Three Cases of Pulmonary Alveolar Proteinosis

폐포 단백증 3예

  • Kim, Yeon-Jae (Department of Internal Medicine, School of Medicine Kyungpook National University) ;
  • Han, Chun-Duk (Department of Internal Medicine, School of Medicine Kyungpook National University) ;
  • Cha, Seung-Ick (Department of Internal Medicine, School of Medicine Kyungpook National University) ;
  • Kim, Chang-Ho (Department of Internal Medicine, School of Medicine Kyungpook National University) ;
  • Lee, Yeung-Suk (Department of Internal Medicine, School of Medicine Kyungpook National University) ;
  • Park, Jae-Yong (Department of Internal Medicine, School of Medicine Kyungpook National University) ;
  • Jung, Tae-Hoon (Department of Internal Medicine, School of Medicine Kyungpook National University) ;
  • Park, Tae-In (Department of Pathology, School of Medicine Kyungpook National University) ;
  • Sohn, Yun-Kyung (Department of Pathology, School of Medicine Kyungpook National University)
  • 김연재 (경북대학교 의과대학 내과학교실) ;
  • 한춘덕 (경북대학교 의과대학 내과학교실) ;
  • 차승익 (경북대학교 의과대학 내과학교실) ;
  • 김창호 (경북대학교 의과대학 내과학교실) ;
  • 이영석 (경북대학교 의과대학 내과학교실) ;
  • 박재용 (경북대학교 의과대학 내과학교실) ;
  • 정태훈 (경북대학교 의과대학 내과학교실) ;
  • 박태인 (경북대학교 의과대학 해부병리학교실) ;
  • 손윤경 (경북대학교 의과대학 해부병리학교실)
  • Published : 1993.08.31

Abstract

Pulmonary alveolar proteinosis(PAP) is thought to be a rare disease of unknown etiology characterized by the accumulation of strong PAS-positive lipoproteinaceous material in the pulmonary alveolar spaces. The defect in the clearance and degradation of intra-alveolar phospholipoproteinaceous material in PAP likely represents dysfunction of type II pneumocytes. Although the causative treatment of PAP is not well known, yet whole lung bronchopulmonary lavage is a relatively safe and effective treatment. We experienced three cases of PAP, which were confirmed by light and electron microscopic examinations of lung tissues obtained by open lung biopsy, transbronchial lung biopsy and lung lavage, so we present 3 cases of PAP with a review of the literature.

경북대학교 의과대학 호흡기내과에서 운동시 호흡곤란을 호소하며 입원하여 개흉폐생검, 경기관지 폐생검 및 기관폐포세척을 하여 광학현미경 및 전자현미경소견상 특징적인 lamellar body가 보이는 폐포단백증으로 진단된 3예를 경험하였기에 문헌고찰과 함께 보고하였다.

Keywords