Kim, Min-Sun;Kim, Jiyeon;Noh, Eu Seon;Kim, Chiwoo;Cho, Sung Yoon;Jin, Dong-Kyu
Journal of mucopolysaccharidosis and rare diseases
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v.5
no.1
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pp.17-21
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2021
Hunter syndrome or mucopolysaccharidosis type II (MPS-II) (OMIM 309900) is a rare lysosomal storage disorder caused by deficiency in the activity of the enzyme iduronate-2-sulfatase. This enzyme is responsible for the catabolism of the following two different glycosaminoglycans (GAGs): dermatan sulfate and heparan sulfate. The lysosomal accumulation of these GAG molecules results in cell, tissue, and organ dysfunction. Patients can be broadly classified as having one of the following two forms of MPS II: a severe form and an attenuated form. In the severe form of the disease, signs and symptoms (including neurological impairment) develop in early childhood, whereas in the attenuated form, signs and symptoms develop in adolescence or early adulthood, and patients do not experience significant cognitive impairment. The involvement of the skeletal-muscle system is because of essential accumulated GAGs in joints and connective tissue. MPS II has many clinical features and includes two recognized clinical entities (mild and severe) that represent two ends of a wide spectrum of clinical severities. However, enzyme replacement therapy is likely to have only a limited impact on bone and joint disease based on the results of MPS II studies. The aim of this study was to review the involvement of joints in MPS II.
Objectives: Bone health in early adulthood, as individuals approach peak bone mass, plays a critical role in preventing osteoporosis later in life. This study aimed to investigate the associations between lifestyle and dietary factors, anthropometric measurements, and urinary bone resorption markers in young adults. Methods: A cross-sectional study was conducted with 100 healthy Korean adults (50 men and 50 women) in their 20s and early 30s. Bone mineral density (BMD), anthropometric measurements, dietary intake (24-hour recall), and urinary bone resorption indicators (deoxypyridinoline and N-terminal telopeptide of type I collagen) were analyzed. Variables were compared between the osteopenia and osteoporosis groups (OSTEO group: 30% men and 60% women) and the healthy control group. Results: Men in the OSTEO group were significantly taller than those in the control group (P < 0.05). Women in the OSTEO group had significantly lower body weight and body composition (muscle and body fat) than those in the normal group (P < 0.01). Men in the OSTEO group had a significantly higher intake of animal calcium (Ca) than those in the normal group (P < 0.05). Women in the OSTEO group had significantly higher dietary fiber, vitamin A, Ca, plant Ca, and potassium intake than did those in the normal group (P < 0.05). There were no significant differences in caffeinated beverage consumption, eating habits, or urinary bone resorption indicators between the OSTEO and control groups of either sex. Conclusions: In our study of young South Korean adults, we observed low bone density levels, with particularly low BMD in taller men and underweight women. We found a higher nutrient intake in the OSTEO group, indicating the possibility of reverse causality, a phenomenon often found in cross-sectional studies. Therefore, there is a need to further elucidate dietary factors related to osteoporosis in young adults through prospective cohort studies involving a larger population.
Nguyen, Hung Vu;Moschis, George P.;Shannon, Randall;Gotthelf, Kristian
Journal of Global Scholars of Marketing Science
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v.19
no.2
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pp.27-39
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2009
Compulsive consumption is regarded as a global phenomenon that can adversely affect consumer well-being. Although the topic has been studied in different cultural settings, we have seen relatively little theory development and explanations of compulsive behavior Nearly all previous empirical studies attempt to explain this behavior by correlating measures of compulsive behavior with independent variables taken within the same time frame. However, recent developments in social sciences suggest that such a phenomenon may best understood in the context of the person's earlier-in-life experiences. Using the life course paradigm as an overarching framework, the present research extends previous work on this topic. Following hypotheses were drawn from literature review: H1: The earlier in childhood and adolescence a person experiences family dislocation, the greater his or her likelihood of exhibiting compulsive behaviors in adulthood. H2: The earlier in life the young person experienced family dislocation, the greater the number of family disruption events the young person experienced prior entering adulthood years. H3: Family dislocation leads to (a) increased frequency of socio.oriented family communications and (b) decreased frequency of concept-oriented family communication. H4: Young adults who were raised in families characterized by a strong socio-oriented communication structure are more likely to exhibit compulsive consumption tendencies than those who were raised in families characterized by a weak socio-oriented family communication structure. H5: Young adults who were raised in families characterized by a strong concept-oriented communication structure are less likely to exhibit compulsive consumption tendencies than those who were raised in families characterized by a week concept-oriented family communication structure. H6: The relationship between family disruption events experienced during adolescence and perceived stressfulness of these events is moderated by (a) global family support, (b) emotional family support, and (c) material family support. Those reporting higher levels of family support as teenagers are less likely to report experiencing stress due to family disruption events. H7: Perceived stressfulness of family disruption events experienced during adolescent years are associated with compulsive consumption tendencies in early adulthood. H8: The greater the number of family disruption events young adults experienced during their adolescent years the more frequent was their communication about consumption with their peers. H9: The more frequent was the young persons' communication with their peers about consumption during their adolescent years, the more likely they are to report compulsive buying tendencies as young adults. We use a sample of 120 Thai undergraduate students attending classes taught in English as part of a four-year international program. Product-moment correlations, hierarchical regression analysis and partial correlation were used to analyze data. Results of testing hypotheses showed that hypothesis 2, 4, 7 and 9 were supported and hypothesis 1, 3, 5, 6 and 8 were not supported. Our study did not find a significant relationship between the age when a person experienced family dislocation and their compulsive behavior tendencies expressed as young adults. We did not find a significant relationship between family dislocation and family communication structures. But we found a significant positive relationship between socio-oriented communication structure and compulsive buying and a significant relationship between our peer communication and compulsive buying measures. Also we found perceived stressfulness due to the disruptive events to have a significant positive relationship between the perceived stressfulness and compulsive buying. Implications from these findings, limitations of this research and future research suggestions were discussed.
The family environment children are exposed to growing up greatly influences their future potential and achievements. Previous findings show that changes in family structure during childhood, particularly those resulting from divorce or death, cause lasting negative consequence that affect the child physically, psychologically, economically, and socially. Unfortunately, single-parent households are becoming increasingly common in Korea, nearly doubling to more than a million cases in the last two decades. Existing domestic and international studies of this area tend to focus on the short-term effects of growing up in a single-parent household. In addition, these studies group their samples in ways that result in findings that may be too broad or are not necessarily an accurate representation of the subjects. This study attempts to address some of these shortcomings by focusing on the long-term effects of how changes in family structure early in children's lives affect achievement during their transition to adulthood. In addition, it takes into account the development cycle the child is in at the time of family restructuring, and what kind of long-term effects result from that. In this analysis, we find that there are several cases of statistically significantly differences in domain achievement depending on the developmental stage the child was in when the parental divorce or death occurred. The findings indicate that changes in family structure during the infant/toddler period influence health condition and depression, while changes in family structure during middle-childhood and adolescence do not. Meanwhile, changes in family structure during any point in the developmental stages have negative effects on educational attainment, with the severity of these negative effects depending on when the family changes occur. The negative effect on educational attainment is most prominent when a change in family structure occurs during the infant/toddler period, followed by adolescence, then middle-childhood.
Journal of the Korea Academia-Industrial cooperation Society
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v.18
no.3
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pp.157-164
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2017
This study investigated the prevalence of scoliosis among undergraduate students who were in early adulthood, and to examine its relationship with body mass index (BMI), which has been associated with scoliosis in previous studies. A descriptive survey of 158 students of universities based in Seoul, Daejeon, Daegu, and Gyeonggi Province was conducted. A structured questionnaire comprising items pertaining to general features, postural features, and BMI was used, and the angle of the trunk rotation (ATR) was measured directly with a scoliometer to assess the spinal angle. Participants with an ATR of greater than $6^{\circ}$ were classified into the scoliosis group. Data were collected for three weeks from November 7, 2014. Scoliosis and BMI were analyzed for their frequencies and percentages, and their relationships were analyzed using the ${\chi}^2$-test and Logistic regression. Overall, 114 (72.2%) participants had an ATR of smaller than $5^{\circ}$, while 44 (27.8%) had an ATR of greater than $6^{\circ}$. After excluding the confounding variables, the overweight group was 2.63 times more likely than the normal BMI group to have an ATR of greater than $6^{\circ}$ which was statistically significant. However, the underweight group was 0.24 times less likely than the normal BMI group to have an ATR of greater than $6^{\circ}$, but this difference was not statistically significant. Although early examination and management of scoliosis in South Korea is generally performed on children and adolescents, the high prevalence of scoliosis among university students found in this study calls for aggressive early examination and management for this age group as well.
Journal of the Korean Academy of Child and Adolescent Psychiatry
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v.11
no.2
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pp.262-281
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2000
We focused on the peer relationship of adolescent patients with schizophrenia, which usually occurs around puberty. Reviewing cases with schizophrenia and the literature extensively, we had come to the conclusion as follows;1) the most robust predictors among factors influencing the prognosis of schizophrenia are premorbid interpersonal relationship and adaptive functions. 2) Especially teachers’ reports about school life and peer relationship during school life are useful for predicting the occurrence of schizophrenia in adolescents. We described characteristic and behavioral childhood features which are important in pathogenesis of schizophrenia, based on high-risk studies and long term follow-up studies. Also, pathological profiles of the interpersonal relationship and pathology in adulthood were presented. We tried to integrate various aspects of interpersonal and social weaknesses of schizophrenics applying 'primary and secondary socialization' concept. Finally, five cases of adolescent schizophrenics were described briefly and proposal for the early detection and intervention for risk factors was introduced.
Journal of the Korean Academy of Child and Adolescent Psychiatry
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v.28
no.2
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pp.84-95
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2017
Attention-deficit hyperactivity disorder (ADHD) is a neuropsychiatric disorder that begins in early childhood and can persist throughout adulthood. ADHD causes difficulties in various area of life, such as academic achievement, peer relationships, family functioning, employment and marriage. Although ADHD is known to respond well to medication, such treatment is more effective when combined with psychosocial (non-pharmacologic) therapy in terms of alleviating the core symptoms and improving appropriate functions. Psychosocial treatment interventions are divided into psychoeducation, behavioral parent training, school intervention, cognitive behavior therapy, social skill training, parent-child interaction therapy, play therapy, other treatments (coaching, complementary and alternative medicine), neurofeedback and Cogmed. Adult ADHD cognitive behavioral therapy is described separately. These practice parameters summarize the evidence for psychosocial treatment. Based on this evidence, specific recommendations are provided for psychosocial interventions.
Young adults are likely to start a new life style which may cause weight gain or obesity since leaving their parents for higher education or university. Whilst young people are slim in general, it is common to see them trying to manage their weight. Few studies have been carried out in Korea to support those changes in lifestyle that can result in weight gain in early adulthood. This study aims to identify prevalence of dieting as well as factors inducing weight gain among university students under unrestricted living conditions. A total of 99 university students were enrolled and asked to complete a questionnaire over a 3-day study period from June to September, 2008. T-test and $X^2$-test were applied to compare each behavior between weight gainers and maintainers. Logistic regression was also used for further analysis. Half of the participants used 'eat less' and 'exercise' as a way of dieting, and weight gainers tried to control their weight significantly more than weight maintainers. Most participants were engaged in very little exercise and were more likely to watch TV and use a computer. The time of lunch was proven as the only factor to predict weight change in the participants. As a result of this study, an intervention to promote exercise with friends or as a group is required to makeup for the lack of exercise among young adults. Further studies are also necessary to investigate how each meal time a day relates to the amount of food eaten. (Korean J Community Nutrition 14(4) : $374{\sim}382$, 2009)
Epidemiological evidence of the effects of dietary sodium, calcium, and potassium, and anthropometric indexes on blood pressure is still inconsistent. To investigate the relationship between dietary factors or anthropometric indexes and hypertension risk, we examined the association of systolic and diastolic blood pressure (SBP and DBP) with sodium, calcium, and potassium intakes and anthropometric indexes in 19~49-year-olds using data from Korean National Health and Nutrition Examination Survey (KNHANES) III. Total of 2,761 young and middle aged adults (574 aged 19~29 years and 2,187 aged 30~49 years) were selected from KNHANES III. General information, nutritional status, and anthropometric data were compared between two age groups (19~29 years old and 30~49 years old). The relevance of blood pressure and risk factors such as age, sex, body mass index (BMI), weight, waist circumference, and the intakes of sodium, potassium, and calcium was determined by multiple regression analysis. Multiple regression models showed that waist circumference, weight, and BMI were positively associated with SBP and DBP in both age groups. Sodium and potassium intakes were not associated with either SBP or DBP. Among 30~49-year-olds, calcium was inversely associated with both SBP and DBP (P = 0.012 and 0.010, respectively). Our findings suggest that encouraging calcium consumption and weight control may play an important role in the primary prevention and management of hypertension in early adulthood.
Background: Atrial septal defect (ASD) is the most common congenital cardiac anomaly, accounting for 30 percent of congenital heart disease detected in the adult. Many patients with ASD are well tolerated and reach adult without significant symptoms. The patients with ASD die 4th and 5th decades, but prolonged survival is not uncommon. In general, the survival depends on whether pulmonary hypertension develops during adulthood or not. The most common cause of death in the patients with ASD is right ventricular failure or arrhythmias. Materials and methods: From January 1988 to June 1997, 33 cases of ASD underwent open heart surgeries in our hospital. Among them, 31 cases were adult ASD, and 2 tricuspid regurgitation, 1 pulmonic stenosis, 1 mitral regurgitation, 1 tricuspid regurgitation, and 1 coronary artery disease were combinded. All of the patients underwent surgical repair using autologus pericardial patch or direct closure. Results: The postoperative course was smooth and uneventful. Most of the patients showed significant improvement in ECG finding, hemodynamic profile, radiologic finding, and echocardiography, after surgery. Conclusions: Conclusively, most of the ASD should be closed even in patients over the age of 60 years, and early surgical repair must be done to prevent pulmonary hypertension, right ventricular failure, and arrythmias.
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