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Archivos argentinos de pediatría
versión impresa ISSN 0325-0075versión On-line ISSN 1668-3501
Resumen
NIETO, Mary et al. Cough and hypoxemia as clinical manifestation of pulmonary alveolar proteinosis: Clinical case report. Arch. argent. pediatr. [online]. 2016, vol.114, n.3, pp.e138-e141. ISSN 0325-0075. http://dx.doi.org/10.5546/aap.2016.e138.
Alveolar proteinosis is a rare chronic lung disease, especially in children, characterized by abnormal accumulation of lipoproteins and derived surfactant in the intra-alveolar space that generates a severe reduction of gas exchange. Idiopathic presentation form constitutes over 90% of cases, a phenomenon associated with production of autoimmune antibodies directed at the receptor for granulocyte-macrophage colony-stimulating factor. A case of a girl of 5 years of age treated because of atypical pneumonia with unfavorable evolution due to persistent hypoxemia is presented. The diagnosis is obtained through pathologic examination of lung biopsy by thoracotomy, as treatment is carried out by 17bronchopulmonary bronchoscopy lavages and the patient evidences marked clinical improvement.
Palabras clave : Pulmonary alveolar proteinosis; Hypoxemia; GM-CSF.