• Journal of Genetic Medicine
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• v.17 no.1
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• pp.39-42
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• 2020

The Senior-Loken syndrome was first described in 1961 as an oculo-renal disease consisting of familial juvenile nephronophthisis and Leber congenital amaurosis. It is a rare autosomal recessive disorder with a prevalence of 1:1,000,000 caused by mutations in nine genes (NPHP 1-8 and NPHP 10). Ocular manifestations (e.g., photophobia, nystagmus, and extreme hyperopia) occur within the first few years of life while renal manifestations (e.g., formation of multiple cysts impairing kidney function and end-stage renal disease) appear in late childhood to adolescence. Here, we report a case of a Filipino male presenting with rotatory nystagmus and progressive deterioration of vision since childhood. He had congenital amaurosis and juvenile nephronophthisis that progressed to end stage renal disease by age 19. All laboratory and imaging findings were consistent with chronic kidney disease. Molecular genetic testing of ciliopathy-related genes was performed revealing a homozygous mutation in exon 11 of the IQCB1/NPHP5 gene, c.1090C>T (p.Arg364^⁎). This sequence change created a premature translational stop signal resulting in a truncated protein product, nephrocystin-5 and its consequent loss of function. His symptoms eventually improved with initiation dialysis. The prognosis of Senior-Loken syndrome remains dismal and a high index of suspicion, early diagnosis and timely intervention of renal complications are warranted.

• Smart Structures and Systems
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• v.31 no.4
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• pp.335-349
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• 2023

Bridges constantly undergo deterioration and damage, the most common ones being concrete damage and exposed rebar. Periodic inspection of bridges to identify damages can aid in their quick remediation. Likewise, identifying components can provide context for damage assessment and help gauge a bridge's state of interaction with its surroundings. Current inspection techniques rely on manual site visits, which can be time-consuming and costly. More recently, robotic inspection assisted by autonomous data analytics based on Computer Vision (CV) and Artificial Intelligence (AI) has been viewed as a suitable alternative to manual inspection because of its efficiency and accuracy. To aid research in this avenue, this study performs a comparative assessment of different architectures, loss functions, and ensembling strategies for the autonomous segmentation of bridge components and damages. The experiments lead to several interesting discoveries. Nested Reg-UNet architecture is found to outperform five other state-of-the-art architectures in both damage and component segmentation tasks. The architecture is built by combining a Nested UNet style dense configuration with a pretrained RegNet encoder. In terms of the mean Intersection over Union (mIoU) metric, the Nested Reg-UNet architecture provides an improvement of 2.86% on the damage segmentation task and 1.66% on the component segmentation task compared to the state-of-the-art UNet architecture. Furthermore, it is demonstrated that incorporating the Lovasz-Softmax loss function to counter class imbalance can boost performance by 3.44% in the component segmentation task over the most employed alternative, weighted Cross Entropy (wCE). Finally, weighted softmax ensembling is found to be quite effective when used synchronously with the Nested Reg-UNet architecture by providing mIoU improvement of 0.74% in the component segmentation task and 1.14% in the damage segmentation task over a single-architecture baseline. Overall, the best mIoU of 92.50% for the component segmentation task and 84.19% for the damage segmentation task validate the feasibility of these techniques for autonomous bridge component and damage segmentation using RGB images.

• Journal of Nuclear Fuel Cycle and Waste Technology(JNFCWT)
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• v.5 no.4
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• pp.309-322
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• 2007

This study deals with the irradiation effects on some selected components which are being used in an Advanced Spent Fuel Conditioning Process (ACP). Irradiation test components have a higher priority from the aspect of their reliability because their degradation or failure is able to critically affect the performance of an ACP equipment. Components that we chose for the irradiation tests were the AC servo motor, potentiometer, thermocouples, accelerometer and CCD camera. ACP facility has a number of AC servo motors to move the joints of a manipulator and to operate process equipment. Potentiometers are used for a measurement of several joint angles in a manipulator. Thermocouples are used for a temperature measurement in an electrolytic reduction reactor, a vol-oxidation reactor and a molten salt transfer line. An accelerometer is installed in a slitting machine to forecast an incipient failure during a slitting process. A small CCD camera is used for an in-situ vision monitoring between ACP campaigns. We made use of a gamma-irradiation facility with cobalt-60 source for an irradiation test on the above components because gamma rays from among various radioactive rays are the most significant for electric, electronic and robotic components. Irradiation tests were carried out for enough long time for total doses to be over expected threshold values. Other components except the CCD camera showed a very high radiation hardening characteristic. Characteristic changes at different total doses were investigated and threshold values to warrant at least their performance without a deterioration were evaluated as a result of the irradiation tests.

• Journal of Korean Society of Environmental Engineers
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• v.38 no.6
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• pp.299-308
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• 2016

Seoul metropolitan has established a vision as 'Healthy water-cycle city' to resolve urban water-environmental deterioration. And it established administrative structure to expand Low Impact Development (LID) facilities to recover aggravated water-cycle and water-environment. Therefore, various LID facilities are constructed and operated, however, benefit analytic plans for systematic valuation are insufficient. In this study, to analyze economic, environmental and social benefits of LID facilities, contents for benefit analysis were selected and categorized as water, energy, air quality and climate changes. As a result of quantification and valuation to the beneficial effects, LID facilities showed the total benefit as 1,191~3,292 won/yr. Characteristics of benefit distribution by analysis contents were various reflecting functional characteristics of each LID facility (Water: 30~90%, Energy: 4~44%, Air quality: <1~2%, Climate change: 5~22%). As a result of Triple Bottom Line analysis, economic benefit showed the greatest portion as 75~90%. As further studies, suggested benefit assessment plans for each LID facility should be applied to inter-connected LID systems on complex-scaled area, and synergy effects by various LID systems would be evaluated such as prevention of heat island and flood disasters.

• Journal of The Korean Ophthalmological Society
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• v.59 no.11
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• pp.1082-1086
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• 2018

Purpose: To report a case of a 60-year-old female with rhegmatogenous retinal detachment, presenting with suprachoroidal hemorrhage after vitrectomy. Case summary: A 60-year-old woman visited our clinic complaining of floaters, flashing, and blurred vision. Best-corrected visual acuity was 0.1 in her left eye, and fundus examination of her left eye revealed macula-involved retinal detachment with a retinal break at the superotemporal quadrant. She underwent cataract surgery, 23-gauge transconjunctival sutureless vitrectomy, and 14% C3F8 gas tamponade under general anesthesia. One hour after anesthesia recovery, she suddenly complained of severe pain in her left eye. The intraocular pressure measured after removal of the pressure patch from her left eye was as high as 58 mmHg. Her ocular pain improved spontaneously within 10 minutes, and the intraocular pressure decreased to 8 mmHg. Fundus examination of her left eye revealed a reddish-brown raised lesion, suggesting suprachoroidal hemorrhage. She was placed in a prone position with a pressure patch over her left eye. Bleeding through the sclerotomy site was observed 1 day after surgery. Subsequently, hemorrhagic choroidal detachment of her left eye continued to decrease without deterioration. Three weeks after surgery, the patient received an intravitreal injection of 100% C3F8 gas into her left eye. At 3 months after surgery, best-corrected visual acuity had improved to 0.8 in her left eye, and the retina was stable. Conclusions: Suprachoroidal hemorrhage may be suspected in a patient complaining of severe ocular pain after vitrectomy.

• 한국노년학
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• v.32 no.4
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• pp.993-1011
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• 2012

The purpose of this study is to investigate the factors associated with the incidence and the transition of disability among Korean older adults. Samples consist of 1,454(42.7%) men and 2,032(58.3%) women aged 65 and over who participated in the 1st and 2nd wave of the Korea Longitudinal Study of Aging: KLOSA. To estimate the level of disability, ADL and IADL disability indexes are used. As the results, major risk factors for ADL/IADL disability incidence include injury, vision problem, cognitive function, depression, health behavior, socioeconomic characteristics and age. Among the normal older adults, the odds ratio of having dementia symptoms at 2nd wave(2008) are 2.0 times greater for the older adults who have less cognitive function than those who don't have at 1st wave(2006). Among the older adults with chronic diseases, the odds ratio of having disability at 2nd wave are 1.8 times greater for the older persons who have depression than those who don't have at 1st wave. Secondly, concerning the predictors affecting the disability transition among the disabled older adults at 1st wave, the likelihood of remaining at the same level or deteriorating the level of IADL disability, as compared with improving the level, is associated with having less instrumental support or being older. These results indicate that it is necessary to prefer multilevel intervention in order not only to prevent the incidence of disability, but also to prolong the deterioration of disability in the older adults.

• KSCE Journal of Civil and Environmental Engineering Research
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• v.29 no.6D
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• pp.745-752
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• 2009

Management of social infrastructure has been advanced from facility management (FM) to asset management (AM), which adopts the aggressive and proactive methods in predicting the deterioration of infrastructure, prevents failures, and eventually saves maintenance cost. Infrastructure asset management is not a simple engineering technique, but it is a new paradigm evolved from facility management practices. To implement the infrastructure asset management successfully, organizational reform is very important. This paper suggests critical success factors and key performance indicators to implement the infrastructure asset management for facility managers of government owned social infrastructures such as roads and bridges. Reorganizing the facility management group requires new vision, objectives, strategies for the paradigm-changing asset management. This paper uses Balanced Score Card (BSC) which is a proven method in measuring and setting new objectives for an organization. Once the performance indicators are reviewed repeatedly by facility managers through experts workshops, developed BSC can be used in practice. This paper discusses the development of robust BSC scoring method through in depth literature reviews and investigation of asset management practices of domestic and international cases.

• Journal of The Korean Ophthalmological Society
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• v.59 no.12
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• pp.1152-1159
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• 2018

Purpose: We prospectively investigated clinical changes and long-term outcomes after administration of the drugs recommended by the Age-Related Eye Disease Study-2 to patients with intermediate age-related macular degeneration (AMD). Methods: This prospective multicenter study enrolled 79 eyes of 55 patients taking lutein and zeaxanthin. The primary endpoint was contrast sensitivity; this was checked every 12 months for a total of 36 months after treatment commenced. The secondary endpoints were visual acuity, central macular thickness, and drusen volume; the latter two parameters were assessed using spectral domain optical coherence tomography. Results: The mean patient age was $72.46{\pm}7.16years$. Contrast sensitivity gradually improved at both three and six cycles per degree. The corrected visual acuity was $0.13{\pm}0.14logMAR$ and did not change significantly over the 36 months. Neither the central macular thickness nor drusen volume changed significantly. Conclusions: Contrast sensitivity markedly improved after treatment, improving vision and patient satisfaction. Visual acuity, central retinal thickness, and drusen volume did not deteriorate. Therefore, progression of AMD and visual function deterioration were halted.