Journal of The Korean Society of Inherited Metabolic disease
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v.18
no.3
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pp.78-86
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2018
Purpose: We aimed to delineate clinical spectrum and short-term effects after enzyme replacement therapy (ERT) for 5 mucopolysaccharidosis type II (MPS II). Methods: Five patients were diagnosed with MPS II by clinical findings, enzyme activity, and genetic testing. Idursulfase was administered by intravenous infusion at a dose of 0.5 mg/kg every week. Observational chart analysis of patients, who underwent systematic investigations more than 12 months after initiation of ERT was done retrospectively. Results: Three patients were classified as having the attenuated type, and 2 patients were classified as having the severe type. The median age at the diagnosis was 9.6 years (range 3.4-26 years). Four different mutations in 5 Korean patients (4 families) with MPS II were identified, among which two were novel mutations (1 small insertion mutation: p.Thr409Hisfs*22, and 1 missense mutation: p.Gly134Glu). Two severe type sibling patients with the same mutation had different clinical manifestation. Urinary glycosaminoglycan excretion decreased within the twelve months of ERT (P=0.043). Liver and spleen volumes showed reductions that were maintained in all patients (P=0.043 and P=0.043, respectively). Improvements were also noted in left ventricular mass index (P=0.042), shoulder flexion (P=0.043), shoulder abduction (P=0.039), knee flexion (P=0.043), elbow flexion (P=0.042), and respiratory distress index (P=0.041). Conclusion: This study demonstrates that Korean patients with MPS II are clinically heterogeneous and indicates that idursulfase is relatively effective in several clinical parameters including heart size and respiratory distress index without infusion-related reactions in patients with MPS II.
Purpose: This study was designed to determine the relationship between health behaviors and high levels of low-density lipoprotein-cholesterol (LDL-cholesterol) according to cardiovascular risk factors among Korean adults. Methods: This cross-sectional study was based on the sixth Korea national health and nutrition examination survey (KNHANES VI). Participants were 13,841 adults aged 19 years and older. Cardiovascular risk factors were stroke, myocardial infarction or angina, diabetes mellitus, smoking, hypertension, aging, high density lipoprotein-cholesterol (HDL-cholesterol) under 40 mg/dL and HDL-cholesterol over 60 mg/dL. Cardiovascular risk groups were classified as very high risk (stroke, myocardial infarction or angina), high risk (diabetes mellitus), moderate risk (over 2 risk factors), and low risk (below 1 risk factor). The prevalence of high LDL-cholesterol was calculated using the LDL-cholesterol target level according to cardiovascular risk group. Results: The prevalence of high LDL-cholesterol was 25.5% in males and 21.7% in females. Complex sample cross tabulation demonstrated that the high LDL-cholesterol and normal groups differed significantly according to age, education, body mass index, percentage of energy from carbohydrate, fat, saturated fat and n-6 in males and females. These two groups were also significantly different according to smoking in males and the percentage of energy from n-3 in females. Complex sample multiple logistic regression analysis adjusted for multiple confounding factors demonstrated that the probability of high LDL-cholesterol was significantly associated with current smoking (OR: 1.66, 95% CI: 1.40-1.99), obesity (OR: 1.95, 95% CI: 1.64-2.31) in males, and current smoking (OR: 1.73, 95% CI: 1.19-2.52), obesity (OR: 1.63, 95% CI: 1.39-1.90), percentage of energy from n-3 (quartile 1 vs. quartile 2; OR: 0.77, 95% CI: 0.62-0.96; quartile 1 vs. quartile 3; OR: 0.73, 95% CI: 0.56-0.94; quartile 1 vs. quartile 4: OR: 0.67, 95% CI: 0.51-0.87) in females. Conclusion: This study reveals the impact of smoking, obesity, energy percentage of nutrient intake on LDL-cholesterol.
Jeon, Hansol;Ryu, Seung-Ho;Ha, Jee Hyun;Jeon, Hong Jun;Park, Doo-Heum
Sleep Medicine and Psychophysiology
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v.25
no.2
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pp.68-73
/
2018
Objectives: The purpose of this study was to explore insomniac demographic characteristics and the type of consultation provided to hospitalized patients asked to the Department of Psychiatry for insomnia and to compare patient insomnia characteristics by consultation type. Methods: We performed a retrospective chart review of 4,966 patients who were hospitalized from August 1, 2005 to December 31, 2011 that received consultation in the Department of Psychiatry. Among them, 236 patients were referred for insomnia. We compared the differences in demographic characteristics and types of consultation between the insomnia patient group and other patient group. We also compared the difference between demographic characteristics and type of consultation by dividing total subjects into 'with reconsultation' and 'without reconsultation' groups. Results: Our results came from the analysis of 9,689 consecutive consultation requests. There were 4,966 patients that participated in the study over 6 years and 6 months. The overall consultation rate was 3.3% of all admissions and insomnia patients comprised 4.8% of those. The ratio of re-consultation for insomnia was 27.5%. There was no significant difference in mean age between the insomnia 'with reconsultation group' and the insomnia 'without reconsultation group', but the 'with reconsultation' group had significantly more male patients and medical patients than the 'without re-consultation' group. For insomnia patients, consultation types were in the order of Mending request (51.3%), Paralle request (36.6%), Complementary request (9.0%) and this composition differed from that of total admission patients. Conclusion: Hospitalized patients referred for insomnia showed a higher proportion of male patients, lower rates of re-consultation compared with other patients, and most of these were for secondary insomnia. Each doctor should be aware of the possibility of inpatient insomnia, conduct positive assessments and referrals as necessary, and psychiatrists who might be asked for consultation need to prepare an active intervention with initial diagnosis and treatment, as well as recommendations for the timing of reconsultation.
Kim, Hyun-Ah;Lee, Jong Wook;Kim, Seung-Jun;Oh, Hong-Seok;Im, Woo Young;Kim, Ji-Woong
Korean Journal of Psychosomatic Medicine
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v.26
no.2
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pp.188-193
/
2018
Objectives : Clozapine is a widely prescribed antipsychotic drug for schizophrenia and is known to increase the risk of cardiovascular disease due to its metabolic side effects. However, little is known about the effect of clozapine on the platelet activation, another important factor in the development of cardiovascular disease. In this study, we tried to investigate the effect of clozapine on platelet activity in patients with schizophrenia by comparing the mean platelet component (MPC) values before and after the clozapine administration. Methods : A retrospective review of medical records of patients with schizophrenia, who newly started clozapine treatment from September 1st, 2003 to April 30th, 2007 at the Department of Psychiatry, Konyang University Hospital in Republic of Korea was performed. The final statistical analysis included 14 participants. Bayer ADVIA $120^{(R)}$ system was used to measure MPC. Results : Among the 14 participants, five subjects were males (28.60%), and ten subjects were females (71.40%). The mean age of participants was $37.50{\pm}11.64years$. Average of duration of illness was $91.00{\pm}93.96months$, with the mean dosage of clozapine taken by participants at the time of the last blood test was $337.50{\pm}109.52mg$. The mean MPC measurement before and after receiving clozapine was $26.12{\pm}2.22g/dL$ and $25.14{\pm}2.08g/dL$ respectively. Wilcoxon signed rank test showed that there was a statistically significant decrease in MPC levels after clozapine administration (V=16, p=0.024). Conclusions : Decreased MPC levels after clozapine administration implies that clozapine may increase platelet activation which could have an adverse effect on the occurrence of thromboembolic disease. Our findings also suggest that careful monitoring of the risk factors of cardiovascular diseases, such as platelets activity, is necessary when administering clozapine.
Objective : This study aims to identify the self-reported driving abilities of elderly drivers and their correlations to the demographic factors that influence them, and to verify the adequacy of the hypothetical model, constructed based on vision, auditory, cognition, motor, and psychological factors, in order to present a path model on the self-reported driving abilities of elderly drivers. Methods : The participants in this study were 122 elderly drivers aged 65 years or older residing in the community. This study evaluated the following factors of the participants: Vision and hearing, motor ability, cognitive ability, depression, self-reported driving abilities. Results : The results of this study are as follows. In the case of men, the self-reported driving ability score was higher than for women, and those driving 6-7 days per week had higher scores than those driving 3 days or less. The period of holding a driver's license and driving experience positively correlated with self-reported driving abilities. The final model of factors influencing the self-reported driving abilities of elderly drivers had a p value (.911) exceeding .05; TLI (1.202), NFI (.949), and CFI (1.000) of over .90; and RMSEA (.000) of lower than 0.1, indicating that the hypothesis model fit the data well. First, the directly influential factors on the self-reported driving abilities of elderly drivers were depression, decreased hearing, and grip strength. Second, age was found to have a direct influence on depression and grip strength; moreover, depression and grip strength as a mediator indirectly influenced their self-reported driving abilities. Third, depression was found to have a direct influence on their delayed cognitive processing and grip strength. Conclusion : The significance of this study is in the identification of direct and indirect factors influencing the self-reported driving abilities of elderly drivers in regional communities, and in the verification of multi-dimensional effects of diverse factors influencing such abilities.
Yun, Ho Hyun;Cheong, Ji Young;Sim, Hyun Bo;Park, Jae Hong
Journal of the Korean Orthopaedic Association
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v.53
no.6
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pp.490-497
/
2018
Purpose: To evaluate the utility of ceramic-on-polyethylene articular bearing surface when cementless total hip arthroplasty is performed in patients older than 65 years through an analysis of the minimum five-year follow-up results using the ceramic femoral head and cross-linked polyethylene liner. Materials and Methods: From March 2010 to September 2012, 51 patients (56 hips) who were older than 65 years were enrolled in this retrospective study. The mean age at surgery was $70.9{\pm}5.1years$ old. A clinical assessment was analyzed using the Harris hip score. For the radiographic assessment, the cup inclination and anteversion, stem alignment, and wear amount were measured. The postoperative complications were also determined. Results: The mean Harris hip score was improved from preoperative 48 points to postoperative 87 points (p<0.05). The mean cup inclination was $40.9^{\circ}{\pm}6.4^{\circ}$ and the mean cup anteversion was $20.3^{\circ}{\pm}8.1^{\circ}$. The mean cup anteversion of the elevated liner-used group (16 cases) was $14.3^{\circ}{\pm}7.9^{\circ}$ and the mean cup anteversion of the neutral liner used group (40 cases) was $22.4^{\circ}{\pm}9.1^{\circ}$ (p<0.05). The mean stem alignment angle was $0^{\circ}$ (range, varus $4^{\circ}$-valgus $4^{\circ}$). The mean linear wear amount was $0.458{\pm}0.041mm$ and the average annual linear wear rate was $0.079{\pm}0.032mm/yr$. Six cases (10.7%) of intraoperative periprosthetic femoral fractures were encountered. Conclusion: Based on these results, the use of a ceramic-on-polyethylene articular bearing surface in elderly patients with cementless total hip arthroplasty is beneficial. On the other hand, careful effort is needed to prevent intraoperative periprosthetic femoral fractures.
The hepatocyte nuclear factor 4 alpha ($HNF4{\alpha}$) gene is related to lipid transport, including abdominal fat and growth, in chickens. Interestingly, the A543G SNP within the $HNF4{\alpha}$ gene has previously been reported to be associated with body weight in both broilers and Korean native chickens (KNCs). However, its exact position within the HNF4 is not yet reported. This study aimed to identify the position of the A543G SNP and to identify additional SNPs that can be used as genetic markers in KNCs. A total of 128 KNCs were used for the sequencing and analysis of these genetic associations. As a result, A543G SNP was located in intron 4 of the $HNF4{\alpha}$ gene; it is reported as rs731246957 in the NCBI database. Fourteen SNPs were detected in the sequenced portion of the $HNF4{\alpha}$ gene; three of these, rs731246957, rs736159604 and new SNP, intron 6 (249), were significantly related with growth in the chickens. In this study, the TT genotype of rs731246957, previously reported as A543G SNP, the GG genotype of rs736159604 and GT of new SNP have are highly associated with body weight from birth to 40 weeks of age in KNCs (P<0.01). These results suggest that rs736159604, rs731246957 and intron 6 (249) SNPs within the $HNF4{\alpha}$ gene could function as growth-related markers in the selective breeding of KNCs.
Lee, Han Hyoung;Park, Ji Hee;Hong, Jong Ouk;Han, Min Su;Seo, Min Suck;Heo, Jun Su
Korean Journal of Heritage: History & Science
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v.45
no.1
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pp.122-147
/
2012
The Four-guardian statues in Song-gwang buddhist temple, Suncheon, Korea, have been remade in AD 1628 and have been repaired and repainted over several times since then. Therefore, the study of the pigments applied on the statues can provide good chance for investigation about pigments used in the late Chosun Period. Pigments on fragments from Gwang-mok(Virupaksha), one of the Four-guardian statues, have been analyzed by optical microscope, SEM-EDX and XRD in order to identify the components and compounds. Six types of materials were found from the fragments, which are soil layer with brown clay band, soil layer containing a lot of fibers, Korean paper with loose texture, Korean paper with dense texture, silk, and hemp textile. Presumably, the soil layer which have brown clay band is basis layer and the other layers are repaired layers. From comparative study for the components of the pigments, applied on upper and lower parts of the repaired layers, we have concluded that those repaired layers had been applied on the statue by the following order; basis layer ${\rightarrow}$ Korean paper with loose texture ${\rightarrow}$ soil containing a lot of fibers ${\rightarrow}$ silk ${\rightarrow}$ hemp textile and Korean paper with dense texture. In addition, the years that those materials were applied on the statue have been estimated as 1720~1891, 1926, 1946 and 1976, respectively. The distinct features of each age are as the following; lead white and copper chloride hydroxide are major white and green pigments before 1891, zinc white, barium white, emerald green, and ultramarine blue appear after 1926 and titanium white uprises around 1976. Our result presented here, study on pigments applied on traditional statues over several different periods, will provide good database for future study on pigments used for traditional painting in Buddist temples and Dancheong.
During the detailed geological survey around the southern Yangsan Fault, we newly found a Quaternary fault outcrop, which cuts unconsolidated sediments. The fault named the Sinwoo site, located in the Sinwoo pasture, Miho-ri, Duseo-myeon, Ulsan metropolitan city, is the first discovered Quaternary fault near the western part of the south Yangsan Fault. In this study, we provide information on characteristics of fault geometry and unconsolidated sediment at Sinwoo site based on the analysis data of topography, drainage, and lineament around the study site. The fault site is situated at pediment slope, but fan-shaped middle terrace, as well as thick sediment exposed at low terrace, indicates that the unconsolidated sediments have been deposited in the alluvial fan environment. The drainage develops to the third-order drainage system, and the first and the second drainage system meet at right angles to each other and form a radial drainage pattern. In addition, the NE-SW direction lineaments can be identified on the basis of the curvature of the river and the step of the topographic relief, running over the Sinwoo site. The fault of $N30-35^{\circ}E/79-82^{\circ}SE$ shows ~ 5.8 m apparent vertical offset and dominantly reverse-slip sense based on slickenline, rotation of pebbles, and drag folding at footwall. However, some discontinuous sediments observed in the footwall are interpreted as fissure-filling materials due to the strike-slip movement. Now, we are under multidisciplinary investigations of additional field survey and age dating in order to determine the evolution of Sinwoo site fault during the Quaternary.
Kim, Kyeong Soon;Shin, Jung;Sim, JiSoo;Yeon, SuJi;Lee, Pyeong An;Chung, Moon Gyu
Korean Journal of Microbiology
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v.55
no.3
/
pp.268-273
/
2019
The intestinal microbiomes vary according to the factors such environment, age and diet. The purpose of this study was to compare the gut microbial diversity between Korean infants receiving breast-fed milk and formula-fed milk. We analyzed microbial communities in stool samples collected from 80 Korean infants using next generation sequencing. Phylum level analysis revealed that microbial communities in both breast-fed infants group (BIG) was dominated by Actinobacteria ($74.22{\pm}3.48%$). Interestingly, the phylum Actinobacteria was dominant in formula-fed infants group A (FIG-A) at $73.46{\pm}4.12%$, but the proportions of phylum Actinobacteria were lower in formulafed infants group B and C (FIG-B and FIG-C) at $66.52{\pm}5.80%$ and $68.88{\pm}4.33%$. The most abundant genus in the BIG, FIG-A, FIG-B, and FIG-C was Bifidobacterium, comprising $73.09{\pm}2.31%$, $72.25{\pm}4.93%$, $63.81{\pm}6.05%$, and $67.42{\pm}5.36%$ of the total bacteria. Furthermore, the dominant bifidobacterial species detected in BIG and FIG-A was Bifidobacterium longum at $68.77{\pm}6.07%$ and $66.85{\pm}4.99%$ of the total bacteria. In contrast, the proportions of B. longum of FIG-B and FIG-C were $58.94{\pm}6.20%$ and $61.86{\pm}5.31%$ of the total bacteria. FIG-A showed a community similar to BIG, which may be due to the inclusion of galactooligosaccharide, galactosyllactose, synergy-oligosaccharide, bifidooligo and improvement material of gut microbiota contained in formula-milk. We conclude that 5-Bifidus factor contained in milk powder promotes the growth of Bifidobacterium genus in the intestines.
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