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Clinical characteristics of congenital myotonic dystrophy diagnosed by molecular genetic method (분자 유전학적 방법으로 진단된 선천성 근육긴장성 이영양증 환자의 임상 양상)

  • Nam, Sook Hyun;Son, Young Bae;Lee, Bo Lyun;Lee, Jeehun;Ki, Chang-seok;Lee, Munhyang
    • Clinical and Experimental Pediatrics
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    • v.50 no.9
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    • pp.868-874
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    • 2007
  • Purpose : We performed this study to investigate the perinatal and developmental features of the patients with congenital myotonic dystrophy (CDM) confirmed by the molecular genetic method and the clinical characteristics of their mother, and to identify the relation between the number of CTG repeats and the clinical severity.Methods : A retrospective review of the medical records and the results of the dystrophia myotonica protein kinase (DMPK) gene test was done for the patients who were confirmed as CDM through gene analysis from January 2001 to September 2006. Results : All of the eight patients (male 2, female 6) showed moderate to severe degree of perinatal distress and feeding difficulty associated with profound hypotonia. Three patients had the history of polyhydramnios and two patients had equinovarus deformity. The developmental milestones were delayed in all patients, which improved gradually with age. All of their mothers demonstrated myotonic symptoms and typical myopathic face. The number of CTG repeats in DMPK gene analysis ranged 1,000-2,083, and there was no significant correlation between the number of CTG repeats and the time of walking alone. Conclusion : All patients with CDM presented with severe hypotonia in perinatal period, and developmental delay thereafter, which were improved with age. All of their mothers manifested myotonic symptoms with typical myopathic face, and the identification of such features greatly contributed to the diagnosis of the patients. The number of CTG repeats had no significant influence on the motor development.

Cytotoxic and Anti-inflammatory Activities of Lipids from the Nuruk (Rhizopus oryzae KSD-815) (누룩(Rhizopus oryzae KSD-815)으로부터 분리한 지질화합물의 세포독성 및 항염증 활성)

  • Kwak, Ho-Young;Lee, Sang-Jin;Lee, Dae-Young;Bae, Nark-Hyun;Jung, La-Koon;Hong, Sung-Youl;Kim, Gye-Won;Baek, Nam-In
    • Applied Biological Chemistry
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    • v.51 no.2
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    • pp.142-147
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    • 2008
  • Nuruk is the Korean traditional Koji that contains various microorganisms and has been used to make the traditional fermented foods including alcoholic beverages. Rhizopus oryzae KSD-815 was isolated from the alcohol-fermenting Nuruk used for manufacturing traditional alcohol. In this study, the authors reported the isolation and identification of four lipids from the Nuruk (Rhizopus oryzae KSD-815) that inoculated wheat with Rhizopus oryzae KSD-815. The dried and powdered Nuruk (Rhizopus oryzae KSD-815) were extracted three times at room temperature with 80% aqueous MeOH. The extracts were partitioned with EtOAc, n-BuOH, and water, successively. The EtOAc extract was suspended in 80% MeOH and partitioned repeatedly with n-hexane. From the n-hexane fraction, four lipids were isolated through the repeated silica gel and ODS column chromatographies. According to the results of physico-chemical data including NMR, GC and MS, the chemical structures of the compounds were determined as linolenic acid methyl ester (1), palmitic acid methyl ester (2), linoleic acid (3), palmitic acid (4). Cytotoxicity was evaluated in huamn breast cancer cells, MDA-MB-231 and human hepatocarcinoma, SK-HEP-1 cells using MTT assay. Exposure of compounds 1 and 3 led to a dose-dependent inhibition of cell viability in both cancer cell lines. In addition, treatment of RAW264.7 cells with compound 3 caused inhibition of lipopolysaccharide/interferon-${\gamma}$-induced nitric oxide production.

Therapeutic Efficacy of Cefotaxime as an Empirical Antibiotic on Ascending Cholangitis after Kasai Operation for Biliary Atresia (Kasai 수술 후 발생한 상행성 담관염에서 경험적 항생제로서 Cefotaxime 치료의 적정성에 대한 평가)

  • Kim, Dong Ho;Lee, Byong Sop;Kim, Yun Kyung;Ko, Jae Sung;Lee, Hoan Jong;Seo, Jeong Kee
    • Clinical and Experimental Pediatrics
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    • v.45 no.4
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    • pp.473-481
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    • 2002
  • Purpose : Ascending cholangitis is the most common complication after Kasai operations. The aim of this study is to evaluate the therapeutic efficacy of cefotaxime as an empirical antibiotic on ascending cholangitis after Kasai operations. Methods : Thirty-nine episodes of cholangitis in twenty-nine children who underwent Kasai operations at Seoul National University Children's Hospital from January 1991 to December 2000 were included in this study. Empirical cefotaxime treatments were divided into three groups : cefotaxime and amikacin treatment group(CA group), cefotaxime and gentamicin treatment group(CG group) and cefotaxime treatment group(C group). A diagnosis of cholangitis was made on the basis of unexplained fever(>$38^{\circ}C$) and either development of acholic stool or elevation of serum total bilirubin (>1.5 mg/dL). Therapeutic efficacy was judged by elimination of fever up to 72 hours, 120 hours, and 168 hours after antibiotic treatment. Results : There were therapeutic responses in 51%(20/39) up to 72 hours after antibiotic treatment : 54%(13/24) in CA group, 43%(3/7) in CG group and 50%(4/8) in C group. There were therapeutic responses in 69%(27/39) up to 120 hours, in 79%(31/39) up to 168 hours and in 82%(32/39) up to 2 weeks. There were no differences in therapeutic efficacy among the three regimens. In 12 of 39 episodes, the etiologic pathogens including Escherichia coli and enterococcus were cultured from the blood. Conclusion : Cefotaxime can be tried as an initial antibiotic in Korean children with ascending cholangitis after Kasai operation prior to the identification of microorganism on culture. However, further evaluation of pathogen and its resistant strain to cefotaxime should be done.

Monitoring of the residues of some pesticides in mulberry leaves and their safety evaluation (뽕잎중 잔류농약의 monitoring 및 안전성 평가)

  • Lee, Jae-Koo;Kwon, Jeong-Wook;Ahn, Ki-Chang;Moon, Jae-Yu
    • The Korean Journal of Pesticide Science
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    • v.2 no.2
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    • pp.45-52
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    • 1998
  • In order to evaluate the safety of mulberry leaves for sericultural purpose to the contamination by pesticide residues, the analyses of dichlorvos, acephate, phenthoate, pyrazophos, deltamethrin, and cypermethrin were done for 24 samples collected in spring and fall over two consecutive years (1996 and 1997), each, from 8 provinces across the country, by selecting three representative farmhouses in each province. In spring samples of 1996, the residual amounts of dichlorvos, acephate, and phenthoate were $0.018{\sim}0.032$, $0.013{\sim}0.072$, and 0.051 ppm, respectively, whereas, pyrazophos was not detected, and the detection frequencies were 29, 58, and 4%, respectively. In fall samples, on the other hand, those of the above pesticides were $0.012{\sim}0.048$, $0.020{\sim}0.156$, and $0.018{\sim}0.050$ ppm, respectively, and pyrazophos was not detected either and the detection frequencies were 42, 17, and 13%, respectively. While in spring samples of 1997, the residual amounts of dichlorvos, acephate, and cypermethrin were $0.014{\sim}0.064$, $0.033{\sim}0.061$, and $0.019{\sim}0.068$ ppm, respectively, and deltamethrin was not detected. The detection frequencies were 75, 13, and 8%, respectively. In fall samples, on the other hand, those of the above pesticides were $0.013{\sim}0.062$, 0.015, and $0.009{\sim}0.013$ ppm, respectively, and deltamethrin was not detected either. The detection frequencies were 88, 4, and 17%, respectively. Almost all of the samples turned out to be non-toxic to silkworms, except that one sample (No. 10, spring 1997) contaminated by a high concentration of cypermethrin (0.068 ppm) was judged to do harm to silkworms. In the GC analyses, the selection of columns, the change in column temperature, and the use of MSD made the separation and identification of the vague compounds possible.

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The effect of nutritional supply on clinical outcomes and nutritional status in critically ill patients receiving continuous renal replacement therapy (지속적 신대체요법을 받은 중환자에서 영양공급이 임상결과와 영양상태에 미치는 영향)

  • Kim, Ju Yeun;Kim, Ji-Myung;Kim, Yuri
    • Journal of Nutrition and Health
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    • v.48 no.3
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    • pp.211-220
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    • 2015
  • Purpose: This study was designed to investigate whether nutritional supply influences biochemical markers and clinical outcomes in patients who received continuous renal replacement therapy (CRRT) by evaluating adequacy of nutritional supply for patients. Methods: From January 2012 to December 2013, 239 adult patients who received CRRT in the intensive care unit for more than 3 days were included. General information from electronic medical records and nutritional status related biochemical data and clinical outcomes on the first day of CRRT and 2 weeks after CRRT were collected. Results: The rate of delivered energy and protein was 68.06% and 43.13% which was much lower than energy and protein supply based on their requirement. When the patients were divided into two groups according to 70% of energy received rate and 50% of protein received rate, the group with more than 70% of energy received rate showed significant decrease of length of hospital stay (p = 0.007), length of stay in intensive care unit (ICU) (p = 0.008), duration of CRRT (p < 0.001), and APACHE II score (p < 0.001) compared to less than 70% of energy received rate after adjusting for age. In addition, the group with more than 50% of protein received rate showed decreased mortality (p = 0.031), length of hospital stay (p = 0.008), length of ICU stay (p = 0.035), duration of CRRT (p < 0.001), and APACHE II score (p < 0.001) after adjusting for age. We found that the level of hematocrit (p = 0.006) was significantly improved in the group with more than 70% of energy received rate, and the level of TLC (p = 0.049), hematocrit (p = 0.041) was significantly improved in the group with more than 50% of protein received rate. We also found that energy delivery was negatively correlated with length of stay in ICU (p = 0.049) and positively correlated with level of calcium (p = 0.037). In addition, protein delivery was correlated with the levels of serum total protein (p = 0.021), serum albumin (p = 0.048), hematocrit (p = 0.009), and total cholesterol (p = 0.021) when dead patients were included, but was correlated with the levels of hematocrit (p = 0.034) and calcium (p = 0.024) when dead patients were excluded. Conclusion: Proper nutritional delivery may help patients' clinical outcomes for patients receiving CRRT. However, their actual intakes of energy and protein were not adequate for their requirements. Identification of patients with malnutrition is necessary and a multidisciplinary approach for systemic management is also required.

Formation of the $CoSi_{2}$ using Co/Zr Bilayer on the Amorphous and the Single Crystalline Si Substrates (단결정과 비정질 Si 기판에서 Co/Zr 이중층을 이용한 $CoSi_{2}$ 형성)

  • Kim, Dong-Wook;Jeon, Hyeong-Tag
    • Korean Journal of Materials Research
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    • v.8 no.7
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    • pp.621-627
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    • 1998
  • The formation of Co-silicide between Co/Zr bilayer on the amorphous and crystalline Si substrates has been investigated. The films of Zr(50$\AA$) and Co(l50$\AA$) were deposited with e-beam evaporation system and were heattreated with the rapid thermal annealing system at the temperatures between 50$0^{\circ}C$ and 80$0^{\circ}C$ with 10$0^{\circ}C$ increments for 30 seconds. The phase identification of Co-silicide was carried out by XRD and the chemical analysis was examined by AES and RBS. The interface morphologies of Co/Zr bilayer films were investigated by cross sectional TEM and HRTEM. $CoSi_2$ was formed epitaxially on the crystalline Si substrate above $700^{\circ}C$ while polycrystalline $CoSi_2$ was grown on the amorphous Si substrate. The formation temperature of Co-silicide on the amorphous Si substrate was about 100 C lower than that on the crystalline Si. The COzSi phase was not identified on the both Si substrates. The formation temperature of first phase of Co-silicide on ColZr bilayer was higher than that on Co mono layer. CoSizlayer formed on the amorphous Si substrate exhibits better uniformity compared to the CoSiz formed on the crystalline substrate. The sheet resistance of CoSiz layer on crystalline Si was lower than that on the amorphous Si at high temperatures.tures.

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Analysis of Potential Infection Site by Highly Pathogenic Avian Influenza Using Model Patterns of Avian Influenza Outbreak Area in Republic of Korea (국내 조류인플루엔자 발생 지역의 모델 패턴을 활용한 고병원성조류인플루엔자(HPAI)의 감염가능 지역 분석)

  • EOM, Chi-Ho;PAK, Sun-Il;BAE, Sun-Hak
    • Journal of the Korean Association of Geographic Information Studies
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    • v.20 no.2
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    • pp.60-74
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    • 2017
  • To facilitate prevention of highly pathogenic avian influenza (HPAI), a GIS is widely used for monitoring, investigating epidemics, managing HPAI-infected farms, and eradicating the disease. After the outbreak of foot-and-mouth disease in 2010 and 2011, the government of the Republic of Korea (ROK) established the GIS-based Korean Animal Health Integrated System (KAHIS) to avert livestock epidemics, including HPAI. However, the KAHIS is not sufficient for controlling HPAI outbreaks due to lack of responsibility in fieldwork, such as sterilization of HPAI-infected poultry farms and regions, control of infected animal movement, and implementation of an eradication strategy. An outbreak prediction model to support efficient HPAI control in the ROK is proposed here, constructed via analysis of HPAI outbreak patterns in the ROK. The results show that 82% of HPAI outbreaks occurred in Jeolla and Chungcheong Provinces. The density of poultry farms in these regions were $2.2{\pm}1.1/km^2$ and $4.2{\pm}5.6/km^2$, respectively. In addition, reared animal numbers ranged between 6,537 and 24,250 individuals in poultry farms located in HPAI outbreak regions. Following identification of poultry farms in HPAI outbreak regions, an HPAI outbreak prediction model was designed using factors such as the habitat range for migratory birds(HMB), freshwater system characteristics, and local road networks. Using these factors, poultry farms which reared 6,500-25,000 individuals were filtered and compared with number of farms actually affected by HPAI outbreaks in the ROK. The HPAI prediction model shows that 90.0% of the number of poultry farms and 54.8% of the locations of poultry farms overlapped between an actual HPAI outbreak poultry farms reported in 2014 and poultry farms estimated by HPAI outbreak prediction model in the present study. These results clearly show that the HPAI outbreak prediction model is applicable for estimating HPAI outbreak regions in ROK.

A Study on Researches of Resource-plants for Special Use or Purpose - Based on the Articles Published in the Journal of Korean Forestry - (특용자원식물(特用資源植物)의 연구(硏究) - 한국임학회지에 게재된 논문을 중심으로 -)

  • Yi, Jae-Seon;Kim, Chul-Woo;Song, Jae-Mo;Bae, Chan-Ho;Kang, Hyo-Jin;Hwang, Suk-In;Moon, Heung-Kyu
    • Journal of Forest and Environmental Science
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    • v.19 no.1
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    • pp.85-98
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    • 2003
  • The articles, published in the Journal of Korean Forestry from Number 1(1962) to Number 6, Volume 91(2002), were surveyed and investigated for the research trend analysis about resource-plants for special use or purpose, i. e., edible plants, medicinal plants, feed resource, landscape plants, fiber plants, industrial usuage, and bee plants. If the purpose or subject matter of the research was construction or furniture timber production, mushrooms and/or pulp and paper, such research was not included in this study. These articles were classified again depending on the content of research into 14 categories: habitat environment, ecology, physiology, propagation, silviculture (tending and culture), genetics and breeding, identification, insect and disease control, animal-related research, component analysis, vegetation survey, biotechnology, management, and review. Among the total 1.434 articles published, 396 ones (27.6%) were related with plants for special use or purpose. Vegetation survey was 60 (15.2%): physiology 56(14.1%) : genetics and breeding 56(14.1%): propagation 53(13.4%): and ecology 37(9.3%). Siviculture research field included 11 articles (2.8%), which indicates that the management of resource-plants is so far from economic income as seen in the low number of management research filed articles, i. e., only 6 reports (1.5%) Korean white pine was most popular for research and included 42 articles: Robinia pseudoacacia 23: Castanea crenata 14: and ginkgo tree 14. Research related with these species had focused mainly on propagation, physiology, genetics and breeding, ecology and pest control. Based on this survey and analysis, the followings are suggested: 1. More research is required on forest herbaceous plants. 2. Cooperative research work with other industrial and/or scientific area is recommendable for commercialization including medicine, cosmetics, and food etc. 3. Research on resource-plant conservation, which includes biology, social education and policy, should be supported for next generation. 4. Mutual correspondence and information exchange about the research results between researchers and institutes is more necessary than now.

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Studies on the Seedling Production of Long Shanny, Stichaeus grigoriewi Herzenstein 1. Natural Spawning in Indoor Tanks and Embryonic Development (장갱이, Stichaeus grigorjewi Herzenstein의 종묘생산에 관한 연구 1. 실내에서의 자연산란과 난발생)

  • Lee Jung-Uie;Jo Jae-Yoon
    • Journal of Aquaculture
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    • v.8 no.3
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    • pp.195-207
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    • 1995
  • In order to study the embryonic development and hatching of wild long shanny, Stichaeus grigorjewi, were caught with the gill nets in the East Sea of Korea, and stocked at indoor tanks to induce natural spawning in February 25, 1994 and February 16 to 24, 1995. They were already matured when stocked, and average body length (50.66 cm) and body weight (1,192.74 g) of 57 females and average body length (48.62 cm) and body weight (612.58g) of 43 males were recorded. Before stocking, they were inserted with identification tags(ID tags) in the dorsal muscle, and spawning was traced by the portable reader (Destron/lDl Ltd.) Forty females among 57 spawned successfully in the average of 4 days after stocking. Females spawned almost all eggs contained in the ovaries at one time in the form of an egg mass and averaging 227,200 eggs Per egg mass. The egg mass was oval in shape, translucent milky in color, 20.32cm long axis and 14.57cm short axis in size, and 803.7g in weight. Male parents guarded their egg masses and circulated water with the tail part of the body. Fertilized egg was spherical in shape, and their average diameter was 1.54 mm. Each egg had a containing single oil globule, and it's average diameter was 0.37 mm. The average water temperature was $13.2^{\circ}C$ and incubation times after fertilization were 5 hours 25 minutes up to 2-cell stage, 13 hours up to morula stage, and 66 hours 35 minutes up to embryo formation stage. Hatching rate was approximately 10 percent in 368 hours 50 minutes after fertilization, and approxionateoly 90 percent of eggs were hatched in 425 hours 30 minutes after fertilization.

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Identification of Flavonoids from Extracts of Opuntia ficus-indica var. saboten and Content Determination of Marker Components Using HPLC-PDA (손바닥선인장 추출물의 플라보노이드 구조 규명 및 HPLC-PDA를 이용한 지표성분의 함량 분석)

  • Park, Seungbae;Kang, Dong Hyeon;Jin, Changbae;Kim, Hyoung Ja
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.46 no.2
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    • pp.210-219
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    • 2017
  • This study aimed to establish an optimal extraction process and high-performance liquid chromatography (HPLC)-photodiode array (PDA) analytical method for determination of marker compounds, dihydrokaempferol (DHK) and 3-O-methylquercetin (3-MeQ), as a part of materials standardization for the development of health functional foods from stems of Opuntia ficus-indica var. saboten (OFS). The quantitative determination method of marker compounds was optimized by HPLC analysis, and the correlation coefficient for the calibration curve showed very good linearity. The HPLC-PDA method was applied successfully to quantification of marker compounds in OFS after validation of the method in terms of linearity, accuracy, and precision. Ethanolic extracts from stems of O. ficus-indica var. saboten (OFSEs) were evaluated by reflux extraction at 70 and $80^{\circ}C$ with 50, 70, and 80% ethanol for 3, 4, 5, and 6 h. Among OFSEs, OFS70E at $80^{\circ}C$ showed the highest contents of DHK and 3-MeQ of $26.42{\pm}0.65$ and $3.88{\pm}0.29mg/OFS100g$, respectively. Furthermore, OFSEs were determined for their antioxidant activities by measuring 1,1-diphenyl-2-picrylhydrazyl (DPPH) radical scavenging and lipid peroxidation (LPO) inhibitory activities in rat liver homogenate. OFS70E at $70^{\circ}C$ showed the most potent antioxidant activities with $IC_{50}$ values of $1.19{\pm}0.11$ and $0.89{\pm}0.09mg/mL$ in the DPPH radical scavenging and LPO inhibitory assays, respectively. To identify active components of OFS, various chromatographic separation of OFS70E led to isolation of 11 flavonoids: dihydrokaempferol, dihydroquercetin, 3-O-methylquercetin, quercetin, isorhamnetin 3-O-glucoside, isorhamnetin 3-O-galactoside, narcissin, kaempferol 7-O-glucoside, quercetin 3-O-galactoside, isorhamnetin, and kaempferol 3-O-rutinoside. The results suggest that standardization of DHK in OFSEs using HPLC-PDA analysis would be an acceptable method for the development of health functional foods.