• Journal of Genetic Medicine
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• v.13 no.2
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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.

• International Journal of Railway
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• v.5 no.3
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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.

• Proceedings of the Korean Society for Noise and Vibration Engineering Conference
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• 2004.05a
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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.

• Proceedings of the KSR Conference
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• 2009.05a
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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.

• Journal of the Korean Society for Railway
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• v.15 no.2
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• pp.162-171
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• 2012

In this study, a new dynamic interaction analysis method for tilting trains and curved track is presented. Three dimensional lumped parameter vehicle elements are used to model tilting train, and the proposed analysis technique can simulate driving direction change of vehicle, the effect of track cant, wheel-rail contact angle, and tilting angle of tilting trains, etc. The proposed method passed several basic verification tests, and it is expected that the suggested method is applicable for practical problems.

• Proceedings of the KSR Conference
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• 2002.10a
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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.

• 시스템엔지니어링워크숍
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• s.4
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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.

• Proceedings of the Korean Society For Composite Materials Conference
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• 2004.10a
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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.

• Tribology and Lubricants
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• v.22 no.1
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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.

• Proceedings of the KSR Conference
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• 2002.10b
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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.