Akademik Veri Yönetim Sistemi
Turkiye Klinikleri Pediatri, cilt.19, sa.3, ss.244-248, 2010 (Scopus)
Pulmonary alveolar proteinosis is an extremely rare cause of respiratory failure in the pediatric age group. PAP is characterized by intra-alveolar accumulation of lipid and proteinaceous material that is periodic acid-Schiff pulmonary alveolar proteinosis positive when visualized on light microscopy. The disease is not associated with inflammation, and lung architecture is typically preserved. The clinical course of pulmonary alveolar proteinosis varies ranges from respiratory failure and death to spontaneous resolution. Classic findings in diagnostic bronchoalveolar lavage include a milky or opalescent aspirate with large alveolar macrophages and increased lymphocytes but few other inflammatory cell types. Open lung biopsy is the criterion standard for the diagnosis of pulmonary alveolar proteinosis. A nine year old girl, presented with respiratory distress, fever, cough and was diagnosed as pulmonary alveolar proteinosis, is discussed in this paper. Copyright © 2010 by Turkiye Klinikleri.