Journal of Yeungnam Medical Science
- Volume 27 Issue 1
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- Pages.63-68
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- 2010
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- 2799-8010(eISSN)
A Case of Lymphangioleiomyomatosis in Lung
폐의 림프관평활근종증 1예
- Park, Jung-Eun (Department of Internal Medicine, College of Medicine, Yeungnam University) ;
- Kim, Hyun-Jung (Department of Internal Medicine, College of Medicine, Yeungnam University) ;
- Woo, Dae-Hyung (Department of Internal Medicine, College of Medicine, Yeungnam University) ;
- Ryu, Yung-Ha (Department of Internal Medicine, College of Medicine, Yeungnam University) ;
-
Lee, Kwan-Ho
(Department of Internal Medicine, College of Medicine, Yeungnam University)
;
- Chung, Jin-Hong (Department of Internal Medicine, College of Medicine, Yeungnam University) ;
-
Shin, Kyeong-Cheol
(Department of Internal Medicine, College of Medicine, Yeungnam University)
- 박정은 (영남대학교 의과대학 내과학교실) ;
- 김현정 (영남대학교 의과대학 내과학교실) ;
- 우대형 (영남대학교 의과대학 내과학교실) ;
- 류영하 (영남대학교 의과대학 내과학교실) ;
-
이관호
(영남대학교 의과대학 내과학교실)
;
- 정진홍 (영남대학교 의과대학 내과학교실) ;
-
신경철
(영남대학교 의과대학 내과학교실)
- Published : 2010.06.30
Abstract
Lymphangioleiomyomatosis (LAM) is a rare, cystic lung disease that is associated with mutation in the tuberous sclerosis genes, renal angiomyolipomas, lymphatic spread and a remarkable female gender predilection. The pathology of LAM is represented by the proliferation of immature smooth muscle cells in the walls of airways, and venules and lymphatic vessels in the lung. The clinical course of LAM is characterized by progressive dyspnea on exertion, recurrent pneumothorax and collections of chylous fluid. The diagnosis of pulmonary LAM can be made on chest X-ray, a high-resolution CT scan and lung biopsy. We experienced a case of pulmonary lymphangioleiomyomatosis in a 28-years-old female patient who had suffered from progressive dyspnea on exertion, so we report on it along with a brief review of the relevant literature.