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A Study on the Perception of Policy Targets to Improve the Effectiveness of Child Safety Measure - Focusing on Children, Guardians, and Workers in Children's Facilities - (어린이 안전대책 실효성 향상을 위한 정책대상자 인식조사 연구 - 어린이, 보호자, 어린이이용시설 종사자 중심으로 -)

  • ChangYoung Song;WonHoi Koo
    • Journal of the Society of Disaster Information
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    • v.19 no.4
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    • pp.869-881
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    • 2023
  • Purpose: This study aims to come up with improvement measures to improve the effectiveness of child safety measures. Method: The current status of child safety accidents was investigated and implications were deduced by analyzing major child safety measures by government department in the past. In addition, a perception survey was conducted on 1,000 people including children, guardians, and children's facility workers who are subject to child safety policies. Result: Regarding the safety of children's living space(environment), 35.3% of guardians answered that more than 1/3 of them were not safe. Both guardians(95.3%) and children's facility workers(89%) answered that there was the highest risk of 'traffic accidents', and the second risk factor was parents(carelessness of workers at children's facilities) and children's facility workers(careless of guardians at home). Looking at the risks by place, "road and sidewalk" was the most dangerous place and for child safety, guardians(64.3%) and workers (78.3%) both said that the role of "parent" is the most important. For improvements to prevent child safety accidents, the response rate of "strengthening safety management of road traffic facilities" is the most necessary with 75.8% for guardians and 65% for child use facilities. Conclusion: The reinforcement measures to strengthen the effectiveness of child safety measures are as follows. First, in order to ensure the continuity of child safety measures, it should be operated effectively so that those subject to the establishment of the Comprehensive Plan for Child Safety, which took effect in August 2022, can feel it. Second, in order to improve the sensitivity of children's policy targets, promotion measures that take into account the characteristics of each child safety field should be continuously strengthened. Third, it is necessary to expand safety infrastructure for each field to secure child safety. Fourth, it is necessary to strengthen safety education that can ensure safety for children themselves and to come up with detailed measures to make safety education for parents(guardians) mandatory.

Analysis of Traffic Safety Effectiveness of Vehicle Seat-belt Wearing Detection System (주행차량 안전벨트 착용 검지시스템 교통안전 효과 분석)

  • Ji won Park;Su bin Park;Sang cheol Kang;Cheol Oh
    • The Journal of The Korea Institute of Intelligent Transport Systems
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    • v.22 no.5
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    • pp.53-73
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    • 2023
  • Although it is mandatory to wear a seat belt that can minimize human injury when traffic accident occurs, the number of traffic accident casualties not wearing seat belts still accounts for a significant proportion.The seat belt wearing detection system for all seats is a system that identifies whether all seat passengers wear a seat belt and encourages their usage, also it can be a useful technical countermeasure. Firstly, this study established the viability of system implementation by assessing the factors influencing the severity of injuries in traffic accidents through the development of an ordered probit model. Analysis results showed that the use of seat belts has statistically significant effects on the severity of traffic accidents, reducing the probability of death or serious injury by 0.054 times in the event of a traffic accident. Secondly, a meta-analysis was conducted based on prior research related to seat belts and injuries in traffic accidents to estimate the expected reduction in accident severity upon the implementation of the system.The analysis of the effect of accident severity reduction revealed that wearing seat belts would lead to a 63.3% decrease in fatal accidents, with the front seats showing a reduction of 75.7% and the rear seats showing a reduction of 58.1% in fatal accidents. Lastly, Using the results of the meta-analysis and traffic accident statistics, the expected decrease in the number of traffic accident casualties with the implementation of the system was derived to analyze the traffic safety effects of the proposed detection system. The analysis demonstrated that with an increase in the adoption rate of the system, the number of casualties in accidents where seat belts were not worn decreased. Specifically, at a system adoption rate of 60%, it is anticipated that the number of fatalities would decrease by more than three times compared to the current scenario. Based on the analysis results, operational strategies for the system were proposed to increase seat belt usage rates and reduce accident severity.

Exploring Mask Appeal: Vertical vs. Horizontal Fold Flat Masks Using Eye-Tracking (마스크 매력 탐구: 아이트래킹을 활용한 수직 접이형 대 수평 접이형 마스크 비교 분석)

  • Junsik Lee;Nan-Hee Jeong;Ji-Chan Yun;Do-Hyung Park;Se-Bum Park
    • Journal of Intelligence and Information Systems
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    • v.29 no.4
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    • pp.271-286
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    • 2023
  • The global COVID-19 pandemic has transformed face masks from situational accessories to indispensable items in daily life, prompting a shift in public perception and behavior. While the relaxation of mandatory mask-wearing regulations is underway, a significant number of individuals continue to embrace face masks, turning them into a form of personal expression and identity. This phenomenon has given rise to the Fashion Mask industry, characterized by unique designs and colors, experiencing rapid growth in the market. However, existing research on masks is predominantly focused on their efficacy in preventing infection or exploring attitudes during the pandemic, leaving a gap in understanding consumer preferences for mask design. We address this gap by investigating consumer perceptions and preferences for two prevalent mask designs-horizontal fold flat masks and vertical fold flat masks. Through a comprehensive approach involving surveys and eye-tracking experiments, we aim to unravel the subtle differences in how consumers perceive these designs. Our research questions focus on determining which design is more appealing and exploring the reasons behind any observed differences. The study's findings reveal a clear preference for vertical fold flat masks, which are not only preferred but also perceived as unique, sophisticated, three-dimensional, and lively. The eye-tracking analysis provides insights into the visual attention patterns associated with mask designs, highlighting the pivotal role of the fold line in influencing these patterns. This research contributes to the evolving understanding of masks as a fashion statement and provides valuable insights for manufacturers and marketers in the Fashion Mask industry. The results have implications beyond the pandemic, emphasizing the importance of design elements in sustaining consumer interest in face masks.

A study on the distribution basis and aspect of teachers holding additional school health (양호겸직교사의 배치근거 및 분포양상)

  • Lee, Jeong Yim
    • Journal of the Korean Society of School Health
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    • v.2 no.1
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    • pp.58-90
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    • 1989
  • This study was attempted to contribute to the development of school health by providing the basic data about the distribution basis and distribution aspect of teachers holding additional school health that are in charge of school health business in parimary schools, middle schools and high schools without any nurse-teacher. This study analyzed literatures about the history, related laws, organization and professional manpower of school health. The emphasis was set on the distribution basis of theachers holding additional school health. The results of this study are as following: 1. The school health of the world dates to the late 18th century in Europe where was free supplying with food for poor children. The school health of Korea orginated from smallpox vaccination which was executed with appearance of modern schools in the late 19th century. 2. The related laws of school health began as a part of Education Law with was constituted in 1949. By the School Health Law constituted in 1967 and the enforcement ordinance of School Health made firm the legal basis of school health. 3. The administrative organs of school health are the Ministry of Education in center and each Board of Education in cities and provinces. For the first time in 1979, the department of school health was established in the organization of the Ministry of Education. And at about the same time of establishment of the department of school health, health section was established in the department of social physical-training in locality. 4. In the manpower of school health which was presented in the related statute of school health, there are the ward chief of education, the superintendent of educational affair, of cities and districts, the mayors, the governors of provinces, the school managers, the principals, the school doctors, the school pharmacists, and the nurse-teachers, including teachers holding additional school health as the practical manpower of school health. 5. In order to get some information on distribution aspect of teachers additional school health, this study made up a questionnaire from August 3 to August 11, 1988. The subjects of this study were 212 leachers who took part in the yearly training for teachers holding additional school health from Kyunggi province, Chungbuk province and Jeonbuk province. The results of the questionnaire are as following: 1. The distribution percentages of teachers holding additional school health according to each Board of Education wich schools are subject to, are as following:70.1% (Kyunggi), 76.5% (Chungbuk), and 81.4% (Jeonbuk). There was a significant difference. The distribution percentages of teachers holding additional school health according to the school levels of 3 provinces are as following: 74.1% (Primary schools), 77.8% (Middle schools), 76.7% (High schools). There were little significant differences. 2. The distribution according to the general characteristics of the subject schools: There were 64.2 percent of primary schools and 35.8 percent of middle schools among 212 schools. 91. 5 percent of schools were located in districts. Public schools formed 55.7% and then national schools were higher in percentage than private schools. 58.5 percent of schools had 1-9 classes, 64.6 percent of schools had 101-500 students, and 90 percents of schools had 1-20 teachers. In considering student sex, the coed school showed the high distribution percentage (Primary schools : 100%, Middle schools: 81.6%). 3. The distribution according to the characteristics of teachers holding additional school health: 93.3 percent of teachers were female, and more than 60 percent of teachers were 20-29 years old. As the age got higher, the percentage became lower. There were little significant differences by marital status. In considering their educational status, 86.8 percent of teachers in primary schools were from teacher's colleges, and 64.5 percent of teachers in middle schools were from education colleges. In considering teaching career, 46.7 percent of teachers had teaching career of less than 2 years. 73.6 percent of teachers had held additional school health for less than one year. More than 80 percent of teachers had participated in the training one time or twice. More than 70 percent of teachers had 1-2 additional jobs except for the school health business. The motivation to hold additional school health is most caused by mandatory order, which accounts for more than 80.0 percent. In considering interesting degree concerning school health, lukewarm answer is the highest of 62.7 percent, followed by affirmative answer of 23.6 percent. In considering their contentment degree respecting additional school health job, "discontent or very discontent"is the highest of 47.6 percent. As a descontent reason of additional school health job, overwork is the highest factor of 37.9 percent. Among addiitional school health job, the most difficult affair is nursing service to be 34.0 percent, followed by health education of 31.6 percent. It testify the need of professional. The source of knowledge about school health has been acquired from masscommunication or private health experience, which account for as much as 56.1 percent. It shows seriousness of lack of professionalism. With regard to neccessity of school health experts, 95.8 percent represents absolute need. With above consideration of study results, I propose as follows : 1. I propose that the authorities concerned unify and improve statute respecting current school health which has not been steadfastly supporting school health business by ambiguity of expression and dualization. 2. I propose that the authorities concerned give the school manager, school staffs and parents of students educational chance with which they can acknowledge the importance of school health and in which they can participate as well as set up alternative policy plan to be albe to vitalize school health committee. 3. I propose that administrative organization practicable to taking totally charge of school health business is established within the Ministry of Education. 4. I propose that the authorities concerned back up and cooperate in an attempt by make school health better and desirable toward development by way of appointing qualitied health teachers on the basis of legally regular teacher staffs.

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Weaning Following a 60 Minutes Spontaneous Breathing Trial (1시간 자가호흡관찰에 의한 기계적 호흡치료로부터의 이탈)

  • Park, Keon-Uk;Won, Kyoung-Sook;Koh, Young-Min;Baik, Jae-Jung;Chung, Yeon-Tae
    • Tuberculosis and Respiratory Diseases
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    • v.42 no.3
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    • pp.361-369
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    • 1995
  • Background: A number of different weaning techniques can be employed such as spontaneous breathing trial, Intermittent mandatory ventilation(IMV) or Pressure support ventilation(PSV). However, the conclusive data indicating the superiority of one technique over another have not been published. Usually, a conventional spontaneous breathing trial is undertaken by supplying humidified $O_2$ through T-shaped adaptor connected to endotracheal tube or tracheostomy tube. In Korea, T-tube trial is not popular because the high-flow oxygen system is not always available. Also, the timing of extubation is not conclusive and depends on clinical experiences. It is known that to withdraw the endotracheal tube after weaning is far better than to go through any period. The tube produces varying degrees of resistance depending on its internal diameter and the flow rates encountered. The purpose of present study is to evaluate the effectiveness of weaning and extubation following a 60 minutes spontaneous breathing trial with simple oxygen supply through the endotracheal tube. Methods: We analyzed the result of weaning and extubation following a 60 minutes spontaneous breathing trial with simple oxygen supply through the endotracheal tube in 18 subjects from June, 1993 to June, 1994. They consisted of 9 males and 9 females. The duration of mechanical ventilation was from 38 hours to 341 hours(mean: $105.9{\pm}83.4$ hours). In all cases, the cause of ventilator dependency should be identified and precipitating factors should be corrected. The weaning trial was done when the patient became alert and arterial $O_2$ tension was adequate($PaO_2$ > 55mmHg) with an inspired oxygen fraction of 40%. We conducted a careful physical examination when the patient was breathing spontaneously through the endotracheal tube. Failure of weaning trial was signaled by cyanosis, sweating, paradoxical respiration, intercostal recession. Weaning failure was defined as the need for mechanical ventilation within 48 hours. Results: In 19 weaning trials of 18 patients, successful weaning and extubation was possible in 16/19(84.2 %). During the trial of spontaneous breathing for 60 minutes through the endotracheal tube, the patients who could wean developed slight increase in respiratory rates but significant changes of arterial blood gas values were not noted. But, the patients who failed weaning trial showed the marked increase in respiratory rates without significant changes of arterial blood gas values. Conclusion: The result of present study indicates that weaning from mechanical ventilation following a 60 minutes spontaneous breathing with $O_2$ supply through the endotracheal tube is a simple and effective method. Extubation can be done at the same time of successful weaning except for endobronchial toilet or airway protection.

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Tetralogy of Fallot Associated with Pulmonary Atresia and Major Aortopulmonary Collateral Arteries -Comparison between the different surgical approaches- (폐동맥폐쇄와 주대동맥폐동맥혈관부행지를 동반한 활로씨사징증 -다양한 외과적 치료접근의 비교-)

  • 이정렬;양지혁;김용진;노준량
    • Journal of Chest Surgery
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    • v.32 no.5
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    • pp.471-479
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    • 1999
  • Background: Patients with tetralogy of Fallot(TOF), pulmonary atresia(PA) and major aortopulmonary collateral arteries(MAPCAs) have been managed by heterogenous surgical strategies. We have taken 5 different surgical approaches to this lesion. In this study, relative advantages and disadvantages of each strategy were discussed by analyzing our surgical results. Material and Method: Between January 1986 and June 1998, 50 patients aged 1 to 177(39${\pm}$31) months at a various morphologic spectrum of this lesion were treated at our institution. The groups, which were classified according to the initial approaches, consisted of one-stage unifocalization(A-1, n=9), staged unifocalization(A-2, n=11), right ventricle to pulmonary artery connection(RV-PA)(B-1, n=11), one-stage RV-PA plus unifocalization (B-2, n=11), and one-stage definitive repair(C, n=8). Morphologic charateristics, operative mortalities, and probabilities of definitive repair were compared between the groups and the causes of death, complications and the follow-up results were described. Result: Mean ages at the first operation were 57${\pm}$18(A-1), 42${\pm}$48(A-2), 18${\pm}$14(B-1), 52${\pm}$55(B-2), and 32${\pm}$34(C) months (p<0.05). Mean numbers of MAPCAs were least in group C (A-1=4.3${\pm}$1.0, A-2=4.5${\pm}$1.3, B-1=4.1${\pm}$1.9, B-2=4.1${\pm}$1.6, C=3.4${\pm}$1.8 : p<0.05). The ratios of the direct and the indirect MAPCAs in each group were not different between the grousps (A-1=91%/9%, A-2=78%/22%, B-1=80%/20%, B-2=80%/20%, C=81%/19% : p>0.05). Nineteen patients had more than 1 dependent MAPCAs. True pulmonary arteries were not present 13 patients and they were confluent in 29. A total of 101 operations were performed. Operative mortalities of initial procedures were 33%(3/9, A-1), 18%(2/11, A-2), 0%(0/11, B-1), 36%(5/11, B-2) and 13%(1/8, C) (p<0.05). Percentages of the definitive repair among the patients of each group were 22%(2/9, A-1), 18%(2/11, A-2). 45%(5/11, B-1), 27%(4/11, B-2), and 100%(8/8, C)(p<0.05). Hypoxic respiratory and cardiac failure(6), hypoxic encephalopathy(2) and sepsis(4) were the major causes of death. Phrenic nerve palsy occured in 5 patients. Thirty-one patients among the survivals have been followed up for a mean duration of 74${\pm}$42(3-145) months. One and five year actuarial survival rates were 73% and 73 %. Conclusion: In conclusion, Right ventricle to pulmonary artery connection used in patients with TOF/PA/MAPCA as an inital procedure appeared to be highly successful in enhancing the chance of satisfactory definitive repair without the significant surgical risks. One-stage total repair at an ealier age group could be performed safely with the resonable outcomes. Unifocalization approach, whether it was performed in a single stage or in the multiple stages, resulted in the high operative mortality and the lowest chance of definitive repair, however more tailored selection of the patients and the long follow-up is mandatory to prove the usefulness of this approach.

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Operative Treatment of Congenitally Corrected Transposition of the Great Arteries(CCTGA) (교정형 대혈관 전위증의 수술적 치료)

  • 이정렬;조광리;김용진;노준량;서결필
    • Journal of Chest Surgery
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    • v.32 no.7
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    • pp.621-627
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    • 1999
  • Background: Sixty five cases with congenitally corrected transposition of the great arteries (CCTGA) indicated for biventricular repair were operated on between 1984 and september 1998. Comparison between the results of the conventional(classic) connection(LV-PA) and the anatomic repair was done. Material and Method: Retrospective review was carried out based on the medical records of the patients. Operative procedures, complications and the long-term results accoding to the combining anomalies were analysed. Result: Mean age was 5.5$\pm$4.8 years(range, 2 months to 18years). Thirty nine were male and 26 were female. Situs solitus {S,L,L} was in 53 and situs inversus{I,D,D} in 12. There was no left ventricular outflow tract obstruction(LVOTO) in 13(20%) cases. The LVOTO was resulted from pulmonary stenosis(PS) in 26(40%)patients and from pulmonary atresia(PA) in 26(40%) patients. Twenty-five(38.5%) patients had tricuspid valve regurgitation(TR) greater than the mild degree that was present preoperatively. Twenty two patients previously underwent 24 systemic- pulmonary shunts previously. In the 13 patients without LVOTO, 7 simple closure of VSD or ASD, 3 tricuspid valve replacements(TVR), and 3 anatomic corrections(3 double switch operations: 1 Senning+ Rastelli, 1 Senning+REV-type, and 1 Senning+Arterial switch opera tion) were performed. As to the 26 patients with CCTGA+VSD or ASD+LVOTO(PS), 24 classic repairs and 2 double switch operations(1 Senning+Rastelli, 1 Mustard+REV-type) were done. In the 26 cases with CCTGA+VSD+LVOTO(PA), 19 classic repairs(18 Rastelli, 1 REV-type), and 7 double switch operations(7 Senning+Rastelli) were done. The degree of tricuspid regurgitation increased during the follow-up periods from 1.3$\pm$1.4 to 2.2$\pm$1.0 in the classic repair group(p<0.05), but not in the double switch group. Two patients had complete AV block preoperatively, and additional 7(10.8%) had newly developed complete AV block after the operation. Other complications were recurrent LVOTO(10), thromboembolism(4), persistent chest tube drainage over 2 weeks(4), chylothorax(3), bleeding(3), acute renal failure(2), and mediastinitis(2). Mean follow-up was 54$\pm$49 months(0-177 months). Thirteen patients died after the operation(operative mortality rate: 20.0%(13/65)), and there were 3 additional deaths during the follow up period(overall mortality: 24.6%(16/65)). The operative mortality in patients underwent anatomic repair was 33.3%(4/12). The actuarial survival rates at 1, 5, and 10 years were 75.0$\pm$5.6%, 75.0$\pm$5.6%, and 69.2$\pm$7.6%. Common causes of death were low cardiac output syndrome(8) and heart failure from TR(5). Conclusion: Although our study could not demonstrate the superiority of each classic or anatomic repair, we found that the anatomic repair has a merit of preventing the deterioration of tricuspid valve regurgitations. Meticulous selection of the patients and longer follow-up terms are mandatory to establish the selective advantages of both strategies.

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A Study of Educational System for Medical Technologists in Korea (한국(韓國)의 의료기사(醫療技士) 교육제도(敎育制度)에 관(關)한 조사(調査) 연구(硏究))

  • Song, Jae-Kwan;Lee, Gun-Sub;Kim, Byong-Lak;Kim, Chung-Rak;Cho, Jun-Suk;Huh, Joon;Lee, Joon-Il
    • Journal of radiological science and technology
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    • v.6 no.1
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    • pp.131-181
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    • 1983
  • After the investigation on, and the analysis of, the educational system for medical technicians and the present educational situation for medical technologies in this country, the following conclusions were drawn. 1. As of March 1983 the current academic system for education in medical technologies included the regular 4-year college courses and those of the 2-year professional junior college courses. But except in the cases on clinical pathology and physical therapy, there were no college-level departments. Particularly, no educational institutions, at whatever level, had a department for working therapies. 2. The total number of credits needed for graduation from a department of medical technologies was 150 points at a regular 4-year college and 85 to 96 points at a 2-year professional college. The obligatory minimum number of credits for a student at a professional college was set at 80 points and above. 3. As for the number of the educational institutions for medical technologies in this country, there were one regular college and 14 professional colleges, a total of 15 institutions. As many as 14 colleges had departments of clinical pathology, 12 had departments of Radiotechnology, 11 had departments of physical therapy, 12 had departments of dental technology, and eight had departments of dental hygiene. 4. The total capacity of the professional colleges in admitting new enrollment each year were 1,920 for clinical pathology, 1,552 for radiology, 1,012 for physical therapy, 1,334 for dental technologies, 828 for dental hygiene, an aggregate of 6,646 for all of the professional college departments. 5. The total number of graduates from the 12 professional colleges by department during the period of 1965-83 were 7,595 for clindical pathology, 4,768 for radiology, 2,821 for physical therapy, 3,000 for dental technologies, and 1,787 for dental hygiene, totalling 19,971 for all departments in the professional colleges. 6. In the state examination for licensed medical technicians, 12,446 have passed from among the total of 26,609 participants, representing a 45% passing ratio. By departments the ratios showed 44% for clinical pathology, 39.7% for radiology, 51.2% for physical therapy, 42.5% for dental technology, 72.5% for dental hygiene and 73.1% for working therapy. 7. As for the degree of satisfaction shown by the people in this field, 52.2 percent of the teaching staffs who responed to the questionaires said they were satisfied with their present profession, while the great majority of medical technicians(66%) replied that they were indifferent to the problem. 8. The degree of satisfaction shown by the students on their enrollment in this particular academic field was generally in the framework of indifference(43.7%), but mere students(36.5%) were satisfied with their choice than those were not satisfied(14.4%) 9. As for the student's opinions on the lectures and practicing hours, a good many students replied that, among such courses as general science and humanities courses the basic medical course, the major course and practicing hours, the hours provided for the general courses(47.1%) and practicing(47.6%) were insufficient. 10. When asked about the contents of their major courses, comparatively few students (23.6%) replied that the courses were too difficult, while a convincing majority(58.5%) said they were neither difficult nor easy. As for the appropriateness of the number of the present teaching staffs, a great majority(71.0%) of the students replied that the level of the teaching personnel in each particular field was insufficient. 11. Among the students who responded to the poll, good part of them(49.5%) wanted mandatory clinical practicing hours, and the the majority of them(64.6%) held the view that the experimental and practicing facilities of their schools were insufficient. 12. On the necessity of the attached hospitals, 71.1% of the teaching staffs and 58.0% of the medical technicians had the opinion that this kind of facility was indispensable. 13. As for the qualifications for applicants to the state examination in the licensing system for medical technicians, 52.2% of the teacher's and 36% of the medical technicians replied that the present system granting the qualifications according to the apprenticeship period should be abolished. 14. On the necessity of improving the present system for education in medical technologies, an overwhelming majority(94.4% of the :caching staffs, 92.0% of the medical technicians and 91.9% of students) of these polled replied that the present system should be changed for the better. 15. On the method of changes for the present educational system, a great majority(89.4% of the teaching staffs, 80.4% of the medical technicians and 90.1% of the students) said that the system must be changed so that it fits into the reality of the present day. 16. As for the present 2-year program for the professional colleges, 61.6% of the teachers, 72.0% of the medical technicians and 38.8% of the students expressed the hope that the academic period would be extended to four regular years, hemming a full-fledged collegelevels program. 17. On the life-long eductional system for medical technicians, there was a considerable number of people who expressed the hope that an open university system(38.9% of the teaching staffs, 36.0% of the medical technicians) and a graduate school system would be set up. 18. As for the future prospects for medical technicians as professionals, the optimists ana pessimists were almost equally divided, and 41.1% of the teaching staffs 36.0% of. the technicians and 50.5% of the students expressed an intermediate position on this issue.

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Clinical Characteristics of Arthrogryposis, Renal Tubular Dysfunction, Cholestasis(ARC) Syndrome in Korea (ARC(Arthrogryposis, Renal Tubular Dysfunction, Cholestasis) 증후군의 발병양상에 관한 연구)

  • Lee Soon Min;Kim Ji Hong;Lee Jae Seung;Han Suk Joo
    • Childhood Kidney Diseases
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    • v.9 no.2
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    • pp.222-230
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    • 2005
  • Purpose : ARC syndrome refers to an association of arthrogryposis, renal tubular dysfunction, and cholestasis. The VPS33B gene was recently identified as the causative gene. So far, 41 cases of ARC syndrome have been reported worldwide, and it has rarely been reported in Korea. This study was conducted to report the clinical findings of seven ARC syndrome cases in Korean children, focusing especially on renal tubular dysfunction. Methods : The hospital records of 7 cases diagnosed as ARC syndrome at Severance Hospital between Mar. 1995 and Aug. 2005 were reviewed and analyzed. Results : Of the 7 cases, 4 were boys and 3 were girls. Six patients(85$\%$) were born with normal birth weight at term, and one was born at preterm. All cases presented with cholestasis and severe jaundice. According to the type of arthrogryposis described by Brown et al, type 3 and 4 were found in 2 patients and type,6, 7, and the undistributed type in one patient respectively. Other associated clinical findings were as follows : failure to thrive in 6(85$\%$), lax skin in 5(71$\%$), and gray platelet syndrome in 4(57$\%$). Urine analysis revealed 6 cases(85$\%$) with proteinuria, 3(43$\%$) with hematuria, 5(71$\%$) with glycosuria, 2(29$\%$) with phosphaturia and 2(29$\%$) with calciuria. Serum electrolytes showed 4 cases(57$\%$) with hyponatremia, 3(43$\%$) with hypokalemia, and 1(14$\%$) with creatinine elevation. Renal tubular dysfunctions were diagnosed as renal tubular acidosis in 6 cases(85$\%$), nephrogenic diabetes insipidus in 2(29$\%$), and Fanconi syndrome in 2(29$\%$). During the follow-up period, 2(29$\%$) had no treatment, 5(85$\%$) had continuous supplementation to correct the electrolyte imbalance and acidosis, and 1(14$\%$) had dialysis. Only one patient had a family history of ARE syndrome in a sibling. Four cases(57$\%$) were diagnosed at the mean age of 8.2 months, and one case was lost during follow-up. Ages of the survived 2 cases were 13 and 25 months, respectively. Conclusion : The rare disease of ARC syndrome Is associated with severe renal dysfunction. However, this study revealed that the renal manifestation of ARC syndrome in Korean children is relatively mild and survival rate is higher than that of previous studies. Contrary to previous reports, this study showed that familial cases are rare and sporadic occurence is possible in Korea. Thus, the diagnosis of this syndrome requires a careful evaluation of the venal function in cases of congenital arthrogryposis, and a mandatory genetic counseling of affected family for prevention of familial occurance. (J Korean Soc Pediatr Nephrol 2005;9:222-230)

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Study of the Impact of Light Through the Vitamin $B_{12}$/Folate Inspection (Vitamin $B_{12}$/Folate 검사 시 빛의 영향에 대한 고찰)

  • Cho, Eun Bit;Pack, Song Ran;Kim, Whe Jung;Kim, Seong Ho;Yoo, Seon Hee
    • The Korean Journal of Nuclear Medicine Technology
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    • v.16 no.2
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    • pp.162-166
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    • 2012
  • Purpose : Vitamin $B_{12}$ and Folate are for anemia work-up which is well known for its sensitivity of light; the screening manual also specifies to be careful with light conditions. According to this, our laboratory minimized the exposure of light when inspecting the Vitamin $B_{12}$ and Folate, but the exposure cannot be wholly blocked due to other various factors such as when conducting specimen segregation. Thus, this inspection is to identify to what extent light can influence and whether the exclusion of light is mandatory during the Vitamin $B_{12}$/Folate test. Materials and Methods : We have conducted two experiments of identifying the extent of light's influence when conducting the Vitamin $B_{12}$/Folate test and also when specimens are under preservation. These experiments were progressed with various concentrations of patients' specimens which were requested to our hospital in March 2012. The first experiment is to verify the results on Vitamin $B_{12}$/Folate dependent on light exposure during the experiment. In the process, we have compared the results of light exposure/exclusion during the incubation process after the reagent division. The second experiment is about the impact of light exposure on the results on Vitamin $B_{12}$/Folate during the preservation. For 1, 2, 7 days the light on the specimen were wholly blocked and were preserved under $-15^{\circ}C$ temperature refrigeration. Then, we compared the results of light-excluded specimen and the exposed one. Results : When conducting first experiment, there were no noticeable changes in the Standard and specimen's cpm, but for Vitamin $B_{12}$, the average result of specimen exposed to light increased 7.8% compare to that of excluded one's. Furthermore, in the significant level 0.05, the significance probability or the p-value was 0.251 which means it has no impact. For Folate, the result being exposed to light decreased 5.4%, the significance probability was 0.033 which means it has little impact. For the second preservation, the result was dependent on the light exposure. The first day of preservation of Vitamin $B_{12}$, the clinical material exposed to light was 11.6%, second day clinical material exposed to light was 10.8%, seventh day clinical material exposed to light increased 3.8%, the significance probability of the $1^{st}$, $2^{nd}$, $7^{th}$ day is 0.372, 0.033, 0.144 respectively, and which indicates that the $1^{st}$ and $7^{th}$ day seems to have no impact. For Folate's case, the clinical material exposed to light has increased 1.4% but hardly had impact, $2^{nd}$ day clinical material being exposed to light was 6.1%, $7^{Th}$ day clinical material being exposed to light decreased 5.2%. The significance probability of Folate on the $1^{st}$, $2^{nd}$, $7^{th}$ day is 0.378, 0.037, 0.217 respectively, and the $1^{st}$ day and the $7^{th}$ day seems to have no impact. Conclusion : After scrutinizing the impact of light exposure/exclusion, Vitamin $B_{12}$ has no impact, while Folate seems to have no noticeable influence but light exclusion is recommended due to its significance probability of 0.033 when conducting experiment. During the preservation, the $2^{nd}$ day result depend on the light exclusion seems to have impact or influence. However, to consider the complication of the experimental process, the experiment including technical errors is predictable. Hence, it is likely to have no impact of light. Nevertheless, it is recommendable to exclude the light during the long preservation as per the significance probability (p-value) of $1^{st}$ and $7^{th}$ day has been diminished.

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