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Enzyme Replacement Therapy in Patients Who Have Mucopolysaccharidosis and Are younger than 5 years old (5세 미만 뮤코다당체침착증 환자에서의 효소 대체 요법)

  • Park, Seong-Won;Son, Yeong-Bae;Kim, Se-Hwa;Jo, Seong-Yun;Ji, Seon-Tae;Jin, Dong-Gyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.10 no.1
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    • pp.59-66
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    • 2010
  • Enzyme replacement of therapy (ERT) is one of the most promising therapeutic strategies for the treatment of lysosomal storage disorders. ERT is available in three types of Mucopolysaccharidosis (MPS): for MPS I (Aludrazyme$^{(R)}$), MPS II (Elaprase$^{(R)}$) and MPS VI (Naglazyme$^{(R)}$) patients who are over 5 years old. But recently, early diagnosis can be done by expert clinicians and even in prenatal case. We describe the case of ERT under 5 years old MPS patients. Up to June, 2010 in Samsung Medical Center, there are 6patients who were diagnosed as MPS and started ERT under 5 years old. 3 patients were MPS I, 3 patients were MPS II. 2 patient who was diagnosed as MPS I was female and others were male. Their age at diagnosis were 4 to 37month-old (4, 13, 16, 25, 27, 37 month-old) and they are now 9 to 60 month-old (9, 39, 32, 81, 60 month-old). The youngest patient was started ERT at 4 month-old and others were started at their 13 to 49 month-old (13, 29, 27, 28, 49 month-old). First manifested symptoms of patients were macrocephaly, kyphosis and coarse face appearance. Especially, in 2 of them, one was MPS I and the other was MPS II had elder brother with same disease. And the youngest one was diagnosed by the iduronate-2-sulfatase (IDS) gene analysis from chorionic villi sampling. His mother knew that she was a heterozygous carrier of IDS gene mutation because her younger brother died from MPS II. All of them confirmed as MPS by the enzyme assay in leukocytes and fibroblast skin culture. We started ERT with ${\alpha}$-L-iduronidase(Aldurazyme$^{(R)}$) to MPS I and did recombinant human iduronate-2-sulfatase (Elaprase$^{(R)}$) to MPS II patients as recommended dose as over 5 years old. But for MPS II patient who was 4 month old, we started ERT by recombinant human IDS (Elaprase$^{(R)}$) with reduced dose 0.1 mg/kg and increased dose every 2 weeks by 0.1mg/kg up to 0.5mg/kg IV infusion. During ERT, all patients had no adverse effects and the excretion of GAGs were decreased. We have evaluated other clinical symptoms such as liver/ spleen volume, heart function and neurologic evaluation. We describe a successful ERT to MPS I and MPS II patient under 5 years old without any adverse event. It indicates that ERT in young children are well tolerated and that it has several effects which may confer clinical benefits with long-term therapy.

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EFFECT OF POLYPHOSPHATE IN ROOT CANAL SEALERS ON THE GROWTH OF ORAL BACTERIA (Polyphosphate가 함유된 근관충전재가 구강세균의 성장에 미치는 영향)

  • 박석범;최기운;최호영
    • Restorative Dentistry and Endodontics
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    • v.26 no.2
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    • pp.141-152
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    • 2001
  • Eliminating the infecting bacteria of the root canal system and preventing reinfection must be the main objectives of all endodontic works. None of commercially available root canal sealers have the properties of desirable tissue compatibility and strong antibacterial activity. The purpose of this study is to develope an ideal root canal sealer using commercially available polyphosphate (polyP), Calgon, which is known to be antibacterial and safe. For the study. resin type AH26, zinc oxide eugenol type Tubli Seal. Ca(OH)$_2$ type Apexit as base sealers for polyP (0~3%) and para formaldehyde containing N2 as a control base were selected. Specimens (3$\times$4mm) of the sealers were prepared in a 37$^{\circ}C$ incubator for 3 and 10 days and their antibacterial activity against streptococci and black pigmented anaerobic rods was observed using an agar diffusion method. The result were as follows: 1. Among 3 day old root canal sealers. N2 as a positive control showed the strongest antibacterial effect. followed by AH26. Tubli Seal and. Apexit which barely showed antibacterial activity against the test bacteria. In contrast. 10 day old AH26 showed a greater antibacterial activity than 10 day old N2. 2. All sealer specimens showed a greater antibacterial activity against black pigmented anaerobic rods than streptococci. Three day old ones appeared to be more antibacterial than 10 day old ones except for Apexit. 3. As compared to N2, 3 day old AH26 demonstrated a similar antibacterial activity against black pig mented anaerobic rods but to a lesser extent to streptococci. Ten day old AH26 showed a greater antibacterial activity against black pigmented anaerobic rods than 10 day old N2. 4. As compared to AH26. Tubli Seal generally revealed a lower antibacterial activity but it showed a greater antibacterial activity aginst S. gordonii Challis. 5. Enhancement of antibacterial activity by polyP was more clearly observed when it was added to Ca(OH)$^{\circ}C$ based root canal sealers. Tubli Seal and N2. 6. The addition of polyP enhanced the antibacterial activity of 3 day old AH26 against S. gordonii G9B (16%) and Challis (29%), and P. gingivalis 2561 (24%) only. Moreover, polyP failed to increase antibacterial activity of 10 day old AH26 against the test strains but P. gingivalis A7A1 28(13%). 7. The addition of polyP increased the antibacterial effect of 3 day old Tubli Seal on several test bacteria including s. mutans GS 5 (50%). s. gordonii G9B (47%) and Challis (122%). and all the test strains of P. gingivalis (13~35%) except for 9 14K 1. The addition of polyP to 10 day old Tubli Seal increased antibacterial activity of the root canal sealer against most test strains. 8. 3 day old Apexit failed to show antibacterial activity. if any very little against S. mutans GS 5 and Pr. intermedia ATCC 49046. However. polyP increased its antibacterial activity by 50 and 69%, respectively. Increase of antibacterial activity of 10 day old Apexit by polyP was more clearly observed than that of 3 day old one.

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The effects of Social Capital of old-old elderly of more than 70-year-old on their health-related quality of life (70세 이상 후기노인의 사회적자본이 건강관련 삶의 질에 미치는 영향)

  • Kim, Kyung-Hee;Lee, Sung-Kook;Yoon, Hee-Jung;Kwon, Gi-Hong
    • Journal of the Korea Academia-Industrial cooperation Society
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    • v.16 no.6
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    • pp.3889-3901
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    • 2015
  • This study was examined the effects of social capital influenced on health related quality of life of old-old-elderly of more than 70-year-old. The subjects were 258 people of old-old-elderly of more than 70-year-old who dwell in M city. The first, October 15 to November 15, 2013, the second, December 1 to 30, 2014 were surveyed. The data were analyzed with SPSS Statistics 18.0 and the structural equation modeling of AMOS 21.0. The Sobel test was conducted to analyze the statistical significance of the mediated effects. The factors of health-related quality of life were influenced significantly by gender, education level, whether or not exercise, amount of sleeping time, family can be a help request, relatives can be a help request, general trust. In the results of the test of the research model, social capital was found to directly affect health behavior, and health behavior was found to directly affect health related quality of life. Therefore, social capital was found to indirectly affect health related quality of life. Social capital of old-old elderly of more than 70-year-old can be recognized as one of the factors influencing their health related quality of life. The results suggest that policy should be established to increasing the level of social capital and improved the health related quality of life.

Selecting Appropriate Seedling Age for Restoration Using Comparative Analysis of Physiological Characteristics by Age in Abies koreana Wilson

  • Seo, Han-Na;Chae, Seung-Beom;Lim, Hyo-In;Han, Sim-Hee;Lee, Kiwoong
    • Journal of Forest and Environmental Science
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    • v.37 no.4
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    • pp.315-322
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    • 2021
  • The aim of this study was to investigate the sensitivity to environmental stress, and changes in the photosynthesis capacity in Abies koreana seedlings by age and to suggest the most effective age for restoration. To identify these physiological characteristics of A. koreana, the chlorophyll fluorescence and photosynthetic capacity of 1-, 2-, 3-, 5- and 6-year-old A. koreana seedlings were observed from June 2020 to June 2021. The maximum quantum efficiency of Photosystem II (Fv/Fm), a chlorophyll fluorescence measurement parameter, was strongly positively correlated with the monthly average temperature (1-year-old seedling: r=0.8779, 2-year-old seedling: r=0.8605, 3-year-old seedling: r=0.8697, 5-year-old seedlings: r=0.8085, and 6-year-old seedlings: r=0.8316). The Fv/Fm values were the lowest in winter (November 2020-March 2021). In addition, the Fv/Fm values of 1-, 2-, and 3-year-old seedlings in winter were lower than that of 5- and 6-year-old seedlings, while the Fv/Fm values in summer were relatively higher than those in winter. Further, the Fv/Fm values of seedlings of all ages decreased in August 2020, when the monthly average temperature was the highest. In particular, 1-year-old to 3-year-old seedlings showed Fv/Fm values less than 0.8. Further, the photosynthetic capacity measured in August 2020 increased with increasing seedling age. The analysis of variance results for summer Fv/Fm values showed significant differences in age-specific averages (p<0.05), and Duncan's multiple range test showed significant differences between 5- and 6-year-old seedlings and 1-, 2-, and 3-year-old seedlings (p<0.05). These results suggested that the 5- and 6-year-old seedlings were less sensitive to environmental stress and showed better photosynthetic capacity than the 1-, 2-, and 3-year-old seedlings. Therefore, 5-year-old or older A. koreana seedlings can be used as restoration materials because they can show increased adaptability and stable growth during transplantation due to their relatively high environmental resistance and photosynthetic capacity.

Factors Relating Quality of Sleep: Comparison between Young-old People and Old-old People (수면의 질 영향 요인: 전기노인과 후기노인 비교)

  • Seo, Yeong-Mi;Kim, Jeong-Sook;Je, Nam Joo
    • Journal of the Korea Academia-Industrial cooperation Society
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    • v.20 no.6
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    • pp.332-341
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    • 2019
  • This study is a descriptive survey study to investigate the quality of sleep and to compare the factors relating quality of sleep in the young-old people and the old-old people. The subjects of the study were 200 elderly people who live in the G-region applying convenient expression method. Using a structured questionnaire, sociodemographic characteristics, lifestyle and health-related characteristics, depression, and quality of sleep were examined. We analyzed ${\chi}^2-test$, t-test, ANOVA(scheffe's test), Pearson's correlation coefficient and multiple regression using IBM SPSS WIN/21.0. The results of the study were as follows. The quality of sleep was significantly worse in the old-old people than in the young-old people. In young-old people, only depression (r=-.22, p=.038) showed a significant negative correlation with sleep quality. Old-old people was a significant negative correlation between sleep quality and depression (r=-.19, p=.045) and subjective health status (r=-.29, p=.002). In the young-old people, job, chronic illness, and economic level were significant influencing factors in the quality of sleep. In the old-old people, chronic disease, subjective health status, and smoking were significant influencing factors. The results of this study can be used as basic data for establishing tailored strategy for ages that can improve health of the elderly.

A Study on the Current Status and Utilization of Old Map in Library and Museums in Korea (국내 도서관·박물관 소장 고지도의 현황 및 활용에 관한 연구)

  • Gi Young Kim
    • Journal of the Korean BIBLIA Society for library and Information Science
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    • v.35 no.1
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    • pp.97-125
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    • 2024
  • The purpose of this study is to increase access to information on old maps and to discuss efficient ways to utilize old maps, such as providing services and information using old maps. To this end, the information search system of domestic institutions that provide old map information was investigated, and methods of searching for old map data and accessing information were searched on the website. In addition, the current status of the collection of old maps in domestic libraries and museums was analyzed by referring to the homepage, book, research book, and publication of each institution. As a result of the analysis, about 2,200 old maps were housed in 76 institutions, including national, public, and university libraries and museums nationwide. Each institution in the collection of old maps was carrying out publication business, such as publication of English manuscripts, exhibitions and books, publication of research document edits such as lists and summaries. However, reading and using of original documents are limited due to the rare nature of old maps and the data characteristics of the only one. In order to effectively utilize old maps, first, it is necessary to improve access to old map information services and expand academic information services. Second, it is proposed to use old maps as data for archival construction that reflects the identity of the region. Third, it is necessary to cultivate professional manpower who selects and provides information based on knowledge of old map data and humanities literacy.

Research and Analysis of Enactment of China's Old and Rare Books Management (중국의 고문헌 관리 법제화의 조사 분석)

  • Han, Mikyung
    • Journal of the Korean Society for Library and Information Science
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    • v.52 no.3
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    • pp.193-214
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    • 2018
  • This study aims to analyze enactment of China's old and rare books management. For the purpose, the review on China's legal system and management related to old and rare books were studied. And this study investigated 4 laws, 6 administrative regulations, 4 sector regulations, and 13 administrative normativity papers. Among those, 5 major sources for old and rare books management were identified as follows : 1) China's National Civilization Protection Law in 2013, 2) China's Old and Rare Book Organization Directive in 1989, 3) Regulation on Management of Research, Organization, and Publication of Old and Rare Books in 1986, 4) Standards on Grading Classification of Civilization Collections in 2001, and 5) Provisional Regulation on Civilization Auctioning in 2003. Based on the analysis of these sources, this study deduced implications in terms of Chinese old and rare books management such as the necessity to give considerations to 1) the importance of old and rare books management policy, 2) nationwide old and rare books management, 3) expansion of eduction and training of human resources, 4) establishment of institution specializing in old and rare books, and 5) special management of science of Chinese medicine.

Study on the Difference in the Level of Preparation for Old Age and Happiness according to the General Characteristics of Senior Welfare Center Attendees.

  • Seo, Jung-Sook;Cho, Sung-Je
    • The Journal of Korea Institute of Information, Electronics, and Communication Technology
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    • v.10 no.4
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    • pp.279-287
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    • 2017
  • The aim of this study is to verify the difference in the level of happiness and preparation for old age according to the general characteristics of senior welfare center attendees. The subjects of the study were 148 attendees of OO senior welfare center in Seoul and a survey was conducted from July 4 to October 7, 2016. The method of analysis was by using the SPSSWIN 18.0 program, a 5% level of significance was verified in a frequency analysis, t-test, one-way ANOVA, correlation analysis, and multiple regression analysis. The results showed that first, from investigating the difference in the level of preparation for old age according to general characteristics, there was a significant difference according to age, occupation, monthly income, and physical condition but there was no significant difference according to gender and level of education. Second, as a result of observing the difference in the level of happiness according to general characteristics, although there was a significant difference according to occupation and monthly income, the difference level according to gender, age, level of education, health condition was less than a 5% level of significance. Third, from observing the correlation between the level of happiness and preparation of old age, it was found that happiness was positively correlated to preparation for old age in the following order: physical preparation for old age ((r=.576, p<.001), economic preparation for old age (r=.570, p<.001), psychological preparation for old age (r=.526, p<.001), social preparation for old age (r=.525, p<.001). It is forecasted that this study will be used as base line data for improvement of happiness and preparation for old age for the elderly according to general characteristics.

Methodology of Field Investigation and Laboratory Test for Distresses of Old Concrete Pavements (노후 콘크리트 포장 파손에 대한 현장조사 및 실내시험 방법)

  • Lee, Ki Sang;Lee, Jun Hyeok;Kang, Min Soo;Cho, Nam Hyun;Jeong, Jin Hoon
    • International Journal of Highway Engineering
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    • v.18 no.5
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    • pp.21-29
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    • 2016
  • PURPOSES : The purpose of this study is to suggest a specific investigation guideline to decide priority of repairing old concrete pavements that pile up substantially. METHODS : In this study, a principle of division of homogeneous sections was proposed to reflect the characteristics of the pavement reasonably in the specific investigation results. In addition, a checklist and guideline of field investigation were suggested for the old concrete pavement sections, which require inspection toward their durability and structural performance. Furthermore, the items of laboratory test necessary to the old concrete pavement were suggested based on the existing laboratory test considering characteristics of the old concrete pavement. The present condition of the old concrete pavement could be analyzed by the test results. RESULTS : A method of division of homogeneous sections suitable for the specific investigation of the old concrete pavement was suggested. The proportions of distress severity of pavement sections were compared by distress type to figure out the present state of the old concrete pavement. Scaling, durability cracking (or alkali-silica reaction), and longitudinal spalling were selected as the most severe distress types. The detailed positions of the sections were also suggested. The checklist of the specific investigation was categorized by field survey and laboratory test, and its evaluation criteria were proposed. The three types of the sections of durability cracking (or alkali-silica reaction), bridge connection, and asphalt overlay were selected as the sections of the field survey. The compressive strength, void structure, and chloride penetration depth were suggested as the items of the laboratory test. CONCLUSIONS : A fundamental level of the guideline was suggested in this study to resolve the problem of old concrete pavement. Appropriate guidelines related to the repair of the old concrete pavement should be provided by performing additional research efforts.

The Effects of Children's Sex, Age and Parental Feeding Styles on Children's Self-help Skills (유아의 성별과 연령 및 부모의 식사지도유형이 유아의 자조기술에 미치는 영향)

  • Lee, Jung-Min;Kim, Jin-Kyung
    • Journal of Families and Better Life
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    • v.28 no.1
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    • pp.37-49
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    • 2010
  • This study investigated the effects of three factors - children's age, sex and parental feeding styles - on children's self-help skills. The subjects were 213 children and their parents who lived in Kwanak-ku, Seoul. The three-way analysis of variance was employed. The main results were as follows: 1. Children's autonomy was varied significantly according to their sex. Girls do better by themselves than boys do in toileting, washing and drying hands, putting on and buttoning up a coat, putting on clothes, washing and drying their faces. Children's autonomy also varied significantly according to age. A majority of the autonomy subsets increased as the children's ages increased. However, drying their hands with a towel, removing their coats, and drinking water did not vary by age. 2. Children's autonomy also varied significantly according to their parents' feeding style. A responsive feeding style encouraged children to drink water by themselves. 3. The group with the highest score in self-help skills in toileting was 6-year-old girls, and the group with the lowest score was 3-year-old boys. Additionally, the groups with the highest scores in toileting were 4-year-old girls whose parents demonstrated a neglective feeding style, 5-year-old girls whose parents demonstrated an authoritative feeding style, 5-year-old girls whose parents demonstrated a responsive feeding style, 3-year-old boys whose parents demonstrated a responsive feeding style and 6-year-old boys whose parents demonstrated a neglective feeding style. Conversely, the groups with the lowest scores in toileting were 3-year-old girls whose parents demonstrated an authoritarian feeding style, 5-year-old girls whose parents demonstrated a neglective feeding style, and 3-year-old and 6-year-old boys whose parents demonstrated an authoritarian feeding style. 4. The group with the highest score in removing their coats was girls whose parnets demonstrated authoritative, responsive or neglective feeding styles. The group with the lowest score in removing their coats was boys whose parents demonstrated authoritative or neglective feeding styles.