Journal of the Korean Society of Radiology (대한영상의학회지)

The Korean Society of Radiology (대한영상의학회)
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Neurofibromatosis Type I presenting with Spontaneous Pneumothorax: A Case Report

자발성 기흉으로 발현된 제 1형 신경섬유종증: 증례 보고

  • Shin, So-Youn (Department of Radiology, East-West Neo Medical Center, Kyung Hee University) ;
  • Lee, Young-Kyung (Department of Radiology, East-West Neo Medical Center, Kyung Hee University) ;
  • Moon, Ah-Lim (Department of Pathology, East-West Neo Medical Center, Kyung Hee University) ;
  • Sung, Dong-Wook (Department of Radiology, Kyung Hee University Hospital, Kyung Hee University)
  • 신소연 (경희대학교 의과대학 동서신의학병원 영상의학과) ;
  • 이영경 (경희대학교 의과대학 동서신의학병원 영상의학과) ;
  • 문아림 (경희대학교 의과대학 동서신의학병원 병리과) ;
  • 성동욱 (경희대학교 의과대학 경희의료원 영상의학과)
  • Received : 2010.05.07
  • Accepted : 2010.07.29
  • Published : 2010.10.01
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Abstract

Neurofibromatosis type I is an autosomal dominant disease with variable clinical manifestations related to dermatologic, neurologic, skeletal, and endocrine system. Lung parenchymal involvement such as lung fibrosis and massive bullous emphysema is infrequent. Here, we report on a 36-year-old man with symptoms of dyspnea, and who has a spontaneous pneumothorax, multiple bullae, and pathologically confirmed neurofibromatosis type I.

제1형 신경섬유종증(Neurofibromatosis type I)은 피부, 신경계, 골격계, 내분비계에 다양한 임상증상을 나타내는 유전질환이다. 또한 드물게 제1형 신경섬유종증은 폐 섬유화와 얇은 벽을 갖는 낭포 등의 폐 병변을 유발할 수 있는 것으로 알려져 있다. 저자들은 호흡곤란으로 내원한 36세 남자에서 시행한 흉부 전산화단층촬영(CT)에서 기흉과 양쪽 폐에 존재하는 다발성 낭포들을 발견하였고 신체 검사상 발견된 피부 병변에 대하여 조직 검사를 실시하여 제1형 신경섬유종증을 확진된 1예를 경험하였고, 이를 보고하고자 한다.

Keywords

References

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