Tuberculosis and Respiratory Diseases

The Korean Academy of Tuberculosis and Respiratory Diseases (대한결핵및호흡기학회)
권호 표지

A Case of Primary Pulmonary Histiocytosis-X Associated with Central Diabetes Insipidus

중추성 요붕증을 동반한 원발성 폐 조직구종 X 1예

  • Kim, Young-Min (Department of Internal Medicine, Pusan National University, College of Medicine) ;
  • Park, Yung-In (Department of Internal Medicine, Pusan National University, College of Medicine) ;
  • Choi, Young-Kuen (Department of Internal Medicine, Pusan National University, College of Medicine) ;
  • Lee, Jae-Seung (Department of Internal Medicine, Pusan National University, College of Medicine) ;
  • Lee, Woo-Chul (Department of Internal Medicine, Pusan National University, College of Medicine) ;
  • Hong, Jin-Hee (Department of Internal Medicine, Pusan National University, College of Medicine) ;
  • Lee, Soo-Bong (Department of Internal Medicine, Pusan National University, College of Medicine) ;
  • Reu, Ki-Chan (Department of Internal Medicine, Pusan National University, College of Medicine) ;
  • Lee, Min-Ki (Department of Internal Medicine, Pusan National University, College of Medicine) ;
  • Lee, Chang-Hun (Department of Pathology, Pusan National University, College of Medicine) ;
  • Lee, Hyoung-Ryel (Department of Thoracic surgery, Pusan National University, College of Medicine) ;
  • Park, Soon-Kew (Department of Internal Medicine, Pusan National University, College of Medicine)
  • 김영민 (부산대학교 의과대학 내과학교실) ;
  • 박융인 (부산대학교 의과대학 내과학교실) ;
  • 최영근 (부산대학교 의과대학 내과학교실) ;
  • 이재승 (부산대학교 의과대학 내과학교실) ;
  • 이우철 (부산대학교 의과대학 내과학교실) ;
  • 홍진희 (부산대학교 의과대학 내과학교실) ;
  • 이수봉 (부산대학교 의과대학 내과학교실) ;
  • 류기찬 (부산대학교 의과대학 내과학교실) ;
  • 이민기 (부산대학교 의과대학 내과학교실) ;
  • 이창훈 (부산대학교 의과대학 병리학교실) ;
  • 이형렬 (부산대학교 의과대학 흉부외과학교실) ;
  • 박순규 (부산대학교 의과대학 내과학교실)
  • Published : 1999.01.30
Download PDF
⟨ Previous Next ⟩

Abstract

Pulmonary histiocytosis X is a granulomatous disorder of the lung of unknown cause. Patients with this disease often complain of cough, dyspnea on exertion and, occasionally, chest pain from pneumothorax or bone involvement. However, DI is uncommon in these patients. We report a case of primary pulmonary histiocytosis X with central diabetes insipidus. A 23-year-old man presented with dyspnea suffered from dry cough, exertional dyspnea, polydipsia and polyuria for 4 months. He was a heavy smoker. He was found to have reticulonodular interstitial opacities on chest X-ray film. High-resolution computed tomography revealed thin-walled cysts of various sizes in both lungs. Open lung biopsy was done. On light microscopic examination revealed proliferation and infiltration of Langerhans cells. Immunohistochemically, Langerhans cells showed strong cytoplasmic staining with S-100 protein and electronmicroscopic examination showed Birbeck granules in Langerhans cells. Water deprivation test showed central-type diabetes insipidus and brain MRI showed no abnormal lesion on suprasellar region. Smoking cessation was recommended. He was treated with oral desmopressin.

저자들은 호흡곤란과 다음, 다뇨로 내원한 23세 남자에서 임상소견, 흉부 X-선 검사, 고해상도 흉부단층 촬영, 수분제한검사, 개흉 폐생검으로 확진된 흔치 않은 중추성 요붕증을 통반한 원발성 폐 조직구증 X를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

Keywords