• Journal of The Korean Society of Inherited Metabolic disease
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• v.24 no.1
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• pp.17-25
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• 2024

Fabry disease (FD) is an X-linked lysosomal storage disorder. It is caused by mutations in the α-galactosidase A gene, which results in deficient or absent activity of α-galactosidase A (α-Gal A). This leads to a progressive accumulation of globotriaosylceramide (Gb3) in various tissues. Manifestations of Fabry disease include serious and progressive impairment of renal and cardiac function. In addition, patients experience pain, gastrointestinal disturbance, transient ischaemic attacks, and strokes. Additional effects on the skin, eyes, ears, lungs, and bones are often seen. Reduced life expectancy and deadly consequences are being caused by cardiac involvement. Chaperone therapy or enzyme replacement therapy (ERT) are two disease-specific treatments for FD. Thus, early detection of FD is critical for decreasing morbidity and mortality. Globotriaosysphingosine (lyso-Gb3) for identifying atypical FD variants and highly sensitive troponin T (hsTNT) for detecting cardiac involvement are both significant diagnostic indicators. This review aimed to offer a basic resource for the early diagnosis and update on the diagnosis of having FD. We will also provide a general diagnostic algorithm and information on ERT and its accompanying treatments.

• The Korean Journal of Nuclear Medicine Technology
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• v.19 no.2
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• pp.112-114
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• 2015

Purpose Vitamin D to Anti- Rickets both steroid compounds showing activity, By acting on bone tissue secretary and the key to maintain serum Ca homeostasis. The blood level of vitamin D is the largest in D3 that the concentration of the metabolite is reflected in the holding state of vitamin D in vivo. Sunlight to change the 7-dehydrocholesterol in the skin and through the skin to D3, In the liver in combination with the D2 and D3 D4 changes. The Radioimmunoassay(RIA) method is measuring the D 3, the sensitivity can be measured also difficult trace substance to measure the normal test because it is very sensitive, but recently, a check is possible, for the Total D3 in Chemimicroparticle immunoassay(CMIA) or Chemiluminescent immunoassay(CLIA) measuring using microparticle RIA and CMIA(Architect i2000SR) / use the CLIA(DXI-800) method to compare and evaluate the correlation between the tests in the same test items. Materials and Methods Commissioned from January 2014 to March 2015 patients were enrolled in a total of 273 people. 29 out of 273 people conducted by RIA were compared with CMIA, 244 patients were compared with CLIA. Using reagents and equipment were used RIA(Diasource), CMIA(Architect i2000SR, Abbott Diagnostics) / CLIA( Unicel DXi-800, Beckman coulter). Results Correlation of the RIA and CLIA was a R2 = 0.1844 (y = 0.7303x + 3.9005), and the correlation of RIA CMIA is R2 = 0.2762 (y = 0.8862x + 4.56) respectively. (According to statistics, during the same period RIA is Deficiency 4.31%, Insufficiency 90.53%, Sufficiency 5.16%, was Excess 0%, CLIA / CMIA is Deficiency 17.02%, Insufficiency 75.91%, Sufficiency 7.03%, indicating the distribution of 0.03 % Excess) Conclusion Serum vitamin D and parathyroid hormone that show an inverse relationship, the level above which are not parathyroid hormone and vitamin D reduced the increase. The density is different for each study, at most 20 is reported to be the maximum between 30 ng / ml. In Korea it requires a proposed standard of vitamin D deficiency, reference to the WHO lack the case more than 10ng/ml, 20ng/ml and defined by the lack of, if not more than, the IOM, but looking at 12ng/ml or less to the normal to lack, at least 20ng/ml, the reference do not match the deficit under 20ng/ml, 21-29ng/ml relative lack between, was also defined as a sufficient condition for more than 30ng/ml. Although not statistically is between RIA and CLIA two ways to vitamin D levels change according to season match, when seasonally seen in summer as commonly known (April to September), winter (October to March) relative to the increase measured than it was found. Finally, the study on the correlation between the two methods have been expected to result in a consistent and apply the same view high reference value on the graph is difficult. However, there may be differences between the test equipment and methods, and could be especially the case of RIA method using an organic solvent is difficult to compare different methods and correlated view similar trend in vitamin D deficiency and quarterly aspect ratio.

• Nuclear Medicine and Molecular Imaging
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• v.42 no.5
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• pp.369-374
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• 2008

Purpose: F-18 FDG PET/CT has excellent sensitivity and specificity for staging non-Hodgkin lymphomas, but to the author's knowledge few studies to date have evaluated FDG PET/CT in peripheral T cell lymphoma. We evaluated the usefulness of F-18 FDG PET/CT in staging of patients with peripheral T cell lymphoma, especially indolent cutaneous T cell lymphomas. Materials and Methods: Twenty five patients (M:F=17:8, age $53.7{\pm}14.8$ yrs) with biopsy-proven indolent cutaneous T cell (CL) or noncutaneous T cell lymphomas (NCL) underwent PET/CT scans for staging at baseline. Peak standardized uptake values (p-SUV) of all abnormal foci were measured and compared between cutaneous and noncutaneous lesions. F-18 FDG PET/CT was performed on 6 patients with indolent CL and on 19 patients with NCL. Results: All 6 patients with indolent CL had no significant FDG avidity in the skin despite histologically positive cutaneous lesions. However, FDG avidity appeared in extracutaneous lesions (lymph nodes) in two patients with CL where CT imaging suggested lymphoma involvement (mean p-SUV $4.26{\pm}0.37$ in noncutaneous lesions in CL). In NCL, FDG avidity was demonstrated in all lesions where CT imaging suggested lymphoma involvement (mean p-SUV, $8.52{\pm}5.00$ in noncutaneous lesions in NCL). Conclusion: F-18 FDG PET/CT has the limitation of usefulness for the evaluation of the skin in indolent CL. In contrast, F-18 FDG PET/CT is sensitive in staging evaluation of extracutaneous lesions regardless of CL or NCL.

• Journal of the Society of Cosmetic Scientists of Korea
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• v.30 no.2
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• pp.181-187
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• 2004

Preparations of mesoporous materials using various templates and their applicability have been intensively investigated for many years. We studied on synthesizing mesoporous Ti02 with pores in which sensitive compounds having weak physico-chemical properties such as thermal or UV irradiation and low solubility in solvent are trapped. Prior to trapping OMC in the pores of mesoporous titania, OMC was nano-emulsified in O/W system using Lecithin. Thereafter the OMC was trapped in the pores of mesoporous titania using sol-gel method. Main focus of this work is to prepare OMC-trapped mesoporous titania and to trace the stability and solubility of nano-emulsified OMC in the pores of mesoporous titania, and compared with that of mesoporous silica. OMC-trapped mesoporous Inorganic-Organic hybrid titania showed higher factors in sun protecting and a skin penetration phenomenon was reduced.

• Journal of Plant Biotechnology
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• v.46 no.3
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• pp.228-235
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• 2019

Apple (Malus pumila) is one of the most economically important fruits in Korea. but virus infection has decreased the sustainable production of apples and caused serious problems such as yield loss and poor fruit quality. Virus or viroid infection including apple chlorotic leaf spot virus (ACLSV), apple stem pitting virus (ASPV), apple stem grooving virus (ASGV), apple mosaic virus (ApMV) and apple scar skin viroid (ASSVd) have been also reported in Korea. In many cases, as apple gets infected with virus and viroid with no specific symptoms, the damage and symptoms caused by the viruses are not detected. In our research, viruses in the rootstock were eliminated for a virus-free apple dwarfing rootstock of M.9 and M.26. The virus elimination methods were apical meristem culture, thermotherapy ($37^{\circ}C$, 6 weeks) and chemotherapy($Ribavirin^{(R)}$). The detection of apple viruses was accomplished by Enzyme-linked Immuno-Sorbent Assay (ELlSA) and reverse transcription-polymerase chain reaction (RT-PCR). RT- PCR method was 10 ~ 30% more sensitive than the ELISA method. The efficiency of virus elimination was enhanced in apical meristem culture method. The acquisition rate of virus-free apple dwarfing rootstocks was 30 ~ 40% higher in apical meristem culture. After the meristem culturing of M.9, the infection ratio of ACLSV, ASPV and ASGV was 45%, 60% and 50%, respectively. In the apple dwarfing rootstock of M.26, the infection ratio of ACLSV, ASPV and ASGV was 40%, 55% and 55%, respectively. Based on this study, the best method for the production of virus-free apple dwarfing rootstocks was the apical meristem culture.

• Horticultural Science & Technology
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• v.30 no.6
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• pp.780-783
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• 2012

A new strawberry cultivar (Fragaria ${\times}$ ananassa Duch.) 'Okmae' was developed from a cross between 'Toyonoka' and 'Maehyang'. This cultivar has a good fruit quality and suitable number of flowers per cluster for labor saving in fruit thinning. 'Gyoengnam No. 1' was selected as an elite line with vigorous plant growth, higher soluble solids content and fruit firmness after examining its characteristics and productivity in forcing cultures from 2007 to 2009. Farmer's field trial of 'Gyoengnam No. 1' was conducted in 2010 and it was registered as 'Okmae' thereafter. The general characteristics of 'Okmae' are vigorous growth habit, erect plant type, less number of leaf and elliptic leaf shape. 'Okmae' has long peduncle and 9-10 flowers per flower cluster which need less labor for fruit thinning. Fruits of 'Okmae' are conical having a bright red and glossy skin color. Although 'Okmae' has a smaller average fruit number per plant of 21.9 than that of the control cultivars, it's possible to produce high yield because of greater fruit weight of 26.0 g. 'Okmae' showed a high soluble solids content of $11.6^{\circ}Brix$, low acidity of 0.37%, and high firmness of $14.5g{\cdot}mm^{-2}$. It is sensitive to anthracnose and powery mildews, but high-quality strawberry can be harvested by using effective control measure.

• Clinical and Experimental Pediatrics
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• v.51 no.3
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• pp.323-328
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• 2008

Purpose : Asthma is defined as chronic inflammation of the lower small airways, and bronchial hyperreactivity (BHR) is a pathophysiologic feature of asthma. It has been proposed that although there is no direct variable capable of assessing the small airways, a forced expiratory flow of between 25 and 75 percent ($FEF_{25-75}$) might be considered a more sensitive early marker of small airway obstruction than the forced expiratory volume in 1 second ($FEV_1$). Thus, we proposed that the presence and degree of positive responses to bronchial methacholine testing were related to the difference (DFF) and ratio (RFF) between $FEV_1$ and $FEF_{25-75}$ in asthmatic children. Methods : The subjects were 583 symptomatic children, including 324 children with BHR and 259 controls. Pulmonary function tests, methacholine challenge tests, and skin prick tests were performed, and the total eosinophil count, total serum IgE, and serum eosinophil cationic protein level were measured in all subjects. From a concentration-response curve, the methacholine concentration required to produce a decrease of 20% from post-saline $FEV_1$ was calculated ($PC_{20}$). Results : The median DFF and RFF values decreased in controls compared to subjects with bronchial hyperresponsiveness, and this trend was found in groups ranked by its severity. $PC_{20}$ had a negative correlation with DFF and RFF. Cutoff values of 0.5 for DFF and 1.042 for RFF were identified, and sensitivity and specificity were calculated. Conclusion : This study revealed that DFF and RFF might be predictive of bronchial hyperresponsiveness in the context of normal $FEV_1$ in children.

• Tuberculosis and Respiratory Diseases
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• v.43 no.1
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• pp.14-21
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• 1996

Objectives: Although outbreak of MDR Tb has been a recent problem in western countries, it has been a longstanding problem in Korea. The poor outcome of MDR Tb is mainly due to poor compliance, high rate of side reaction of secondary drugs, and limitation in number of available drugs. Thus, to improve the outcome of MDR Tb, it is crucial to make individualized adequate prescription based on the knowledge of the patterns of resistance to each drugs in the community as well as the natural history. The purpose of present study is to evaluate the clinical features of Korean MDR Tb patients including patterns of drug resistance and success rate of treatment which was prescribed according to the sensitivity tests. Methods: Retroscpective analysis of 71 Korean patients with MDR Tb was made. All strains isolated from patients showed resistence to at least two first line drugs. Patients profile, previous treatment history, patterns of drug resistance, outcome of treatment was analysed. Initial treatment regimen was selected according to the previous treatment history and was modified according to the sensitivity reports. The regimen was composed to include at least 4 sensitive drugs when possible. Results: The patients showed resistance to 4.1 drugs on average. 90% of them were resistant to INH and RFP. Among 71 patients, 35 patients(49%) had cavitary lesions in CXR. Treatment outcome was analysed in 55 patients. 35 patients(67%) were improved after treatment and 18 patients(33%) showed treatment failure. 5 patients showed primary resistance. Treatment outcome could be evaluated in 4 of them and all showed improvement after treatment. 14 patients(20%) had to change their regimens due to drug side effects. The most frequent side effect was elevation of liver enzymes(6 patients). Others included dizziness, hyperuricemia, tinnitus, skin rash, GI troubles. More than 50% of side effects developed within 3 months. In repeated drug sensitivity test, the concordance rate of resistance to INH was 100% and RFP 98%. EMB, PZA showed 80% concordance rate. But in the other drugs, the concordances were less than 50%. Operation was done in 5 patient - 1 patients as a adjunctive means of chemotherapy -. In that case, negative conversion of sputum AFB was done. Conclusion: 2/3 patients of multidrug-resistant tuberculosis were improved by appropriate prescription and regular medication suggesting that more aggressive management and monitoring is indicated in multidrug-resistant tuberculosis.

• Tuberculosis and Respiratory Diseases
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• v.45 no.6
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• pp.1154-1166
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• 1998

Background : In the management of patients whose primary chemotherapy has failed, careful assessment is essential. It is important to find out as accurate a chemotherapy history as possible. Preferably it should contain the drugs which has never used before. The purpose of present study is establishment of retreatment regimen for pulmonary tuberculosis. The present report concerns the results of retreatment of pulmonary tuberculosis patients treated at National Masan Tuberculosis Hospital. Methods : Retrospective cohort study was made of 104 drug-resistant pulmonary tuberculosis patients who were treated by five regimens between Jan. 1994 and Nov. 1996. All the patients taken medicine for second anti-tuberculosis regimens for the first time. We separated the patients by three groups(Group I ; OFX+PTA+CS+PAS+Aminoglycoside, Group II : PZA+PTA+CS+PAS+Aminoglycoside, Group III : PZA+OFX+PTA+PAS+Aminoglycoside). Results : The age distribution was most frequent in fourth decade(36patients, 34.6%) and the mean age was 42.6 year. The sex distribution was more frequent in the males(81 patients, 85.7%). There was 31 patients(29.8%) with combined diseaes, 18 patients with complication and 24 patients(27.9%) with family history. Primary chemotherapy regimens were HERZ(S or K) in 48 patients (46.2%), HER(S or K) in 41 patients(39.4%) and others in 15 patients(14.4%). Result of drug sensitivity test showed that the resistance to INH and RFP is in 68 patients(65.4%), RFP is 12 patients(11.5%), INH is in 3 patients(2.9%) and all sensitive to INH and RFP is 3 patients(2.9%). The clinical symptoms on admission were coughing(89.4%), sputum(69.2%), dyspnea on exertion(37.5%), weight loss(33.7%) blood tinged sputum(15.4%) and others. The extent of disease on the radiograph was far-advanced in 73 patients(70.2%), moderate in 28 patients(26.9%) and minimal in 3 patients(2.9%). The side effects for drugs were gastrointestinal troubles in 31 patients(29.8%), arthralgia in 22 patients(21.2%), skin rash in 12 patients(11.5%) and others. The negative conversion rate on sputum AFB smear was 85.6%(87.5% in Group I, 80.0% in Group II and 90.5% in Group III). The average negative conversion time on sputum was 4 month(4.0 month in Group I, 4.6 month in Group II and 3.0 month in Group III). Conclusion : In the retreatment of pulmonary tuberculosis, ofloxacin is useful drug for the patients who are not available to use PZA and combination of PZA and OFX can be use effectively substituting for CS.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.20 no.1
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• pp.103-110
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• 2019

Recently, Korea has been affected by extreme weather events including extended summers and increased temperatures caused by global warming and climate change. Environmental temperature is especially important to the livestock industry because it is closely related to livestock productivity. This study was conducted to investigate the influence of different environmental temperatures on respiration rate, rectal temperature and body-surface temperature in finishing pigs. Pigs ($98.3{\pm}6.6kg$) were housed in individual cages inside an experimental chamber and exposed continuously to one of five environmental treatments ($22^{\circ}C$, $24^{\circ}C$, $26^{\circ}C$, $28^{\circ}C$, $30^{\circ}C$) for 10 days without providing additional rest time. Feed and water intake, respiration rate, rectal temperature and body-surface (head, ear, neck, back, side) temperature were measured two times daily during the experimental period. A significant increase in respiration rate from $26^{\circ}C$ and in body-surface temperature from $24^{\circ}C$ (p<0.05) was observed. At $30^{\circ}C$, the respiration rate had almost doubled and the body-surface temperature increased by about $5^{\circ}C-7^{\circ}C$. Moreover, ear skin temperature was very sensitive to environmental temperature. However, feed intake, water intake and rectal temperature did not change significantly during the experiment.