Lesch-Nyhan 증후군 1례

김준성,이재승,노하영,김병주,우영종,박지민,김명관,김구환,유한욱,Kim, Joon-Sung,Lee, Jae-Seung,Noh, Ha-Young,Kim, Byung-Ju,Woo, Young-Jong,Park, Jee-Min,Kim, Myung-Gwan,Kim, Gu-Hwan,Yoo, Han-Wook 대한소아청소년과학회 2003 Clinical and Experimental Pediatrics (CEP) Vol.46 No.5

저자들은 빈번한 구토와 성장 장애, 발달 지연 등을 주소로 내원한 1년 7개월된 남아에서 HPRT에 대한 생화학적 효소 분석을 통해 진단하고, HPRT 유전자에 대한 분자유전학적 분석을 시행하여 병인이 되는 돌연변이가 확인된 Lesch-Nyhan 증후군 1례를 경험하였기에 이를 문헌고찰과 함께 보고하는 바이다. Lesch-Nyhan syndrome is an X-linked recessive disorder characterized by hyperuricemia, choreoathetosis, spasticity, mental retardation, and compulsive, self-injurious behavior. This disorder results from a complete deficiency of the purine salvage enzyme, hypoxanthine-guanine phosphoribosyl transferase(HPRT). We report here on a case of Lesch-Nyhan syndrome in a 1-year, 7-month-old male who presented with frequent vomiting, failure to thrive, and developmental delay. The diagnostic work-up revealed hyperuricemia, hyperuricosuria, and medullary nephrolithiasis. The HPRT activity in the erythrocytes was undetectable with a biochemical assay. We also identified de novo mutation which was a deletion of the 649th base, adenosine, in HPRT gene(649delA) by analysis of cDNA using RT-PCR technique coupled with direct sequencing.

Polyethylene Terephthalate (PET) FOAM의 전과정 환경성 평가(LCA)적 고찰

김준성,Kim, Jeun-Seong 한국복합신소재구조학회 2013 복합신소재학회논문집 Vol.4 No.1

$Mg_2$Ni계 수소저장합금전극의 퇴화거동에 미치는 불화 처리 영향

김준성,최재웅,이창래,강성군,Kim, Jun-Seong,Choe, Jae-Ung,Lee, Chang-Rae,Gang, Seong-Gun 한국재료학회 2001 한국재료학회지 Vol.11 No.4

기계적 합금화 방법을 이용하여 제작한 Ni-MH 전지용 $Mg_2$Ni전극의 표면 불화처리에 대한 전기화학적 충.방전 특성이 조사되었다. 20시간 밀링을 통해 제조된 $Mg_2$Ni합금은 나노결정을 가졌으며 그 $Mg_2$Ni전극의 KOH전해질내에서 충.방전 실험 결과, 초기 방전 용량이 280mAh/g이상으로 증가하였으나 10cyc1e이내에 급격히 퇴화되었다. 전극표면에서 지속적이고 안정한 불화층 형성을 목적으로 KOH용액에 잉여의 불소이온이 첨가된 $Mg_2$Ni전극의 내구성은 크게 향상되었으며 특히 2N KF를 첨가했을 경우 전극의 내구성이 가장 크게 향상되었다. 고율 방전실험의 경우도 그 성능이 90-100mAh/g으로 유지되었다 이러한 내구성 향상의 이유는 표면에 얕고 다공성인 $Mg_2$Ni층의 형성으로 인해 퇴화의 주요인인 Mg(OH)$_2$의 생성이 억제되었기 때문이었다. Effects of the surface fluorination on the electrochemical charge-discharge properties of $Mg_2$Ni electrode in Ni-MH batteries fabricated by mechanical alloying were investigated. After 20h ball milling, Mg and Ni powder formed nanocrystalline $Mg_2$Ni. Discharge capacity of this alloy increased greatly at first one cycle, but due to the formation of Mg(OH)$_2$ passive layer, it showed a rapid degradation in alkaline solution within 10cyc1es. In case of 6N KOH +xN KF electrolyte (x = 0.5, 1, 2), a continuous and stable fluorinated layer formed by adding excess F$^{-}$ ion, increased durability of $Mg_2$Ni electrode greatly and high rate discharge capability(90-100mAh/g). 2N KF addition led to the highest durability of all tested here. The reason of the improvement is due to thin MgF$_2$, which can prevent the $Mg_2$Ni electrode from forming Mg(OH)$_2$layer that is the main cause of degradation. Effects of the surface fluorination on the electrochemical charge-discharge properties of $Mg_2$Ni electrode in Ni-MH batteries fabricated by mechanical alloying were investigated. After 20h ball milling, Mg and Ni powder formed nanocrystalline $Mg_2$Ni. Discharge capacity of this alloy increased greatly at first one cycle, but due to the formation of Mg(OH)$_2$ passive layer, it showed a rapid degradation in alkaline solution within 10cyc1es. In case of 6N KOH +xN KF electrolyte (x = 0.5, 1, 2), a continuous and stable fluorinated layer formed by adding excess F$^{[-10]}$ ion, increased durability of $Mg_2$Ni electrode greatly and high rate discharge capability(90-100mAh/g). 2N KF addition led to the highest durability of all tested here. The reason of the improvement is due to thin MgF$_2$, which can prevent the $Mg_2$Ni electrode from forming Mg(OH)$_2$layer that is the main cause of degradation.

온라인 쇼핑몰에서의 리뷰 속성이 브랜드 태도, 구매결정 및 온라인 구전의도에 미치는 영향

김준성,Kim, Joon-Sung 한국디지털정책학회 2021 디지털융복합연구 Vol.19 No.7

본 연구는 온라인 쇼핑몰에서 리뷰의 속성을 평점, 댓글의 양 및 이미지정보로 분류하여 브랜드 태도와 구매결정 및 온라인 구전의도에 미치는 영향을 검증하였다. SPSS 23.0을 이용하여 빈도분석, 요인분석, 회귀분석 등을 실시하였다. 분석 결과, 리뷰 속성이 브랜드 태도, 구매결정 및 온라인 구전의도에 정(+)의 영향을 미치는 것이 확인되었으나 댓글의 양은 구매결정에 영향을 미치지 않은 것으로 나타났다. 브랜드 태도는 구매결정에, 온라인 구전의도에 정(+)의 영향을 미치는 것이 확인되었다. 더불어 평점, 이미지정보와 구매결정 간의 영향관계에서, 또한 리뷰 속성과 온라인 구전의도 간의 영향관계에서 브랜드 태도의 매개효과가 확인되었다. 이와 같은 결과는 소비자가 무조건 댓글의 양이 많은 것을 선호하지 않으며, 리뷰를 작성할 때 최대한 높은 평점과 포토후기에 초점을 맞출 수 있도록 해야 함을 시사한다. This study classifies review attributes into ratings, number of comments and image information in online shopping mall to verify their impact on brand attitude and purchase decision and e-WOM intention. Use SPSS 23.0 for frequency analysis, factor analysis and regression analysis. The results showed that review attributes have a positive effect on brand attitudes, purchase decision and e-WOM intention, but the number of comments has not affect on purchase decision. Brand attitude has a positive effect on purchase decision and e-WOM intention. Brand attitude has media effect in the relationship between ratings, image information and purchase decision, and in the relationship between review attributes and e-WOM intention. As these results, consumers don't always like to have a lot of comments. and should allow to focus on high ratings and photo reviews as much as possible when writing reviews.

Calcium sulfate와 혈소판 유래성장인자의 혼합사용이 치주인대세포에 미치는 영향

김준성,최성호,유윤정,채중규,김종관,조규성,Kim, Jun-Seong,Choi, Seong-Ho,Yu, Yun-Jung,Chai, Jung-Kiu,Kim, Chong-Kwan,Cho, Kyoo-Sung 대한치주과학회 1997 Journal of Periodontal & Implant Science Vol.27 No.4

It was well known that calcium sulfate was biocompatible, resorbed rapidly in the body, had potential as a good barrier membrane. Platelet-derived growth factor(PDGF) was one of polypeptide growth factor that had been reported as a biological mediator which regulates activities of wound healing process including the cell proliferation, migration and metabolism. The purpose of this study was to evaluate the effects of a combination of calcium sulfate and PDGF on periodontal ligament cells in vitro to use as a regeneration promoting agent of periodontal tissue. Human periodontal ligament cells were prepared from the premolar tooth extracted for the orthodontic treatment. Cells were cultured in ${\alpha}-MEM$ contained with 20% FBS, at the $37^{\circ}C$, 100% of humidity, 5% $Co_2$ incubator. Cells were inoculated and cultured into 96 well culture plate with $1{\times}10^4cells/well$ of ${\alpha}-MEM$ for 1 day. After discarding the medium, those cells were cultured in ${\alpha}-MEM$ contained with 10% FBS alone(control group), in calcium sulfate(calcium sulfate group), in calcium sulfate treated with 15ng/ml of PDGF-BB(calcium sulfate+PDGF group), in ${\alpha}-MEM$ contained with 10% FBS treated with 15ng/ml of PDGF-BB(PDGF group) for 1, 2, 3 day respectively. And then each group was characterized by examining of the cell counting, MTT assay, collagen synthesis. The results were as follows. 1. In the analysis of cell proliferation by cell counting, both calcium sulfate group and calcium sulfate plus PDGF group showed no stastically significant difference compared to control group, but there was stastically significant difference between PDGF group and calcium sulfate group at 1, 2 day(P<0.05). 2. In the analysis of cell proliferation by MTT assay in calcium sulfate extracts, both calcium sulfate group and calcium sulfate plus PDGF group showed no stastically significant difference compared to control group, but there was stastically significant difference between PDGF group and calcium sulfate group at 2, 3 day, and between calcium sulfate plus PDGF group and calcium sulfate group at 2 day(P<O.05). 3. In the analysis of cell proliferation by MTT assay in transwell, both control group and PDGF group showed stastically significant difference compared to both calcium sulfate group and calcium sulfate plus PDGF group at 1 day, but there was no stastically significant difference compared to both calcium sulfate group and calcium sulfate plus PDGF group at 2, 3 day(P(0.05). 4. In the analysis of collagen synthesis by immunoblotting assay in calcium sulfate extracts, high level was detected on calcium sulfate group at 3 day, on calcium sulfate plus PDGF group at 1 day, on PDGF group at 2 day. On the basis of these results, calcium sulfate was biocompatible on the periodontal ligament cells and might have potential possibility as a vehicle of PDGF in the periodontal tissue regeneration.

국제 협력 - 한 발자국 가까워진 체코 원전 수출

김준성,Kim, Jun-Seong 한국원자력산업회의 2017 원자력산업 Vol.37 No.12

청소년에서 컴퓨터 작업의 종류와 작업 시간이 눈깜박임 횟수와 안구건조에 미치는 영향

김준성,조경준,송종석.Jun Sung Kim. M.D.. Kyung Jun Cho. M.D.. Jong-Suk Song. M.D. 대한안과학회 2007 대한안과학회지 Vol.48 No.11

Purpose: To evaluate the change in blink rate in adolescents according to the type and duration of computer tasks and to analyze the risk of ocular dryness using an ocular protection index. Methods: Fifteen male high school students played computer games and viewed Internet lectures for 20 minutes each. Blink rate was measured by USB camera, and ocular protection index (OPI, the ratio of BUT to inter-blink interval) was calculated for each subject during the two tasks. In addition, 7 of the 15 subjects played computer games again for 40 minutes on another day, and the change in blink rate according to the duration of computer games was observed. Results: The average blink rate was 5.44±3.29 per minute (median 5, range 2-12.75) while playing computer games and 20.63±11.21 per minute (median 18.25, range 4.93-42.2) while viewing Internet lectures, and this difference was statistically significant (p=0.001). The percentage of subjects with OPI values less than 1 was 53% for computer games and 13% for Internet lectures (p=0.025). While playing computer games for 40 minutes, the mean of total blinks during ten-minute intervals decreased over time: 59.43 times (median 57) during the first 10 minutes, 33.86 times (median 41) during the last 10 minutes. Conclusions: The blink rate in adolescents was significantly lower and the risk of ocular dryness was higher while playing computer games compared with that of viewing internet lectures. As playing time increased, the blink rate decreased and risk of ocular dryness increased.

하천에서 유해화학물질 확산 예측을 위한 2차원 수질모형 CTM-2D-TX의 개발

김준성,정성현,윤세훈,서일원,Kim, Jun-Seong,Jeong, Seong-Hyeon,Yun, Se-Hun,Seo, Il-Won 한국수자원학회 2021 물과 미래(한국수자원학회지) Vol.54 No.3

조경수관리

김준성,Kim, Jun-Seong 한국조경수협회 2009 造景樹 Vol.112 No.-

RAMS+를 이용한 하천에서 오염물질의 2차원 체류시간 분포 모델링

김준성,서일원,신재현,정성현,윤세훈,Kim, Jun Song,Seo, Il Won,Shin, Jaehyun,Jung, Sung Hyun,Yun, Se Hun 한국수자원학회 2021 한국수자원학회논문집 Vol.54 No.7

With the recent industrial development, accidental pollution in riverine environments has frequently occurred. It is thus necessary to simulate pollutant transport and dispersion using water quality models for predicting pollutant residence times. In this study, we conducted a field experiment in a meandering reach of the Sum River, South Korea, to validate the field applicability and prediction accuracy of RAMS+ (River Analysis and Modeling System+), which is a two-dimensional (2D) stream flow/water quality analysis program. As a result of the simulation, the flow analysis model HDM-2Di and the water quality analysis model CTM-2D-TX accurately simulated the 2D flow characteristics, and transport and mixing behaviors of the pollutant tracer, respectively. In particular, CTM-2D-TX adequately reproduced the elongation of the pollutant cloud, caused by the storage effect associated with local low-velocity zones. Furthermore, the transport model effectively simulated the secondary flow-driven lateral mixing at the meander bend via 2D dispersion coefficients. We calculated the residence time for the critical concentration, and it was elucidated that the calculated residence times are spatially heterogeneous, even in the channel-width direction. The findings of this study suggest that the 2D water quality model could be the accidental pollution analysis tool more efficient and accurate than one-dimensional models, which cannot produce the 2D information such as the 2D residence time distribution.