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Pulmonary Lymphangioleiomyomatosis: A Rare, Diffuse Parenchymal Lung Disease

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Author(s): Levent Cansever | Celalettin İbrahim Kocatürk | Füsun Şahin | Pınar Yıldız | Mehmet Ali Bedirhan

Journal: Turk Toraks Dergisi
ISSN 1302-7808

Volume: 12;
Issue: 03;
Start page: 124;
Date: 2011;
Original page

Keywords: Lymphangioleiomyomatosis | diagnosis | pneumothorax | dyspnea

ABSTRACT
Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease affecting fertile women and is characterized by progressive dyspnea on exertion. Clinically, it could also be characterized by recurrent pneumothorax, haemoptysis or chylous pleural effusions. It is characterized by peribronchial, perivascular, and perilymphatic proliferation of atypical smooth muscle, resulting in vascular and airway obstruction, cyst formation, and a progressive decline in lung function. The diagnosis of LAM usually requires an open lung biopsy. Here we presented 3 cases that were diagnosed as LAM by surgical pathology.
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