• Journal of Genetic Medicine
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• 제17권2호
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• pp.73-78
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• 2020

Purpose: Congenital myasthenic syndrome (CMS) is a clinically and genetically heterogeneous group of disorders characterized by impaired neuromuscular transmission. This study aims to provide the clue for early diagnosis and improved therapeutic strategies in CMS. Materials and Methods: Through the targeted panel sequencing including twenty CMS causative genes, eleven patients were genetically confirmed and enrolled in this study. A retrospective medical record review was carried out for the clinical and laboratory data analysis. Results: The age of patients ranged from 5 to 23 years, with the median age of 16 years. The peak age at onset of symptoms was the neonatal period. Seven out of the eleven patients were symptomatic at birth. The most commonly reported initial finding was generalized hypotonia with poor sucking and crying. Mean time to accurate diagnosis was 9.3±5.0 years. Total fifteen different variants in seven genes associated with CMS (DOK7, AGRN, RAPSN, CHRNE, COLQ, SLC5A7, and GFPT1) were identified. Conclusion: We describe the clinical and genetic characteristics of CMS patients and treatment outcome in a single tertiary center. High clinical suspicion and timely molecular diagnosis is particularly important for the tailored therapy to maximize clinical improvement in CMS.

• Nuclear Engineering and Technology
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• 제55권3호
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• pp.884-894
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• 2023

Design and safety assessment of fuel pins for application in innovative Generation IV fast reactors calls for a dedicated nuclear fuel modelling and for the extension of the fuel performance code capabilities to the envisaged materials and irradiation conditions. In the INSPYRE Project, comprehensive and physics-based models for the thermal-mechanical properties of U-Pu mixed-oxide (MOX) fuels and for fission gas behaviour were developed and implemented in the European fuel performance codes GERMINAL, MACROS and TRANSURANUS. As a follow-up to the assessment of the reference code versions ("pre-INSPYRE", NET 53 (2021) 3367-3378), this work presents the integral validation and benchmark of the code versions extended in INSPYRE ("post-INSPYRE") against two pins from the SUPERFACT-1 fast reactor irradiation experiment. The post-INSPYRE simulation results are compared to the available integral and local data from post-irradiation examinations, and benchmarked on the evolution during irradiation of quantities of engineering interest (e.g., fuel central temperature, fission gas release). The comparison with the pre-INSPYRE results is reported to evaluate the impact of the novel models on the predicted pin performance. The outcome represents a step forward towards the description of fuel behaviour in fast reactor irradiation conditions, and allows the identification of the main remaining gaps.

• Tuberculosis and Respiratory Diseases
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• 제86권3호
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• pp.234-244
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• 2023

Background: Effective treatment of fluoroquinolone-resistant multidrug-resistant tuberculosis (FQr-MDR-TB) is difficult because of the limited number of available core anti-TB drugs and high rates of resistance to anti-TB drugs other than FQs. However, few studies have examined anti-TB drugs that are effective in treating patients with FQr-MDR-TB in a real-world setting. Methods: The impact of anti-TB drug use on treatment outcomes in patients with pulmonary FQr-MDR-TB was retrospectively evaluated using a nationwide integrated TB database (Korean Tuberculosis and Post-Tuberculosis). Data from 2011 to 2017 were included. Results: The study population consisted of 1,082 patients with FQr-MDR-TB. The overall treatment outcomes were as follows: treatment success (69.7%), death (13.7%), lost to follow-up or not evaluated (12.8%), and treatment failure (3.9%). On a propensity-score-matched multivariate logistic regression analysis, the use of bedaquiline (BDQ), linezolid (LZD), levofloxacin (LFX), cycloserine (CS), ethambutol (EMB), pyrazinamide, kanamycin (KM), prothionamide (PTO), and para-aminosalicylic acid against susceptible strains increased the treatment success rate (vs. unfavorable outcomes). The use of LFX, CS, EMB, and PTO against susceptible strains decreased the mortality (vs. treatment success). Conclusion: A therapeutic regimen guided by drug-susceptibility testing can improve the treatment of patients with pulmonary FQr-MDR-TB. In addition to core anti-TB drugs, such as BDQ and LZD, treatment of susceptible strains with later-generation FQs and KM may be beneficial for FQr-MDR-TB patients with limited treatment options.

• Pediatric Infection and Vaccine
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• 제31권1호
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• pp.25-36
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• 2024

목적: 테트라사이클린 (tetracycline, TC)은 소아청소년을 대상으로 복용하였을 시 영구적 치아 변색의 위험이 증가된다는 보고에 따라 미국에서는 8세 이하, 국내에서는 12세 미만으로 처방이 가이드라인상 추천되지 않고 있는 실정이다. 이에 본 연구에서는 TC에 노출된 소아청소년을 대상으로 치아 변색의 발생률을 분석하고, TC에 노출되지 않은 일반 인구 집단과의 발생률 차이를 비교하고자 하였다. 방법: 본 코호트 연구는 2008년 1월부터 2020년 12월 사이 최소 1일 이상 TC에 노출된 소아청소년(0-12세)에 대한 건강보험심사평가원 데이터베이스 정보를 기반으로 분석하였다. TC 노출 6개월 이후 치아 변색 관련 진단코드의 입력여부를 기준으로 치아 변색 발생률을 도출하였고, 추가적으로 연령 보정이 된 TC에 노출되지 않은 일반 인구집단을 추출하여 이를 변수로 한 표준화한 치아 변색 발병률(standardized incidence ratio, SIR)을 구하였다. 결과: 총 56,990명이 포함되었으며, 이 중 8세 미만은 1,735명, 그리고 8-12세는 55,255명이었다. 이 중 61%가 14일 미만 동안 TC를 처방받았으며, 독시사이클린(61%)과 미노사이클린(35%)을 포함한 2세대 TC가 가장 많은 비중을 차지하였다. 0-7세 연령군에서의 5년 및 10년 누적발생률은 4.1% (95% confidence interval [CI], 3.0% to 5.7%) 및 5.7% (95% CI, 4.1% to 7.8%)으로 확인되었고, 이에 비해 8-12세 연령군에서는 0.8% (95% CI, 0.7 to 0.9%) 및1.3 (95% CI, 1.1% to 1.4%)으로 상대적으로 낮았다. TC노출 후 치아 변색의 발생률은 연령 보정된 일반 인구 집단과 비교하였을 때 통계학적으로 유의미한 차이는 없었다 (SIR, 1.08; 95% CI, 0.69 to 1.60). 결론: TC 노출은 일반 인구에 비해 치아 변색 위험을 유의하게 증가시키지 않았으며, 특히 8-12세 사이의 TC 노출군은 그 이하 연령군에 비해 유의미하게 낮은 발생률을 보였다. 따라서, 국내에서 TC 처방에 대한 연령 제한 완화를 고려할 필요가 있다.

• 한국안전학회지
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• 제31권5호
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• pp.22-27
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• 2016

The Electrostatic Charge Prevention Technology is a core factor that highly influences the yield of Ultra High Resolution Flat Panel Display and high-integrated semiconductor manufacturing processes. The corona or x-ray ionizations are commonly used in order to eliminate static charges during manufacturing processes. To develop such a revolutionary x-ray ionizer that is free of x-ray radiation and has function to control the volume of ion formation simultaneously is a goal of this research and it absolutely overcomes the current risks of x-ray ionization. Under the International Commission on Radiological Protection, it must have a leakage radiation level that should be lower than a recommended level that is $1{\mu}Sv/hour$. In this research, the new generation of x-ray ionizer can easily control both the volume of ion formation and the leakage radiation level at the same time. In the research, the test constraints were set and the descriptions are as below; First, In order not to leak x-ray radiation while testing, the shielding box was fully installed around the test equipment area. Second, Implement the metallic Ring Electrode along a tube window and applied zero to ${\pm}8kV$ with respect to manage the positive and negative ions formation. Lastly, the ion duty ratio was able to be controlled in different test set-ups along with a free x-ray leakage through the metallic Ring Electrode. In the result of experiment, the maximum x-ray radiation leakage was $0.2{\mu}Sv/h$. These outcome is lower than the ICRP 103 recommended value, which is $1{\mu}Sv/h$. When applying voltage to the metallic ring electrode, the positive decay time was 2.18s at the distance of 300 mm and its slope was 0.272. In addition, the negative decay time was 2.1s at the distance of 300 mm and its slope was 0.262. At the distance of 200 mm, the positive decay time was 2.29s and its slope was 0.286. The negative decay time was 2.35s and its slope was 0.293. At the distance of 100 mm, the positive decay time was 2.71s and its slope was 0.338. The negative decay time was 3.07s and its slope was 0.383. According to these research, the observation was shown that these new concept of ionizer is able to minimize the leakage radiation level and to control the positive and negative ion duty ratio while ionization.

• 대한한의학원전학회지
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• 제18권4호통권31호
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• pp.1-14
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• 2005

Zhu Dan Xi's name is ZhenHeng(震亨) and was also called by the title of YanXiu(彦修). Early in his life, he started to study JuZiYe(擧子業), and went on to study DaoDeXingMingXue(道德性命學) under the teachings of XueQian(許謙), who as one of fourth generation disciple of ZhuZi(朱子) was teaching in BaHuaShan(八華山). His well-known literary works are ${\ulcorner}$JuFangFaHui(局方發揮)${\lrcorner}$, ${\ulcorner}$GeZhiYuLun${\lrcorner}$, ${\ulcorner}$ShangHanBianYi(傷寒辨疑)${\lrcorner}$, ${\ulcorner}$BenCaoYanYiBuYi(本草衍義補遺)${\lrcorner}$, ${\ulcorner}$WaiKeJingYaoXinLun(外科精要新論)${\lrcorner}$. Zhu Dan Xi learnt the studies of Liu(劉), Zhang(張), Li(李) from LouZhiTi(羅知悌) and adopted the advantages and abolished disadvantages from it. The southern district being low and damp, which also leads to a geographical condition with a lot of ShiReXiangHuo(濕熱相火) disease and with the social background of people exhausting their QingYu(情欲) and damaging QLXie(氣血), he came out with the theory of 'YangYouYuYinBuZu(陽有餘陰不足)', 'XiangHuo(相火)' and became a well renowned expert in diagnosis and treatment of QiXieTanYuHuo(氣血痰鬱火). As a result, the writer has performed a research based on Liu's works and related theories, GuWuZhiZhi theory, the understanding of TaiJiZhiLi(太極之理), the inner meaning of YinYang and YouYuBuZu(redundancy-and-deficit), YinYangDongJingGuan, physiology and pathology, the medical reason of lust damaging QingYuYangYin and YangSheng(養生)(preservation of health), which are the main medical theory of ZhuDanXi, comments of later generations and is reporting the outcome.

• Asian Pacific Journal of Cancer Prevention
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• 제16권18호
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• pp.8503-8512
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• 2016

The purpose of this study was to provide a detailed explanation of the mechanism of bisanthracycline, WP 631 in comparison to doxorubicin (DOX), a first generation anthracycline, currently the most widely used pharmaceutical in clinical oncology. Experiments were performed in SKOV-3 ovarian cancer cells which are otherwise resistant to standard drugs such as cis-platinum and adriamycin. As attention was focused on the ability of WP 631 to induce apoptosis, this was examined using a double staining method with Annexin V and propidium iodide probes, with measurement of the level of intracellular calcium ions and cytosolic cytochrome c. The western blotting technique was performed to confirm PARP cleavage. We also investigated the involvement of caspase activation and DNA degradation (comet assay and immunocytochemical detection of phosphorylated H2AX histones) in the development of apoptotic events. WP 631 demonstrated significantly higher effectiveness as a pro-apoptotic drug than DOX. This was evident in the higher levels of markers of apoptosis, such as the externalization of phosphatidylserine and the elevated level of cytochrome c. An extension of incubation time led to an increase in intracellular calcium levels after treatment with DOX. Lower changes in the calcium content were associated with the influence of WP 631. DOX led to the activation of all tested caspases, 8, 9 and 3, whereas WP 631 only induced an increase in caspase 8 activity after 24h of treatment and consequently led to the cleavage of PARP. The lack of active caspase 3 had no outcome on the single and double-stranded DNA breaks. The obtained results show that WP 631 was considerably more genotoxic towards the investigated cell line than DOX. This effect was especially visible after longer times of incubation. The above detailed studies indicate that WP 631 generates early apoptosis and cell death independent of caspase-3, detected at relatively late time points. The observed differences in the mechanisms of the action of WP631 and DOX suggest that this bisanthracycline can be an effective alternative in ovarian cancer treatment.

• 방송공학회논문지
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• 제19권3호
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• pp.362-371
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• 2014

카메라 초점에 의해 발생하는 흐림(blur)의 변화는 깊이값을 측정하는데 사용한다. DFD(Depth from Defocus)는 깊이값과 흐림의 비례 관계를 이용하여 흐림의 양을 측정하는 기술이다. 기존 DFD 방법은 입력으로 두 장의 비초점 영상(defocused image)을 사용하는데, 기술적인 문제로 낮은 품질의 복원된 초점 영상(infocused image)과 깊이맵을 얻고 있다. 상기 문제점을 해결하는 방법으로 초점영상과 비초점 영상을 이용함으로써 복원된 초점 영상의 품질 저하를 해결한다. 제안 방법에서는 Subbaro가 제안한 DFD 방법에 새로운 에지 흐림 측정 방법을 결합하여 보다 정확한 흐림 값을 구한다. 또한 명암의 변화가 적은 영역에서는 흐림의 양을 측정하기가 어렵기 때문에, 관심맵(saliency)을 이용하여 비에지 영역을 채울 수 있도록 하였다. 실험에서는 초점 조절 기능이 있는 카메라로부터 20장의 2K FHD 해상도의 초점 및 비초점 영상을 생성한 후에 제안 방법을 이용하여 깊이맵을 생성하고, 마지막으로 입력 초점 영상과 깊이맵으로부터 3D 입체영상을 제작하였다. 3D 모니터로 시청한 결과 안정된 3D 공간감과 입체감을 얻을 수 있었다.

• 한국응용약물학회:학술대회논문집
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• 한국응용약물학회 2001년도 추계학술대회 및 정기총회
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• pp.3-6
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• 2001

Inflammation is an outcome or an end effect of disruption of complex immunological balance. A variety of approaches to control immunological unbalance have been tried, and some of them are in practice in the clinic. Since inflammatory disorders are reflection of very complex immunological responses, it should be difficult to have such disorders under complete control. Thus, most of the drugs, being marketed and under development, possess some degrees of undesired side offsets originating from disruption of immunological balance. Steroids are excellent drugs suppressing inflammation in short term, however, long-term use of steroids would incur a serious side effect of "rebound". Another example is TNF-${\alpha}$-neutralizing agents, such as enbrel and infliximab. TNF-${\alpha}$ has been known to play a key role in the exacerbation of inflammation, and knock-out of TNF-${\alpha}$ is regarded essential to control of chronic inflammation. The TNF-${\alpha}$-neutralizing drugs in the market are regarded very efficient in the management of rheumatoid arthritis. Upon long term use, however, those drugs cause sepsis to a certain proportion of patients. It is ironical that a high plasma level of TNF-${\alpha}$ is known to be responsible for sepsis, and that the drugs scavenging TNF-${\alpha}$ cause sepsis. The above two examples illustrate well the difficulty of discovering an anti-inflammatory drug without unwanted immunological side effects. An anti-inflammatory drug would make a case in the market, as long as the drug has huge therapeutic benefits compared to its expected but unwanted immunological side effects, where cyclooxygenase-2 inhibitors are positioning. In this presentation, will be discussed general aspects of cyclooxygenase-2 inhibition in conjunction with 3(2Η)furanone derivatives, a novel class of COX-2 inhibitors.

• 한국발생생물학회지:발생과생식
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• 제21권2호
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• pp.121-130
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• 2017

4-Nonylphenol (NP) is a surfactant that is a well-known and widespread estrogenic endocrine disrupting chemical (EDC). Although it has been known that the affinity of NP to ERs is low, it has been suggested that low-dose NP has toxicity. In the present study, the endocrine disrupting effects on reproduction, and the weight of gonads, epididymis, and uterus were evaluated with the chronic lower-dose NP exposing. This study was designed by following the OECD test guideline 443 and subjected to a complete necropsy. In male, NP had an effect on the weight of the testis and epididymis in both $F_0$ and $F_1$. In females, NP decreased the weight of ovary and uterus in $F_0$ but not in pre-pubertal $F_1$ pubs. Fertility of male and female in $F_0$ or $F_1$ was no related with NP administration. The number of caudal-epididymal sperm by body weight (BW) was not different between groups in both $F_0$ and $F_1$. Besides, the difference of the sperm number between generations was not detected. The number of ovulated oocytes was similar between groups in $F_0$, but significantly decreased in NP 50 group of $F_1$. The litter size and sex ratios of offspring in $F_1$ and $F_2$ were not different. The accumulated mating rate and gestation period were not affected by the NP administration. Those results shows that chronic lower-dose NP administration has an effect of endocrine disruptor on the weight of gonads and epididymis of $F_0$ and $F_1$ but not in reproduction. Based on the results, it is suggested that chronic lower-dose NP exposing causes endocrine disruption in the weight of gonad and epididymis but not in the reproductive ability of next generations.