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The Perception and Emotional Experiences of Rare and Intractable Diseases in Caregivers and Pediatric Patients with Mitochondrial diseases (미토콘드리아 질환 소아 환자 보호자에서의 질환 인식 및 정서변화)

  • Eom, Soyong;Lee, Joo Young;Hyun, Jiah;Lee, Young-Mock
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.17 no.1
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    • pp.1-10
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    • 2017
  • Purpose: This study aimed to investigate the perception and emotional experiences in rare and intractable diseases for caregivers of pediatric patients with mitochondrial diseases in order to provide therapeutic interventions for patients, caregivers, and families. Methods: A total of 83 caregivers of pediatric patients with mitochondrial diseases were recruited from the pediatric mitochondrial disease clinics of the Gangnam Severance Hospital in South Korea. Participants completed the survey about their perception of mitochondrial disease and emotional experiences after the diagnosis, and these clinical data were analyzed accordingly. Results: Surveys from a total of 83 caregivers of patients were analyzed, and the patients' age ranged from 6 to 12 years (33%), followed by ages 1 to 6 years (30%). Children with mitochondrial diseases were between 0 and 0.5 years of age at the time of first symptom onset (43%), and the duration of illness lasted more than 10 years in most cases (42%). Prior to diagnosis of mitochondrial diseases, the amount of awareness the caregivers had was 'Not at all' for both rare and intractable diseases and mitochondrial diseases in 44 cases and 68 cases, respectively. For the caregivers' emotional experiences, the most common initial responses were 'Discouraged/despair', 'Helpless/lethargic', and 'Disconcerted'. 'Anxious', 'Committed to treatment', and 'Responsibility as family members' were the most common emotional responses from the caregivers, followed by 'Disconcerted' and 'Helpless/lethargic'. Conclusion: It is important to consider the level of perception and emotional experiences of caregivers and patients with rare and intractable mitochondrial diseases for planning treatment programs.

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A Case of Glycogen Storage Disease Type Ia Confirmed by Biopsy and Enzyme Assay (제Ia형 당원병 1례 (Glycogen Storage Disease , Type Ia))

  • Meen Sang-Ae;Rho Kwang-Sik;Kim Pyung-Kil;Jeong Hyeon-Joo;Park Young-Nyeon;Kim Myung-Joon;Kim Ji-Hong
    • Childhood Kidney Diseases
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    • v.2 no.1
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    • pp.77-81
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    • 1998
  • The author exprienced a case of glycogen storage disease type Ia(GSD-I) in an 18-year-old male patient who was admitted to our hospital due to proteinuria and hypertension. he was suspected to have GSD when 12 years old because of his family history of short stature and hepatomegaly. On admission, physical examination revealed short stature, heparomegaly, and The diagnosis of GSD-I was confirmed by compatible liver biopsy finding and enzyme assay which erealeddeficiency of glcose-6-phosphatase if hepatocyte. Sympromatic treatment was done using antihypertensive drugs and allopurinol with diet control. The authors report a case of glycogen storage disease type Ia completely confirmed by typical clinical manifestation, pathologic findings of the liver and the kidney, and the result of enzyme assay which revealed deficiency of glucose-6-phosphatase in hepatocytes with brief review fo related literatures.

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A RURAL HEALTH SERVICE MODEL FOR KOREA BASED OH A PRIMARY CARE NURSING SERVICE SYSTEM

  • Hong, Yeo-Shin
    • Journal of Korean Academy of Nursing
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    • v.11 no.2
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    • pp.5-8
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    • 1981
  • This study concerns itself with the development of a new model of comprehensive health service for rural communities of Korea. The study was conceived to resolve the problems of both underservice in rural communities and underutilization of valuable health manpower, namely the nurses, the disenchanted elite health personnel in Korea. On review of the current situation, the greatest deficiencies in the Korean health care system were found in the availability of primary care at the peripheries of md communities, in the dissemination of knowledge of disease prevention and health care, and in the induction of and guidance for active participation by the clientele in health maintenance at the personal, family and community level Abundant untapped health resources were identified that could be brough to bear upon the national effort to extend health services to every member of the Korean Population. Therefore, it was Postulated that the problem of underservice in rural communities of Korea can be structurcturally resolved by the effective mobilization and organization of untapped health resources, and that. a primary care Nursing Service System offers the best possibility for fulfillment of rural health service goals within the current health man-power situation. In order to identify appropriate strategies to combat the present difficulties in Korean rural health services and to utilize nurses and other health personnel in community-centered health programs, a search was made for examples of innovative service models throughout the world. An extensive literature survey and field visits to project sites both in Korea and in the United States were made. Experts in the field of world health, health service, planners, administrators, and medical and nursing practitioners in Korea, in the United States as well as visitors from other Asian countries were widely consulted. On the basis of information and inputs from these experts a new rural health service model has been constructed within the conceptual framework of community development, especially of the innovation diffusion Model. It is considered especially important that citizens in each community develop capacities for self-care with assistance and supports from available health professionals and participate in health service-related decisions that affect their own well-being. The proposed model is based upon the regionalization of health care planning utilizing a comprehensive Nursing Service System at the immediate delivery level The model features: (1) a health administration unit at each administrative level; (2) mechanisms for community participation; (3) a continuous source of primary health care at the local community level; (4) relative centralization of specialty care and provision of tertiary or super-specialty care only at major national metropolitan centers; and (5) a system for patient referral to the appropriate level of care. This model has been built around professional nurses as the key community health workers because their training is particularly suited and because large numbers of well-trained nurses are currently available and being trained. The special element in this model is a professional nurse-guided, self-care facilitating primary care Community Nursing Service System. This is supported by a Nursing Extension Service as a new training and support structure. (See attached diagrams). A broad spectrum of programs was proposed for the Community Nursing Service System. These were designed to establish a balance of activities between the clinic-centered individual care component and the field activity-centered educational and supportive component of health care services. Examples of possible program alternatives and proposed guidelines for health care in specific situations were presented, as well as the roles and functions of the key health personnel within the Community Nursing Service System. This Rural Health Service Model was proposed as a real alternative to the maldistributed, inequitable, uncoordinated solo-practice, physician-centered fee-for-service health care available to Koreans today.

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Sputum Smear Conversion During mDOT (Modified Directly Observed Treatment) (변형된 복약확인 치료(mDOT) 수행 후의 균음전율에 대한 연구 부제: 보건소 균양성 폐결핵 환자를 대상으로)

  • Hwang, Taik Gun;Kim, Soon Deok;Yoo, Se Hwa;Shin, Yoo Chul
    • Tuberculosis and Respiratory Diseases
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    • v.56 no.5
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    • pp.485-494
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    • 2004
  • Background : To assess the effects of mDOT implementation on sputum smear conversion for AFB (Acid fast bacilli) positive pulmonary tuberculosis patients, modified Directly Observed Treatment (mDOT) was started on October $8^{th}$ 2001 at a health center in Seoul. mDOT was defined through weekly interviewing and supervising of a patient by a supervisor (doctor, nurse, or lay health worker). The sputum smear conversion of a mDOT group was compared with that of a self-medication (self) group. Methods : This study included 52 AFB positive pulmonary tuberculosis patients registered at a health center in Seoul between October $8^{th}$ 2001 and April $23^{rd}$ 2002. 24 and 28 patients were enrolled in the mDOT and self medication groups, respectively. Paired (1:1) individual matching, by gender, extent of disease, relapse and age-matching variables, was performed between the two groups, resulting in 20 paired matches. This prospective study was planned as an unblinded, non-randomized quasiexperimental pilot project. Outcomes were identified from results of sputum smear examinations for AFB in both groups at 2 weeks, and 1 and 2 months. The paired matching data were analyzed using the SAS program version 8.1 by McNemar test. Results : At the end of 2 weeks of treatment, the sputum smear conversion of the mDOT group was somewhat higher than that of the self medication group (78.57 vs. 50%, p-value=0.289), and after 1 month of treatment no statistically significant difference was shown between the two groups (83.33 vs. 50, p-value=0.125). At the end of 2 months of treatment (initial intensive phase), the sputum smear conversions of the mDOT and self groups were 95 and 75%, respectively (p-value=0.219). Conclusions : The implementation of mDOT did not result in clinically significant increases in the sputum smear conversion at 2 weeks, and 1 and 2 months compared with that of the self medication group. However, the increases experienced might contribute to diminishing the infectious period of AFB positive patients, and this approach may act as a guide for a specific group of patients. In this study, mDOT was performed for one hundred percent of the intensive treatment phase. It can also be an effective treatment for pulmonary tuberculosis patients, and may be useful for some high risk tuberculosis patients.

Pulmonary tuberculosis misdiagnosed as lung Metastasis in childhood cancer patients (소아암 환자에서 암의 전이로 오인된 폐결핵)

  • Lee, Hyun-Jae;Kim, Dong-Whan;Lee, Kang-Min;Park, Kyung-Duk;Lee, Jun-Ah;Cho, Soo-Yeon;Kook, Yoon-Hoh;Kim, Hee-Youn;Kim, Dong-Ho
    • Clinical and Experimental Pediatrics
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    • v.52 no.8
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    • pp.904-909
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    • 2009
  • Purpose : The differential diagnosis for a pulmonary nodule is intriguing in cancer patients. Metastasis might be a preferential diagnosis, and yet possibilities of other medical conditions still exist. Pulmonary tuberculosis should be enlisted in the differential diagnosis for a pulmonary nodule in cancer patients in Korea. This study was aimed at analyzing the incidence and clinical features of pulmonary tuberculosis that were misdiagnosed as pulmonary metastasis during radiologic follow-up in pediatric cancer patients. Methods : We retrospectively studied 422 cancer patients less than 18 years old in the Korea Cancer Center Hospital from January 2001 to June 2007. We collected episodes of lung metastasis of primary tumor and tuberculosis during treatment or follow-up, and analyzed medical records. Results : There were 5 cases of tuberculosis confirmed after surgery which were initially regarded as cancer. Two patients had respiratory symptoms such as cough and sputum but the other 3 patients did not. One patient had a family history of tuberculosis. Acid-fast M. tuberculosis was found in one case upon tissue specimen analysis. Two cases were Mantoux positive and the sputum examination was negative in all cases. The polymerase chain reaction for tuberculosis on a pathologic specimen was used to differentiate M. tuberculosis from non-tuberculosis mycobacterium (NTM). It was positive in one case. Lung lesions in one case showed a concurrence of tuberculosis along with lung metastasis. One of these patients died after cancer recurrence. Conclusion : It is necessary to consider the possibility of tuberculosis when a lung mass is newly detected during treatment or follow-up in patients with childhood cancer.

A Study on Implementation of Primary Health Care Delivery System meet to Rural Area in Korea -Village Health Voluntary Worker Development- (우리 나라 농촌지역(農村地域)에 부합하는 1차(次) 보건의료전달체계(保健醫療傳達體系) 정착구현(定着具現)에 관한 연구(硏究) -마을 보건임원(保健任員) 개발(開發)-)

  • Koo, Y.C.;Wie, J.H.;Hwang, S.J.;Choi, S.S.
    • Journal of Preventive Medicine and Public Health
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    • v.12 no.1
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    • pp.13-23
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    • 1979
  • A study was carried out from October 1977 to September 1978 in order to develope health care delively system which will meet to rural area in Korea. For the study objective a model of health care delivery system of Myun (township) area was developed which is adopted the net-work of village health voluntary worker who will play the role of bridge for communication related with health and illness between families or village people and health subcenter, and :he model health care delivery system net-work was set in the area of Soodong Myun, Yangju Gun. which is the rural health demonstration area of Ewha Womans University since 1972. The activities and attitude of 22 village health voluntary workers were observed and analized. during the study period. The results are as follows; 1. For the field activities of village health voluntary workers. a guide line which is described with specific behavioral objectives was developed and used for not only training of the workers but also evaluation of their field activities. 2. During the study period, the number of 971 village people were served primary health care service by village health voluntary worker and the service was classified largely into symptomatic medications (92%) and preventive measures (8%). 3. Comparative percentage of the number of 894 symptomatic cases cared by village health voluntary workers to 5,695 cases of patient treated by Soodong Health Subcenter during the same period was 15.7%. 4. Annual utility rate of village health voluntary worker by Myun total people was 16.1% but utility rate by Rie was varied from 38.2% to 2.8% which shown there were considerable differences in each Rie. In order to settle the village health care service, the obstructive factors of utility should be detected and their counter measure must be taken. 5. As the health need of village people increases, it is expected that the supplement of drug excluding present sit basic drugs is inevitable, but considering the ability of village health voluntary worker, the selection of additional drugs and education, plan should be carefully studied. 6. It is desirable that a financial resource for supplementary purchase of first aid kit, drugs and materials whould be alloted from village public fund like Saemaeul Women's Club fund, which has already practiced in a few villages in the study area. 7. As pointed out by village health voluntary workers, in order to improve the village health, village leaders should be in the center of it and the cooperation of whole village people is a core of healthful village development, and it is reasonable that the health subcenter backs up these voluntary health activities by village people in techniques. 8. It seems effective that a supplementary education for village health voluntary worker be accomplished by a planned education through regular meetings like worker's monthly meeting and irregular post guide when Myun Health Workers can handle the problems found during the round trip of villages. 9. It is desirable that village health voluntary workers, who are recommended by a civil voluntary organization like Saemael Woman's Club, are charged by natural villagc unit, are given a function of village health care service and used through basic education at health subcenter. 10. It is advisable that the village health voluntary worker's service is compensated not by a form of money, but by other way such as an exemption of medical fee of worker herself or her families in health subcenter can be one method. 11. Daily health activities of each village health voluntary worker should be reported to health subcenter by biweekly or monthly in order to get not only for basic data of the program but also for evaluation the program. It is recomandable that the report form should be simple and clear enough for village health voluntary worker to fill it effectively. 12. Village health care service should be developed into a Saemaeul Movement in which village people actively participate. For this, the appointed function of village health voluntary worker should be absorbed into those of living Environment Betterment Section or Family Planning Section of Saemaeul Women's Club or it is desirable that establish a new section, Village Health Promoting Section and make it involve the appointed functions of those sections mentioned above.

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Postoperative Atrial Fibrillation after Isolated Coronary Artery Bypass Graft Surgery (단독관상동맥우회로술 후 발생한 심방세동)

  • Suh, Jong-Hui;Park, Chan Beom;Moon, Mi-Hyoung;Kweon, Jong Bum;Kim, Young-Du;Jin, Ung;Moon, Seok-Whan;Kim, Chi-Kyung
    • Journal of Chest Surgery
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    • v.42 no.1
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    • pp.14-21
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    • 2009
  • Background: Postoperative atrial fibrillation (AF) is a common complication after coronary artery bypass graft (CABG) surgery. Although postoperative AF is regarded as benign, transient and self-limited, it has been associated with increased morbidity, thromboembolic events and an increased duration and cost of hospitalization. Material and Method: From January 1994 to December 2007, 190 patients that had isolated CABG surgery were divided into two groups. Group 1 (n=139) involved those who had postoperative atrial fibrillation, and group 2 (n=51) did not have any such events. We reviewed the medical records retrospectively including the incidence of postoperative AF, patient characteristics, surgery related factors and the outcome of the patients with postoperative AF. Result: The frequency of postoperative AF was 26.8%, the conversion rate to regular sinus rhythm before discharge was 82.4%; 82.4% of the AF developed within the first three postoperative days. Although the postoperative AF group was significantly older and had a prolonged postoperative Intensive care unit (ICU) stay, there was no difference in the aortic crossclamp time or duration of hospitalization. No spontaneous defibrillation at declamping, and longer duration of cardiopulmonary bypass were significantly related to the development of postoperative AF. However, postoperative treatment with a beta blocker was associated with a decreased incidence of postoperative AF. The multivariate analysis showed that age and ICU stay were significantly associated with the development of POAF. Spontaneous defibrillation and postoperative beta blocker treatment were significantly associated with a decreased frequency of POAF. Conclusion: AF after CABG surgery is a common complication associated with increased morbidity and a longer ICU stay. Therefore, various strategies aimed at reducing AF, and its complications, such as postoperative treatment with a beta blocker should be considered.

Factors upon revisit and intention of recommendation for dental care service (치과의료 재이용 및 권유의사 관련 요인)

  • Lee, Hyang-Nim;Shim, Hyung-Sun
    • Journal of Korean society of Dental Hygiene
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    • v.12 no.2
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    • pp.317-326
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    • 2012
  • Objectives : This study aims to examine satisfaction of dental treatment and revisit and intention of recommendation to dental patients and to supply as a basic resource for dental management improvement. Methods : 645 dental patients to dental hospitals in G City were subjects of this study. 27 dental hospitals were randomly chosen per 5 or 6 in 5 Districts. The intention of the survey was explained to 645 patients and it was agreed to have questionnaire survey. Questionnaire survey was carried out for 2 weeks in April 2011. Total 593 were used for the analysis except for insufficient answer sheets. The analysis was executed by using SPSS 18.0 and the results are as follows. Results : 1. The highest figures are as follows: female was 53.29% in sex, 20-29 age group was 44.52%, graduates of university were 38.62%, students were 28.50%, monthly family income with 300-399 million won was 31.70%. As to the treatment, dental caries were highest with 46.21% followed by scaling with 40.81%. As to the number of visit, 39.80% were over 5 times, and as to the opportunity to use, 29.34% answered it because it is close to home. 2. As to satisfaction, male was 4.15, over 60years old was 4.58, self-employed was 4.33, friendship with dentist as opportunity to use was 4.37. And as to revisit intention, over 60 years old was 4.60, over postgraduate in education level was 4.38, self-employed was 4.43, over 5 times visit was 4.32, and fame as opportunity to use was 4.39 which were the highest. As to the intention of recommendation to others, female was 4.24, over 60 years old was 4.65, self-employed was 4.36, friendship with dentist as opportunity was 4.43 which were the highest and showed significant differences (p<0.05). 3. As to patient satisfaction, there were strong relevance between revisit intention and intention of recommendation with 0.769 and 0.744 respectively. And, as to revisit intention, it had significant relation with revisit intention with 0.791. 4. The explanatory power of the regression analysis on the factors affecting revisit intention was 61.20%. The revisit intention was high when the satisfaction of dental hospital was high (${\beta}$=0.767, p<0.001), the number of visit (${\beta}$=0.026, p<0.01), reason for a visit (${\beta}$=0.070, p<0.01), education level (${\beta}$=-0.063, p<0.05) and occupation (${\beta}$=0.078, p<0.05). 5. The explanatory power of the regression analysis on the factors affecting intention of recommendation was 59.9%. The intention was taken patients satisfaction(${\beta}$=0.601, p<0.001), dental hygienist's kindness (${\beta}$=0.218, p<0.001), female (${\beta}$=0.079, p<0.05), reason for a visit(${\beta}$=-0.059, p<0.05), dissatisfaction treat(${\beta}$=0.084, p<0.05), dentist's kindness (${\beta}$=-0.080, p<0.05), age (${\beta}$=0.054, p<0.05). Conclusions : To improve revisit rate of patients, the refurbishing hospital facility, treatment and kindness of dental hygienists and satisfaction of treatment must be improved. And it also needs to accept complaint from female patients in a positive manner, and understand and correct complaints actively from the perspective of patients.

Clinical and Cytogenetic Analysis of Children with Maternal Chromosomal Balanced Translocation (모체의 염색체 균형전좌를 가진 환아들의 임상적 세포 유전학적 관찰)

  • Lim, Han Hyuk;Jeong, Hee Jeong;Park, Kyung Duk;Kim, Sook Ja
    • Clinical and Experimental Pediatrics
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    • v.48 no.7
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    • pp.701-705
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    • 2005
  • Purpose : Parents' genetic information plays an important role in their children's genetic expression. Human chromosome has 23-paternal chromosomes and 23-maternal chromosomes. Parental chromosomal translocation can induce clinical problems in their children because of imbalance in genetic information. We intent to analyze the cytogenentic and clinical features about children with maternal balanced translocation between chromosome 15 and 18. Methods : We detected by one family's FISH study of chromosome 15. We have evaluated children born to clinically normal parents about peripheral bood analysis, endocrine, metabolic, radiologic study, electroencephalogram and social & intelligence scale. and We analysis their clinical manifestation by hospital records. Results : Patient's father and elder sister are normal clinically and genetically. Her mother's chromosome show balanced translocation, 46, XX, t(15;18)(p11.2;p11.3). One child has 46, XX, der(18) t(15;18)(p11.2;p11.3), mental retardation, growth retardation, speech & social developmental delay, recurrent infection and mild mitochondria dysfunction. Her young brother has 46, XY, der(15) t(15;18) (p11.2;p11.3), mental retardation, aggressive behavior, obesity and speech developmental delay. Conclusion : In this study we observed the children with developmental delay, dysmorphic facial features, mental retardation, growth retardation associated with growth hormone deficiency and aggressive behavior due to unbalanced translocation between chromosome 15 and 18.

A Study of the Construction of Nursing Theory in Korean Culture - View of Medicine- (한국문화에 따른 간호정립을 위한 기초조사연구 III -의료관을 중심으로-)

  • Park, Jeong-Sook;Ok, Yun-Jung
    • Research in Community and Public Health Nursing
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    • v.9 no.1
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    • pp.143-162
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    • 1998
  • This is a study for the construction of nursing care based upon the Korean attitude toward medicine. Factors which were investigated include the source of nursing care, the reason for choosing care, the type of heath care chosen, the accessability of caregivers, and the desired location of death. The population examined in this study consisted of 517 adults distributed in six large cities and 191 adults from five rural communities. Data was analyzed using frequency, percent, Cronbach alpha, $X^2$ - test, t - test, F - test and scheffe post hoc contrast with an SAS program. The results of this study are summarized as follows: 1. Among sources of nursing care used, first rank rated-pharmacy(54.4), private hospital(18.2), general hospital(8.4), folk remedies in house (5.0), chinese hospital(2.8), prayer(2.8) and others(8.4), and the reasons for choosing nursing care rated 'the easiest method' (63.6), 'the best method'(15.7), 'reliable'(10.8) and 'lower cost burden'(4.6) in order of preference. 2. The type of nursing care chosen rated western medicine(6.80), chinese medicine(6.15), folk remedies(5.46), faith remedies(3.51) and divination remedies (1.41). There were significant differences in the effect recognition degree to various kinds of medicine. 3. The difference of the type of nursing care chosen according to general characteristics showed that urban residents were higher than rural community residents(t=2.15, p=0.0320) in western medicine, and urban residents, women, and singles were higher than rural community residents(t=2.04, p=0.0414), men (t= -2.89, p=0.0039), and married(t=2.50, p= 0.0126) on folk remedies. With repect to age and education those 21-30, under 20 and 31-40, graduated from college and graduate school were higher than above 51, above 61 (F = 7.76, p = 0.0001), graduated from elementary school(F=4.39, p=0.0006) on folk remedies. In other categories, rural community residents, women, younger people. Christians were higher than urban residents ( t = -2.73, p=0.0305), men(t= -4.15, p=0.0001), older people (F=2.48, p=0.0307), Catholic, Buddhist, or atheist (F= 70.18, p=0.0001) on faith remedies. Those graduated from high school and Buddhist were higher than unschooled, graduated from middle school(F=3.18, p= 0.0075), atheist, Catholic or Christian(F=18.32, p=0.0001) on divination redemies. There were significant differences concerning age and education level. 4. The accessibility of caregivers rated 'caregivers should be nearby if the patients need them' (50.0), 'caregivers must be there all day (24 hours)' (39.6), 'caregivers must be there at night only'(5.0), 'caregivers must be there during the day only'(2.6), 'caregivers always should visit during visiting hours' 0.4), 'caregivers don't need to be there at all' (1.2). The frist rank of suitable caregivers were rated as spouse(66.6), mother(24.2), daughter (3.6), daughter-in-law(1.9), and the reasons of thinking thus were rated as 'the most comfortable' (81.5), 'people should correctly with regards to family they'(7.1), 'the easiest' (5.4), 'take good care of the patient' (5.1) and 'lower cost burden' (0.4). 5. The desired location of death rated as the following: his/her house (91. 6) to the hospital(8. 4). A person going to encounter death in the hospital wanted his house(78.5) over the hospital(21.5), and a person dieing in the hospital prefered his house(52.9) over the hospital(47.1) as a funeral ceremony place. The following suggestions are made based on the above results. 1. A sampling method that enhances the re presentativeness should be used in regional and/or national related research and replicated to confirm the result of this study. 2. This study should be used to understand the Korean view of medical centers and to meet the expectations of patients in Korean nursing. 3. Research on the Korean traditional view of humans and expectations of the sick, health and illness, and health behavior, the perception of dying, the decision to heal, and the view of general medicine should continue to be conducted continuosly so that Korean nursing theory can be advanced on these concepts.

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