Tuberculosis and Respiratory Diseases

The Korean Academy of Tuberculosis and Respiratory Diseases (대한결핵및호흡기학회)
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A Case of Multiple Micronodular Pneumocyte Hyperplasia of the Lung in a Man with Tuberous Sclerosis

결절성경화증 남자 환자에서 동반된 폐의 다발성 미세결절폐세포증식증 1예

  • Nam, Dong Hyuk (Department of Internal Medicine, National Health Insurance Corporation Ilsan Hospital) ;
  • Choi, Yoon Jung (Department of Pathology, National Health Insurance Corporation Ilsan Hospital) ;
  • Lee, Ju Hyun (Department of Internal Medicine, National Health Insurance Corporation Ilsan Hospital) ;
  • Na, Hyoung Jung (Department of Internal Medicine, National Health Insurance Corporation Ilsan Hospital) ;
  • Kim, Dong Hwan (Department of Internal Medicine, National Health Insurance Corporation Ilsan Hospital) ;
  • Kim, Chong Ju (Department of Internal Medicine, National Health Insurance Corporation Ilsan Hospital) ;
  • Lee, Sun Min (Department of Internal Medicine, National Health Insurance Corporation Ilsan Hospital) ;
  • Hong, Yong Kug (Department of Radiology, National Health Insurance Corporation Ilsan Hospital) ;
  • Han, Chang Hoon (Department of Internal Medicine, National Health Insurance Corporation Ilsan Hospital)
  • 남동혁 (국민건강보험공단 일산병원 내과) ;
  • 최윤정 (국민건강보험공단 일산병원 병리과) ;
  • 이주현 (국민건강보험공단 일산병원 내과) ;
  • 나형중 (국민건강보험공단 일산병원 내과) ;
  • 김동환 (국민건강보험공단 일산병원 내과) ;
  • 김정주 (국민건강보험공단 일산병원 내과) ;
  • 이선민 (국민건강보험공단 일산병원 내과) ;
  • 홍용국 (국민건강보험공단 일산병원 영상의학과) ;
  • 한창훈 (국민건강보험공단 일산병원 내과)
  • Received : 2008.03.26
  • Accepted : 2008.04.21
  • Published : 2008.05.30
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Abstract

Tuberous sclerosis (TS) is an autosomal dominant disorder that is characterized by cutaneous lesions, seizures, mental retardation and hamartomas in various organs including the skin, kidney and brain. Pulmonary involvement is extremely rare, and occurs in approximately 0.1 to 1% of TS cases. Recent reports have indicated multiple micronodular pneumocyte hyperplasia (MMPH) as another rare form of pulmonary involvement of tuberous sclerosis. We report a case of a 35 year-old-male patient who had no pulmonary symptoms but showed multinodular pulmonary shadows on his chest CT scan. The patient was finally diagnosed with TS with MMPH of the lung. MMPH does not appear to have any malignant potential but the clinical significance of MMPH in TS patients is unknown.

결절성경화증은 유전성 신경피부 증후군으로 피부, 뇌, 신장 등의 여러 장기에 과오종을 형성하는 질환이다. 결절성경화증의 폐 침범은 드물지만 다발성 미세결절의 소견을 보이는 경우, 정확한 진단을 위해서는 고해상 흉부 전산화 단층 촬영 및 조직 검사가 필요하겠으며, 다발성 미세결절폐세포증식증을 감별 진단으로 고려해야 할 것이다. 이 질환의 임상적 의의 등 아직 알려진 것이 많지 않으나 현재까지의 보고로는 특별한 치료는 없으며 예후는 좋은 것으로 알려져 있다.

Keywords

References

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