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.

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.

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.

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.

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.

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.

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.

국제 협력 - 한 발자국 가까워진 체코 원전 수출

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

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.