[Pulmonary alveolar proteinosis: report of two cases and review of the literature]. / Proteinosis alveolar pulmonar: presentación de dos casos y revisión de la bibliografía.
Rev Fac Cien Med Univ Nac Cordoba
; 65(1): 23-31, 2008.
Article
em Es
| MEDLINE
| ID: mdl-20803936
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.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Proteinose Alveolar Pulmonar
Tipo de estudo:
Diagnostic_studies
Limite:
Adult
/
Female
/
Humans
/
Male
/
Middle aged
Idioma:
Es
Revista:
Rev Fac Cien Med Univ Nac Cordoba
Ano de publicação:
2008
Tipo de documento:
Article
País de afiliação:
Argentina
País de publicação:
Argentina