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.

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.

부동산협회 운영 시스템을 통한 중개 일반화

김준성,Kim, Jun-Seong 한국주택협회 2006 주택과 사람들 Vol.189 No.-

캐나다의 부동산 거래는 대부분 다중 중개 매물 서비스인 MLS(Multiple Listing Service)를 통해 이뤄진다. 어떤 방식과 절차를 거쳐 부동산 거래가 이뤄지는지, 그 과정에서 주의해야 할 점들은 무엇인지 알아보자.

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.

Sequential Therapy versus Triple Therapy for the First Line Treatment of Helicobacter pylori in Korea: A Nationwide Randomized Trial

김준성,김병욱,Su Jin Hong,김진일,Ki-Nam Shim,김지현,백광호,Sang Wook Kim,Hyun Joo Song,Ji Hyun Kim 거트앤리버 소화기연관학회협의회 2016 Gut and Liver Vol.10 No.4

Background/Aims: Eradication of Helicobacter pylori infection with standard triple therapy (TT) has declined primarily because of increased antibiotic resistance. Sequential therapy (ST) has been suggested as an alternative to TT for the first-line treatment of H. pylori. The purpose of this study was to compare the efficacy of ST with TT. Methods: This was a multicenter, randomized open-label trial performed at nine centers in Korea. Patients with H. pylori infection were randomly assigned to receive either 7 day TT or 10 day ST. Eradication rates, drug compliance, and adverse events were compared among the two regimens. Results: A total of 601 patients were enrolled between March 2011 and September 2014. The intention-to-treat eradication rates were 70.8% for TT and 82.4% for ST (p=0.001). The corresponding per protocol eradication rates were 76.9% and 88.8% for TT and ST, respectively (p=0.000). There were no statistically significant differences between the two regimens with respect to drug compliance and adverse events. Conclusions: ST achieved better eradication rates than TT as a first-line therapy for H. pylori eradication in Korea.

$Mg_2$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.

궤양성 대장염에 동반된 간정맥 혈전증 1예

김준성,김경모,Kim, Joon-Sung,Kim, Kyung-Mo 대한소아소화기영양학회 2009 Pediatric gastroenterology, hepatology & nutrition Vol.12 No.2

저자들은 궤양성 대장염으로 진단되어 치료 중이던 15세 소아에서 발생한 간정맥 혈전증(Budd-Chiari 증후군) 1예를 경험하였기에 문헌고찰과 함께 보고한다. 간정맥 혈전증은 소아 궤양성 대장염의 매우 드문 혈관계 합병증으로 이환율과 사망률의 주요한 원인이 될 수 있으므로 진단과 치료에 보다 세심한 주의가 필요하다. Patients with inflammatory bowel disease are known to have hypercoagulability and an increased risk for venous thromboembolism. The deep veins of the lower extremities and the pulmonary veins are the most common sites of thrombosis in ulcerative colitis. However, hepatic vein thrombosis (Budd-Chiari syndrome) is a very rare extra-intestinal complication of ulcerative colitis in children. We describe a case of hepatic vein thrombosis in a 15-year-old girl with ulcerative colitis who presented with abdominal pain and hematochezia. Doppler ultrasonography and an abdominal CT scan revealed the characteristic filling defects caused by large thrombi in both hepatic veins. These lesions were successfully treated with conventional management for ulcerative colitis and anticoagulation therapy.

캐나다 서부지역 전체 평균의 두배 이상 올라

김준성,Kim, Jun-Seong 한국주택협회 2006 주택과 사람들 Vol.193 No.-

캐나다 주택 시장이 국내 투자자들의 주목을 받고 있다. 캐나다의 밝고 쾌적한 날씨와 자연 녹지가 풍부한 주거 환경 덕분에 이곳에 고급 주택이나 콘도, 아파트의 매입하는 투자자들이 늘고 있다. 유학과 어학 연수뿐만 아니라 이민을 위해 이곳을 찾고 있는 캐나다의 주택 시장을 알아보았다.

LDPC 부호의 효율적인 복호 방법에 관한 연구

김준성,신민호,송홍엽,Kim, Joon-Sung,Shin, Min-Ho,Song, Hong-Yeop 한국통신학회 2004 韓國通信學會論文誌 Vol.29 No.9C

본 논문에서는 LDPC 부호의 복호 알고리즘의 하나인 Normalized-BP 알고리즘의 각각의 비트에 업데이트되는 값에 대한 신뢰도에 따라 복호 알고리즘에 필요한 표준화 인수값을 달리함으로써 성능을 향상시킬 수 있는 복호 알고리즘을 제안하였다. 제안된 복호 알고리즘은 기존의 Normalized-BP 알고리즘에 비하여 복잡도를 거의 증가시키지 않으면서 LLR-BP 알고리즘의 BER 성능에 약 0.02dB 정도까지 근접하는 성능을 나타냄을 컴퓨터 모의실험을 통하여 확인하였다. In this paper, we propose a modified Normalized-BP algorithm by changing the normalization factor according to the reliability of updated messages. Proposed algorithm has almost same decoding complexity as Normalized-BP algorithm and achieves a bit-error probability of $10^4$within 0.02dB away from compared with LLR-BP algorithm.

국제 협력 - 한 발자국 가까워진 체코 원전 수출

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