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Clinical Findings of Genotypes in Korean Patients with Glycogen Storage Disease Type Ia (한국인 당원병 제 Ia형에서 유전형의 임상 양상)

  • Ko, Jae Sung;Yang, Hye Ran;Kim, Jong Won;Seo, Jeong Kee
    • Clinical and Experimental Pediatrics
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    • v.48 no.8
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    • pp.877-880
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    • 2005
  • Purpose : Glycogen storage disease type Ia(GSD Ia) is an autosomal recessive disorder caused by the deficiency of glucose-6-phosphatase(G6Pase). The aim of the study was to investigate the spectrum of G6Pase gene mutations and relationship between genotype and clinical findings in Korean patients with GSD Ia. Methods : Genomic DNA was extracted from peripheral leukocytes of 20 patients with GSD Ia. The five exons of G6Pase gene were amplified and PCR products were directly sequenced. The frequency of short stature, hypoglycemia, hypercholesterolemia, hyperuricemia, hypercalciuria, nephrocalcinosis and hepatic adenoma was compared between 727G>T homozygotes and 727G>T compound heterozygotes. Results : A total of 5 different mutations were identified. The most common mutation was the 727G>T with an allele frequency of 80%. All patients were either homozygous(12/20) or heterozygous(8/20) for the 727G>T mutation. G122D was found in 3 patients, P178A in 1, G222R in 2, and S339R in 2. There was no difference in the frequency of short stature, hypoglycemia, hypercholesterolemia, hyperuricemia, nephrocalcinosis, and hepatic adenoma between 727G>T homozygotes and heterozygotes. Conclusion : Diagnosis of GSD Ia can be based on clinical and biochemical abnormalities combined with mutation analysis instead of enzymatic diagnosis that requires liver biopsy. Homozygosity for the 727G>T does not seem to alter the disease phenotype as compared with the heterozygous state.

Seismic Performance Evaluation of Post-Northridge WUF-B Connections Frame (Post-Northridge WUF-B 접합부 골조의 내진 성능 평가)

  • Han, Sang Whan;Moon, Ki Hoon;Jung, Ji Eun;Lee, Ki Hak
    • Journal of Korean Society of Steel Construction
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    • v.19 no.6
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    • pp.751-760
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    • 2007
  • The purpose of this study is to evaluate the seismic performance of the frame which is assumed to be used with Welded Unreinforced Flange-Bolted web (WUF-B) connections and detailed in compliance with FEMA 350 recommended seismic design criteria. In FEMA 350, these types of connections are only valid for Ordinary Moment-Resisting Steel Frames (OMRSFs). For this purpose, based on test results, we proposed an analytical model for the Post-Northridge WUF-B connections with different panel zone strength ratios. Using the connection model, SAC Phase II three and nine-story frames were modeled and analyzed. From a nonlinear static pushover analysis, ductility, maximum strength, and the maximum interstory drift ratio were investigated for buildings with Post-Northridge details. Moreover, the maximum interstory drift ratio of each performance level (IO and CP) was also investigated through Incremental Dynamic Analysis (IDA). Analytical results were compared with those of buildings with either Pre-Northridge connection or ductile connections with no fracture. The analytical results showed that buildings with Post-Northridge WUF-B connections provide superior strength and interstory drift ratio capacity than buildings with Pre-Northridge WUF-B connections.

단섬유강화 금속복합재료의 계면분리 및 균열

  • Kim, Jin;Koh, Byeong-Cheon
    • Journal of the KSME
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    • v.31 no.3
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    • pp.293-299
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    • 1991
  • 단섬유보강 금속복합재료의 2차가공은 금속복합재료의 넓은 범위 응용을 위해 필히 요구된다. 여러 가공방법 중 하나인 열간압출시 보강섬유파괴 및 계면에서의 접합분리 및 균열발생이 없는 제조공정의 최적화를 위해서 가공시 내부조직의 소성변형 기구 규명보다 압출력에 의한 응력분 포와 기지재료와 보강섬유 사이 계면 변화 및 기계적 특성 관계규명이 정량적으로 요구된다. 본 글에서는 유한요소법을 이용하여 계면에서의 접합상태를 임의로 가정하여 압출조건에 따른 압 출후의 보강섬유 방향 및 계면균열 및 접합분리를 거시적으로 예측하고, advanced shear-lag을 이용하여 균열 전, 후의 응력. 변형관계를 미시적으로 규명할 수 있음을 제시하였다. 그러나 향후 현상적 모델인 shear-lag 모델을 수학적 모델인 균질화법에 도입하면 미시적. 거시적 거 동해석이 함께 요구되는 금속복합재료의 열간압출거동 해석을 일체적으로 행할 수 있어 효율적 이고 정확한 예측이 가능하리라고 사료된다.

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Micro Soldering Process and Its Reliability (Micro Soldering의 프로세스와 그 신뢰성)

  • 신영의
    • Journal of Welding and Joining
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    • v.13 no.4
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    • pp.14-23
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    • 1995
  • Micro soldering 기술의 응용은 주로 전자제품에 이용되며, 특히 컴퓨터 정보 통신 기기의 집합.접속 기술의 중추적인 역할을 하고 있다. 아울러 이 분야는 일본, 미국이 선구적인 역할을 하고 있으며, 예를 들어 일본 전자산업의 1994년도 생산액이 앤 고의 열쇠에 불구하고 민생용.산업용 전자기기 및 전자 부품의 3부분에서 약 .yen.30조(250조원)에 달한 것으로 보면 그 규모 및 중요성을 알 수 있다. 이것은 기본 적으로 반도체의 집적도가 높아진것(LSI.rarw.VLSI.rarw.ULSI)과 아울러 소자를 접합 접속시키는 기술이 확보되었기 때문에 이루어진 결과라고 말할 수 있다. 따라서 본 기술 해설에서는 접합.접속 기술의 하나인 Micro soldering의 각종 프로세스 중에서 도 특히 기본이 되는 리플로우 프로세스(reflow process)를 중심으로 기술하였으며 아울러 신뢰성의 제반사항에 관하여 간략하게 기술하였다.

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海洋開發用 海洋構造物의 熔接 I

  • 김태영
    • Journal of Welding and Joining
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    • v.4 no.2
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    • pp.12-20
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    • 1986
  • 본 고에서는 해양개발 수단으로서의 해양구조물을(선박, 해저탐사선등 분야는 제외한) 대상으로 하여 이의 제작을 위한 용접에 관련하여 제한적 범위내에서 아래와 같이 제목을 설정하여 서술 코저 한다. 1) 해양구조물의 정의 2) 해양구조물의 역사 3) 해양구조물의 분류 4) 해양구조물용 강자 5) 해양구조물 용접용 용접재료 6) 해양구조물 거조 현황 일차로 위 항목들 중 1)-3)까지를 1회분으로 하고저 한다.

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Cracking in Welds and Its Prevention(II) - Cracks in Welds and Hot Cracking(I) - (용접부의 균열 및 그 방지(II) - 균열의 종류 및 고온균열(I)-)

  • 박화순
    • Journal of Welding and Joining
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    • v.20 no.2
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    • pp.1-3
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    • 2002
  • 전 호에서는 용접부와 밀접한 관계가 있는 가스의 성질 및 가스가 용접부에 미치는 영향에 대하여 서술하였다. 본 호에서는 용접부의 균열 및 그 방지에 대하여, 먼저 용접 부에서 발생하는 균열의 종류와 고온균열 중의 일부를 소개하기로 한다 고온균열은 응고균열, 연성저하균열, 액화균열로 크게 나눌 수 있으며, 총 3회에 나누어 서술한다.

AC-DC Transfer Characteristics of a Bi-Sb Multijunction Thermal Converter (Bi-Sb 다중접합 열전변환기의 교류-직류 변환 특성)

  • 김진섭;이현철;함성호;이종현;이정희;박세일;권성원
    • Journal of the Korean Institute of Telematics and Electronics D
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    • v.35D no.11
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    • pp.46-54
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    • 1998
  • A planar Bi-Sb multijunction thermal converter, which is consisted of a linear or bifilar thin film NiCr-heater and a thin film Bi-Sb thermopile, has been fabricated, and its ac-dc transfer characteristics were examined in a frequency range from 10 Hz to 10 KHz. In order to increase the thermal sensitivity and to decrease the ac-dc transfer error of a thermal converter, the heater and the hot junctions of a thermopile were prepared on a Si$_3$N$_4$/SiO$_2$/Si$_3$N$_4$-diaphragm which acts as a thermal isolation layer, and the cold junctions on the Si$_3$N$_4$/SiO$_2$/Si$_3$N$_4$-thin film supported with the silicon rim which functions as a heat sink. The respective thermal sensitivities in air and in a vacuum of the converter with a built-in bifilar heater were about 14.0 ㎷/㎽ and 54.0 ㎷/㎽, and the ac-dc voltage and the current transfer difference ranges in air were about $\pm$0.60 ppm and $\pm$0.11 ppm, respectively, indicating that the ac-dc transfer accuracy of the converter are much higher than that of a commercial 3-dimensional multijunction thermal converter. However, the output thermoelectric voltage fluctuation of the converter was rather high.

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Numerical Analysis of Chromium Distributions in Laser-Arc Hybrid Welding of Structural Steel Using Stainless Steel Solid Wire (스테인리스강 솔리드와이어를 이용한 구조용 강재의 레이저-GMA 하이브리드 용접에서 크롬 분포의 해석적 분석)

  • Cho, Won-Ik;Na, Suck-Joo;Cho, Min-Hyun;Lee, Jong-Sub
    • Proceedings of the KWS Conference
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    • 2009.11a
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    • pp.110-110
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    • 2009
  • 본 연구에서는 전산유체역학적 방법을 이용하여 레이저-GMA 하이브리드 용접에서 용융풀 거동에 대한 3차원 과도해석을 수행하였다. 철강재의 주요 합금원소 중 하나인 크롬의 용융풀에서의 거동을 구현하기 위해 크롬에 대해 추가적인 보전 방정식을 도입하였다. 이를 이용하여 합금원소 분포에 대한 용접 변수별 영향을 해석적으로 분석하였으며 이를 EPMA(Electron Probe Micro Analyzer)를 통해 분석한 시험결과와도 비교하였다. 결과적으로 용접변수 중 선행 조건이 합금원소 분포에 구별할만한 영향을 미침을 알 수 있었고 이는 용융풀의 거동을 지배하는 주요 유동 패턴의 상이함에 기인한 것으로 보여진다.

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Structural Behavior of Bolted Lap-Joint Connection in the Pultruded FRP Structural Members (볼트로 겹침이음된 펄트루젼 복합재 접합부의 구조적 거동)

  • Lee, Young-Geun;Shin, Kwang-Yeoul;Joo, Hyung-Joong;Nam, Jeong-Hun;Yoon, Soon-Jong
    • Composites Research
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    • v.23 no.1
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    • pp.37-43
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    • 2010
  • In this paper, we present the result of an experimental investigation pertaining to the structural behavior of bolted lap-joint connection of pultruded fiber reinforced plastic structural shapes. In the experimental investigation, in order to find the mechanical property of the material, tension and shear tests on the pultruded structural composite specimen are conducted prior to the investigation on the structural behavior of bolted lap-joint connection of the member. Based on the result, number of bolts, type of placement and location of bolt are determined to be a test variable. Three different types of experimental specimens are prepared. Tensile load is applied through the center of the specimen with lap-joint connection and the structural behavior and failure mode of the test specimens with respect to the tensile load increment are investigated. As a result, it is found that most of the failure mode at the lap-joint connection is shear failure mode. Consequently, it is also found that the data obtained through this experimental program could be used for the structure connection design as a basis.

Evaluation of Design Formulae for T-joints on the Branch Plate and Hollow Steel Sections welded connections (지관 플레이트가 주관에 용접된 각형강관 접합부의 설계내력 평가에 관한 연구)

  • Park, Keum Sung;Bae, Kyu Woong;Moon, Tae Sup
    • Journal of Korean Society of Steel Construction
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    • v.17 no.5 s.78
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    • pp.581-591
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    • 2005
  • This paper proposes a design formulae that evaluates the design strength of T-joints made of cold-formed square hollow steel sections with longitudinal branch plate. The T-joints had a configuration that a branch member used to longitudinal plate to the main chord in the plane. This study focused on the branch plate T-joints governed by the main chord flange failure mode among the experimental results. Based on the test results of the longitudinal branch plate T-joint in the square hollow sections, the ultimate strength on the T-joints was defined as 1.5 times the load at 1% B the strength of joints that governed the serviceability in control for $16.7{\leq}2\gamma(B/T){\leq}31.3$ and $0.20{\leq}{\beta}(b1/B){\leq}0.75$. Existing yield line models for normal T-joints were investigated to be the main chord flange failure for the branch plate T-joint, and this proposal design formula was based on the theory of the yield line model. Finally, the value of the finite element method compared with the value of the test and theory for the T-joints verified the validity of the design formulae.