• 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)

• Journal of Nutrition and Health
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• v.42 no.6
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• pp.536-546
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• 2009

This study was done to investigate the effect of stress on appetite and eating habits, and other health-related behaviors. The subjects of this study consisted of 188 males and 224 females in Ulsan area. The results were as follows: When stressed, 56% (n = 231) of the subjects experienced a change in appetite and of these, 32% (n = 132) experienced an increased appetite. Stress-induced eating may be one factor contributing to the development of obesity. There was a gender-specific response to stress in which women are more likely to use food to deal with stress, whereas men are more likely to use alcohol consumption or smoking. It was found that types of stressors were individual (52.9%), social (50.7%), family relations (34.5%), work demands (34.2%) and physical environment (32.3%). Stress-induced symptoms of the subjects were anxiety (38.3%), headache (36.7%) and neck or shoulder aches (36.2%), and females experienced those symptoms more than males. Those older than 50 years had a higher eating habit score and lower stress score compared with younger subjects. There were significant differences between sex, age, occupation, family type, BMI, exercise, sleeping hours and eating habits or stress level. This study may be helpful in advancing findings in this area to better provide health professionals with appropriate counseling tools to improve the health of all individuals.

• Journal of Korean Academy of Nursing Administration
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• v.2 no.1
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• pp.35-56
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• 1996

Nowerdays, the increase of traffic accidents and old age population make the Femoral Neck Fracture(FNF) patients increase. By the improvement of education and standard of living the patients demand better medical service than before. This study is designed to give practical help for the FNF patients by observing their hospital life and establish practical nursing strategies for the FNF patients. For these purposes the Ethnographic Participant Observation was adopted. By this study is focused on the hospital life patient's view. For this end, the field study adopted orthopedic ward in the C University Hospital with 400 beds in Seoul. The object patients of the study were twelve patients. The patients experienced five stages : Embarrassment, Conflict, Stability, Independent, and Extension Stage. The findings and prepared nursing strategies are stated as follows. First, in the Embarrassment Stage they suffered embarrassment, anxiety, pain, they could not do ordinary things. The patients who accidental fractures had anxiety from unfamiliar tests and from hospitalization itself. They lamented that they could not ordinary things, and do nothing but obeying the hospital, and endure the pain. They recognized the changed environment and resigned themselves to life in the ward. In this stage, full openness by the nurses is needed. Second, the attribute of the Conflict Stage were conflict, fear, curiosity, belief, reflection. When they sign the consentment form, they experience conflicts about the possibility of complication, fear of recovery from anesthesia, curiosity about the operation procedure, post - operation state, reflection on their past life, and promise to care for their family members after discharge and keep their religious life faithfully. And they accepted the operation depending on God, believing in modern medicine, and the surgeon. Asking for their changed informations, they expected positive results from the operation. In this stage, an empathic attitude by the nurses is needed. Third, the attribute of the Stability Stage were relief, gratitude, difficulty with excretion, and pain. When they awoke from anesthesia, they felt relief because of a the end of the operation, but they experienced extreme pain, difficulty of excretion in bed. They accepted the changed environment and expected recovery. In this stage, support by the nurses is needed. Fourth, the attributes of the Independence Stage were freedom, exercise, nurturing, anxiety, and discomfort. When they ambulated and exercised, they experienced freedom. They showed exhibited weakness of the digestive organs and discomfort hospital's space, structure, and facilities, the delay of medical certificate issue the lack of prompt response by the medical agents. They ate nurturious food and felt anxiety on the end of hospital life and returning to their ordinary life. They showed the independence of overcoming their environment by increasing exercise and expected their discharges. In this stage, respect by the nurses is needed for the patients to, overcome their environment and prepare for their independence. Fifth, the attributes of the Extension Stage were pessimism, isolation, dissatisfaction, and pain. Accompanied injury and old age made their ward life extend to over seven weeks. They exhibited weariness, melancholy, skeptisis, general pessimistic feeling, and desperation caused by their isolated life. They experienced the digestive discomfort caused by the prolonged medication and psycological pain caused by long-time hospitalization. As a, result, their dissatisfaction on the human, physical, and systematic environments had been increased. They acquired critical power and sought for something to do spending their time. They expected vaguely about the returning of their ordinary life. In this stage, counseling is needed by the nurse to overcome positively their psychological, social, and physical problems. The process of the FNF patient's ward life starts from the dependent state, when they are hospitalized, and gradually progresses to self-fulfillment in order to keep independent life. As a result, the FNF patients showed "Response in Challenge" or "Adaptation in Conflict" through their experiences of social, physical, and psychological difficulties.

• Journal of Genetic Medicine
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• v.4 no.2
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• pp.133-141
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• 2007

Purpose : Cri-du-Chat syndrome (CdCs) is a rare but clinically recongnizable condition with an estimated incidence of 1:50,000 live births. The clinical characteristics of the syndrome include severe psychomotor and mental retardation, microcephaly, hypertelorism, hypotonia, and slow growth. Also the size of the chromosome 5p deletion ranges were known from the region 5p13 to the terminal region. In this study, we report the spectrum of 5p deletion in Korean 20 pts. with CdCs and genotype-phenotype associations in CdCs. Methods : In order to delineate genotype-phenotype correlation, molecular cytogenetic studies including GTG banding and clinical characterization were performed on Korean 20 pts with CdCs including parents. CGH array and Fluorescence in situ hybridization (FISH) analysis were used to confirm a terminal deletion karyotype and map more precisely the location of the deletion breakpoint. Results : Molecular analysis of the spectrum of 5p deletion revealed 9 pts (45%) with a del (5)(p14), 7 pts. (35%) a del (5)(p13), 3 pts. (15%) a del (5)(p15.1) and 1 pt. (5%) a del (5)(p15.2) in 20 pts with CdCs. 4(20%)pts were identified to have additional chromosome abnormalites of deficiency and duplication involving chromosomes of 6, 8, 18, & 22. Parental study identified 3 familial case (2 paternal and 1 maternal origin) showing parents being a balanced translocation carrier. And the comparison study of the deletion break points among these 20 pts. with their phenotype has showed the varying clinical pheno-types in the CdCs critical region. Conclusion : The characterization of 5p deletion including parental study may help to delineate the genotypephenotype correlation in CdCs. Also these molecular cytogenetic analyses will be able to offer better information for accurate genetic diagnosis in CdCs and further make possible useful genetic counseling in pts. and family.

• Clinical and Experimental Pediatrics
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• v.51 no.6
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• pp.576-583
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• 2008

Purpose : The identification of specific behaviors conducive to overeating or inactivity is the cornerstone of obesity management. The Committee on Nutrition of the Korean Pediatric Society developed parent and self-reporting questionnaires about eating behavior and physical activity in 2006. The aim of this study was to evaluate the usefulness of the questionnaires in assessing modifiable lifestyle factors related to obesity. Methods : A retrospective chart review was performed for 177 children (6-11 years old) and 134 adolescents (12-16 years old) from 10 hospitals between May 2006 and January 2007 who had completed parent or self-reporting questionnaires. Cases were divided into normal and overweight groups at or above the age-gender-specific 85th percentile based on 2007 Korean national growth charts. Results : Compared to children, the adolescents tended to have a significantly more sedentary lifestvle and inappropriate dietary behaviors significantly (P<.05). Overweight mothers were significantly associated with overweight children and adolescents (P<.05). Being overweight was significantly associated with a family history of adult diseases for children and adolescents (P<.05). Inappropriate eating behaviors (strong appetite, eating fast, eating until they were full, binge eating, favoring greasy foods) were associated with being overweight in children and adolescents. Sedentary activity such as TV viewing and using a computer were significantly associated with overweight in children and adolescents (P<.05). Conclusion : Intervention to modify obesity-related lifestyle factors is needed before adolescence. These questionnaires are useful in identifying modifiable lifestyle factors and in individual counseling for overweight children and adolescents in pediatric clinics.

• The Korean Journal of Elementary Counseling
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• v.5 no.1
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• pp.159-184
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• 2006

Lately, the rapid social and family system change, the entrance examination-centered circumstance in education have a negative influence not only on making up the affirmative self-esteem but also on building up the personal relationship through mutual communication with friends of the same age. Making children adapt well to school life and develop a good relationship in the class is an important subject in educational field. Thus, various school activities should offer lots of opportunities to increase their affirmative self-esteem and more specified performance-centered programs are required toform a harmonious relationship among parties of students. The purpose of this study is to improve an affirmative self-esteern and a desirable personal relationship of children through developing friend-praise program and adapting it to children. Based on the above purpose, the following subjects for this study 'are suggested. Firstly, is the friend-praise program effective in forming an affirmative self-esteem of elementary students? Secondly, is the friend-praise program successful in improving a desirable personal relationship in an elementary school? In addition, two hypotheses of study are decided on the basis of theoretical background and the former study. Hypothesis 1. The friend-praise program will have a meaningful effect on improving an affirmative self-esteem. Hypothesis 2. The friend-praise program will have a meaningful effect on improving a desirable personal relationship. In order to improve the above hypotheses, the experiment was carried out with two classes on the fourth grade in M elementary school in Wonju with being divided into one experimental group and the other comparative group. The friend-praise program which was adapted to the experimental group was composed with four steps as 'introduction', 'preparation', 'operation' and 'closing'. This program which has twelve categories was executed once or twice in a week and each class was continued for forty or sixty minutes. Contrastively, comparative group had no application of the main program. For the purpose of proving the hypotheses after adapting the program to students, I have executed both self-esteem test and personal relationship test. The tests were performed with using SPSS/Windows V10.0 and the statistic was conducted through independant sample t-test for the difference between groups and contrastive sample t-test for the individual difference in each group. The results of this study can be summarized like the followings: Firstly, from the result of previous and after tests, there was no meaningful change in the comparative group, but the experimental group showed meaningful difference in all categories of self-esteem(t=-4.496, p=.000). Especially, the general self-esteem(t=-3.216, p=.003), social self-esteem(t=-2.680, p=.012), homely self-esteem(t=-3.732, p=.001), and school self-esteem(t=-3.902, p=.000) showed a meaningful difference. At the same time, the experimental group also showed meaningful difference with the comparative group in self-esteem(t=-4.758, p=.000). Especially, the general self-esteem(t=2.581, p=.017), social self-esteem(t=3.160, p=.003), homely self-esteem(t=4.283, p=.000), and school self-esteem(t=4.110, p=.000) showed a meaningful difference. As a result of this experiment, the friend-praise program will have a meaningful effect on improving an affirmative self-esteem was proved. Secondly, there was no meaningful change in the comparative group, but the experimental group showed meaningful difference in all categories of personal relationship(t=-4.131, p=.000). Specifically, satisfaction(t=-2.113, p=.045), communication(t=-3.381, p=.002), confidence(t=-3.517, p=.001), intimacy(t=-3.958, p=.000), sensibility(t=-2.955, p=.006), openness(t=-4.318, p=.000) and interest(t=-2.941, p=.000) showed a meaningful difference. In the same instant, the experimental group also showed meaningful difference with the comparative group in all categories of personal relationship(t=3.897, p=.000). Especially, satisfaction(t=2.257, p=.003), communication(t=3.527, p=.001), confidence(t=3.704, p=.001), intimacy(t=3.904, p=.000), sensibility (t=4.382, p=.000), openness(t=2.648, p=.013) and interest(t=2.944, p=.006) showed a meaningful difference. Accordingly, the friend-praise program will have a meaningful effect on improving a desirable personal relationship was proved. Judging from all these results, we should provide primary students with various opportunities to take part in active programs which help them to set up their righteous sense of value, to solve their own problems and to develop their potentials. Consequently, many kinds of practice-centered program like the friend-praise program should be developed more systematically and teachers should apply all those programs to students according to their individual level and developmental stage.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.96-102
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• 2017

Glycogen storage disease type IX (GSD IX) is caused by deficiency of phosphorylase kinase which plays a role in breakdown of glycogen. Mutations in PHKA2 are the most common cause of GSD IX (GSD IXa). Clinical manifestations of GSD IXa include hepatomegaly, elevation of liver enzyme, growth retardation, fasting hypoglycemia, and fasting ketosis. However, the symptoms overlap with those of other types of GSDs. Here, we report Korean familial cases with GSD IXa whose diagnosis was confirmed by targeted exome sequencing. A 4-year old male patient was presented with hepatomegaly and persistently elevated liver enzyme. Liver biopsy revealed swollen hepatocyte filled with glycogen storage, suggesting GSDs. Targeted exome sequencing was performed for the differential molecular diagnosis of various types of GSDs. A hemizygous mutation in PHKA2 were detected by targeted exome sequencing and confirmed by Sanger sequencing: c.3632C>T (p.Thr121Met), which was previously reported. The familial genetic analysis revealed that his mother was heterozygous carrier of c.3632C>T mutation and his 28-month old brother had hemizygous mutation. His brother also had hepatomegaly and elevated liver enzyme. The hypoglycemia was prevented by frequent meals with complex carbohydrate, as well as cornstarch supplements. Their growth and development is in normal range. We suggest that targeted exome sequencing could be a useful diagnostic tool for the genetically heterogeneous and clinically indistinguishable GSDs. A precise molecular diagnosis of GSD can provide appropriate therapy and genetic counseling for the family.

• Journal of Hospice and Palliative Care
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• v.13 no.4
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• pp.216-224
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• 2010

Purpose: The purpose of this study was to evaluate a community-based cancer patient management program (CBPCMP) which was collaborated between a hospice center and public health centers. Methods: The CBPCMP proceeded on four steps; 1) Signing agreements with three public health centers, 2) Enrolling the domiciliary terminal cancer patients, 3) Providing home hospice service, and 4) Inquiring patient's level of satisfaction. From February 1 to December 31 in 2009, 43 terminal cancer patients were referred and provided with home hospice service. The hospice team made a total of 605 visits. Medical records for each visit and data from satisfaction surveys were analyzed. Results: 76.7% of patients were older than 60 years, and 90.7% of the patients were alert. The level of functional status for 76.7% of patients rated as lower than ECOG grade 1. 62.8% of the patients or their caregivers signed hospice service agreements. On the initial evaluation, the most frequent reasons for referral were general weakness (86.0%), followed by anorexia (72.1%). Nurses visited the patients' most frequently (371 visits), followed by volunteers (216 visits). Nurses provided emotional support and health promotion counseling on 95.1% and 22.9% of visits, respectively. The mean satisfaction score rated by patients and their family was 4.45 out of 5. Conclusion: This study tested CBPCMP in collaboration with hospice centers and public health centers. CBPCMP showed a possibility to improve the quality of end of life care. To insure the quality care, however, the guidelines for home hospice service should be developed.

• Journal of Korean Public Health Nursing
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• v.16 no.2
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• pp.354-377
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• 2002

Aging population is a global trend and Korea is no exception. Due to the progress of the medical and scientific sectors, increase in the average lifetime incurred increase in the number of elderly people. Meanwhile, the family function of supporting the elderly has become a major issue in a society due to the changes in the society's structure and increased entry of the women into the workforce. As a means to substitute the protection of the elderly who are not protected at their respective homes and to replace the tending needs, weekly protection centers and short-stay centers for the elderly were established and operated in Seoul since 1992. However, structured and satisfactory services are not yet to be provided, thus this research intends to understand the current situation at the short-stay centers for the elderly, to identify users' characteristics and to conduct study on nursing need for the health reasons so that the results of the research can be utilized as basic data for the development of nursing care program. Towards this end, this research targeted 142 elderly people who were undergoing treatment at the 11 short-stay centers for the elderly out of total of 13 located in Seoul that were willing to cooperate. The research was conducted from April to May 2002, with the measurement tool developed by Rhee, Seon-ja (2001) to measure the level of nursing need. Then data was collected and analyzed using SPSS program. The results are as follows. The general characteristics of users of the short-stay centers for the elderly were: 123 females $(86.6\%)$, 19 males $(13.4\%)$ and 72 elderly were over the age of 80 $(50.7\%)$. Among these, 24$(16.9\%)$ were living by themselves. Among the illnesses that the subjects were inflicted with were: dementia $(54.2\%)$, stroke $(54.1\%)$ and high blood pressure $(50.7\%)$, in the respective order. Despite the fact that they were diagnosed with illnesses, $47.2\%$ responded that they do not undergo special treatment due to financial reasons. When the level of needs for nursing care among the elderly who use the short-stay centers is divided into informational. physical. and emotional need levels and scored between zero to two, the level of emotional need is the highest with 1.80, followed by physical (1.45) and informational (1.44) need levels. As for the realities of services at the centers and improvement areas, restriction on term was cited as the greatest inconvenience with $38.7\%$, followed by lack of budget (24 respondents, $16.9\%$). Wishful services were financial assistance $(46.5\%)$, medical and nursing services $(40.1\%)$, meal provision service $(8.5\%)$ and counseling service $(4.9\%)$, in the respective order. Based on the above mentioned results, the research identified the need to develop nursing program for the patients of dementia that can be applied onto the short-stay centers and the need to develop volunteer service, backed by societal support. Also, placement of specialized cadre of professionals is needed so that medical and nursing service functions can be provided in a complementary manner.

• Journal of Intelligence and Information Systems
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• v.16 no.3
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• pp.181-199
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• 2010

As rapid progress of population aging and strong interest in health, the demand for new healthcare service is increasing. Until now healthcare service has provided post treatment by face-to-face manner. But according to related researches, proactive treatment is resulted to be more effective for preventing diseases. Particularly, the existing healthcare services have limitations in preventing and managing metabolic syndrome such a lifestyle disease, because the cause of metabolic syndrome is related to life habit. As the advent of ubiquitous technology, patients with the metabolic syndrome can improve life habit such as poor eating habits and physical inactivity without the constraints of time and space through u-healthcare service. Therefore, lots of researches for u-healthcare service focus on providing the personalized healthcare service for preventing and managing metabolic syndrome. For example, Kim et al.(2010) have proposed a healthcare model for providing the customized calories and rates of nutrition factors by analyzing the user's preference in foods. Lee et al.(2010) have suggested the customized diet recommendation service considering the basic information, vital signs, family history of diseases and food preferences to prevent and manage coronary heart disease. And, Kim and Han(2004) have demonstrated that the web-based nutrition counseling has effects on food intake and lipids of patients with hyperlipidemia. However, the existing researches for u-healthcare service focus on providing the predefined one-way u-healthcare service. Thus, users have a tendency to easily lose interest in improving life habit. To solve such a problem of u-healthcare service, this research suggests a u-healthcare recommender system which is based on collaborative filtering principle and social network. This research follows the principle of collaborative filtering, but preserves local networks (consisting of small group of similar neighbors) for target users to recommend context aware healthcare services. Our research is consisted of the following five steps. In the first step, user profile is created using the usage history data for improvement in life habit. And then, a set of users known as neighbors is formed by the degree of similarity between the users, which is calculated by Pearson correlation coefficient. In the second step, the target user obtains service information from his/her neighbors. In the third step, recommendation list of top-N service is generated for the target user. Making the list, we use the multi-filtering based on user's psychological context information and body mass index (BMI) information for the detailed recommendation. In the fourth step, the personal information, which is the history of the usage service, is updated when the target user uses the recommended service. In the final step, a social network is reformed to continually provide qualified recommendation. For example, the neighbors may be excluded from the social network if the target user doesn't like the recommendation list received from them. That is, this step updates each user's neighbors locally, so maintains the updated local neighbors always to give context aware recommendation in real time. The characteristics of our research as follows. First, we develop the u-healthcare recommender system for improving life habit such as poor eating habits and physical inactivity. Second, the proposed recommender system uses autonomous collaboration, which enables users to prevent dropping and not to lose user's interest in improving life habit. Third, the reformation of the social network is automated to maintain the quality of recommendation. Finally, this research has implemented a mobile prototype system using JAVA and Microsoft Access2007 to recommend the prescribed foods and exercises for chronic disease prevention, which are provided by A university medical center. This research intends to prevent diseases such as chronic illnesses and to improve user's lifestyle through providing context aware and personalized food and exercise services with the help of similar users'experience and knowledge. We expect that the user of this system can improve their life habit with the help of handheld mobile smart phone, because it uses autonomous collaboration to arouse interest in healthcare.