• Journal of mucopolysaccharidosis and rare diseases
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• v.3 no.1
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• pp.9-13
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• 2017

Tandem mass spectrometry and other new technologies for the multiplex and quantitative analysis of dried blood spots have emerged as powerful techniques for the early screening and assessment of newborns for lysosomal storage diseases (LSDs). Screening newborns for these diseases is important, since treatment options, including enzyme replacement therapy or hematopoietic transplantation, are available for some LSDs, such as infant-onset Pompe disease, Fabry disease, some types of mucopolysaccharidoses (MPSs), and Krabbe disease. For these diseases, early initiation of treatment, before symptoms worsen, often leads to better clinical outcomes. Several problems, however, are associated with newborn screening for LSDs, including the development of accurate test methods to reduce low false-positive rates and treatment guidelines for late-onset or mild disease variants, the high costs associated with multiplex assays, and ethical issues. In this review, we discuss the history, current status, and ethical problems associated with the newborn screening for LSDs, including MPSs.

• Clinical and Experimental Pediatrics
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• v.57 no.8
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• pp.337-344
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• 2014

Inherited bone marrow failure syndrome (IBMFS) encompasses a heterogeneous and complex group of genetic disorders characterized by physical malformations, insufficient blood cell production, and increased risk of malignancies. They often have substantial phenotype overlap, and therefore, genotyping is often a critical means of establishing a diagnosis. Current advances in the field of IBMFSs have identified multiple genes associated with IBMFSs and their pathways: genes involved in ribosome biogenesis, such as those associated with Diamond-Blackfan anemia and Shwachman-Diamond syndrome; genes involved in telomere maintenance, such as dyskeratosis congenita genes; genes encoding neutrophil elastase or neutrophil adhesion and mobility associated with severe congenital neutropenia; and genes involved in DNA recombination repair, such as those associated with Fanconi anemia. Early and adequate genetic diagnosis is required for proper management and follow-up in clinical practice. Recent advances using new molecular technologies, including next generation sequencing (NGS), have helped identify new candidate genes associated with the development of bone marrow failure. Targeted NGS using panels of large numbers of genes is rapidly gaining potential for use as a cost-effective diagnostic tool for the identification of mutations in newly diagnosed patients. In this review, we have described recent insights into IBMFS and how they are advancing our understanding of the disease's pathophysiology; we have also discussed the possible implications they will have in clinical practice for Korean patients.

• Structural Monitoring and Maintenance
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• v.2 no.1
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• pp.19-34
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• 2015

One of the main causes of a limited use of nondestructive evaluation (NDE) technologies in bridge deck assessment is the speed of data collection and analysis. The paper describes development and implementation of the RABIT (Robotics Assisted Bridge Inspection Tool) for data collection using multiple NDE technologies. The system is designed to characterize three most common deterioration types in concrete bridge decks: rebar corrosion, delamination, and concrete degradation. It implements four NDE technologies: electrical resistivity (ER), impact echo (IE), ground-penetrating radar (GPR), and ultrasonic surface waves (USW) method. The technologies are used in a complementary way to enhance the interpretation. In addition, the system utilizes advanced vision to complement traditional visual inspection. Finally, the RABIT collects data at a significantly higher speed than it is done using traditional NDE equipment. The robotic system is complemented by an advanced data interpretation. The associated platform for the enhanced interpretation of condition assessment in concrete bridge decks utilizes data integration, fusion, and deterioration and defect visualization. This paper concentrates on the validation and field implementation of two NDE technologies. The first one is IE used in the delamination detection and characterization, while the second one is the USW method used in the assessment of concrete quality. The validation of performance of the two methods was conducted on a 9 m long and 3.6 m wide fabricated bridge structure with numerous artificial defects embedded in the deck.

• Journal of Technology Innovation
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• v.25 no.3
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• pp.197-231
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• 2017

We investigate the effect of technology convergence and spillover on firm performance. In doing so we specifically examine how automobile firms adapting aircraft technologies perform in stock market and product market. In order to use technology convergence and spillover, we investigate the patent citation relationship between aircraft and automobile related patents and construct the measures on automobile firms' adaptation of aircraft technologies. We also measure automobile firms' performance based on market value of firms in the stock market and revenues in product market. We find that the market value increases as automobile firms use the knowledge of aircraft technologies more. However, there is no relationship between the use of the aircraft technology and the revenue of automobile firms. This suggests that the use of advance aircraft technology plays a positive signaling role in financial market while it is not associated with generating revenues in product market.

• Korean Chemical Engineering Research
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• v.45 no.4
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• pp.319-327
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• 2007

Incineration has been regarded as the best developed technology available for organically hazardous waste. However, permitting and siting incinerators to treat hazardous waste such as a waste containing PCBs is very difficult due to the public concerns associated with toxic air emissions. Recently, a lot of alternatives to an incineration have been developed and these technologies have the potential of alleviating public concerns by decreasing emissions of hazardous materials such as dioxins and furans. This paper reviews currently available alternative incineration technologies for various hazardous waste streams. Various categories of non-thermal and thermal alternative incineration technologies have been evaluated in terms of their process operating condition, applicability of a waste stream and their emission of secondary waste. Detailed descriptions of operating principles of several technologies are also provided.

• International Journal of Naval Architecture and Ocean Engineering
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• v.6 no.2
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• pp.307-322
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• 2014

With the rate of worldwide LNG demand expected to grow faster than that of gas demand, most major oil companies are currently investing their resources to develop floating LNG-FLNG (i.e. LNG FSRU and LNG FPSO). The global Floating LNG (FLNG) market trend will be reviewed based on demand and supply chain relationships. Typical technical issues associated with FLNG design are categorized in terms of global performance evaluation. Although many proven technologies developed through LNG carrier and oil FPSO projects are available for FLNG design, we are still faced with several technical challenges to clear for successful FLNG projects. In this study, some of the challenges encountered during development of the floating LNG facility (i.e. LNG FPSO and FSRU) will be reviewed together with their investigated solution. At the same time, research of new LNG-related technologies such as combined containment system will be presented.

• Biomaterials and Biomechanics in Bioengineering
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• v.2 no.4
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• pp.225-235
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• 2015

Treatments for asthma are largely pharmaceutical, with some therapies also utilising alternative breathing techniques. The objective of both medical and alternative methods is to relax contracted airway smooth muscle (ASM). In normal subjects, tidal breathing- and deep inspiration-oscillations are believed to have a bronchodilatory effect. Similarly, application of length oscillations to isolated, contracted ASM also elicits muscle relaxation. As a means of investigating more-effective alternative treatment methods for contracted airways, we analyse the combined effects of bronchodilators and length oscillations on isolated, contracted ASM. The contractile state of the muscle tissue prior to treatment is of primary interest. Thereafter, the effect of applying a combination of small superimposed length oscillations with tidal breathing-like oscillations to ASM is studied alone and in combination with a common bronchodilator, isoproterenol (ISO). This work suggests that relaxation of isolated, contracted ASM following application of combined oscillations and ISO is larger than treatments of either combined oscillations or ISO alone. Further, the observed oscillation-associated relaxation is found to be amplitude- rather than frequency-dependent. This study gives additional insight into the role of oscillations and bronchodilators on contracted airways.

• Proceedings of the Korean Vacuum Society Conference
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• 2015.08a
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• pp.82-82
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• 2015

Industrial crystalline silicon (c-Si) solar cells with using a screen printing technology share the global market over 90% and they will continue to be the same for at least the next decade. It seems that the $2^{nd}$ generation and the $3^{rd}$ generation technologies have not yet demonstrated competitiveness in terms of performance and cost. In 2014, new world record efficiency 25.6% (Area-$143.7cm^2$, Voc-0.740V, $Jsc-41.8mA/cm^2$, FF-0.827) was announced from Panasonic and its cell structure is Back Contact $HIT^*$ c-Si solar cell. Here, amorphous silicon passivated contacts were newly applied to back contact solar cell. On the other hand, 24.9% $TOPCon^{**}$ cell was announced from Fraunhofer ISE and its key technology is an excellent passivation quality applying tunnel oxide (<2 nm) between metal and silicon or emitter and base. As a result, to realize high efficiency, high functional technologies are quite required to overcome a theoretical limitation of c-Si solar cell efficiency. In this presentation, Si solar cell technology summarized in the International Technology Roadmap for Photovoltaics ($^{***}ITRPV$ 2014) is introduced, and the present status of R&D associated with various c-Si solar cell technologies will be reviewed. In addition, national R&D projects of c-Si solar cells to be performed by Korea University are shown briefly.

• New & Renewable Energy
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• v.2 no.2 s.6
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• pp.102-107
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• 2006

In Japan, research and development were undertaken on gas hydrate-side industrial processes associated with power generation system connections that may particularly be necessary to develop gas hydrated technology-based industrial systems. In so doing, data and engineering technologies useful n formulating guidelines on design of practical process were accumulated. In addition, basic research into theoretical evidence were carried out to promote and support the development of technological elements for those processes. In basic research designed to promote and support the research and development of elemental technologies, microanalyses were conducted to understand the decomposition mechanism of mixed gas hydrate. Moreover, measurement technologies that can be applied in industrial processes, such as numerical analyses and concentration measurement, were examined. Japan has developed a highly efficient gas hydrate formation process using micro-bubbles with a tubular reactor. Higher formation rate over conventional systems has been obtained by the process. As mentioned above, the technical problems were clarified and the economics were studied from a view point of the NGH technology in this study. The results can be applied for utilization and must contribute to popularization of gas hydrate production.

• Journal of the Society of Naval Architects of Korea
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• v.51 no.3
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• pp.220-230
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• 2014

Typical technical issues associated with Floating LNG (FLNG: FSRU and LNG FPSO) design are categorized in terms of global performance evaluation. Although many proven technologies developed through LNG carrier and oil FPSO projects are available for FLNG design, we are still faced with several technical challenges to clear for successful FLNG projects. In this paper, some of the challenges encountered during development of the floating LNG facility (i.e. LNG FPSO and FSRU) will be reviewed together with their investigated solution. At the same time, research of design improvement including new LNG-related technologies such as combined containment system will be presented to overcome the unrevealed challenges for the FLNG development.