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Clinical Spectrum and Short-term Effects of Enzyme Replacement Therapy for Mucopolysaccharidosis Type II (제2형 뮤코다당증의 임상적 스펙트럼과 효소대치요법의 단기간 효과)

  • Cheon, Chong Kun;Hwu, Wuh-Liang
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.18 no.3
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    • pp.78-86
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    • 2018
  • Purpose: We aimed to delineate clinical spectrum and short-term effects after enzyme replacement therapy (ERT) for 5 mucopolysaccharidosis type II (MPS II). Methods: Five patients were diagnosed with MPS II by clinical findings, enzyme activity, and genetic testing. Idursulfase was administered by intravenous infusion at a dose of 0.5 mg/kg every week. Observational chart analysis of patients, who underwent systematic investigations more than 12 months after initiation of ERT was done retrospectively. Results: Three patients were classified as having the attenuated type, and 2 patients were classified as having the severe type. The median age at the diagnosis was 9.6 years (range 3.4-26 years). Four different mutations in 5 Korean patients (4 families) with MPS II were identified, among which two were novel mutations (1 small insertion mutation: p.Thr409Hisfs*22, and 1 missense mutation: p.Gly134Glu). Two severe type sibling patients with the same mutation had different clinical manifestation. Urinary glycosaminoglycan excretion decreased within the twelve months of ERT (P=0.043). Liver and spleen volumes showed reductions that were maintained in all patients (P=0.043 and P=0.043, respectively). Improvements were also noted in left ventricular mass index (P=0.042), shoulder flexion (P=0.043), shoulder abduction (P=0.039), knee flexion (P=0.043), elbow flexion (P=0.042), and respiratory distress index (P=0.041). Conclusion: This study demonstrates that Korean patients with MPS II are clinically heterogeneous and indicates that idursulfase is relatively effective in several clinical parameters including heart size and respiratory distress index without infusion-related reactions in patients with MPS II.

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Exposure and Risk Assessments of Multimedia of Arsenic in the Environment (환경 중 비소의 매체통합 노출평가 및 위해성평가 연구)

  • Sim, Ki-Tae;Kim, Dong-Hoon;Lee, Jaewoo;Lee, Chae-Hong;Park, Soyeon;Seok, Kwang-Seol;Kim, Younghee
    • Journal of Environmental Impact Assessment
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    • v.28 no.2
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    • pp.152-168
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    • 2019
  • The element arsenic, which is abundant in the Earth's crust, is used for various industrial purposes including materials for disease treatment and household goods. Various human activities, such as the disposal of soil waste, metal mining and smelting, and combustion of fossil fuels, have caused the pollution of the environment with arsenic. Recently, guidelines for arsenic in rice have been adopted by the Korean ministry of food and drug safety to prevent health risks based on rice consumption. Because of the exposure to arsenic and its accumulation in the human body through various channels, such as air inhalation, skin contact, ingestion of drinking water, and food consumption, integrated multimedia risk assessment is required to adopt appropriate risk management policies. Therefore, integrated human health risk assessment was carried out in this study using integrated exposure assessment based on multimedia (e.g., air, water, and soil) and multi-route (e.g., oral, inhalation, and dermal) scenarios. The results show that oral uptake via drinking water is the most common pathway of arsenic into the human body, accounting for 57%-96% of the total arsenic exposure. Among various age groups, the highest exposures to arsenic were observed in infants because the body weight of infants is low and the surface areas of infant bodies are large. Based on the results of the exposure assessment, the cancer and non-cancer risks were calculated. The cancer risk for CTE and RME is in the range of 2.3E-05 to 6.7E-05 and thus is negligible because it does not exceed the cancer probability of 1.0E-04 for all age groups. On the other hand, the cancer risk for RME varies from 6.4E-05 to 1.8E-04 and from 1.3E-04 to 1.8E-04 for infants and preschool children, exceeding the excess cancer risk of 1.0E-04. The non-cancer risks range from 5.4E-02 to 1.9E-01 and from 1.5E-01 to 6.8E-01, respectively. They do not exceed the hazard index 1 for all scenarios and all ages.

The Life Experiences of the Deaf Elderly (농아노인의 생활 경험)

  • Park, Ina;Hwang, YoungHee;Kim, Hanho
    • 한국노년학
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    • v.36 no.3
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    • pp.525-540
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    • 2016
  • The purpose of this study was to investigate what kind of experiences the deaf elderly would have in the course of life. It also aimed to promote the understanding of their living difficulties and culture among people with normal hearing and provide basic data to help them live with others as members of the community. Phenomenological qualitative research was conducted as part of the methodology. The subjects include seven deaf old people. Based on the results of in-depth interview and analysis, the life experiences of the deaf elderly were categorized into "unforgettable wounds," "life in the community," "life with the family," "marriage of the deaf elderly", and "living by adjusting to reality." First, the subcategories of "unforgettable wounds" include "receiving no treatment for fever," "damage by the Korean War," "alienation from the family," and "people's cold eyes." It turned out that the deaf elderly had led a life, suffering from the heart wounds that they could not forget. Second, the subcategories of "life in the community" include "inconvenience in life," "disadvantages in life," and "severed life." The deaf elderly were not only subjected to inconvenience and disadvantages in life, but also suffered loneliness, being cut off from the community. Third, the subcategories of "life with the family" include "not communicating with children," "being abandoned again," "being used by the family," "being lonely even with the family," and "wishing to live independently from the family." The deaf elderly were not supported by their families and were abandoned or used by them, leading a solitary life. Fourth, the subcategories of "marriage of the deaf elderly" include"send as a surrogate mother," "frequent remarriage and divorce," "lean on as a married couple." Deaf elderly form their own culture of the marriage and lean on each other. Finally, the subcategories of "living by adjusting to reality" include "getting help from neighbors," "behaving oneself right in life," "learning Hangul," "living by working," "living freely," "living by missing," and "controlling the impulse to end life," "resorting to religion." The deaf elderly made the most alienated and vulnerable group with no access to benefits due to their limitations as a linguistic and social minority, but they made efforts to form their own culture and adjust to reality for themselves. Based on those findings, the study made the following proposals: first, there is a need for practical approaches to heal the ineffaceable wounds in the hearts of deaf elderly. Second, there is a need for policies to help them experience no inconvenience and disadvantages as members of community and communicate with people with normal hearing. Third, there should be practical approaches to enable them to get recognition and support from their families and share love with them. Finally, there should be practical policy approaches to help people with normal hearing understand the culture of deaf elderly and assist the deaf elderly to receive supports from the community and live with others within the community.

Changes in Korean Consumers' Perception on Food Preservatives by a Risk Communication Booklet

  • Kim, Suna;Kim, Ji-Sun;Kang, Hee-Jin;Lee, Gunyoung;Lim, Ho Soo;Yun, Sang Soon;Kim, Jeong-Weon
    • Journal of Food Hygiene and Safety
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    • v.33 no.6
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    • pp.417-426
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    • 2018
  • Food preservatives are very important food additives for the biological and chemical safety of processed foods. The purposes of this study were to investigate Korean consumer's perception and information needs on food preservatives, to develop an educational booklet as a risk communication material on food preservatives, and to assess the educational effect of the developed booklet. To understand perception on food preservatives, a self-administered questionnaire survey was conducted by 381 parents having elementary school students at Seoul and Geoynggi area in Korea. Based on the survey results, brain storming of the authors along with consultation from the professionals, we developed a risk communication booklet about food preservatives. It was exposed to 35 parents of elementary school children, and their evaluation was collected by using a questionnaire and analyzed statistically. Respondents considered food safety (44.8%) as the most important factor while purchasing processed foods. They still perceived food additives as the most hazardous one (41.5%), and among those, food preservatives were the most concerned (45.9%). Total 67.7% of the respondents considered the consumption of food preservatives as hazardous or very hazardous. However, 90.6% of respondents did not have any educational experience about food additives and food preservatives. Based on their information needs, a science-based booklet consisting of the definition, classification, safety, intake, and management of food preservatives was developed. When the booklet titled as 'Food preservatives, Just Know Them!' was exposed to the parents via elementary school teacher, their negative perceptions on food additives and food preservatives were changed positively by increasing the understanding level on preservatives from 18.9% to 90.9% and obtaining 72.7% positive answers on their safety. Therefore, it could be used as an effective risk communication material on food preservatives.

Palliative Care Practitioners' Perception toward Pediatric Palliative Care in the Republic of Korea (소아완화의료에 대한 호스피스 완화의료 전문기관 종사자의 인식)

  • Moon, Yi Ji;Shin, Hee Young;Kim, Min Sun;Song, In Gyu;Kim, Cho Hee;Yu, Juyoun;Park, Hye Yoon
    • Journal of Hospice and Palliative Care
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    • v.22 no.1
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    • pp.39-47
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    • 2019
  • Purpose: This study was performed to investigate the current status of pediatric palliative care provision and how it is perceived by the palliative care experts. Methods: A descriptive study was conducted with 61 hospice institutions. From September through October 2017, a questionnaire was completed by experts from the participating institutions. Data were analyzed using SPSS 21.0. Results: Among 61 institutions, palliative care is currently provided for pediatric cancer patients by 11 institutions (18.0%), all of which are concentrated in Seoul, Incheon and Gyeonggi and Gyengsang provinces; 85.2% of all do not plan to provide specialized pediatric palliative care in the future. According to the experts, the main barriers in providing pediatric palliative care were the insufficient number of trained specialists regardless of the delivery type. Experts said that it was appropriate to intervene when children were diagnosed with cancer that was less likely to be cured (33.7%) and to move to palliative care institutions when their conditions worsened (38.2%); and it was necessary to establish a specialized pediatric palliative care system, independent from the existing institutions for adult patients (73.8%). Conclusion: It is necessary to develop an education program to establish a nationwide pediatric palliative care centers. Pediatric palliative care intervention should be provided upon diagnosis rather than at the point of death. Patients should be transferred to palliative care institutions after intervention by their existing pediatric palliative care team at the hospital is started.

Glomerular Filtration Rate Test Methods and Guidelines (Glomerular Filtration Rate 검사방법 및 가이드라인)

  • Park, Min-Ho;Lee, Ha-Young;Ryu, Hwa-Jin;Yoo, Tae-Min;Noh, Gyeong-Woon
    • The Korean Journal of Nuclear Medicine Technology
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    • v.22 no.2
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    • pp.97-100
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    • 2018
  • Purpose The glomerular filtration rate (GFR) test is an important indicator of glomerular filtration and has been used to test renal function and the extent of its function. The GFR test is performed by intravenous injection of radioactive medicines made of $^{51}Cr$-EDTA, and blood concentration is measured by taking blood according to the elapsed time. also, PET-CT, bone scan, transfusion and so on will affect the outcome. Therefore, we will improve the quality of the test by providing guidelines for the GFR test for more accurate testing. Materials and Methods 5 mL of physiological saline solution and 2 mL of $^{51}Cr$-EDTA solution are used to make 5 mL of the radiopharmaceutical solution to be injected into the patient. First, the syringe weight is measured before the injection, and then the radioactive medicine is injected into the patient's vein and the syringe weight is measured after the injection. Blood sampling is performed twice in total. In adults, blood is collected 3 hours / 5 hours after injection and in children 2 hours / 5 hours after injection. The blood sample is centrifuged at 3300 rpm for 5 minutes. Standard solution is prepared by filling diluent water up to the scale indicated in the 200-mL volumetric flask, discarding $500{\mu}L$, injecting $500{\mu}L$ of GFR reagent and mixing well. $500{\mu}L$ each of the standard solution is dispensed into two test tubes, and $500{\mu}L$ of each of the plasma samples collected in time is dispensed into two test tubes and measured with a Cobra Counter. Results At present, the reference range applied in this study is $119.5{\pm}30.3ml/min/1.73m2$ for males and $125.2{\pm}28.2ml/min/1.73m^2$ for females. Conclusion The GFR test is conducted using radioactive medical products. GFR testing is performed as a scheduled test, but PET-CT, dialysis and transfusion, which may affect GFR testing, may be scheduled during GFR testing. Therefore, we could get accurate GFR test results by notifying the ward and department beforehand when booking.

Changes in Occupational Therapy Students' Occupational Balance and Quality of Life in Epidemic of COVID-19 (COVID-19 유행으로 인한 작업치료(학)과 학생들의 작업균형과 삶의 질 변화)

  • Lee, Hyang-sook;Han, Gyeong-ju;Park, In-yeong;Hwang, Eun-bi;Chae, Hyun-ah;Noh, Chong-su;Cha, Jung-jin
    • The Journal of Korean society of community based occupational therapy
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    • v.11 no.1
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    • pp.11-22
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    • 2021
  • Objective : The purpose of this study was to investigate the changes in occupational balance and quality of life caused by COVID-19 in occupational therapy students. Methods : From May 27 to June 26, 2020, questionnaires were distributed to a total of 35 universities among 62 occupational therapy departments nationwide. General characteristics, COVID-19 related characteristics, OBQ and WHOQOL-BREF were used to evaluate and analyze occupational balance and quality of life. The SPSS/PC 24.0 program was used to analyze frequency analysis, crossover analysis, chi-square test, independent t-test, analysis of variance, and Pearson correlation analysis. Results : There were significant differences in school system(years), class, life pattern, quality of life, personal and public schedule depending on whether they are interested in occupational balance. There were significant differences in occupational balance(OBQ) and quality of life(WHOQOL-BREF), 'Hobby', 'new hobbies after COVID-19', 'life patterns', 'use of public transportation', 'maintenance of occupational balance', and 'quality of life'. There was a significant positive correlation occupational balance and quality of life. Conclusion : This study showed that the more people who have changed their lives due to COVID-19 are interested in work balance, and the better they maintain their work balance and emotional well-being, the higher the work balance and quality of life, and the positive correlation between work balance and quality of life was confirmed. This will be the basis for studies related to intervention strategies that can improve occupational balance and quality of life in a time when social isolation is easy due to the COVID-19 epidemic.

A Comparison of Body Shape Changes Between Deep Tissue Massage and Illite-Combined Deep Tissue Massage - Focusing on women in their 30s - (딥티슈마사지와 일라이트병행 딥티슈마사지의 체형변화 비교 -30대 여성을 대상으로-)

  • Jeong, In-Sun;Park, Jeong-Yeon
    • Journal of Korea Entertainment Industry Association
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    • v.14 no.6
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    • pp.279-287
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    • 2020
  • This study aims to put forth an efficient way of improving body shapes by examining the effects of deep tissue massage and illite-combined deep tissue massage on body shape changes, and identifying body shape changes when applying each method. This study targeted twenty women in their thirties, and ten separate subjects were placed in different groups. Then deep tissue massage and illite-combined deep tissue massage were performed once a week, for a total of eight weeks. Moire Topography was applied before the experiments, four weeks later and eight weeks later to compare changes in spinous process inclination, shoulders and hips. The data collected were analyzed using SPSS v. 21.0, and the study results are as follows. In relation to general characteristics of the subjects, professionals occupied the highest proportion of them, and 90% of them were married. Here, 77.8% of them had experience in giving birth, and 78.6% of them chose natural birth. In addition, 57.1% of the subjects holding a majority had two children. When measuring spinous process inclination, shoulders and hips in the illite-combined deep tissue massage group and in the deep tissue massage group before the experiments, the illite-combined deep tissue massage group showed somewhat higher values in every area than the deep tissue massage group, but no statistically significant differences were not found, which means the homogeneity existed between them. When comparing body shape changes between the two massage methods, there were significant differences(p<.05, p<.01), because the illite-combined deep tissue massage group showed a much higher decline in spinous process inclination, shoulders and hips than the deep tissue massage group. This implies illite-combined deep tissue massage was more effective in improving body shapes than deep tissue massage. Therefore, illite-combined deep tissue massage is considered to be helpful in improving body shapes, and it is anticipated that this massage method can be used in relevant fields, including the skin care industry.

From Frankenstein to Torture Porn -Monstrous Technology and the Horror Film (프랑켄슈타인에서 고문 포르노까지 -괴물화하는 테크놀로지와 호러영화)

  • Chung, Young-Kwon
    • Journal of Popular Narrative
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    • v.26 no.1
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    • pp.243-277
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    • 2020
  • This paper examines a social and cultural history of horror films through the keyword "technology", focusing on The Spark of Fear: Technology, Society and the Horror Film (2015) written by Brian N. Duchaney. Science fiction film is closely connected with technology in film genres. On the other hand, horror films have been explained in terms of nature/supernatural. In this regard, The Spark of Fear, which accounts for horror film history as (re)actions to the development of technology, is remarkable. Early horror films which were produced under the influence of gothic novels reflected the fear of technology that had been caused by industrial capitalism. For example, in the film Frankenstein (1931), an angry crowd of people lynch the "monster", the creature of technology. This is the action which is aroused by the fear of technology. Furthermore, this mob behavior is suggestive of an uprising of people who have been alienated by industrial capitalism during the Great Depression. In science fiction horror films, which appeared in the post-war boom, the "other" that manifests as aliens is the entity that destroys the value of prosperity during post-war America. While this prosperity is closely related to the life of the middle class in accordance with the suburbanization, the people live conformist lives under the mantle of technologies such as the TV, refrigerator, etc. In the age of the Vietnam War, horror films demonize children, the counter-culture generation against a backdrop of the house that is the place of isolation and confinement. In this place, horror arises from the absolute absence of technology. While media such as videos, internet, and smartphones have reinforced interconnectedness with the outside world since the 1980s, it became another outside influence that we cannot control. "Found-footage" and "torture porn" which were rife in post-9/11 horror films show that the technologies of voyeurism/surveillance and exposure/exhibitionism are near to saturation. In this way, The Spark of Fear provides an opportune insight into the present day in which the expectation and fear of the progress of technology are increasingly becoming inseparable from our daily lives.

An Qualitative Study on Correctional institution Counselors' Perception of Ex-Offender's Experience regarding Reintegration into Family (수감자의 출소 후 가족복귀 경험에 관한 교정기관 상담자의 인식)

  • Dong Hun Lee ;Su Eun Kang ;Seung Hee Jee
    • Korean Journal of Culture and Social Issue
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    • v.22 no.4
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    • pp.595-622
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    • 2016
  • This study aims to understand the process of family reunion of the ex-offenders. To this end, Korea Rehabilitation Agency under Ministry of Justice and Healthy Family Support Center conducted intensive interviews with ex-offenders, their families and with 8 counselors who are in charge of ex-offenders and their families' residential, psychological, and educational support. The data collected through the interviews were analyzed by Consensus Qualitative Research(COR). The followings are the results: the counselors found out that most of ex-offenders had experienced unhappy childhood which was lack of healthy relationship with their parents. Secondly, counselors noticed a common feature among the families of ex-offenders. The common feature was that they keep the fact that one of their parents was imprisoned to their children as a secret. Thirdly, through the data analysis, counselors could understand various factors that affect reunion of ex-offenders' families: the factors that helped successful reunion were ex-offenders' sense of responsibility, open and healthy communication among family members, and mutual understanding of being a good family member, whereas, irresponsible dependance to other family members, denier and avoidance from the family members against ex-offenders, and lost sense of being a family member were the factors that discouraged the reunion. It turned out that the kinds of crime that ex-offenders committed also affected family reunion. The processes of reunion were easier for those who served their time with fraud, embezzlement, whereas, it was much more challenging for those who served their time with rape, violence, or murder. Fourthly, counselors learned that "relaxation" is the key factor in the process of reunion of ex-offenders' families. They also emphasized that there should be thorough monitoring process before the intervention in the reunion process. This study contributes in terms of finding healthy ways of intervention with ex-offenders' families and developing programs that help ex-offenders to recover their relationship with their family.