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KABUKI SYNDROME WITH PHONETIC & DENTAL PROBLEM: A CASE REPORT (구강내 이상소견과 언어 장애를 보이는 Kabuki 증후군환자의 증례보고)

  • Lee, Jong-Seok;Ko, Seung-O;Leem, Dae-Ho;Baek, Jin-A;Shin, Hyo-Keun
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.33 no.6
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    • pp.681-683
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    • 2007
  • Kabuki(Niikawa-Kuroki) syndrome was first reported by Niikawa et al(1981). The faces of the patients are similar to the make-up of traditional Japanese Kabuki actors: long palpebral fissures, an ectropium of the lateral third of the eyelids, and arching eyebrows with sparse lateral halves. Craniofacial findings include a depressed nasal tip, short nasal septum, large and prominent ears, and micrognathia. Other main features area mild to moderate mental deficiency, short stature, skeletal and dermatoglyphic abnormalities, including prominent finger tip pads. Oral anomalies are common in KS(over 60%) and include abnormal dentition, widely spaced teeth, cleft palate or lip, high vault of palate, hypodontia, conical incisors, screw driver-shaped incisors and ectopic upper 6-year molars. The increased occurrence of cleft lip and palate or the development of a high vault of palate has been described by a number of authors. This condition is believed to be common in Japan, but has been reported from other parts of the world. The objective of this presentation is to report a case of this syndrome in six-year-old girl, with characteristic findings.

Experience of Single Stage Treatment of Caniosynostosis, Hypertelorism, Exophthalmos Patient (두개조기유합증, 안와격리증, 안구돌출증 환자의 동시 교정 치험례)

  • Chung, Chul Hoon;Eun, Seok Chan;Seo, Dong Kuk;Jo, Woo Sung;Park, Se Hyuck
    • Archives of Plastic Surgery
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    • v.33 no.2
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    • pp.237-240
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    • 2006
  • The simultaneous correction of the hypertelorism and exophthalmos combined with craniosynostosis is very rarely performed operative procedures in the world. The craniosynostosis is the congenital anomaly that designates premature fusion of one or more sutures in either cranial vault or cranial base. Hypertelorism is not a distinct clinical syndrome in itself, but is a physical finding secondary to facial and cranial maldevelopment and it is defined as a increase in the distance between the medial orbital walls. Exophthalmos can occur following the decrease in the size of the orbit in patients with developmental skeletal disorders such as craniofacial synostosis. The authors experienced 9-year-old male patient, who has complex cranio-facial abnormality. The craniosynostosis was oxycephaly type and primary fronto-orbital advancement surgery had been performed in other hospital. The abnormal cranial vault combined with hypertelorism and exophthalmos due to maldeveloped both orbital walls. Surgical correction was obtained by various cranio-fronto-orbital remodeling technique such as calvarial bone craniotomy, fronto-orbital advancement, paramedian resection, medial canthopexy, Tessier-Wolfe three wall orbital expansions. We achieved a quite satisfactory result both functionally and aesthetically in a complex cranio-facial deformity patient by combination and modification of previously developed various cranio-facial plasty technique and hereby report the case with brief discussion and review of literature.

A Study on the Meaning of New Brutalism in Sigurd Lewerentz's Late Church Architecture (지구르트 레버렌츠의 후기 교회당 건축에 나타난 뉴 브루탈리즘의 의미에 관한 연구)

  • Kim, Hyon-Sob
    • Journal of architectural history
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    • v.19 no.5
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    • pp.65-80
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    • 2010
  • The purpose of this paper is to investigate into the meaning of the New Brutalism sensed in the late church buildings of the Swedish architect Sigurd Lewerentz (1885-1975). St Mark's (1956-64) and St Peter's (1962-66) churches, his grand finale designs, have a unique architectural vocabulary of rough brickwork. The brick treatment is the basis on which Reyner Banham (1966) discussed him concerning the New Brutalism, and the point that this research focuses on. This paper explores the brutalist character of the buildings from two aspects - interpretational level of individual buildings and historical level of a broader view. First, the character of two churches could be interpreted with the phrase of 'play between brutality and sacredness'. The rough surface of brick and mortar in the buildings symbolises brutality and the vault of their chapels' ceiling sacredness. And the two characteristics meet and play on the rough vault surface. Second, in the historical point of view, this paper argues that the buildings made a giant leap for Swedish modern architecture, which had been at a deadlock owing to the compromising attitude of the New Empiricism since 1940s. And the Swedish New Empiricism (or the New Humanism), spread to Britain as "Welfare State architecture" after World War II, brought about reaction of the young British architects such as the Smithsons and became the background that made the new brutalist mood. However, considering that the term of the New Brutalism was first used in Sweden by Hans Asplund, Lewerentz's brutalist late churches - which seemingly had nothing to do with the British nor the Corbusian lineage - are also meaningful in that they confirmed the tendency in its homeland. In conclusion, this paper argues that St Mark's and St Peter's churches with the brutalist characteristics should be regarded as crucial buildings not only in Lewerentz's personal career but also in Swedish and international architectural history.

The usefulness of intermolar traction wiring for restoration of maxillary & mandibular dental arch in facial bone fracture (안면골 골절에서 상하악 치열궁 복원을 위한 양측 대구치간 철사견인술의 유용성)

  • Jeong, Jae Ho;Shin, Seung Kyu;Lee, Jun Ho;Kim, Yong Ha
    • Archives of Plastic Surgery
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    • v.36 no.1
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    • pp.56-60
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    • 2009
  • Purpose: Palatal fracture and mandible fracture result in instability of dental arch. Because they divide the maxillary and mandibular alveolus sagittally and / or transversely and comminute the dentition, they permit rotation of dental alveolar segments and significantly increase the potential for fracture malalignment, complicating fracture treatment. Previous treatment of palatal fracture consisted of palatal splint application and rigid palatal vault stabilization. This procedure result in patient's oral discomfort and removal of palate and screw. Mandible fracture often results in malocclusion due to widening of posterior aspect of dental arch. So we introduce more simple method using intermolar traction wiring, which can protect the widening of dental arch and rotation of dental alveolar segment. Methods: Arch bar and intermolar traction wiring with wire 1 - 0, or 2 - 0 was applied. After exposure of fracture line, neutrooclusion was maintained with intermaxillary fixation. And then open reduction & internal fixation on maxillary fracture line, commonly maxillary buttress, alveolar ridge, pyriform aperture except palatal vault or mandibular fracture line. After 1 week, intermolar traction wiring was removed. We checked occlusion and postoperative radiologic finding. Results: From June of 2007 to October of 2007, 10 patient, who have maxillary fracture with palatal fracture and mandible fracture, underwent open reduction & internal fixation with intermolar traction wiring. All have satisfactory occlusion and there were no complication, like gingiva disease, mouth opening impairment and nonunion. Conclusion: The intermolar traction wiring accompany open reduction and internal fixation can be alternative method for restoration of dental arch in facial bone fracture.

Outcome Analysis of Cranial Molding Therapy in Nonsynostotic Plagiocephaly

  • Yoo, Han-Su;Rah, Dong Kyun;Kim, Yong Oock
    • Archives of Plastic Surgery
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    • v.39 no.4
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    • pp.338-344
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    • 2012
  • Background It is known that nonsynostotic plagiocephaly does not spontaneously improve, and the craniofacial deformities that result from it. This study was conducted to analyze the effectiveness of helmet therapy for the nonsynostotic plagiocephaly patient, and to suggest a new treatment strategy based on this analysis. Methods A total of 108 pediatric patients who had undergone helmet therapy after being diagnosed with nonsynostotic plagiocephaly were included in this study. The patients were classified according to the initiation age of the helmet therapy, severity, and helmet wearing time. The treatment effect was compared using cranial vault asymmetry (CVA) and the cranial vault asymmetry index (CVAI), which were obtained from diagonal measurements before and after therapy. Results The discrepancy of CVA and CVAI of all the patients significantly decreased after helmet therapy. According to the initiation time of helmet therapy, the treatment effect was best at 5 months old or less. The helmet wearing time per day was proportional to the treatment effect up to 20 hours. In addition, the rate of the successful treatment (final CVA ${\leq}$ 5 mm) significantly decreased when the initiation age was 9.1 months or older and the treatment period was less than 7.83 months. Conclusions This study showed the effectiveness of the helmet therapy for nonsynostotic plagiocephaly patients. Based on analysis of this study, helmet therapy should be started at the age of 9 months or younger for 7.83 months or more, and the helmet wearing time should be more than 20 hours a day.

Criticality Safety Determination of Spent Fuel Storage Vault (기사용(旣使用) 핵연료저장시(核燃料貯藏時) 핵임계(核臨界) 안전성(安全性) 결정(決定))

  • Yook, Chong-Chul
    • Journal of Radiation Protection and Research
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    • v.4 no.1
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    • pp.1-4
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    • 1979
  • Effective multiplication factor has been calculated for one PWR fresh fuel assembly immersed in a spent fuel storage vault on the basis of the neutron transport theory. A numerical calculation has been carried out by means of Sn approximation. The method employed in this study is that the energy domain is broken into 16 groups, the angular variable is divided into four discrete direction, i.e., $S_4$, and the spatial variable which is divided into fine meshes at the interface between different materials is discretized into 27 mesh points. The calculated $K_{eff}$ value of 0.6145 seems to be far small in comparison with the value obtained by other author for an infinite array of fuel assemblies.

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Management and role of Baekdudaegan National Arboretum Seed Vault in readiness for unification (통일대비 국립백두대간수목원 시드볼트의 운영과 역할)

  • Bae, Kee Hwa
    • Proceedings of the Plant Resources Society of Korea Conference
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    • 2019.04a
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    • pp.20-20
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    • 2019
  • 전 지구적으로 학명이 보고된 식물 종은 약 36만종이다. 그 중 종자를 생산하는 관속식물은 310,422종으로 전체 식물종의 85.7%에 달한다(IUCN, 2018). 그리고 2015년 Primely Forest Organization (PFO)에서는 야생식물의 80%가 산림 내에 서식한다고 밝혔다. 식물이 인류에게 주는 다양한 이로움은 인류의 생존과 복지증진에 막대한 영향을 미친다. 2016년 State Worlds Plants (SWP)에 따르면 인류가 현재까지 이용하는 식물종은 31,128종으로 알려져 있다. 이중 약용으로 17,810종, 식량으로 5,538종을 유용하게 이용하고 있다. 하지만 현재 전 지구적 생물멸종속도는 산업혁명 이전 보다 약 1,000배정도 빠르게 진행이 되고 있다(Science, 2017). 이에 전 지구적 생물다양성보전을 위해 생물다양성 협약, 지구식물보전전략 등이 지속적으로 발효 이행되고 있다. 시드볼트는 이러한 보전전략을 이행하기 위해 적합한 시설이다. 우리나라도 노르웨이와 전 세계적으로 2곳뿐이 없는 시드볼트를 가지게 되었다. 우리의 시드볼트는 전 지구적 재난과 재앙에 대비해 야생식물종자를 안전하게 영구 저장하는 시설이고, 우리나라는 봉화군에 위치한 국립백두대간수목원 내에 200만점 저장 규모로 조성이 되어 있다. 현재 국내 외 수집 수탁종자 48,327점이 안전하게 저장이 되어 있다. 국내 23개 종자관련 기관, 단체, 개인과 카자흐스탄 등 해외 3개국 6개 기관이 야생식물종자의 영구저장을 요청하였다. 앞으로 시드볼트는 통일시대에 대비하여 지구적 야생식물종자 저장의 컨트롤타워 역할과 종자연구의 중심기관으로 발돋움 할 것이다. 이에 따른 목표로 2050년까지 백두대간 글로벌시드볼트(BGSV)는 전세계 식물종의 13%, 40만점을 저장할 것이다. 특히 종자연구부분에서는 현재 종 맞춤형 활력검정시스템개발, 분류군별 장기저장 특성분석, 장기저장 종자수명예측 등을 고도화하여 한반도 야생식물 종자정보 플랫폼 구축, 고대 매장종자 보전 모델링 연구, 지구 종자연구 브릿지 구축 등으로 개발, 발전시킬 것이다.

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Comparison of SIEM Solutions for Network Security (네트워크 보안을 위한 SIEM 솔루션 비교 분석)

  • Lee, Jong-Hwa;Bang, Jiwon;Kim, Jong-Wouk;Choi, Mi-Jung
    • KNOM Review
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    • v.22 no.1
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    • pp.11-19
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    • 2019
  • As technology develops, the latest security threats on the network applied to users are increasing. By attacking industrial or corporate systems with malicious purposes, hackers cause many social problems such as confidential information leakage, cyber terrorism, infringement of information assets, and financial damage. Due to the complex and diversified threats, the current security personnel alone are not enough to detect and analyze all threats. In particular, the Supervisory Control And Data Acquisition (SCADA) used in industrial infrastructures that collect, analyze, and return static data 24 hours a day, 265 days a year, is very vulnerable to real-time security threats. This paper introduces security information and event management (SIEM), a powerful integrated security management system that can monitor the state of the system in real time and detect security threats. Next, we compare SIEM solutions from various companies with the open source SIEM (OSSIM) from AlienVault, which is distributed as an open source, and present cases using the OSSIM and how to utilize it.

Controlled Environmental Vault for High Quality Internal Service (고품질 인터넷 서비스를 위한 CEV (환경 조절형 맨홀))

  • Baik Song-Hoon;Kang Wang-Kyu;Shin Key-Soo;Kang Young-Sug
    • 한국정보통신설비학회:학술대회논문집
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    • 2002.08a
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    • pp.79-81
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    • 2002
  • 초고속 인터넷 서비스는 정보화 시대에 살고 있는 우리에게 없어서는 안 되는 존재가 되고 있다. 이에 맞추어 KT는 기존의 가입자 동선 케이블을 이용하여 초고속 인터넷 서비스를 제공하기 위해 서비스 속도 향상과 음영지역 해소를 위한 다양한 해결방안을 모색하여왔다. 그 결과로써. 옥외 통신장비를 수용하기 위한 방안으로 광전송장비를 지하에 수용할 수 있는 CEV의 필요성이 대두되었으며 CEV의 개발이 완료되는 시점이 다가옴에 따라 KT는 언제 어디서든 고품질의 인터넷 서비스를 제공할 수 있는 인프라를 갖추게 되었다.

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Management of Le Fort I fracture

  • Kim, Hak Su;Kim, Seong Eun;Lee, Hyun Tae
    • Archives of Craniofacial Surgery
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    • v.18 no.1
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    • pp.5-8
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    • 2017
  • Among the classification of maxillary fracture, the Le Fort classification is the best-known categorization. Le Fort (1901) completed experiments that determined the maxilla areas of structural weakness which he designated as the "lines of weakness". According to these results, there are three basic fracture line patterns (transverse, pyramidal and craniofacial disjunction). A transverse fracture is a Le Fort I fracture that is above the level of the apices of the maxillary teeth section, including the entire alveolar process of the maxilla, vault of the palate and inferior ends of the pterygoid processes in a single block from the upper craniofacial skeleton. Le Fort fractures result in both a cosmetic and a functional deficit if treated inappropriately. In this article, authors review the management of a Le Fort I fracture with a case-based discussion.