• Clinical and Experimental Pediatrics
• /
• 제51권6호
• /
• pp.646-649
• /
• 2008

Hallermann-Streiff 증후군은 새 모양의 두개 기형, 치아 이상, 털 감소증, 피부위축, 선천성 백내장, 양측 소안구증, 비례적 왜소증 등의 7가지 특징을 동반한다. 소하악증이나 후두 연화증으로 인한 상기도 폐쇄가 문제가 되며, 이로 인한 수면 무호흡증이나, 호흡기 감염, 폐성심, 섭식 곤란 등이 자주 나타난다. 전 세계적으로도 150례 정도가 보고되어 있는 드문 질환으로써, 이에 저자들이 경험한 무수정체를 동반한 Hallermann-Strieff 증후군 1례를 보고하는 바이다.

• Childhood Kidney Diseases
• /
• 제11권1호
• /
• pp.100-105
• /
• 2007

Cockayne 증후군은 지능저하와 발육부전을 특징으로 하며 피부의 광과민성, 색소성의 망막증, 백내장, 신경전도성 귀머거리 외에 다기관 이상을 동반하는 드문 질환이다. 신장학적 이상 소견 또한 드물지 않게 동반되는 합병증 중 하나이며 병리학적으로 사구체의 유리질화, 세뇨관의 위축 및 간질 내 섬유화가 관찰될 수 있다. 저자들은 7세된 남아에서 Cockayne 증후군의 특징적인 외모와 임상양상을 확인하고 동반된 국소 분절성 사구체 경화증을 진단하였기에 문헌 고찰과 함께 보고하는 바이다.

• 복식문화연구
• /
• 제18권2호
• /
• pp.229-244
• /
• 2010

This study aims to discuss the type and characteristics of primitivism in the modern fashion of the $21^{st}$ century and, as a research method, the concept of primitivism as well as the transition of the patterns of primitivism expressed in modern art have been considered and reviewed through a variety of references. In particular, an empirical analysis of the works that have been created from 2000 to 2009 has been performed using domestic and overseas fashion and collection magazines. The characteristics of primitivism in modern fashion possess the following types of expression: First, Sensuality can be cited as one of the characteristics, either by using direct or indirect exposure of the human body, a silhouette which fits tightly to the body, or creating the effect of sensual beauty using animal fur or bird feathers. Second, Incantation: Masks symbolizing primitive incantation are used to cover the human face or primitive incantation is incorporated as a theme of hair accessories or fashion trinkets, etc. In addition, such decorations as tattoos and the body colorations of ancient tribes are reproduced in modern fashion by means of body painting, printing or other accessories, emphasizing the image of occult primitiveness. Third, Naturalness can be cited as one of the characteristics. Naturalness is emphasized in modern fashion not through artificial decorations and processing, but rather through different patterns of exposure by which natural purity can be felt or through the use of non-artificial materials which recalls primitive civilization. Forth, Playfulness is expressed in the form of graffiti or abstract letters and paintings, and the character of the play is often expressed by the use of grotesque images based on various distortions and exaggerations of the human body, the utilization of symbols of primitive incantation and body and/or facial painting. Fifth, Lastly 'folkishness is emphasized. Folk-like objects, facial decorations, exposure of the body and intense color contrasts typically represent the folkish characteristics.