| Title | [Pulmonary alveolar proteinosis] |
| Author(s) | Sobiecka M, Korzeniewska-Koseła M, Kuś J |
| Institution | I Kliniki Gruźlicy i Chorób Płuc Instytutu Gruźlicy, i Chorób Płuc w Warszawie. |
| Source | Pneumonol Alergol Pol 2000; 68(9-10):441-9. |
| MeSH | Adult
Bronchoalveolar Lavage Fluid
English Abstract
Female
Humans
Lung
Male
Middle Aged
Pulmonary Alveolar Proteinosis
Radiographic Image Enhancement
Respiratory Function Tests
Tomography, X-Ray Computed
|
| Abstract | Pulmonary alveolar proteinosis (PAP) is a rare disease characterised by the accumulation of proteinaceous material within alveoli. In order to evaluate the clinical features and the course of PAP we reviewed 7 cases (2F/5M) diagnosed during a 11-year period (1989-1999). The mean age of patients was 40.7 +/- 11.2 years. Diagnosis was obtained by open lung biopsy in all cases. Clinical findings included dyspnea (43%), cough (28%) and crackles (28%). Lung function tests were normal in 5 cases and showed a moderate restrictive pattern in 1 and mild airflow obstruction in 1. Three patients had reduced Dlco (mean was 63% of predicted). Four patients had hypoxemia at rest. Chest X-ray revealed bilateral alveolar opacities (71%), involving perihilar areas and lower lobes. HRCT scans demonstrated diffuse ground glass opacities (83%) with interlobular septa thickening (50%). Three patients were treated with repeated segmental BAL (2 improved). The spontaneous partial remission occurred in 4. |
| Language | pol |
| Pub Type(s) | Clinical Trial
Journal Article
|
| PubMed ID | 11276975 |
