• 제목/요약/키워드: cant

검색결과 166건 처리시간 0.023초

Three Korean patients with Cantú syndrome caused by mutations in ABCC9 and their clinical manifestations

  • Jang, Jin Hee;Ko, Jung Min;Yang, Sei Won;Chae, Jong Hee;Bae, Eun Jung
    • Journal of Genetic Medicine
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    • 제13권2호
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    • pp.99-104
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    • 2016
  • $Cant{\acute{u}}$ syndrome (CS, OMIM 239850) is a very rare autosomal dominantly inherited genetic disease characterized by congenital hypertrichosis, neonatal macrosomia, a distinct facial features such as macrocephaly, and cardiac defects. Since the first description by $Cant{\acute{u}}$ et al. in 1982, about 50 cases have been reported to date. Recently, two causative genes for CS has been found by using exome sequencing analyses: ABCC9 and KCNJ8. Most cases of clinically diagnosed CS have resulted from de novo mutations in ABCC9. In this study, we report three independent Korean children with CS resulting from de novo ABCC9 mutations. Our patients had common clinical findings such as congenital hypertrichosis, distinctive facial features. One of them showed severe pulmonary hypertension and hypertrophic cardiomyopathy, which require medical treatment. And, two patients had a history of patent ductus arteriosus. Although two of our patients had shown early motor developmental delay, it was gradually improved during follow-up periods. Although CS is quite rare, there are the concerns about development of various cardiac problems in the lifetime. Therefore, an accurate diagnosis followed by appropriate management and genetic counseling should be provided to CS patients.

Evaluating New Track Construction Costs of Two Different Options: Conventional Trains vs. Tilting Trains

  • Rho, Hag Lae;Han, Seong Ho;Kim, Gang Seog
    • International Journal of Railway
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    • 제5권3호
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    • pp.103-113
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    • 2012
  • Infrastructure costs could be greatly reduced if the need for bridges and tunnels was reduced, or if the line could be shortened. Tilting trains might be a less costly alternative to building new tracks with large curve radii, because tilting trains can negotiate tighter curves without having to decrease their speeds. Tracks built for tilting trains would be cheaper, as they require fewer bridges and tunnels. This paper compares the construction costs of two different options for the new Dodam-Yeoncheong section on the Central line, which includes 148.65 km of new electrified double-track with a design speed of 250 km/h. The first option is to straighten the high-speed line. The second option is to build a line with small radii curves and run tilting trains on the line. In the first option, tunnels would account for about 51% of the new section. In comparison, the second option would have shorter curves and fewer tunnels and bridges which would reduce construction costs. Furthermore, alignment modifications could be made to several segments on the straight line, making the most of the existing roadbed. The analysis concluded that the line suited to tilting trains would be 95.7 million USD cheaper to build the straight route. That is a savings of 2.8% of the total project cost. However, this option would increase the total travel time of the route by 1.2 minutes, which means it is not necessarily the best choice.

한국형 고속전철의 기존선 주행 진동가속도 특성 분석 - 곡선 통과속도 중심으로 (Analysis of the Acceleration Characteristics on the Conventional line for Korean High Speed Train- in il point of passing speed on the curve)

  • 김영국;김석원;목진용;박찬경
    • 한국소음진동공학회:학술대회논문집
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    • 한국소음진동공학회 2004년도 춘계학술대회논문집
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    • pp.222-227
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    • 2004
  • Korean High Speed Train(KHST) has been tested on the high speed test line in Osung site, since it was developed through the G7 Project Plan in 2002. It was also tested on the conventional line such as KyongBu and Honan Line to know the possibility of increasing the limited speed for the high speed trains. This paper introduces the method to improve the speed on the conventional line with body lateral acceleration among the several considered issues and explains the parameters related to those analysis, such as the cant deficiency, the radius of curve, speed and etc. When a train pass on the curved track, the lateral accelerations of body are divided into the quasi-static and the maximum accelerations according to the UIC 518 which is the international specification for testing and approval of railway vehicles from the point of view of their dynamic behaviour, especially for safety and ride comfort. This paper shows that it is safe and comfort from the results of test when KHST runs on the conventional line with the curves and proposes that the limited speed of conventional curved line could be changed to a little higher speed if the analysises of other fields are completed.

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틸팅차량 완화곡선 증속 주행시 궤도작용력 검토 (A Study on the Rail Pone of Transition Curve Track to Speed Up of Tilting train)

  • 신태형;최정열;엄기영;박용걸
    • 한국철도학회:학술대회논문집
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    • 한국철도학회 2009년도 춘계학술대회 논문집
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    • pp.529-538
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    • 2009
  • A trial run of locally-developed tilting train has been in process on Chungbuk line since the test vehicle was first produced. For the system stabilization, interface verification among the systems including track, structure, catenary and signaling system, not to mention the rolling stock, is very crucial. Therefore, in this study, the dynamic rail force of the tilting (Hanvit 200), high-speed (KTX) and general (Mugunghwa) vehicle caused by driving in transition curve track was measured. And, it compared the tilting response with the other by using the measured wheel load data in transition curve track, and then evaluated probability the range of wheel load fluctuation for the variable dynamic vertical and lateral wheel load. As a result, a range of wheel load by occured a change of cant from the high-speed and general vehicle which had fixed bogie structure was distributed throughout small deviation (${\Delta}8{\sim}13kN$). Otherwise, in case of the tilting train which was consisted of the pendulum bogie structure was distributed wide range about large deviation (${\Delta}25{\sim}28kN$) by changed of cant.

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틸팅열차 주행시 곡선부 궤도에서의 동적상호작용 (Dynamic Interaction Analysis of Tilting Train and Curved Track)

  • 정근영;고태훈
    • 한국철도학회논문집
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    • 제15권2호
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    • pp.162-171
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    • 2012
  • 본 연구에서는 곡선부를 주행하는 틸팅열차와 궤도와의 동적상호작용을 고려한 동적해석을 수행하기 위한 해석기법을 제시하였다. 본 연구에서는 3차원 차량요소를 이용하여 틸팅열차를 모형화하였으며, 틸팅열차의 곡선부 주행시 차량의 주행방향이 크게 변화하는 상황에서 주행속도 및 원곡선 반경에 상응하는 원심력의 표현, 선로의 캔트량, 차륜-레일의 접촉각 및 열차의 틸팅각 등을 고려한 동적상호작용해석 방법을 제안하였다. 제안된 해석방법은 기본적인 검증예제들에서 만족할만한 결과를 보여주었으며, 실제적인 문제에 적용이 가능할 것으로 기대된다.

GIS를 이용한 철도선형최적화 (Optimization of Railway Alignment Using GIS)

  • 강인준;이준석;김수성
    • 한국철도학회:학술대회논문집
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    • 한국철도학회 2002년도 추계학술대회 논문집(I)
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    • pp.727-732
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    • 2002
  • This study is to develop the model of alignment optimization based on design criteria by approaching through alignment of railway design and problems in economy, environment and technology for satisfying traffic volume of the main roads caused by economical and social developments. Now, Geographic Information System isn't applied when designing a present railway in home. And the design of railway alignment is still set on importance of transition curves and cant according to passenger comfort in abroad so tile study of railway alignment is at initiation phase so far. This paper is about decision of optimal alignment between two stations such as starting point and ending point automatically using GIS in optimization of railway alignment. A route between Sungsan city and Shinpung city is the training area and the study compared and evaluated optimal railway route by GIS automatically with present railway route designed. Present optimal fomulas was used in this study for optimization of railway alignment. The model of optimization of railway alignment was developed through topographical elements and it was mentioned by the model of road alignment because of the similarity in design of alignment. But the design of lateral track irregularities, cant fur passenger comfort and motion sickness fellowed by train rolling have to be considered more. Anyway, this study farmed the basis of using GIS and the study should be keep going on in the future.

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고속 틸팅 차량설계를 위한 전산통합 환경 구축 연구 (Product Data Management for The system Engineering of Train Tilting express)

  • 한성호;송용수
    • 시스템엔지니어링워크숍
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    • 통권4호
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    • pp.65-69
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    • 2004
  • Abstract Tilting train has been developed to increase the oprational speed of the trains on conventional lines which have many curves. This train are tilted at curves to compensate for unbalanced carbody centrifugal acceleration to a greater extent than compensation produced by the track cant, so that passengers do not feel centrifugal acceleration and thus trains can run at higher speed at curves. This paper developed PDM(product data management) to make a system engineering of TTX(Tilting Train eXpress) with maximum operation speed 180 km/h.

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SE관리기법(PDM)을 이용한 틸팅차량(TTX) 설계기술 연구 (Design Techniques of Tilting Train(TTX) using the system engineering (PDM))

  • 한성호;송용수
    • 한국복합재료학회:학술대회논문집
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    • 한국복합재료학회 2004년도 추계학술발표대회 논문집
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    • pp.203-206
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    • 2004
  • Tilting train has been developed to increase the operational speed of the trains on conventional lines which have many curves. This train are tilted at curves to compensate for unbalanced carbody centrifugal acceleration to a greater extent than compensation produced by the track cant, so that passengers do not feel centrifugal acceleration and thus trains can run at higher speed at curves. This paper developed PDM(product data managemnet) to make a system engineering of TTX(tilting train express) with maximum operation speed 180 km/h.

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고속열차 차륜 플랜지 마모 (Flange Wear of the High Speed Train)

  • 이희성;강부병
    • Tribology and Lubricants
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    • 제22권1호
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    • pp.33-39
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    • 2006
  • During the high speed train operation in the conventional line, it was found to have extremely severe flange wear, due to the curvy routes. This paper looks at the actions that have been taken to bring the wear to an acceptable level. In particular, it have been presented for flange wear variation depending on curve radius, cant, transition curve length by using numerical method. And the impacts on the flange wear by the application of stick type solid lubricant are also presented.

철도차량의 Kinematic Envelope에 관한 연구 (A Study on the Kinematic Envelope of the Railway Vehicle)

  • 양희주;이강운;박길배
    • 한국철도학회:학술대회논문집
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    • 한국철도학회 2002년도 추계학술대회 논문집(II)
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    • pp.863-869
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    • 2002
  • Studied in this paper was the kinematic envelope of the railway vehicle to calculate the lateral displacement using the multi-body dynamic simulation program (VAMPIRE) and the BASS 501. The lateral displacement of railway vehicle is occurred by the clearance between wheel flange and rail, the track irregularity, the property of each suspension of vehicle and the cant of track etc. The results of analysis shown that Vehicle is not interfere with subway platform in any conditions namely the tare and full load condition, the wheel wear condition and the stationary and running of vehicle.

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