• Journal of Genetic Medicine
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• v.10 no.2
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• pp.109-112
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• 2013

Treacher Collins syndrome (TCS) is the most common and well known mandibulofacial dysostosis with characteristic clinical features including downward slanting of palpebral fissures, coloboma of the lower eyelid, hypoplastic zygomatic arches, micrognathia, macrostomia, microtia, and other deformities of the ears. TCS is caused by mutations in at least 3 genes involved in pre-rRNA transcription: TCOF1, POLR1D and POLR1C. We experienced a 1-day-old female infant with characteristic clinical features of TCS. A novel, heterozygotic mutation within the TCOF1 gene (c.3874_3875insG, p.Ala1292Glyfs*30) was identified to cause a premature stop codon.

• Journal of the Korean Society for Railway
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• v.9 no.3 s.34
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• pp.264-270
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• 2006

In this paper we present a safety plan to be applied to the development of the TCS(Train Control System). The safety plan that can be applied to the life cycle of a system, from the conceptual design to the dismantlement, shows the whole process of the paper work in detail through the establishment of a goal, analysis and assessment, the verification. In this paper we study about the making a plan, the preliminary hazard analysis, the hazard identification and analysis to guarantee the safety of the TCS. The process far the verification of the system safety is divided into several steps based on the target system and the approaching method. The guarantee of the system safety and the improvement of the system reliability is fellowed by the recommendation of the international standards.

• Transactions of the Korean Society of Automotive Engineers
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• v.7 no.5
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• pp.194-205
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• 1999

The prevalence of microprocessor-based controllers in automotive systems has greatly increased the need for tools which can be used to validate and test control systems over their full range of operation. The objective of this paper is to develop a real time simulator of traction control system by the methodology of using hardware-in-loop simulation based on a personal computer. By use of this simulator, the analysis of commercial electronic control units and components for TCS were performed successfully. The simulator of this research can be applied to development of more advanced control systems(suck as vehicle dynamics control system) and other automotive system.

• Progress in Superconductivity and Cryogenics
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• v.22 no.4
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• pp.24-30
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• 2020

The test results of the ITER toroidal field conductors demonstrated a decrease of the current sharing temperature (Tcs) with an increase in the number of electromagnetic cycles in general. This is associating with several factors. One of them is the superconducting Nb3Sn filaments cracking and another one is the redistribution of the relative deformation of the Nb3Sn strands under Lorentz forces. Despite these factors, some conductors have shown the absence or significantly less degradation of Tcs during electromagnetic cycling. This article considers another possible reason for a more stable conductors Tcs behavior, namely, the local compression of Nb3Sn wires in the cross section of a conductor. In this article presents the results of a quantitative analysis Nb3Sn superconducting filaments cracking of strands extracted from a conductor that has passed electromagnetic cycling and the model of a conductor compaction, as well as calculation results based on this model are presented also.

• Journal of the Korean earth science society
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• v.36 no.2
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• pp.171-180
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• 2015

Regarding the tropical cyclone (TC) genesis frequency, TCs between 1999 and 2013 were generated more frequently in the northwest waters of the tropical- and subtropical western North Pacific than TCs between 1977 and 1998. TCs over the period from 1977-1998 showed a northward track trend generated mostly from the distant sea in east of the Philippines via the mainland of the Philippines and the South China Sea to the west toward Indochina or from the distant sea in east of the Philippines to the distance sea in east of Japan. TCS over the period from 1999-2013 showed a northward shift pattern to the mid-latitude region mostly in East Asia. Therefore, TCs over the period from 1999-2013 tended to move to much higher latitudes than TCs over the period from 1977-1998, which also resulted in the high possibility of maximum TC intensity occurred in higher latitudes during the former period than the latter period. In the difference of 500 hPa streamline between two periods, the anomalous anticyclonic circulations were strengthened in $30-50^{\circ}N$ whereas the anomalous monsoon trough was placed in north of the South China Sea, which was extended to the east up to $145^{\circ}E$. The mid-latitude in East Asia is affected by the anomalous southeasterlies due to the above anomalous anticyclonic circulations and anomalous monsoon trough. The anomalous southeasterlies play a role in anomalous steering flows that directed TCs to the mid-latitude regions in East Asia, which made the latitudes of the maximum intensities in TCs over the period from 1999 - 2013 further to the north than those in TCs over the period from 1977-1998.

• Journal of Korean Neurosurgical Society
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• v.46 no.5
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• pp.468-471
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• 2009

Objective : The adolescent presentation of tethered cord syndrome (TCS) is well-recognized, but continues to pose significant diagnostic and management controversies. The authors conducted a retrospective study of clinical outcomes after surgical intervention in 24 school-aged children, adolescents, and young adults with TCS. Methods : All 83 patients with a lipomyelomeningocele (LMMC) underwent untethering surgery for caudal cord tethering between 1987 and 2007. The clinical charts and follow-up data were reviewed. Of these patients, 24 school-aged children, adolescents, and young adults with TCS were studied with respect to the clinical, radiologic, pathologic features, and surgical outcomes. Results : Untethering procedures were performed in 24 patients (age range, 7-25 years) for TCS of various origins (lipoma, lipomyelomeningocele, and tight filum terminale). Specific circumstances involving additional tugging of the already tight conus, and direct trauma to the back precipitated the onset of symptom in 50% of the patients. Diffuse and non-dermatomal leg pain, often referred to the anorectal region, was the most common presenting symptom. Progressive sensorimotor deficits in the lower extremities, as well as bladder and bowel dysfunction, were also common findings, but progressive foot and spinal deformities were noted less frequently. The most common tethered lesions were intradural lipomas, thickened filum and fibrous band adhesions into the placode sac. The surgical outcome was gratifying in relation to pain and motor weakness, but disappointing with respect to resolution of bowel and bladder dysfunction. Of the 24 patients with TCS, pre-operative deficits improved after surgery in 14 (58.3%). remained stable in 8 (33.4%). and worsened in 2 (8.3%). Conclusion : The pathologic lesions of tethered cord syndrome in school-aged children, adolescents, and young adults, are mostly intradural lipomas and tight filum. It is suggested that the degree of cord traction results in neurologic dysfunction in late life due to abnormal tension, aggravated by trauma or repeated tugging of the conus during exercise. Early diagnosis and adequate surgical release might be the keys to the successful outcome in school-aged children, adolescents, and young adults with TCS.

• Atmosphere
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• v.24 no.3
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• pp.283-301
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• 2014

In 2010, only 14 tropical cyclones (TCs) were generated over the western North Pacific (WNP), which was the smallest since 1951. This study summarizes characteristics of TCs generated in 2010 over the WNP and investigates the causes of the record-breaking TC genesis. A long-term variation of TC activity in the WNP and verification of official track forecast in 2010 are also examined. Monthly tropical sea surface temperature (SST) anomaly data reveal that El Ni$\tilde{n}$o/Southern Oscillation (ENSO) event in 2010 was shifted from El Ni$\tilde{n}$o to La Ni$\tilde{n}$a in June and the La Ni$\tilde{n}$a event was strong and continued to the end of the year. We found that these tropical environments leaded to unfavorable conditions for TC formation at main TC development area prior to May and at tropics east of $140^{\circ}E$ during summer mostly due to low SST, weak convection, and strong vertical wind shear in those areas. The similar ENSO event (in shifting time and La Ni$\tilde{n}$a intensity) also occurred in 1998, which was the second smallest TC genesis year (16 TCs) since 1951. The common point of the two years suggests that the ENSO episode shifting from El Ni$\tilde{n}$o to strong La Ni$\tilde{n}$a in summer leads to extremely low TC genesis during La Ni$\tilde{n}$a although more samples are needed for confidence. In 2010, three TCs, DIANMU (1004), KOMPASU (1007) and MALOU (1009), influenced the Korean Peninsula (KP) in spite of low total TC genesis. These TCs were all generated at high latitude above $20^{\circ}N$ and arrived over the KP in short time. Among them, KOMPASU (1007) brought the most serious damage to the KP due to strong wind. For 14 TCs in 2010, mean official track forecast error of the Korea Meteorological Administration (KMA) for 48 hours was 215 km, which was the highest among other foreign agencies although the errors are generally decreasing for last 10 years, suggesting that more efforts are needed to improve the forecast skill.

• Journal of the Korean earth science society
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• v.31 no.4
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• pp.322-334
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• 2010

Spatiotemporal characteristics of precipitation in Korea, associated with the weakening of Tropical Cyclones (TCs) around the Korean Peninsula ($32-36^{\circ}N$, $122-132^{\circ}E$) over the last 30 years (1979-2008), were investigated. Weakened TCs are classified as WEC (Weakened to Extratropical Cyclone) and WTD (Weakened to Tropical Depression). In WEC, precipitation was evenly distributed all over the Korean Peninsula and the greater precipitation was recorded in the southern coast. In WTD, the most precipitation was recorded in the southern coast but low precipitation was recorded in the central and inland areas of Korea. The difference of precipitation between WEC and WTD was not statistically significant in Region 2 (Jeollanam-do, Gyeongsangnam-do, southeastern part of Gyeongsangbuk-do, Jeju-do); however, the precipitation resulting from WEC was greater than that resulting from WTD in Region 1 (central area of Korea, Jeollabuk-do, inland of Gyeongsangbuk-do). In WEC, the developed upper-level potential vorticity (PV) and low-level temperature trough shifted to the northwest of TCs approaching Korea. In addition, an upper-level jet stream and strong divergence field were observed to the northeast of the TCs. It was assumed that these meteorological factors had induced baroclinic instability and diabatic process, which created a large precipitation area around the TCs. However, the intense PV, temperature trough, jet stream were not observed in WTD, which created a small precipitation area around the TCs.

• Journal of the korean academy of Pediatric Dentistry
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• v.37 no.3
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• pp.374-380
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• 2010

Treacher Collins syndrome(TCS) is a rare, incurable condition occurring in approximately 1 of 25,000 to 50,000 births. It may occur as a spontaneous mutation out of genetically normal parents or it may be manifested as an autosomal dominant pattern. TCS is characterized by facial deformities such as, underdevelopment of the maxilla, mandible and zygoma, malocclusion, bilateral deformities of auricles, antimongoloid slant of the palpebral fissures. The syndrome is often associated with cleft lip and palate, ear malformations and hearing loss, short stature, and anomalies of the heart and skeleton. Respiratory difficulty associated with air way obstruction may also be observed, and there is considerable difficulty in airway management during general anesthesia. It is necessary that dentists provide safe dental treatments and guidelines to TCS patients by providing adequate understanding about the characteristics of the syndrome and proper ways of managements. The purpose of this study is to report the dental and medical characteristics of the patient who visited the Department of Pediatric Dentistry, Yonsei University for multiple dental caries treatment and to review the literatures of TCS.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.79-83
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• 2019

Treacher Collins syndrome(TCS) is an autosomal craniofacial development disorder which results from mutations in the gene TCOF1. Major features include midface hypoplasia, micrognathia, microtia, conductive hearing loss. Oral manifestations are characterized by cleft palate, shortened soft palate, malocclusion, anterior open bite and enamel hypoplasia. The purpose of this presentation is to describe the interesting aspects of dental treatment of a patient with TCS. A 6-year-old boy with TCS visited Seoul National University Dental Hospital for dental caries. Multiple caries was observed from clinical and radiographic examination. Because of multiple caries and behavior management ploblem, dental treatment under general anesthesia was planned. Treatment of posterior teeth was performed and some primary teeth were extracted. General anesthesia was induced and maintained with sevoflurane, nitrous oxide and oxygen. Under general anesthesia, successful dental procedure was done. Considering behavior management problem and medical condition of patient with TCS, general anesthesia can be useful.