• Journal of mucopolysaccharidosis and rare diseases
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• 제3권1호
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• pp.1-8
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• 2017

Mucopolysaccharidosis (MPS) type III, or Sanfilippo syndrome is a rare autosomal recessive lysosomal storage disorder. It is caused by a deficiency of one of four enzymes involved in the degradation of the glycosaminoglycan (GAG) heparan sulfate. The resultant cellular accumulation of heparan sulfate causes various clinical manifestations. MPS III is divided into four subtypes depending on the deficient enzyme: MPS IIIA, MPS IIIB, MPS IIIC and MPS IIID. All the subtypes show similar clinical features and are characterized by progressive degeneration of the central nervous system (CNS). Main purpose of the treatment for MPS III is to prevent neurologic deterioration. However, conventional enzyme replacement therapy has a limitation due to inability to cross the blood-brain barrier. Several experimental treatment options for MPS III are being developed.

• 한국소음진동공학회논문집
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• 제25권1호
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• pp.58-64
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• 2015

There are a lot of different types of noise from construction site and it depends on construction stage, equipment type and number of operating equipments. The problem is that the almost noise from construction site is high level and some kinds of noise levels are over 100 dB. In addition, if construction site is located in downtown, there are a few options to reduce noise level. Therefore several site mitigation plans were developed to reduce different types of construction noises such as breaker work, gang form and asphalt saw. They were applied in site and evaluated noise reduction performance. The test result of site insertion loss was between 4~8 dB depends on the equipment and type of work.

• 한국통계학회:학술대회논문집
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• 한국통계학회 2002년도 추계 학술발표회 논문집
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• pp.191-196
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• 2002

Sales of equity-indexed annuities (EIAs) have rapidly increased, but the growth rates in sales have recently shown signs of slowing down because the current volatile equity market increases the costs of guarantees in EIAs. New EIAs need to be designed that are similar to existing EIAs but have a cheaper guarantee and a higher participation rate. This paper proposes three types of EIAs with higher participation rates: up-and-in barrier EIA, aulual reset EIA with up-and-in barriers, and partial-time lookbackEIA. It also presents a probability distribution and the method of Esscher transforms, with which explicit pricing formulas for these EIAs are derived.

• Journal of mucopolysaccharidosis and rare diseases
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• 제4권2호
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• pp.37-41
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• 2018

Mucopolysaccharidosis III (MPS III, Sanfilippo syndrome) is a rare autosomal recessive disease caused by a deficiency of one of four enzymes involved in the degradation of glycosaminoglycan (GAG). The resultant cellular accumulation of GAG causes various clinical manifestations. MPS III is divided into four subtypes depending on the deficient enzyme. All the subtypes show similar clinical features and are characterized by progressive degeneration of the central nervous system. A number of genetic and biochemical diagnostic methods have been developed. However, there is no effective therapy available for any form of MPS III, with treatment currently limited to clinical management of neurological symptoms. Main purpose of the treatment for MPS III is to prevent neurologic deterioration. Because conventional intravenous enzyme replacement therapy (ERT) has a limitation due to inability to cross the blood-brain barrier, several innovative therapeutic approaches for MPS III are being developed. This review covers the currently developing new therapeutic options for MPS III including high dose ERT, substrate reduction therapy, intrathecal or intraventricular ERT, fusion protein delivery using bioengineering technology, and gene therapy.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제49권4호
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• pp.218-222
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• 2023

Re-ankylosis is a common postoperative complication of temporomandibular joint (TMJ) ankylosis surgery. Various surgical options to prevent re-ankylosis, both with and without interpositional material, have been discussed in the literature. However, no standardized protocol has been suggested for management or prevention of TMJ ankylosis. This paper discusses the probable causes behind TMJ re-ankylosis and presents a case of unilateral TMJ re-ankylosis, which was managed by gap arthroplasty using an autologous abdominal dermal fat graft as an interpositional material and closely monitored for signs of relapse. Autologous fat graft acted as an effective barrier between the glenoid fossa and mandibular condyle, thus preventing dead space, hematoma and heterotrophic bone formation. A brief review of the literature and update on TMJ re-ankylosis are also presented.

• 생명과학회지
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• 제33권9호
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• pp.754-765
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• 2023

엑소좀은 단백질, mRNA 및 miRNA를 포함하고 모든 유형의 세포에서 분비되는 세포 외 소포의 일종이다. 방출된 엑소좀은 인접하거나 멀리 있는 다른 세포에 의해 선택적으로 흡수되어 그 내용물을 방출하고 표적 세포를 재프로그래밍한다. 엑소좀은 세포에 의해 생성되는 작은 천연 소포이므로 무독성과 비면역원성의 특징이 있는 것으로 받아들여지고 있다. 최근에는 엑소좀이 중추신경계에 대한 약물 전달체로 과학적 관심을 받고 있다. 중추신경계에는 약물의 침투를 어렵게 하는 혈뇌장벽이 있고 이는 퇴행성신경질환의 치료제 개발에 큰 걸림돌이 되어왔다. 그러나 축적된 연구결과들을 볼 때, 엑소좀이 주로 트랜스사이토시스를 통해 혈뇌장벽을 통과할 수 있음이 제시되었다. 이러한 결과를 종합하면, 엑소좀은 혈뇌장벽을 넘어 뇌 실질조직에 약물을 전달할 수 있는 새로운 전달 수단이 될 것으로 기대된다. 또한 세포의 종류와 질병상태에 따라 분비되는 엑소좀의 종류가 다르기 때문에 엑소좀은 중추신경계 질환의 진단을 위한 바이오마커로도 활용될 수 있다. 본 총설 논문에서는 중추신경계 질환에 대한 바이오마커 및 치료 옵션으로서의 임상시험을 포함한 엑소좀에 대한 최근 연구동향을 정리하였다.

• 한국지반환경공학회 논문집
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• 제11권8호
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• pp.15-23
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• 2010

토석류는 사면 붕괴, 산사태, 홍수 등과 같은 인명 및 재산피해를 수반하는 자연재해의 일종이다. 2006년 7월 집중호우로 인하여 대규모 토석류가 발생하였으며, 이에 따라 고속국도, 일반국도, 철도 등의 국가 기간망이 통제되어 언론에 보도되는 사태가 발생하곤 하였다. 토석류에 대한 연구는 지금까지 충실히 수행되지 않은 것이 사실이다. 원인으로는 예측이 매우 어려웠을 뿐 아니라, 발생위치가 산지에서 주로 발생하였으며 도심지 및 주택가에서 발생하지 않아 국민들에게 부각되지 않았던 이유도 있다. 본 연구에서는 토석류를 방지하기 위하여 사방댐 등의 대책방안이 수립되어 적용되어 오고 있으나, 단점 및 보완점을 개발하여 새로운 기법을 적용하였다. 또한, 계곡부의 특성 및 형상에 따라 다단계의 유연성 시설물을 설치함하여 인명 및 재산피해를 최소화 할 수 있도록 고안하였다. 대상지역을 2개소 선정하여 적용성에 대한 평가를 실시하고 결과를 제시하였다. 연구결과, Site A, B 지역에 토석류 방지시설이 각각 17개와 13개가 설치요구되었다. 향후 토석류에 대한 체계적인 지침 및 방안에 대하여 정립해 나갈 필요가 있을 것으로 생각된다.

• 대한두개안면성형외과학회지
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• 제15권1호
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• pp.14-21
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• 2014

Background: There are several options for replacement of the dermal layer in fullthickness skin defects. In this study, we present the surgical outcomes of reconstruction using acellular dermal substitutes by means of objective and subjective scar assessment tools. Methods: We retrospectively reviewed the medical records of 78 patients who had undergone autologous split-thickness skin graft with or without concomitant acellular dermal matrix (CGDerm or AlloDerm) graft. We examined graft survival rate and evaluated postoperative functional skin values. Individual comparisons were performed between the area of skin graft and the surrounding normal skin. Nine months after surgery, we compared the skin qualities of CGDerm graft group (n=25), AlloDerm graft group (n=8) with skin graft only group (n=23) each other using the objective and subjective measurements. Results: The average of graft survival rate was 93% for CGDerm group, 92% for AlloDerm group and 86% for skin graft only group. Comparing CGDerm grafted skin to the surrounding normal skin, mean elasticity, hydration, and skin barrier values were 87%, 86%, and 82%, respectively. AlloDerm grafted skin values were 84%, 85%, and 84%, respectively. There were no statistical differences between the CGDerm and AlloDerm groups with regard to graft survival rate and skin functional analysis values. However, both groups showed more improvement of skin quality than skin graft only group. Conclusion: The new dermal substitute (CGDerm) demonstrated comparable results with regard to elasticity, humidification, and skin barrier effect when compared with conventional dermal substitute (AlloDerm).

• 대한치과의사협회지
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• 제58권6호
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• pp.336-345
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• 2020

Peri-apical implant lesion, also known as 'retrograde peri-implantitis' can occur with multifactorial etiological factors. The purpose of this case report is to demonstrate resolution of periapical implant lesion by removal of causative factors and saving implant by regenerative therapy. A 54-year old male patient with mild dull pain around implant on the right mandibular second premolar area due to persistent peri-apical infection of the adjacent first premolar was treated. Extraction of tooth with symptomatic apical periodontitis and regenerative therapy on the buccal fenestration area of the implant and extraction site were performed. After 6-month reentry, notable regenerated bone tissue around implant was found, and implant placement on the previous extraction site was performed. After 14-month follow-up from the regenerative therapy, neither biological nor mechanical complication could be found around the implant, evidenced by high implant stability, normal clinical probing depth, and absence of discomfort spontaneously and during masticatory function. In conclusion, surgical intervention including regenerative therapy using bone graft and barrier membrane on periapical implant lesion can be suggested as one of the treatment options considering the extent of periapical lesion.

• Nuclear Engineering and Technology
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• 제55권2호
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• pp.530-545
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• 2023

As an alternative to conventional management options for a lot of concrete waste from decommissioning of nuclear power plants, a set of scenarios for controlled recycling of decommissioning concrete waste as engineered barriers of a radioactive waste repository was proposed, and a comprehensive safety assessment model and framework covering both pre-and post-closure phases was newly developed. The new methodology was applied to a reference vault-type repository, and the ratios of derived concentration limits to unconditional clearance levels of eighteen radionuclides for controlled recycling were provided for three sets of dose criteria (0.01, 1, and 20 mSv/y for the pre-closure and 0.01 mSv/y for the post-closure phases). It turns out that decommissioning concrete waste whose concentration is much higher than the unconditional clearance level can be recycled even when the dose criterion 0.01 mSv/y is applied. Moreover, a case study on ABWR bio-shield shows that the fraction of recyclable concrete waste increases significantly by increasing the dose criterion for the radiation worker in the pre-closure phase or the duration of storage prior to recycling. The results of this study are expected to contribute to demonstrating the feasibility of controlled recycling of a lot of decommissioning concrete waste within nuclear sectors.