Journal of The Korean Society of Inherited Metabolic disease
/
v.15
no.1
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pp.1-8
/
2015
Purpose: 3-methylcrotonyl CoA carboxylase deficiency (3MCCD) is leucine metabolic disorder caused by mutation in MCCC1 or MCCC2 gene. Clinical manifestations are variable, ranging from fatal neonatal onset to asymptomatic individuals. There is no retrospective study of Korean patients undergoing long-term treatment for 3MCCD. We reported this study to find out clinical symptoms and gene analysis of 3MCCD patients. Methods: This study was based on data of patients diagnosed with 3MCCD in Soonchunhyang university hospital between April 2009 and September 2013. We report clinical, enzymatic and mutation data of 3MCCD patients found by newborn screening. Results: In tandem mass spectrometry, 3-OH-isovalerylcarnitine (C5OH) of all patients increased. And all 7 patients were elevated 3-methylcrotonylglycine (3MCG) and 3-hydroxyisovaleric acid (3HIVA) in urine. MCCC mutation was identified in 2 patients and MCCC2 was mutated in 5 patients. We found mutation occurred in 8 different parts of nucleotide and such mutation caused 7 different types of changes in amino acid. All patients are on medication of L-carnitine and L-glycine. 4 patients are taking biotin. And 4 patients are eating leucine free formula. After starting treatment, there were no significant changes of urine 3MCG and 3HIVA levels. Conclusions: According to our data, MCCC2 gene mutation was more common than MCCC1 gene mutation. But the level of 3HIVA or 3MCG in urine has no correlation with phenotype. All patients has no symptoms and are shown normal development.
Kim, Hye-Young;Lee, Jung-Sug;Hwang, Ji-Yun;Kwon, Sehyug;Chung, Hae Rang;Kwak, Tong-Kyung;Kang, Myung-Hee;Choi, Young-Sun
Journal of Nutrition and Health
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v.50
no.2
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pp.142-157
/
2017
Purpose: The purpose of this study was to develop a nutrition quotient for adolescents (NQ-A) to assess overall dietary quality and food behavior of Korean adolescents. Methods: Development of the NQ-A was undertaken in three steps: item generation, item reduction, and validation. Candidate items of the NQ-A checklist were selected based on literature reviews, results of the fifth Korea National Health and Nutrition Examination Survey data, dietary guidelines for Korean adolescents, expert in-depth interviews, and national nutrition policies and recommendations. A total of 213 middle and high school students participated in a one-day dietary record survey and responded to 41 items in the food behavior checklist. Pearson's correlation coefficients between the responses to the checklist items along with nutritional status of the adolescents were calculated. Item reduction was performed, and 24 items were selected for the nation-wide survey. A total of 1,547 adolescents from 17 cities completed the checklist questionnaire. Exploratory factor and confirmatory factor analyses were performed to develop a final NQ-A model. Results: Nineteen items were finalized as the checklist items for the NQ-A. Checklist items were composed of five factors (balance, diversity, moderation, environment, and practice). The five-factor structure accounted for 47.2% of the total variance. Standardized path coefficients were used as weights of the items. The NQ-A and five-factor scores were calculated based on the obtained weights of the questionnaire items. Conclusion: Nutrition Quotient for adolescents (NQ-A) would be a useful instrument for evaluating dietary quality and food behavior of Korean adolescents. Further research on NQ-A is needed to reflect changes in adolescent's food behavior and environment.
Na, Hee Ra;Park, Eun Ju;Yang, Soo Jin;Cha, Youn-Soo;Lee, Min A
Journal of Nutrition and Health
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v.50
no.2
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pp.192-200
/
2017
Purpose: The purposes of this study were to analyze visitors' food needs and identify the Push-Pull factor in Jeonju Hanok Village. Methods: A questionnaire was developed based on previous research to survey Korean adults who visited Jeonju city. A total of 580 questionnaires were used for the analysis. Results: Most of the subjects who visited Jeonju Hanok Village had food purchase experiences in Jeonju Hanok Village (96.4%). 'Traditional Korean food (26.5%)' was the most purchased food, followed by 'foreign food (25.8%)' and 'Korean food combined with foreign food (16.8%)'. Satisfaction of food purchases (3.35 points) was higher than average. The primary reason for satisfaction was 'the food is delicious (23.0%)', and the reason for dissatisfaction was 'the food is expensive (48.1%)'. In the push and pull factor analysis for identifying visit motivation, 'local food seeking', 'experience seeking', 'relaxation seeking', and 'friendship seeking' were push factors while 'traditional culture', 'facility convenience', 'experience activity', and 'food experience' were pull factors. There was a significant correlation between the push and pull factors. Regression analysis showed that all push factors influenced satisfaction. However, among pull factors, only 'food experience' and 'traditional culture' influenced satisfaction. Conclusion: In conclusion, this study indicates that food-related factors are destination characteristics (Pull factor) influencing intrinsic visit motivation (Push factor) and satisfaction. It is necessary to develop various food tourism products to satisfy visitors' needs and continuous visits in Jeonju Hanok Village. This study suggests the importance of research on food factors and provides useful basic data to establish positioning strategies for food cultural development in Jeonju Hanok Village.
BACKGROUND: This experiment was conducted to investigate the distribution and burden characteristics of heavy metal in the rainwater sampled at Taean area, in the middle part of Korea, from April 2002 to October 2003. METHODS AND RESULTS: The relationship between concentration of heavy metal and other chemical properties in the rainwaters was also evaluated. Chemical properties in the rainwater were various differences with raining periods and years. It appeared that a weighted average pH values of rainwater was ranged from 5.0 to 5.1. Heavy metal concentrations in the rainwater were ranked as Pb > Zn > Cu > Ni > As > Cr > Cd. As compared with heavy metal concentrations of rainwater in 2002, Cu, Pb, and Zn were higher than other elements in 2003. There were positive correlation between major ionic components, such as ${NH_4}^+$, $Ca^{2+}$, $Mg^{2+}$, $K^+$, $Na^+$, ${SO_4}^{2-}$ and ${NO_3}^-$, and As, Cd, Zn, Cr, and Ni concentrations in rainwater. For heavy metal distribution of rainwater, the order of average enrichment factor was Cd > Pb > As > Cu > Zn > Ni > Cr, and these were relatively higher than the natural components such as Fe, Mg and Ca. The monthly enrichment factor were relatively high, from August to October at Taean. The monthly amount of heavy metal precipitation was high in the rainy season from July to August because of great influence of rainfall. CONCLUSION(s): The results of this study suggest that the heavy metals(Cd, Pb, As, Cu, and Zn) of rainwater is strongly influenced by anthropogenic sources rather than natural sources.
Purpose: The purpose of this study is to provide baseline data for the development of an educational program for hospice care for college students. Methods: A survey was conducted at three universities in B city with 143 students from September 5, 2014 through September 26, 2014. The data were analyzed by the SPSS 18 program. This study was approved by IRB. Results: The college students' spiritual well-being was at a medium level. Their attitudes to death were neutral, and perception of hospice care was at a medium level. Most of the participants (93.0%) had no training in hospice care. The participants' awareness of the purpose of hospice care was at a relatively high level. Their awareness of the need for hospice care was at a moderate level. The participants' spiritual well-being and their attitudes to death showed a weak but positive correlation (r=0.179, P=0.030). The relationship between their spiritual well-being and awareness of hospice care were positively correlated (r=0.203, P=0.015). Conclusion: The participants' perception of hospice care was low. Most of them had no experience of hospice care education. Also, the higher the spiritual stability was, the higher the participants' perception of the purpose and the necessity of hospice care was. And their perception of the hospice care varied depending on their family relationship, satisfaction with school life, and cognition of hospice care. Therefore, we need consider these variables to develop a hospice education program to enhance college students' attitudes to death and their perception of hospice care.
Kim, Eun-Young;Kim, Jung-Hyun;Lee, Nam-Yun;Kim, Soo-Jeong;Rhyu, Mee-Ra
Korean Journal of Food Science and Technology
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v.35
no.5
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pp.818-824
/
2003
Capillary electrophoresis (CE) and near-infrared spectroscopy (NIRS) were performed to discriminate astragalus roots (Astragalus membranaceus) according to geographical origin (domestic or foreign). Two-hundred-and-four astragalus roots were extracted with 30% methanol in 0.1 M phosphate buffer (pH 2.5) and separated in a uncoated fused-silica $(50\;{\mu}m{\times}27\;cm)$ capillary. Conditions for optimal analysis included: temperature $-45^{\circ}C$, voltage -14 kV, and pressure injection time -8 sec. The optimal separation buffer was 0.1 M phosphate buffer (pH 2.5) containing 40 mM hexane sulfonic acid with 20% 2-methoxy ethanol. Raw NIR spectra were obtained using NIRS, and modified partial least square regression was used to develop the prediction model. The correlation coefficient and standard error of prediction were 0.915 and 14.3%, respectively. Under the optimal conditions established for CE and NIRS, the geographical origins of the astragalus roots were correctly identified in 80 and 97%, respectively. Astragalus roots that were not discriminated by NIRS were correctly discriminated by CE. Hence, CE and NIRS are potential methods for discriminating the geographical origins of astragalus roots that complement one another.
Objecives : The purpose of the study was to evaluate treatment outcome of mandibular advancement device(MAD) in obstructive sleep apnea (OSA) patients using full night polysomnography and cephalometry. Methods : Twenty-seven OSA patients were confirmed by full night, lab-attended polysomnography. Cephalometric examinations were conducted to obtain SNA, ANB, $AH{\bot}MP$, AH-C3, SPT, PNS-U, NAS, SOAS, MOAS, and HAS. Mandibular advancement devices (MADs) were fabricated and delivered for all subjects. After acclimation period of MAD, the second polysomnographic examinations were conducted in the same manner. Polysomnographic variables were compared between before and after MAD placement. Also, correlation coefficients were calculated between apnea-hypopnea index (AHI) and each item of cephalometric parameters. Results : There were significant improvements in total AHI, lowest $SpO_2$, and total arousal index after MAD therapy. Also, there were significant improvements in NREM $SpO_2$ and NREM AHI, but not in REM $SpO_2$ and REM AHI with MAD. Stratifying the sleep stage, there were significant decrease in stage I and significant increases in stage II and REM, but change in stage III was not significant. SNA and SOAS were significantly correlated with total AHI and NAS was significantly correlated with supine AHI. ANB was significantly associate with the effect of MAD. Conclusions : MAD is an effective treatment in OSA patients comparing polysomnographic variables before and after treatment. Cephalometric examination can be useful to evaluate OSA patients and predict treatment outcome of MAD.
Purpose: The aim of this study was to compare the film thicknesses of several resin cements as a function of time after mixing and to examine the effect of working time on the film thicknesses. Materials and methods: The film thickness (${\mu}m$) of 4 resin cements (n=10), 1 composite resin (Panavia F 2.0), 3 self-adhesive resin (Clearfil SA luting, Zirconite, RelyX U200) cements was measured at 20-second intervals after mixing of the cements up to 200 seconds under a load of 50 N. Linear regression was fitted to verify the effect of working time on the film thickness of each cement. Data were compared to the working time recommended by manufacturers using Wilcoxon test ($\alpha$=.05). Results: All of the materials showed a positive linear correlation between the film thickness and working time. There was no statistically significant difference between the working time based on our results and the values recommended by the manufacturers even though there was a discrepancy between those two values. Conclusion: The film thickness of resin cements could increase with the increase of working time. Working time to meet the ISO standard of $50-{\mu}m$ maximum film thickness could be different from the manufacturer's recommended value.
Objectives : Patients with obstructive sleep apnea syndrome(OSAS) often complain of nocturnal enuresis. There are a few reports that OSAS patients have altered renal function, and there are some evidences that the increased release of atrial natriuretic peptide(ANP) may be involved in the pathogenesis of nocturnal urinary symptoms of OSAS patients. In this study, we measured plasma ANP concentrations during waking and sleep in OSAS patients and normal controls to investigate whether there were differences of ANP concentrations between OSAS patients and normal subjects. Methods : 27 patients with OSAS and 10 normal subjects were studied. All subjects underwent a full-night polysomnographic study. Venous blood samples were separately drawn during waking and sleep. Plasma ANP concentrations were measured using radioimmunoassay. Results : In OSAS patients, ANP concentrations during sleep($122.9\;{\pm}\;29.9pg/ml$) were significantly higher than ANP concentrations during waking($60.2\;{\pm}\;5.8pg/ml$)(p < 0.05). However, in normal subjects, there was no significant difference between ANP concentrations during waking($59.2\;{\pm}\;5.7pg/ml$) and sleep($69.6\;{\pm}\;3.0pg/ml$)(p > 0.05). There was no significant difference of ANP concentrations during waking between OSAS patients($60.2\;{\pm}\;5.8pg/ml$) and normal controls($59.2\;{\pm}\;5.7pg/ml$)(p > 0.05), and also there was no significant difference during sleep between OSAS patients($122.9\;{\pm}\;29.9pg/ml$) and normal subjects($69.6\;{\pm}\;3.0pg/ml$)(p > 0.05). Plasma ANP concentrations during sleep showed significant positive correlations with apnea index(r = 0.3846, p < 0.05) and respiratory disturbance index(r = 0.3939, p < 0.05) in OSAS patients. Conclusion : These data suggest that, in OSAS patients, plasma ANP concentrations during sleep are significantly higher than plasma ANP concentrations during waking, and there is a positive correlation between the plasma ANP concentration during sleep and the severity of sleep apnea.
Kim, Dae-Wook;Kim, Sung-Gon;Kim, Ji-Hoon;Yang, Young-Hui;Jung, Woo-Young;Lee, Jin-Seong
Sleep Medicine and Psychophysiology
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v.20
no.2
/
pp.69-74
/
2013
Introduction: Although it is well known that medical students are not getting an adequate amount of sleep, there have been only a few studies on the sleep patterns of medical students and the related factors. Therefore, the present study aimed to investigate the medical students' sleep patterns and the related factors. Methods: A questionnaire package was administered to the $1^{st}$ to $4^{th}$ year medical students at one medical school. It consisted of questions asking about their lifestyles as well as Pittsburgh sleep quality index (PSQI), Epworth sleepiness scale (ESS), global assessment of recent stress scale (GASS), the center for epidemiologic studies-depression scale (CES-D), and Moudsley obsessive-compulsive inventory (MOCI). A total of 352 students (206 males and 146 females) responded to the survey and the result was analyzed using the independent t-test, the chi-square test, the paired t-test, Pearson's correlation and ANOVA. p-values of less than 0.05 were considered statistically significant in analyses. Results: The weekend bedtime was significantly delayed (0 : 49 on weekday ; 1 : 34 on weekend ; t=-5.23, p<0.001), the weekend rise time was delayed (6 : 58 on weekday ; 9 : 30 on weekend ; t=-24.48, p<0.001) and the total sleep time was increased on weekends (5 : 36 on weekday ; 7 : 39 on weekend ; t=15.94, p<0.001). The PSQI score of all subjects was 6.43{\pm}2.64. PSQI was positively correlated with ESS (r=0.383, p<0.001), GASS (r=0.326, p<0.001), CES-D (r=0.393, p< 0.001), and MOCI (r=0.247, p<0.001), but not with GPA (r=0.072, p=0.228. The more senior students had lower PSQI, GASS, CES-D, and MOCI score (p<0.05). Conclusion: Medical students were experiencing a lack of sleep during weekdays as they have a later bedtime and earlier rise time, and consequently had more hours of sleep on weekends. Overall, the medical students were experiencing poor sleep quality and sleep deprivation. Poor sleep quality is associated with psychological problems (daytime sleepiness, stress, depression, and obsessive tendency).
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