buildings and plant facilities. Conclusions are as follows. (1) The research works on the thermal and fluid engineering have been reviewed as groups of flow, heat and mass transfer, the reduction of pollutant exhaust gas, cooling and heating, the renewable energy system and the flow around buildings. CFD schemes were used more for all research areas. (2) Research works on heat transfer area have been reviewed in the categories of heat transfer characteristics, pool boiling and condensing heat transfer and industrial heat exchangers. Researches on heat transfer characteristics included the results of the long-term performance variation of the plate-type enthalpy exchange element made of paper, design optimization of an extruded-type cooling structure for reducing the weight of LED street lights, and hot plate welding of thermoplastic elastomer packing. In the area of pool boiling and condensing, the heat transfer characteristics of a finned-tube heat exchanger in a PCM (phase change material) thermal energy storage system, influence of flow boiling heat transfer on fouling phenomenon in nanofluids, and PCM at the simultaneous charging and discharging condition were studied. In the area of industrial heat exchangers, one-dimensional flow network model and porous-media model, and R245fa in a plate-shell heat exchanger were studied. (3) Various studies were published in the categories of refrigeration cycle, alternative refrigeration/energy system, system control. In the refrigeration cycle category, subjects include mobile cold storage heat exchanger, compressor reliability, indirect refrigeration system with $CO_2$ as secondary fluid, heat pump for fuel-cell vehicle, heat recovery from hybrid drier and heat exchangers with two-port and flat tubes. In the alternative refrigeration/energy system category, subjects include membrane module for dehumidification refrigeration, desiccant-assisted low-temperature drying, regenerative evaporative cooler and ejector-assisted multi-stage evaporation. In the system control category, subjects include multi-refrigeration system control, emergency cooling of data center and variable-speed compressor control. (4) In building mechanical system research fields, fifteenth studies were reported for achieving effective design of the mechanical systems, and also for maximizing the energy efficiency of buildings. The topics of the studies included energy performance, HVAC system, ventilation, renewable energies, etc. Proposed designs, performance tests using numerical methods and experiments provide useful information and key data which could be help for improving the energy efficiency of the buildings. (5) The field of architectural environment was mostly focused on indoor environment and building energy. The main researches of indoor environment were related to the analyses of indoor thermal environments controlled by portable cooler, the effects of outdoor wind pressure in airflow at high-rise buildings, window air tightness related to the filling piece shapes, stack effect in core type's office building and the development of a movable drawer-type light shelf with adjustable depth of the reflector. The subjects of building energy were worked on the energy consumption analysis in office building, the prediction of exit air temperature of horizontal geothermal heat exchanger, LS-SVM based modeling of hot water supply load for district heating system, the energy saving effect of ERV system using night purge control method and the effect of strengthened insulation level to the building heating and cooling load.

Recent Progress in Air Conditioning and Refrigeration Research - A Review of Papers Published in the Korean Journal of Air-Conditioning and Refrigeration Engineering in 2002 and 2003 - (공기조화, 냉동 분야의 최근 연구 동향 -2002년 및 2003년 학회지 논문에 대한 종합적 고찰 -)

  • Chung Kwang-Seop;Kim Min Soo;Kim Yongchan;Park Kyoung Kuhn;Park Byung-Yoon;Cho Keumnam
    • Korean Journal of Air-Conditioning and Refrigeration Engineering
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    • v.16 no.12
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    • pp.1234-1268
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    • 2004
  • A review on the papers published in the Korean Journal of Air-Conditioning and Refrigerating Engineering in 2002 and 2003 has been carried out. Focus has been put on current status of research in the aspect of heating, cooling, air-conditioning, ventilation, sanitation and building environment/design. The conclusions are as follows. (1) Most of fundamental studies on fluid flow were related with heat transportation in diverse facilities. Drop formation and rivulet flow on solid surfaces were interesting topics related with condensation augmentation. Research on micro environment considering flow, heat transfer, humidity was also interesting to promote comfortable living environment. It can be extended considering biological aspects. Development of fans and blowers of high performance and low noise were continuing research topics. Well developed CFD technologies were widely applied for analysis and design of various facilities and their systems. (2) Heat transfer characteristics of enhanced finned tube heat exchangers and heat sinks were extensively investigated. Experimental studies on the boiling heat transfer, vortex generators, fluidized bed heat exchangers, and frosting and defrosting characteristics were also conducted. In addition, the numerical simulations on various heat exchangers were performed and reported to show heat transfer characteristics and performance of the heat exchanger. (3) A review of the recent studies shows that the performance analysis of heat pump have been made by various simulations and experiments. Progresses have been made specifically on the multi-type heat pump systems and other heat pump systems in which exhaust energy is utilized. The performance characteristics of heat pipe have been studied numerically and experimentally, which proves the validity of the developed simulation programs. The effect of various factors on the heat pipe performance has also been examined. Studies of the ice storage system have been focused on the operational characteristics of the system and on the basics of thermal storage materials. Researches into the phase change have been carried out steadily. Several papers deal with the cycle analysis of a few thermodynamic systems which are very useful in the field of air-conditioning and refrigeration. (4) Recent studies on refrigeration and air-conditioning systems have focused on the system performance and efficiency enhancement when new alternative refrigerants are applied. Heat transfer characteristics during evaporation and condensation are investigated for several tube shapes and new alternative refrigerants including natural refrigerants. Efficiency of various compressors and performance of new expansion devices are also dealt with for better design of refrigeration/air conditioning system. In addition to the studies related with thermophysical properties of refrigerant mixtures, studies on new refrigerants are also carried out. It should be noted that the researches on two-phase flow are constantly carried out. (5) A review of the recent studies on absorption refrigeration system indicates that heat and mass transfer enhancement is the key factor in improving the system performance. Various experiments have been carried out and diverse simulation models have been presented. Study on the small scale absorption refrigeration system draws a new attention. Cooling tower was also the research object in the respect of enhancement its efficiency, and performance analysis and optimization was carried out. (6) Based on a review of recent studies on indoor thermal environment and building service systems, it is noticed that research issues have mainly focused on several innovative systems such as personal environmental modules, air-barrier type perimeterless system with UFAC, radiant floor cooling system, etc. New approaches are highlighted for improving indoor environmental conditions and minimizing energy consumption, various activities of building energy management and cost-benefit analysis for economic evaluation.