Pulmonary alveolar proteinosis: report of two cases and update
Pulmonary alveolar proteinosis is a rare disease characterized by extensive radiological and tomographic pulmonary lesions and a variable clinical picture ranging from pulmonary insufficiency to spontaneous remission. Among its three described forms, the so called Idiopathic or Adult form is respons...
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| Autores principales: | , , , , , , , , |
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| Formato: | Artículo revista |
| Lenguaje: | Español |
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Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología
2020
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| Materias: | |
| Acceso en línea: | https://revistas.unc.edu.ar/index.php/med/article/view/27687 |
| Aporte de: |
| id |
I10-R10article-27687 |
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| record_format |
ojs |
| institution |
Universidad Nacional de Córdoba |
| institution_str |
I-10 |
| repository_str |
R-10 |
| container_title_str |
Revistas de la UNC |
| language |
Español |
| format |
Artículo revista |
| topic |
pulmonary alveolar proteinosis lung diseases therapy proteinosis alveolar pulmonar enfermedades pulmonares terapia |
| spellingShingle |
pulmonary alveolar proteinosis lung diseases therapy proteinosis alveolar pulmonar enfermedades pulmonares terapia Cambursano, VH Langer, MD Cazaux, A Fossati, F Gonzalez Achaval, CJ Azcurra, S Mendoza, C Schiarolli , H Marino, M Pulmonary alveolar proteinosis: report of two cases and update |
| topic_facet |
pulmonary alveolar proteinosis lung diseases therapy proteinosis alveolar pulmonar enfermedades pulmonares terapia |
| author |
Cambursano, VH Langer, MD Cazaux, A Fossati, F Gonzalez Achaval, CJ Azcurra, S Mendoza, C Schiarolli , H Marino, M |
| author_facet |
Cambursano, VH Langer, MD Cazaux, A Fossati, F Gonzalez Achaval, CJ Azcurra, S Mendoza, C Schiarolli , H Marino, M |
| author_sort |
Cambursano, VH |
| title |
Pulmonary alveolar proteinosis: report of two cases and update |
| title_short |
Pulmonary alveolar proteinosis: report of two cases and update |
| title_full |
Pulmonary alveolar proteinosis: report of two cases and update |
| title_fullStr |
Pulmonary alveolar proteinosis: report of two cases and update |
| title_full_unstemmed |
Pulmonary alveolar proteinosis: report of two cases and update |
| title_sort |
pulmonary alveolar proteinosis: report of two cases and update |
| description |
Pulmonary alveolar proteinosis is a rare disease characterized by extensive radiological and tomographic pulmonary lesions and a variable clinical picture ranging from pulmonary insufficiency to spontaneous remission. Among its three described forms, the so called Idiopathic or Adult form is responsible for more than 80% of published cases. It’s physiopathology depends on an autoimmune process directed against the GM-CSF (Granulocite Macrophage- Colony Stimulating Factor) that induces a functional defect of the macrophage with consequent intraalveolar accumulation of surfactant. Pulmonary and extrapulmonary infections, are common, often with unusual pathogens. Diagnosis can be made from the combination of clinical, radiological and bronchoalveolar lavage data, although sometimes histopathologic material is needed for confirmation. Total pulmonary lavage is currently the treatment of choice. Mortality is usually low and related mostly to infectious complications. We hereby describe our experience with two patients. |
| publisher |
Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología |
| publishDate |
2020 |
| url |
https://revistas.unc.edu.ar/index.php/med/article/view/27687 |
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