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Effect of Enzyme Replacement Therapy on Growth in Korean Patients with Mucopolysaccharidosis Type I (I형 뮤코다당증 환자들에서 효소 보충 요법이 성장에 미치는 영향)

  • Huh, Rimm;Cho, Sung Yoon;Chang, Mi Sun;Lee, Jieun;Kwun, Younghee;Kim, Su Jin;Sohn, Young Bae;Park, Sung Won;Maeng, Se Hyun;Kwon, Eun-Kyung;Han, Sun Ju;Jung, Jooyoun;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.13 no.2
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    • pp.111-119
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    • 2013
  • Purpose: Mucopolysaccharidosis type I (MPS I) is a lysosomal storage disorder caused by deficiency of the enzyme ${\alpha}$-L-iduronidase, which leads to a broad spectrum of multisystemic manifestations. Short stature and decreased growth velocity are prominent features of MPS I. The aim of the present study was to evaluate the effect of enzyme replacement therapy (ERT) on growth of Korean MPS I patients from a single center. Methods: Height data were obtained by retrospective chart review of 10 Korean patients with MPS I who had received ERT for a minimum of 3 years. Height was expressed as standard deviation scores (SDS) based on normative data. Annual growth rates were calculated before and during ERT. A piecewise regression model was used to analyze height z-scores before and after treatment. Individual analysis was performed for impact of phenotype [(severe (Hurler) versus attenuated (Hurler-Scheie, Scheie)] on growth. Results: Annual growth was 3.3 cm (z-score= -0.21) in the year before ERT and 6.2 cm (z-score= 0.17), 5.8 cm (z-score= 0.07), and 3.8 cm (z-score= -0.4) in the first, second, and third years of ERT, respectively. Regression analysis showed improvement in the slope after ERT (difference= 0.04; P=0.022). Estimated slope differences between severe and attenuated phenotypes were statistically significant before (P=0.001) and after treatment (P<0.0001), although no significant difference was noted when stratified by phenotype. Conclusion: ERT with aldurazyme appears to have a positive impact on linear growth in patients with MPS I.

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Allergenic potential and enzymatic resistance of buckwheat

  • Lee, Sujin;Han, Youngshin;Do, Jeong-Ryong;Oh, Sangsuk
    • Nutrition Research and Practice
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    • v.7 no.1
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    • pp.3-8
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    • 2013
  • Buckwheat is known as a health food but is one of the major food allergens triggering potentially fatal anaphylaxis in Asia, especially in Japan and Korea. This study was conducted to investigate the characteristic of enzymatic resistance of buckwheat protein and allergenic potential. Enzymatic resistance of buckwheat protein was performed with in vitro digestibility test in simulated gastric fluid (SGF), pH 1.2, using pepsin and simulated intestinal fluid (SIF) using chymotrypsin. Reactivity of buckwheat proteins to human IgE was performed using six allergic patients sensitized to buckwheat. Buckwheat's IgE levels were measured using the Phadia UniCAP-system. Buckwheat protein, 16 kDa, still remained after 30 min treatment of pepsin on SDS-PAGE. Even though 16 kDa almost disappeared after 60 min treatment, two out of the six buckwheat patients' sera showed reactivity to hydrolysate after 60 min treatment, indicating that allergenicity still remained. In simulated intestinal fluid (SIF) using chymotrypsin, buckwheat protein, 24 kDa, showed resistance to hydrolysis with chymotrypsin on SDS-PAGE, and still had allergenicity based on the result of ELISA. Our results suggest that buckwheat proteins have strong resistance to enzyme degradation. This may be attributed in part to the allergenic potential of buckwheat. Further study should be continued regarding buckwheat allergy.

Analysis of Security Vulnerability in U2U Authentication Using MEC in IoD Environment (IoD 환경에서 MEC를 활용한 U2U 인증에서 보안 취약점 분석)

  • Choi, Jae Hyun;Lee, Sang Hoon;Jeong, Ik Rae;Byun, Jin Wook
    • Journal of the Korea Institute of Information Security & Cryptology
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    • v.31 no.1
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    • pp.11-17
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    • 2021
  • Due to the recent development of the Internet of Things (IoT) and the increase in services using drones, research on IoD is actively underway. Drones have limited computational power and storage size, and when communicating between drones, data is exchanged after proper authentication between entities. Drones must be secure from traceability because they contain sensitive information such as location and travel path. In this paper, we point out a fatal security vulnerability that can be caused by the use of pseudonyms and certificates in existing IoD research and propose a solution.

Effects of the Self-help Group Program for Promoting Self-Transcendence in Women with Breast Cancer (유방암 환자의 자기초월감 증진을 위한 자조집단과정 개발 및 운영 효과)

  • Kwon, In-Gak;Park, Eun-Young;Ham, Yun-Hee;Rhoo, Sung-Suk;Lee, Eun-Ok
    • Asian Oncology Nursing
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    • v.1 no.2
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    • pp.217-228
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    • 2001
  • Cancer survivor's self-help group may promote psychosocial adjustment in cancer patients. Self-transcendence has been shown to be related with crisis and adjustment in women with breast cancer. The purpose of this study is to determine the effectiveness of self-transcendence promoting program to facilitate self-transcendence, physical, and emotional well-being in women with breast cancer. Thirty patients who received mastectomy were recruited, but 9 subjects of experimental group and 14 subjects of control group participated in this study. Subjects of experimental group attended the self-help group for self-transcendence once a week for 6 weeks. Each session of program was proceeded for 2hours and the program contained activities for promoting self-transcendence, stress management, communication skill, exercise and relaxation, symptom management, and family support. Data were collected by self-reporting questionnaires within seven days after mastectomy and after the program was finished in experimental group, within 7 days after mastectomy and after 3 months in control group. Self-transcendence was assessed using the self-transcendence scale (STS) developed by Reed. Physical well being was assessed using Symptom distress scale (SDS) developed by McCorkle and Young and emotional well being was assessed using Profile of mood states (POMS). As a result, no significant differences were found between both groups in changes of self-transcendence, emotional well-being, and physical well-being. Self-transcendence score was significantly decreased in control group (p =0.16). But that of experimental group showed no decrease. In conclusions, when the self-transcendence promoting program is provided to the patients with breast cancer, it can be helpful for promoting self- transcendence. For the further study, repeated research with appropriate sample size and more intensive nursing intervention to promote self-transcendence is suggested. And reassessment after 6 months is recommended for testing long term effects of the program.

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On Finding a Convenient Path in the Hierarchical Road Network

  • Sung, Ki-Seok;Park, Chan-Kyoo;Lee, Sang-Wook;Doh, Seung-Yong;Park, Soon-Dal
    • Management Science and Financial Engineering
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    • v.12 no.2
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    • pp.87-110
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    • 2006
  • In a hierarchical road network, all roads can be classified according to their attributes such as speed limit, number of lanes, etc. By splitting the whole road network into the subnetworks of the highlevel and low-level roads, we can reduce the size of the network to be calculated at once, and find a path in the way that drivers usually adopt when searching out a travel route. To exploit the hierarchical property of road networks, we define a convenient path and propose an algorithm for finding convenient paths. We introduce a parameter indicating the driver's tolerance to the difference between the length of a convenient path and that of a shortest convenient path. From this parameter, we can determine how far we have to search for the entering and exiting gateway. We also propose some techniques for reducing the number of pairs of entries and exits to be searched in a road network. A result of the computational experiment on a real road network is given to show the efficiency of the proposed algorithm.

A Dynamic Key Lifetime Change Algorithm for Performance Improvement of Virtual Private Networks (가상사설망의 성능개선을 위한 동적 키 재생성 주기 변경 알고리즘)

  • HAN, Jong-Hoon;LEE, Jung Woo;PARK, Sung Han
    • Journal of the Institute of Electronics Engineers of Korea TC
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    • v.42 no.10 s.340
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    • pp.31-38
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    • 2005
  • Ipsec is a security protocol suite that provides encryption and authentication services for IP messages at the network layer of the internet. Internet Key Exchange (IKE) is a protocol that is used to negotiate and provide authenticated keying materials in a protected manner for Security Associations (SAs). In this paper, we propose a dynamic key lifetime change algorithm for performance enhancement of virtual private networks using IPSec. The proposed algorithm changes the key lifetime according to the number of secure tunnels. The proposed algorithm is implemented with Linux 2.4.18 and FreeS/WAN 1.99. The system employing our proposed algorithm performs better than the original version in terms of network performance and security.

Growth Hormone Therapy in PWS with Mobile Application-Living with PWS

  • Im, Minji
    • Journal of mucopolysaccharidosis and rare diseases
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    • v.4 no.2
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    • pp.42-44
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    • 2018
  • Prader-Willi syndrome (PWS) is a multisystemic complex disorder characterized by hyperphagia and impaired satiety which lead to severe and early obesity. In infancy, hypotonia and poor suck are main problems, and a child goes through Failure-to-thrive. During childhood, clinical manifestations change to food seeking as well as excessive weight gain, short stature, developmental delay, cognitive disability and behavioral problems. Also, growth hormone insufficiency is frequent. Most patients receive the recombinant growth hormone (rGH) therapy that provides improvement in growth, body composition, and physical attributes. The clinical care guideline for rGH therapy in PWS had been noticed in 2013. The rGH therapy helps in body fat, lean body mass, height SDS and head circumference. Also, the rGH therapy helps motor function, psychomotor development and cognition and behavioral issues.In Samsung medical center, there are clinical care guidelines for rGH therapy in PWS and an useful application for the patients. 'Living with PWS', the name of an moblie application for PWS patients, was introduced in the lecture. The application revised to version 2. It was made more convenient to users than in version 1. It helps caregivers to schedule the rGH therapy and to monitor height and weight.

Relations among Security Models for Authenticated Key Exchange

  • Kwon, Jeong Ok;Jeong, Ik Rae
    • ETRI Journal
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    • v.36 no.5
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    • pp.856-864
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    • 2014
  • Usually, key-establishment protocols are suggested in a security model. However, there exist several different security models in the literature defined by their respective security notions. In this paper, we study the relations between the security models of key establishment. For the chosen security models, we first show that some proven key-establishment protocols are not secure in the more restricted security models. We then suggest two compilers by which we can convert a key-establishment protocol that is secure in a specific security model into a key-establishment protocol that is still secure in a more restricted security model.

Robust investment model for long range capacity expansion of chemical processing networks using two-stage algorithm

  • Bok, Jinkwang;Lee, Heeman;Park, Sunwon
    • 제어로봇시스템학회:학술대회논문집
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    • 1997.10a
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    • pp.1758-1761
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    • 1997
  • The problem of long range capacity expansion planing for chemical processing network under uncertain demand forecast secnarios is addressed. This optimization problem involves capactiy expansion timing and sizing of each chemical processing unit to maximize the expected net present value considering the deviation of net present values and the excess capacity over a given time horizon. A multiperiod mixed integer nonlinear programming optimization model that is both solution and modle robust for any realization of demand scenarios is developed using the two-stage stochastic programming algorithm. Two example problems are considered to illustrate the effectiveness of the model.

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A hypercube + + approach for multiblock structured grids (하이퍼큐브 ++를 이용한 다중블록 격자생성)

  • Park, Sang-Geun;Lee, Geon-U
    • Transactions of the Korean Society of Mechanical Engineers B
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    • v.21 no.7
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    • pp.900-910
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    • 1997
  • Multiblock structured grids are, to a large extent, capable of filling up topologically complex flow domains in an efficient way. The proposed approach enables to use different flow models in each different block and the easy incorporation of different grid refinement strategies for different blocks. Furthermore, it may be expected that this multiblock structured approach will naturally lead to the parallel executions of calculations per block on different vector processors. In this paper, the hypercube + + structure is proposed for topological informations on multiblock grids and the B-spline volume for geometrical informations. Three samples of the-three dimensional results are presented to demonstrate the capabilities of the present approach.