• Journal of mucopolysaccharidosis and rare diseases
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• 제4권1호
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• pp.7-10
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• 2018

Rare diseases are life threatening or chronically debilitating diseases with a low prevalence (less than 2,000 people in a population), which includes lysosomal storage diseases. These diseases are often seen as unimportant especially in developing countries, such as Indonesia, due to small number of patients. National Rare Disease Center in Indonesia was pioneered almost 20 years ago and officially established in 2017 by the Indonesian Minister of Health. Lysosomal storage disease become the most commonly found inborn errors of metabolism (IEM) in Indonesia due to easily accessible diagnostic facilities. Currently there are 7 patients receiving ERT in this mixed-donation scheme, one patient with Gaucher disease and 6 patients with MPS type II. Few challenges for ERT in Indonesia include importation through special access scheme, preparation of ERT infusion in intensive care settting, and cost of treatment. Even with limited resources, healthcare professionals in Indonesia have been giving the best care possible for rare disease patients, especially to provide diagnostic facilities through collaboration and treatment options for treatable rare diseases. Improvements in care for rare disease patients are still needed.

• Journal of mucopolysaccharidosis and rare diseases
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• 제3권2호
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• pp.37-40
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• 2017

Mucopolysaccharidosis (MPS) is caused by accumulation of the glycosaminoglycans in all tissues due to decreased activity of the lysosomal enzyme. Patients exhibit multisystemic signs and symptoms in a chronic and progressive manner, especially with changes in the skeleton, cardiopulmonary system, central nervous system, cornea, skin, liver, and spleen. In the past, treatment of MPS was limited to enzyme replacement therapy (ERT). The outcome for affected patients improved with the introduction of new technologies as hematopoietic stem cell transplantation, relegated to specific situations after ERT became available. Intrathecal ERT may be considered in situations of high neurosurgical risk but still it is experimental in humans. New insights on the pathophysiology of MPS disorders are leading to alternative therapeutic approaches, as gene therapy, inflammatory response modulators and substrate reduction therapy. In this paper, we will highlight the recent novel treatment and clinical trials for MPS and discuss with the goal of fostering an understanding of this field.

• 한국기계가공학회지
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• 제19권10호
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• pp.87-97
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• 2020

In general, tires require various sensors and power supply devices, such as batteries, to obtain information such as pressure, temperature, acceleration, and the friction coefficient between the tire and the road in real time. However, these sensors have a size limitation because they are mounted on a tire, and their batteries have limited usability due to short replacement cycles, leading to additional replacement costs. Therefore, vibration energy harvesting technology, which converts the dynamic strain energy generated from the tire into electrical energy and then stores the energy in a power supply, is advantageous. In this study, the output voltage and power generated from piezoelectric elements are predicted through finite element analysis under static state and transient state conditions, taking into account the dynamic characteristics of tires. First, the tire and piezoelectric elements are created as a finite element model and then the natural frequency and mode shapes are identified through modal analysis. Next, in the static state, with the piezoelectric element attached to the inside of the tire, the voltage distribution at the contact surface between the tire and the road is examined. Lastly, in the transient state, with the tire rotating at the speeds of 30 km/h and 50 km/h, the output voltage and power characteristics of the piezoelectric elements attached to four locations inside the tire are evaluated.

• Advances in concrete construction
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• 제11권3호
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• pp.183-192
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• 2021

Due to economic and environmental benefits, increasing the substitution ratio of ordinary cement by industry by-products like fly ash (FA) is one of the best approaches to reduce the impact of the concrete industry on the environment. However, as the substitution rate of FA increases, it will have an adverse impact on the performance of cement-based materials, so the actual substitution rate of FA is limited to around 10-30%. Therefore, in order to increase the early-age strength of high replacement (30-70%) low-calcium ultrafine FA blended cement paste, sodium sulfate and calcium sulfate dihydrate were used to improve the reactivity of FA. The results show that sodium sulfate has a significant enhancement effect on the strength of the composite pastes in the early and late ages, while calcium sulfate dihydrate has only a slight effect in the late ages. The addition of sodium sulfate in the cement-FA blended system can enhance the gain rate of non-evaporation water, and can decrease the Ca(OH)2 content. In addition, when the sulfate chemical activators are added, the ettringite content increases, and the surface of the FA is dissolved and hydrated.

• 대한물리의학회지
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• 제18권2호
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• pp.93-101
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• 2023

PURPOSE: The Western Ontario and McMaster Universities Arthritis (WOMAC) index has been used to measure the outcome of total knee replacement (TKR), but studies on its reliability and validity are limited. The present study examined the reliability and validity of this index for patients with knee osteoarthritis who underwent TKR. METHODS: Seventy-one inpatients and outpatients who underwent bilateral TKR for knee osteoarthritis were included in this study. The pain assessment scale and WOMAC index were used to evaluate the participants every two weeks to examine the test-retest reliability, internal consistency, and construct validity. RESULTS: The test-retest reliability scores for pain, stiffness, and physical function were .75-.92, .85-.90, and .75-.95, respectively. The corresponding intraclass correlation coefficients were .75-.88, .76-.88, and .71-.95, respectively. The internal consistency score in the first and second examinations was .92. Furthermore, the construct validity scores for pain, stiffness, and physical function were .83, .41, and .58, respectively. CONCLUSION: The application of the WOMAC index in patients who underwent TKR showed high test-retest reliability and internal consistency with the use of the WOMAC index and good validity with the use of the pain assessment scale.

• 한국프랜차이즈경영연구
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• 제6권1호
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• pp.1-20
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• 2015

Home Meal Replacement (HMR) products are ready-to-eat or pre-cooked food products that are consumed at daily home. HMR market has grown rapidly due to societal changes: increases in female social activities, silver population, and one-person households. Consumption channels of HMR can be classified into take-out, delivery, and retail. In Korean HMR market, retail sector is largely growing, but companies are focusing their business on the home delivery sector. Moreover, franchise companies are expanding their areal coverage in the HMR market based on their multi-unit strategy. However, more research on the HMR market is needed as existing studies are limited in conceptualization, classification, and processed food from malls or home-shopping channels. Therefore, we conducted the efficiency analysis on Gukseonsaeng, one of franchises that applied the take-out channel, using DEA method. According to the research on 29 franchisees of Gukseonsaeng, 77.9% of input appeared inefficient for technical efficiency, while 53.3% of input appeared inefficient for scale efficiency. Thus, we found that franchises of Gukseonsaeng are structured in increasing returns to scale (IRS), so enhancing efficiency by expanding scales need to be implemented.

• Journal of Chest Surgery
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• 제56권6호
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• pp.452-455
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• 2023

Patients who have undergone mechanical valve replacement require anticoagulation therapy with warfarin to prevent thromboembolism. However, administering warfarin to pregnant patients increases their risk of warfarin embryopathy or central nervous system disorders. Consequently, safer alternatives, such as heparin or low-molecular-weight heparin injection, are substituted for warfarin. However, limited research has been conducted on this subject, with no large-scale studies and particularly few investigations involving multiparous patients. A patient who had previously undergone mechanical mitral valve replacement for atrial septal defect and mitral stenosis received anticoagulant therapy with enoxaparin during 2 pregnancies. Upon confirmation of pregnancy, warfarin was replaced with subcutaneously injected enoxaparin with a dosage of 1 mg/kg at 12-hour intervals. The enoxaparin dosage was controlled using an anti-factor Xa assay, with a target range of 0.3-0.7 IU/mL. Intravenous heparin injections were administered starting 3 days prior to the expected delivery date and were continued until delivery, after which warfarin was resumed. No complications were observed during the deliveries.

• 지질공학
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• 제13권3호
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• pp.345-358
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• 2003

근래에 와서 천연골재자원의 보호와 건설 폐기물의 재활용의 측면에서 폐 콘크리트에서 분리한 재생골재의 재활용이 모색되어 왔으나, 재생골재는 주로 콘크리트 도로기층용과 구조물 됫채움재와 같은 저급재료로 사용되고 있다. 본 연구는 재생골재의 콘크리트 용 골재로의 효율적인 재활용 가능성을 위하여, 재생골재의 구성광물, 화학조성 및 공학적 물성 특성 파악하고 재생콘크리트(RCA)의 시공성과 역학적 특성에 대한 연구를 수행하였다. 재생골재에 잔존하는 폐 콘크리트의 모르타르 및 시멘트 페이스트는 골재의 기초 물성 특성, 재생콘크리트의 시공성 및 굳은 콘크리트의 공학적 특성에 주요한 영항을 미치는 것으로 나타났다. 그러나, 적절한 양의 재생굵은골재를 대체한 재생콘크리트는 압축강도와 동결융해 내구성에서 보통 콘크리트의 기준에 근접하는 것으로 나타났다 따라서, 재생골재의 제조과정에서 폐 콘크리트의 모르타르 및 시멘트 페이스트의 제거가 효율적으로 이루어지고 적절한 대체율이 적용되면, 재생골재는 콘크리트용 골재로서 효율적인 사용이 확대 될 수 있을 것으로 사료된다.

• Journal of Microbiology and Biotechnology
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• 제19권9호
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• pp.1070-1076
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• 2009

Telomerase reverse transcriptase (TERT), which prolongs the replicative life span of cells, is highly upregulated in 85-90% of human cancers, whereas most normal somatic tissues in humans express limited levels of the telomerase activity. Therefore, TERT has been a potential target for anticancer therapy. Recently, we described a new approach to human cancer gene therapy, which is based on the group I intron of Tetrahymena thermophila. This ribozyme can specifically mediate RNA replacement of human TERT (hTERT) transcript with a new transcript harboring anticancer activity through a trans-splicing reaction, resulting in selective regression of hTERT-positive cancer cells. However, to validate the therapeutic potential of the ribozyme in animal models, ribozymes targeting inherent transcripts of the animal should be developed. In this study, we developed a Tetrahymena-based trans-splicing ribozyme that can specifically target and replace the mouse TERT (mTERT) RNA. This ribozyme can trigger transgene activity not only also in mTERT-expressing cells but hTERT-positive cancer cells. Importantly, the ribozyme could selectively induce activity of the suicide gene, a herpes simplex virus thymidine kinase gene, in cancer cells expressing the TERT RNA and thereby specifically hamper the survival of these cells when treated with ganciclovir. The mTERT-targeting ribozyme will be useful for evaluation of the RNA replacement approach as a cancer gene therapeutic tool in the mouse model with syngeneic tumors.

• 중환자간호학회지
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• 제9권1호
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• pp.40-50
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• 2016

Purpose: Continuous renal replacement therapy (CRRT) has become the preferred dialysis method to support critically ill children and neonates with acute kidney injury. Using CRRT on neonates has increased, but reports about experience are limited. The aim of this study is to describe the clinical application, outcomes, and complications of CRRT in children and neonates. Methods: A retrospective review was performed in 135 children and 36 neonates who underwent CRRT at a tertiary hospital from 2008 to 2015. Results: At the initiation of CRRT, the median age of children was 72 months and the corrected age of neonates was 37.1 weeks. Median body weight of neonates was 3.2 kg. In neonates, initial degree of fluid overload [FO%], blood flow rate [BFR] and ultrafiltration rate [UFR] rate during CRRT were higher than in children. Median real time of CRRT was 90.5 and 53.5 hours in children and neonates, respectively. Downtime of CRRT was 0.7 and 1.3 hours/day. Median mortality rates (44.4% vs.47.2%) and complication rates were similar between the groups. Conclusion: CRRT can be used for a wide range of critically ill children and neonates. Different application methods of CRRT can contribute to increased survival of neonates.