• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제23권6호
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• pp.558-566
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• 2020

Purpose: Progressive familial intrahepatic cholestasis (PFIC) is a rare genetic autosomal recessive disease caused by mutations in ATP8B1, ABCB11 or ABCB4. Mutational analysis of these genes is a reliable approach to identify the disorder. Methods: We collected and analyzed relevant data related to clinical diagnosis, biological investigation, and molecular determination in nine children carrying these gene mutations, who were from unrelated families in South China. Results: Of the nine patients (five males, four females) with PFIC, one case of PFIC1, four cases of PFIC2, and four cases of PFIC3 were diagnosed. Except in patient no. 8, jaundice and severe pruritus were the major clinical signs in all forms. γ-glutamyl transpeptidase was low in patients with PFIC1/PFIC2, and remained mildly elevated in patients with PFIC3. We identified 15 different mutations, including nine novel mutations (p.R470HfsX8, p.Q794X and p.I1170T of ABCB11 gene mutations, p.G319R, p.A1047P, p.G1074R, p.T830NfsX11, p.A1047PfsX8 and p.N1048TfsX of ABCB4 gene mutations) and six known mutations (p.G446R and p.F529del of ATP8B1 gene mutations, p.A588V, p.G1004D and p.R1057X of ABCB11 gene mutations, p.P479L of ABCB4 gene mutations). The results showed that compared with other regions, these three types of PFIC genes had different mutational spectrum in China. Conclusion: The study expands the genotypic spectrum of PFIC. We identified nine novel mutations of PFIC and our findings could help in the diagnosis and treatment of this disease.

• 대한용접접합학회:학술대회논문집
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• 대한용접접합학회 2009년 추계학술발표대회
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• pp.45-45
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• 2009

원가 측면에서 유리한 저항점용접(Resistance Spot Welding)이 차체 용접에 80%이상으로 가장 많이 적용되고 있다. 첨단고강도강(Advanced High Strength Steel)의 저항점용접성 및 용접부 특성에 미치는 공정 변수의 영향에 대한 연구결과는 많으나, 합금원소의 영향에 대해서는 전무하다. 특히, Si는 DP(Dual Phase)강에 첨가 시 균일한 마르텐사이트의 분포를 촉진하는 원소로 저항 점용접성 및 용접부 특성에 영향을 미칠 것으로 예상되며, 이에 대한 연구는 보고된바 없다. 본 연구에서는 냉연 DP강의 저항 점용접시 중요한 인자 중 하나인 너깃경과 전단인장강도에 미치는 Si함유량의 영향을 검토하였다. 사용된 강재 및 용접기는 1.2mm 두께의 Si함유량(0, 0.5, 1.0, 1.5wt%)이 다른 인장강도 780~1000MPa급 냉연 DP강과 단상 AC용접기를 사용하였다. 용접조건은 ISO 18278-2규격에 따라 가압력 4kA, 초기가압시간 40cycle, 유지시간 17cycle로 고정하고, 용접전류만 변화하여 용접을 실시하였다. 너깃경은 용접부 단면을 컷팅 후 폴리싱 하여, 광학현미경과 Image Pro plus를 이용하여 측정했으며, 인장시편규격은 JIS Z 3137를 이용하였다. Si함유량이 증가에 따라 스패터 발생 전류는 감소했고, 너깃경은 직선적으로 증가했다. Si함유량 증가에 따른 너깃경 증가 이유는 저항(R) 측정결과, Si함유량 증가에 따라 모재의 저항이 높아져, 따라서 입열량($Q=I^2Rt$)이 많아지기 때문으로 판단되었다. 인정전단강도는 Si함유량 증가에 따라 직선적으로 증가했다. 이러한 이유는 Si함유량 증가에 따라 너깃경이 증가되기 때문으로 판단되었고, 너깃경과 인장전단강도 사이에 직선적 관계(PL(kN)=$3.2N_{dia.}$-0.81, $R^2$=0.93)를 가지고 있었다. 파단양상은 Si함유량에 상관없이 5.4kA이하에서는 계면파단이 일어났고, 6.0kA이상에서는 풀 아웃 파단이 일어났다. 계면파단주원인은 용접부 가장자리에 지름이 약 $5{\mu}m$이하의 예리한 노치가 존재하여 노치응력집중과 HAZ계면 근처에 미접합부가 존재하기 때문으로 판단되었다. 6.0kA이상에서는 예리한 노치가 없었고, HAZ부가 완전히 접합되어 있기 때문에 풀 아웃 파단이 일어난 것으로 판단되었다. 따라서, Si함유량 증가에 따라 적정용접전류 구간은 감소했고, 너깃경은 직선적으로 증가했다. 또한, Si함유량 증가에 따라 인장전간강도는 증가 했으며, 너깃경과 인장전단강도 사이에 직선적 관계를 가지고 있었다. 파단 양상은 Si함유량에 상관없이 5.2kA이하에서는 계면파단이, 6.0kA이상에서는 풀 아웃 파단이 일어났다.

• Journal of Preventive Medicine and Public Health
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• 제22권1호
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• pp.153-161
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• 1989

Authors studied on the prevalence of 94 cases of pneumoconiosis who were found out through the screening test on 1,062 workers engaged in welding process at 36 shipyard in Pusan area from March 1st, 1986 to November 30th, 1986. The result were as follows; 1. Dust concentration was measured $4.49{\pm}0.54mg/m^3$ in the small scale shipyard while it was $6.25{\pm}1.08mg/m^3$ in the large one. 2. The prevalence of welder's lung was 8.9% (male:8.5%, female:12.0%) and this is consist of 4.9% suspected pneumoconiosis and 4.0% pneumoconiosis more than category 1/0. 3. The prevalence was significantly increased according to the duration of dust exposure(p<0.05), and it showed the increasing tendency by the age group. 4. The prevalence was significantly higher in the large scale shipyard than is the small scale one(p<0.01). 5. The type and shape of opacities were 71.4% of p type and 28.6% of q type, however no pneumoconiosis with r type observed in this study. 6. The main subjective symptoms were the sputum(29.8%), coughing(25.5%), shortness of breath(20.2%), fatique(6.4%), and chest pain(5.3%). In other hand, 57.4% of pneumoconiosis were asymptomatic. 7. The prevalence of pulmonary tuberculosis was radiologically 1.7% in all subjects including 5.3% in pneumoconiosis and 1.3% in no pneumoconiosis(p<0.01).

• 동의생리병리학회지
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• 제19권5호
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• pp.1169-1180
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• 2005

First, I read all the materials, including Dongeuibogam(Encyclopedia Medica Koreana), classical writings on Lacca sinica exsiccata, herbal writings on lacquer poison, and herbal books on how to treat lacquer poison. And then after 1 examined all the details on qi and taste of Lacca sinica exsiccata, its efficacy, detoxification, lacquer poison, and its effects on body symptom, 1 got the following results. The order of frequency that Lacca sinica exsiccata is used in Dongeuibogam is pressure-feeling, blood circulation, and insect biting. Its way of intake is not so much through herb-boiling or powdered medicine as through hand-made pills. When medicine is used in the form of pills, the Lacca sinica exsiccata is more included among other ingredients. When old doctors treated pressure-feeling in the chest, they mixed up other herbs, with not putting more emphasis on the efficacy of lacquer 010 doctors believed that toxicity of Lacca sinica exsiccata is not having its own poison, but having biased dominance in the use of its qE and taste. The way or detoxification or Lacca sinica exsiccata is used in the order of crab-boiled water, egg, Xanthoxylum piperitum, Perilla frutescens, Astar tataricus, a weeping willow, iron-tempered water, and Allium toberosum. Special point in detoxificating lacquer poison is that they used medicines for well-ciruculating pulmonary stream, medicines for promoting to urinate or discharge by helping large colons to move, medicines for making the lacquer scar small, medicines for helping digest, and medicines for improving vessel function in the poisoned area. With the above results, the more profound study, based on the crab-boiled water and egg, is expected to go on to increase the effect on the one hand, and to make the new way of lessening or removing the toxicity of lacquer with more safe use on the other hand.

• 자원환경지질
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• 제16권3호
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• pp.163-221
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• 1983

Main aspects of this study are to clarify geochronology and petrogenetic processes of the so-called Hongjesa granite, which is a member of various intrusive rocks exposed in the northeastern part of the Ryongnam Massif, one of the Precambrian basements of South Korea. In this study, the Hongjesa grainte is divided into four rock units based on the geologic age, mineralogical and chemical constituents, and texture: the Precambrian Hongjesa granite gneiss (Hongjesa granite Proper) and leucogranite gneiss, the Paleozoic gnessic two mica granite, and the Jurassic muscovite granite. The Hongjesa granite gneiss is identified by its grayish color, slight foliation, and porphyroblastic texture. The leucogranite gneiss is distinct by its light gray color, sand medium to coarse grained texture. The gneissic two mica granite is distinguished from others by its strong foliation, containing gray-colored feldspar phenocrysts with biotite and muscovite in varying amounts. The muscovite granite occurs as a small stock containing feldspar phenocrysts along margin of the stock. These granitic rocks vary widely in composition, reflecting the facts that they partly include highly metamorphosed xenolith and schlierens as relics of magmatic and anatectic processes. In particular, grayish porphyroblasts of microcline perthite is characteristic of the Hongjesa granite gneiss, whereas epidote and garnet occur in both the Hongjesa granite gneiss and leucogranite gneiss. These minerals are considered to be formed by potassic metasomatism and contamination of highly metamorphosed rocks deeply buried under the level of the Hongjesa granite emplacement. The individual synchronous granitic rocks plotted on Harker diagram show mostly similar trends to the Daly's values. The plots of the Hongjesa granite gneiss and gneissic two mica granite concentrate near the end part of the calc-alkalic rock series on the AMF diagrams, whereas those of the leucogranite gneiss and muscovite granite indicate the trend of the Skaergaard pluton. These granitic rocks plotted on a Q-Ab-Or diagram (petrogeny's residua system) fall well outside the trough of the system. This can be attributed to the potassic matasomatism of these rocks. On the ACF diagram, these rocks appear to be dominantly I-type prevailing over S-type. The K-Ar ages, obtained from a total of 7 samples of the leucogranite gneiss, gneissic two mica granite, muscovite granite, porphyritic alkali granite, and rhyolitic rock, in addition to the Rb/Sr ages of the Hongjesa granite gneiss by previous workers, permit the rock units to be arranged in the following chronological order: The middle Proterozoic Hongjesa granite gneiss (1714-1825 m.y.), the upper proterozoic leucogranite gneiss (875-880 m. y.), the middle Paleozoic gneissic two mica granite (384 m. y.) the upper Jurassic muscovite granite (147 m. y.), the Eocene alkali granite (52 m. y.), and the Eocene rhyolitic rock (45 m. y.). From the facts and data mentioned above, it is concluded that the so-called Hongjesa granite is not a single granitic mass but is further subdivided into the four rock units. The Hongjesa granite gneis, leucogranite gneiss, and gneissic two mica granite are postulated to be either magmatic or parautochtonous, intrusive, and the later muscovite granite is to be magmatic in origion.

• 대한유전성대사질환학회지
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• 제2권1호
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• pp.77-79
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• 2002

The urea cycle, consisting of a series of six enzymatic reactions, plays key roles to prevent the accumulation of toxic nitrogenous compound and synthesize arginine de novo. Five well characterized diseases have been described, resulting from an enzymatic defect in the biosynthesis of one of the normally expressed enzyme. This presentation will focus on two representative diseases; ornithine transcarbamylase(OTC) deficiency and citrullinemia(argininosuccinate synthetase deficiency). OTC deficiency is one of the most common inborn error of urea cycle, which is inherited in X-linked manner. We identified 17 different mutations in 20 unrelated Korean patients with OTC deficiency; L9X, R26P, R26X, T44I, R92X, G100R, R141Q, G195R, M205T, H214Y, D249G, R277W, F281S, 853 del C, R320X, V323M and 10 bp del at nt. 796-805. These mutations occur at well conserved nucleotide sequences across species or CpG hot spot. The L9X and R26X lead to the disruption of leader sequences, required for directing mitochondrial localization of the OTC precursor. Their phenotypes are severe, and neonatal onset. The G100R, R277W and V323M mutations were uniquely identified in patients with late onset OTC deficiency. The other genotypes are associated with neonatal onset. Out of 20 patients with OTC deficiency, only 6 patients are alive; two were liver transplanted, and normal in growth and development at 2, 4 years after transplantation respectively. Citrullinemia is an autosomal recessive disease, caused by the mutations in the argininosuccinate synthetase(ASS) gene. We identified in 3 major mutations in 11 unrelated Korean patients with citrullinemia; G324S, $IVS6^{-2}$ A to G, and 67 bp ins at nt 1125-1126. Among these, the 67 base pair insertion mutation is novel. The allele frequency of each mutation is; G324S(45%), IVS6-2 A to G(32%), and 67 base pair insertion(14%). All patients are diagnosed at neonatal or infantile age. Interestingly, two patients presented with stroke like episode. Out of 11 patients, 5 patients died. Among 6 patients alive, one patient was successfully liver transplanted.

• The Korean Journal of Physiology and Pharmacology
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• 제17권3호
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• pp.223-228
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• 2013

The calcium-activated $K^+$ ($BK_{Ca}$) channel is one of the potassium-selective ion channels that are present in the nervous and vascular systems. $Ca^{2+}$ is the main regulator of $BK_{Ca}$ channel activation. The $BK_{Ca}$ channel contains two high affinity $Ca^{2+}$ binding sites, namely, regulators of $K^+$ conductance, RCK1 and the $Ca^{2+}$ bowl. Lysophosphatidic acid (LPA, 1-radyl-2-hydroxy-sn-glycero-3-phosphate) is one of the neurolipids. LPA affects diverse cellular functions on many cell types through G protein-coupled LPA receptor subtypes. The activation of LPA receptors induces transient elevation of intracellular $Ca^{2+}$ levels through diverse G proteins such as $G{\alpha}_{q/11}$, $G{\alpha}_i$, $G{\alpha}_{12/13}$, and $G{\alpha}s$ and the related signal transduction pathway. In the present study, we examined LPA effects on $BK_{Ca}$ channel activity expressed in Xenopus oocytes, which are known to endogenously express the LPA receptor. Treatment with LPA induced a large outward current in a reversible and concentration-dependent manner. However, repeated treatment with LPA induced a rapid desensitization, and the LPA receptor antagonist Ki16425 blocked LPA action. LPA-mediated $BK_{Ca}$ channel activation was also attenuated by the PLC inhibitor U-73122, $IP_3$ inhibitor 2-APB, $Ca^{2+}$ chelator BAPTA, or PKC inhibitor calphostin. In addition, mutations in RCK1 and RCK2 also attenuated LPA-mediated $BK_{Ca}$ channel activation. The present study indicates that LPA-mediated activation of the $BK_{Ca}$ channel is achieved through the PLC, $IP_3$, $Ca^{2+}$, and PKC pathway and that LPA-mediated activation of the $BK_{Ca}$ channel could be one of the biological effects of LPA in the nervous and vascular systems.

• 한국자원식물학회:학술대회논문집
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• 한국자원식물학회 2018년도 춘계학술발표회
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• pp.77-77
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• 2018

미역의 뿌리부분인 미역귀에는 알긴산과 후코이단 성분이 풍부하고 고혈압과 당뇨등과 같은 성인병을 예방하는데 도움이 된다. 후코이단은 미역과 다시마 같은 갈조류에서 추출된 천연물질로 특히 면역력을 활성화하고 항암작용으로 잘 알려져 있다. 알긴산 등 유용성분이 함유된 것으로 알려진 미역귀의 유용성분을 분석하여, 향후 미역귀를 활용한 제품개발의 기초자료를 제공하고자 본 연구를 수행하였다. 본 연구에 사용된 미역귀는 2015년 12월 완도 금일수산에서 제공받아 연구재료로 활용하였다. AOAC방법에 따라 분석한 미역귀의 일반성분은 수분이 대부분을 차지하였으며, 조섬유와 가용성 무질소물 함량이 높게 나타났다. 이는 시료로 사용한 미역귀가 수확 후 바로 냉동보관 되어서 수분함량이 높은 것으로 보여진다. 유리당은 미역귀 1g에 증류수를 가하여 $60^{\circ}C$에서 4시간 추출 한 후, 원심분리(3,000 rpm, 30 min)하여 $0.45{\mu}m$ membrane filter (Millipore Co., USA)로 여과한 여액을 HPLC (High Performance Liquid Chromatography)를 이용하여 분석한 결과, 미역귀의 유리당은 Galactose 1종만이 검출되었다. 미역귀의 구성아미노산과 유리아미노산은 $0.45{\mu}m$ membrane filter로 여과하여 얻은 여액을 일정량 취하여 AccQ-Tag 시약을 사용하여 유도체화 시킨 후 HPLC로 분석하였다. 미역귀의 구성아미노산은 glutamic acid, aspartic acid, alanine, lysine, threonine, valine, arginine 순으로 높은 함량을 나타내었으며, 유리아미노산은 glutamic acid, alanine, threonine, arginine, lysine 순으로 높은 함량을 나타내었다. 미역귀의 유리아미노산함량은 표고에 비하여 현저히 낮은 함량이 나타났으며, 주요아미노산은 해조류의 풍미를 대표하는 아미노산인 glutamic acid와 alanine으로 나타났다.

• Journal of the Korean Data and Information Science Society
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• 제27권5호
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• pp.1375-1387
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• 2016

본 연구는 65세 이상의 정상 노인을 대상으로 운동요법이 인지기능에 미치는 영향을 체계적 문헌고찰 및 메타분석을 통해 확인하고자 수행되었다. 본 연구는 국내 주요 DB를 이용하여 논문검색을 하였으며 3.197편 중 선정기준에 적합한 최종 14편의 논문을 체계적 문헌고찰하였으며 이 중 효과크기를 산출할 수 있는 11편을 대상으로 메타분석을 실시하였다. 분석은 랜덤효과모형을 이용하였으며, 운동중재의 효과크기를 산출하였다. 본 연구에서 운동중재의 인지기능에 대한 효과크기는 Hedges' g=1.05 (95% CI: 0.61~1.50)로 큰 효과크기를 보였으며, 전체 이질성이 $I^2=82.3%$ (Q=56.61, p<.001)로 중재방법, 주당 중재횟수, 중재기간, 측정도구를 조절변수로 하여 조절효과를 분석하였다. 분석결과 중재방법, 주당 중재횟수, 중재기간에서는 유의한 차이가 없었으며, 측정도구에서 유의한 차이가 있는 것으로 나타났다. 본 연구는 우리나라 노인의 인지기능에 운동이 미치는 영향에 대한 국내연구결과들을 체계적으로 분석하고, 그 효과크기를 객관적으로 제시하여 향후 인지기능 향상을 위한 중재전략으로 운동요법을 설계할 때 기초자료를 제공하였다는 데에 의의가 있다.

• 한국병원경영학회지
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• 제22권3호
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• pp.61-73
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• 2017

Purposes : The purpose of this study is to identify factors that have effects on safety activities of hospital personnels by investigating causality between patient safety culture, job stress, safety system and safety activities of faculty and staff member who are working for oriental medicine hospital of university. Methodology : The subjects were 246 employees working in 4 oriental medicine hospitals of university in Daejeon and Chungcheongnam-do. The data were collected from January 16 to January 25, 2017 using a structured questionnaire. For data analysis, descriptive statistics, Pearson correlation coefficient, t-test, ANOVA and Duncan test with SPSS 22.0 were used. Findings : The activity score for patient safety of faculty and staff member, who were experienced at job training program after joining a company and regular training course for qualification or license, was meaningfully higher than that of group who had no job training experience. The result indicated that the higher there is level of safety culture and safety system and the lower there is work stress, the more the activity has positive effect on patient safety. The level score of awareness for safety culture of faculty and staff member in C hospital, which is facing financial crisis in business circumstances recently, showed average value of 3.29. It was significantly lower than that of the other three hospital. Also, The activity score for patient safety was markedly lower than that of the other three hospital. This result become interested in the process of linking non-financial performance and financial performance. The level score of safety activity in A hospital which obtained healthcare accreditation was remarkably higher than that of the other three hospital which didn't certify healthcare accreditation. Pratical Implications : Subjects about Q.I or patient's experience management must be included in curriculum of Oriental medicine. It is necessary to get the effect of job training program for faculty and staff member through the process of preparation for obtaining healthcare accreditation. When the hospital director is appointed, it must be considered that he/she has the ability to attach importance to analysis and management of the factors creating safety accident, and has business mindset for healthcare delivery of customer- centricity. This research showed that financial performance of hospital, which of business environment is favorable; located in metropolitan city and having large scale of hospital and quality of residence rating but low-level of safety culture and safety activity, was lower than that of general hospital situated in small and medium-sized cities. More research needs to be done for answering this result.