• Journal of Genetic Medicine
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• 제7권1호
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• pp.37-44
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• 2010

목적: Kabuki 증후군은 정신 지체를 동반하는 선천성 다발성 기형 증후군이다. 우리나라에서는 현재까지 6례의 Kabuki 증후군 증례가 산발적으로 보고 된 바 있다. 본 연구에서는 저자들이 경험한 Kabuki 증후군 환자 6명의 임상 및 유전학적 특징을 조사하고, 이를 외국 문헌들과 비교 분석해 보고자 하였다. 대상 및 방법: 2003년부터 2009년까지 아주대학교 병원 유전질환 전문센터에서 Kabuki 증후군으로 진단되어 추적 중인 6명의 한국인 여아를 대상으로 하였으며, 의무기록을 후향적으로 검토하여 이들의 임상 및 검사 소견을 수집하고 분석하였다. 결 과: 6명의 환자 모두가 특징적인 얼굴 모습 및 발달 지연 소견을 보이고 있었고, 손끝의 태아 패드 또한 모든 환자에서 확인되었다. 이외에도 대부분의 환자가 생후 성장 지연(83.3 %) 및 근력 저하(83.3%) 소견을 보였다. 안과적인 이상 또한 흔하게 동반되었는데, 특히 사시(83.3%)가 가장 흔한 안과적 이상 소견이었다. 선천성 심장 기형은 50%의 환자에서 동반되었으며, 골격계통의 증상으로는 짧거나(83.3%) 굽은(50%) 5번째 손가락, 관절의 과신전(50%) 및 고관절 탈구(16.7%) 등으로 다양하였다. Kabuki 증후군의 가족력을 가진 환자는 없었으며, 핵형 분석 및 array CGH를 포함한 세포유전학적 분석에서 Kabuki 증후군의 원인으로 생각되는 이상 소견은 발견되지 않았다. 결 론: 한국인 Kabuki 증후군 환자들이 보이는 임상 양상은 매우 광범위하며 다양한 신체 기관을 침범하고 있다. 비록 Kabuki 증후군의 임상적특징들이 비교적잘 알려져 있기는 하지만, 아직질환의 원인으로 추정되는 유전적 이상은 확실히밝혀지지 않았다. 적절한 질환의 관리및 유전상담이 이루어지기 위해서는 Kabuki 증후군의 자연 경과 및 유전적 배경에 대한 추가적인 연구가 필요하다고 생각된다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제11권1호
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• pp.60-64
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• 2008

저자들은 어린 영아기에 선천성 간섬유증으로 진단받은 후 특징적인 얼굴 모습과 함께 발달 및 지능 저하를 보이는 10세 남아에서 가부키 증후군으로 진단된 1예를 경험하였기에 문헌고찰과 보고하는 바이다.

• Archives of Plastic Surgery
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• 제38권2호
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• pp.203-206
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• 2011

Purpose: Kabuki syndrome is a multiple malformation syndrome that was first reported in Japan. It is characterized by distinctive Kabuki-like facial features, skeletal anomalies, dermatoglyphic abnormalities, short stature, and mental retardation. We report two cases of Kabuki syndrome with the surgical intervention and speech evaluation. Methods: Both patients had velopharyngeal insufficiency and had a superior based pharyngeal flap operation. The preoperative and postoperative speech evaluations were performed by a speech language pathologist. Results: In case 1, hypernasality was reduced in spontaneous speech, and the nasalance scores in syllable repetitions were reduced to be within normal ranges. In case 2, hypernasality in spontaneous speech was reduced from severe level to moderate level and the nasalance scores in syllable repetitions were also reduced to be within normal ranges. Conclusion: The goal of this article is to raise awareness among plastic surgeons who may encounter such patients with unique facial features. This study shows that pharyngeal flap operation can successfully correct the velopharyngeal insufficiency in Kabuki syndrome and post operative speech therapy plays a role in reinforcing surgical result.

• 복식
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• 제60권1호
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• pp.14-27
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• 2010

The purpose of this study is to analyze how the aesthetic characteristics of Brecht's V-Effekt is visually expressed of the costume in the Traditional Play of Korea, China and Japan. The method and the contents of the study were to refer to the antecedent studies and the related documents to peruse the characteristics of the traditional plays of the East and West, the origin and the concept of Brecht's Verfremdung and considered the relation of Brecht's V-Effekt and the Oriental plays, and then the researcher derived the aesthetic properties of Brecht's V-Effekt. This study analyzed how those qualities of the aesthetic characteristics on the V-Effekt are manifested on the stage costumes of Korean mask, Beijing opera and Kabuki. The aesthetic qualities of Brecht's V-Effekt are classified into symbolism, grotesque disposition, character of sing and dance, comicality. The symbolism in the traditional play costumes of the three nations is shown in the colors of the masks in Korean and the tone of the colors on the costumes and the make-up in Beijing opera and Kabuki. While the properties of bizarrerie and deformation coexist in masque and Beijing opera costumes in terms of grotesque disposition, Kabuki strongly displays bizarre grotesquerie. The character of sing and dance is visually expressed through the transformation of sleeves on the three nation's traditional play costumes; Masque on the Hansam and Chengsam, Beijing opera on the Water sleeves and Kabuki on Hurisode. The comic aspection is expressed in a humorous and comical way through the distortion and transformation of forms in Masque and Beijing opera but it cannot be seen in Kabuki costume. The study as above will form the aesthetic properties of the Oriental traditional play costumes and also it will contribute to establishing the identity of Korean mask costumes.

• 비교문화연구
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• 제25권
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• pp.291-312
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• 2011

The academic world in Korea demands internationally competitive researches at the moment. However, there is a lack of understanding on research trends of Western countries. This research aims to understand how discussions on early modern Japanese plays are developed in Western countries such as America. Also, it aims to consider and compare the present situation of researches on early modern Japanese plays in Western countries, Japan and Korea. In Western countries, modern Japanese plays such as Kabuki and Joruri is accepted and enjoyed as universal plays of the world. It is not only because there is a background of enjoying plays such as Shakespeare's but also researches and translations are accumulated continuously from the early 20th century. In the early 20th century, the main theme of researches was about Kabuki actors in Ukiyoe which was collected because of an interest in orientalism. After 1930s, Japanese arts and handcrafts were sent to art museums in America in order to promote Japanese culture. Also, many Japanese books were donated to university libraries in order to form institutes of Japanology. The war was started when the foundation of Japanology was about to be laid. Students who were interested in Japan were mobilized to the war as agents to understand Japan. After the war, those students started to do researches on Japan on a full scale. There were researchers such as Donald Keen who did researches on Kabuki and Joruri. In western countries, there are researches on patterns of behavior and thoughts of Japanese people, comparison with Shakespeare's plays, dramaturgie, theatricalism as well as censorship of Kabuki during the war and mobilization of Kabuki to the war. It is necessary to understand where my researches are positioned in those research trends. It is also necessary to break away from overlapped and repeated researches of the same point of view and find out new research paradigms.

• Clinical and Experimental Pediatrics
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• 제58권7호
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• pp.256-262
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• 2015

Purpose: Kabuki syndrome is a multiple congenital malformation syndrome, with characteristic facial features, mental retardation, and skeletal and congenital heart anomalies. However, the cardiac anomalies are not well described in the Korean population. We analyzed the cardiac anomalies and clinical features of Kabuki syndrome in a single tertiary center. Methods: A retrospective analysis was conducted for a total of 13 patients with Kabuki syndrome. Results: The median age at diagnosis of was 5.9 years (range, 9 days to 11 years and 8 months). All patients showed the characteristic facial dysmorphisms and congenital anomalies in multiple organs, and the diagnosis was delayed by 5.9 years (range, 9 days to 11 years and 5 months) after the first visit. Noncardiac anomalies were found in 84% of patients, and congenital heart diseases were found in 9 patients (69%). All 9 patients exhibited left-side heart anomalies, including hypoplastic left heart syndrome in 3, coarctation of the aorta in 4, aortic valve stenosis in 1, and mitral valve stenosis in 1. None had right-side heart disease or isolated septal defects. Genetic testing in 10 patients revealed 9 novel MLL2 mutations. All 11 patients who were available for follow-up exhibited developmental delays during the median 4 years (range, 9 days to 11 years 11 months) of follow-up. The leading cause of death was hypoplastic left heart syndrome. Conclusion: Pediatric cardiologist should recognize Kabuki syndrome and the high prevalence of left heart anomalies with Kabuki syndrome. Genetic testing can be helpful for early diagnosis and counseling.

• 한국의류학회지
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• 제32권10호
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• pp.1629-1639
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• 2008

The purpose of this study is to research the similarity and difference of color application in the culture of Korea and Japan. The subject of this research is the costume color of Changgeuk and Kabuki. This survey analyzes Munsell's 3 attributes(Hue, Value, Chroma), tone, and 3D color analysis by extracted color data. And representative color according to Obangsaek is proposed. The results of this study are as follows: 1. High chroma red in Kabuki costume is more used as symbolic color(passion and luxurious) in order to show character's personality than that in Changguek costume. 2. Low chroma YR color(no-dyeing color) in Changgeuk costume much more used because of Korean white robe preference and eco-friendly thinking. But high chroma yellow is restrictive color for symbolic color of emperor in Korea and Japan. 3. Blue is most frequently used in both costumes because blue is encouraging color by the theory of exponents of the five elements doctrine. 4. White in Korean Changguek costume is more used for white robe preference thinking, but black in Kabuki costume is much more used for symbol of power in Japan. The similarity of Korean and Japanese cultural color is to use much Obangsaek, less Ogansaek by the theory of exponents of the five elements doctrine, but the difference of Korean and Japanese cultural color is to use color differently according to preference thinking system.

• 대한장애인치과학회지
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• 제13권2호
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• pp.104-107
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• 2017

본 증례는 가부키 증후군 환아의 상악 좌측 제2유구치의 심한 우식으로 인하여 좌측 협부 종창이 관찰되었고, 물리적 속박 하에 절개 및 배농술을 시행 후 유구치는 발거되었다. 이후 전신마취 하에 남은 우식치아의 치료가 진행되었다. 가부키 증후군은 환자에 따라 다양한 의학적 소견을 보이므로 전신마취 하 치과 치료가 계획 될 경우, 의과와의 협진 및 철저한 치료계획이 필수적이다.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제33권6호
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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.

• 비교문화연구
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• 제31권
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• pp.35-64
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• 2013

This thesis seeks to compare China's Jing-ju and Japan's Kabuki for their common aspects and differences and examine how they are performed on the stage. Jing-ju is often called as Beijing Opera to refer to a musical play completed during the mid-Ching era and developed around Beijing. Jing-ju is composite arts of music, dance and play which are remarkable in its strict patterns in move along with luxurious costume and heavy make-up. Kabuki which was developed during the Edo-period, is expressional arts also structured with music, dance and play coupled with extravagant costume as well as even more strictly controlled move and emphasis on the beauty of form. The two plays seem very similar to each other in their time setting to gain popularity or features of play. It may look obvious that Jing-ju which had developed earlier than Kabuki, affected the latter's formation. However, general social practices or cultural trends in China and Japan at the time of their development also influenced literature and arts thus affecting play contents and performance expressions. Although the two plays have similar stage structure, they developed in different ways with detailed differences and actors' performance on the stage, way of using a stage and other ways of directing play are largely distinctive from each other. If a play's primary goal is to gain recognition of audience and draw their positive response, the relationship between play and stage becomes essential. With this understanding, this thesis aims to identify where such similarities and differences between the two plays are from by comparing historical background, stage structural development and directing manner development at a basic level.