title: [Cough and hypoxemia as clinical manifestation of pulmonary alveolar proteinosis. Clinical case report].
abstract: 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.
authors.affiliation: Servicio de Endoscopía Respiratoria. Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Ciudad Autónoma de Buenos Aires, Argentina.
authors.forename: Mary
authors.initials: M
authors.lastname: Nieto
authors.affiliation: Servicio de Neumonología. Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Ciudad Autónoma de Buenos Aires, Argentina. manu_dice@hotmail.com.
authors.forename: Manuela
authors.initials: M
authors.lastname: Dicembrino
authors.affiliation: Servicio de Endoscopia Respiratoria. Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Ciudad Autónoma de Buenos Aires, Argentina.
authors.forename: Rubén
authors.initials: R
authors.lastname: Ferraz
authors.affiliation: Servicio de Neumonología. Hospital de Niños D. Villegas, Tandil, Buenos Aires, Argentina.
authors.forename: Fernando
authors.initials: F
authors.lastname: Romagnoli
authors.affiliation: Servicio de Neumonología. Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Ciudad Autónoma de Buenos Aires, Argentina.
authors.forename: Hilda
authors.initials: H
authors.lastname: Giugno
authors.affiliation: Servicio de Neumonología. Hospital Británico, Ciudad Autónoma de Buenos Aires, Argentina.
authors.forename: Glenda
authors.initials: G
authors.lastname: Ernst
authors.affiliation: Servicio de Anatomía Patológica. Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Ciudad Autónoma de Buenos Aires, Argentina.
authors.forename: Monica
authors.initials: M
authors.lastname: Siminovich
authors.affiliation: Servicio de Endoscopía Respiratoria. Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Ciudad Autónoma de Buenos Aires, Argentina.
authors.forename: Hugo
authors.initials: H
authors.lastname: Botto
mesh_terms: D002675:Child, Preschool; D003371:Cough / Q000209:etiology; D005260:Female; D006801:Humans; D000860:Hypoxia / Q000209:etiology; D011649:Pulmonary Alveolar Proteinosis / Q000150:complications / Q000175:diagnosis*
keywords: GM-CSF; Hypoxemia; Pulmonary alveolar proteinosis