Titulo:
.
Guardado en:
0121-5426
2538-9513
29
2017-08-01
55
62
Revista Colombiana de Neumología - 2017
info:eu-repo/semantics/openAccess
http://purl.org/coar/access_right/c_abf2
| id |
metarevistapublica_asoneumocito_revistacolombianadeneumologia_28_article_239 |
|---|---|
| record_format |
ojs |
| spelling |
Pulmonary alveolar proteinosis is a rare disease characterized by accumulation of surfactant within the alveolar space, which leads to a deranged gas exchange. Its clinical course is variable, ranging from spontaneous resolution to respiratory failure and death. Three forms have been described: genetic, secondary, and primary or idiopathic. The latter is the most common form in the adult population. The pathophysiology is given by an autoimmune process against the granulocyte-macrophage colony-stimulating factor (GM-CSF); this process conditions a functional impairment of macrophages that leads to accumulation of surfactant within the alveoli, as well as pulmonary and extrapulmonary infectious manifestations, often due to unusual germs. The bulk of data yielded by image studies, clinical-radiological dissociation, and bronchoalveolar lavage material is usually enough for reaching diagnosis, although histopathology is sometimes required for confirmation. Recommended treatment is total pulmonary lavage. The mortality is low and is fundamentally related to infectious complications. We describe a case of pulmonary alveolar proteinosis that required confirmation by histopathologic study of thoracoscopic lung biopsy. We also offer a review of the literature. Bula G., Carlos Santiago H., Ernesto Martínez O., Brenda Reyes, Melisa Pabón R., Ricardo alveolar proteinosis dyspnea total pulmonary lavage proteinosis alveolar disnea lavado pulmonar total 29 1 Artículo de revista Journal article 2017-08-01T00:00:00Z 2017-08-01T00:00:00Z 2017-08-01 application/pdf Asociación Colombiana de Neumología y Cirugía de Tórax Revista Colombiana de Neumología 0121-5426 2538-9513 https://revistas.asoneumocito.org/index.php/rcneumologia/article/view/239 10.30789/rcneumologia.v29.n1.2017.239 https://doi.org/10.30789/rcneumologia.v29.n1.2017.239 spa https://creativecommons.org/licenses/by-nc-sa/4.0/ Revista Colombiana de Neumología - 2017 55 62 https://revistas.asoneumocito.org/index.php/rcneumologia/article/download/239/224 info:eu-repo/semantics/article http://purl.org/coar/resource_type/c_6501 http://purl.org/coar/resource_type/c_dcae04bc http://purl.org/redcol/resource_type/ARTREV info:eu-repo/semantics/publishedVersion http://purl.org/coar/version/c_970fb48d4fbd8a85 info:eu-repo/semantics/openAccess http://purl.org/coar/access_right/c_abf2 Text Publication |
| institution |
ASOCIACION COLOMBIANA DE NEUMOLOGIA Y CIRUGIA DE TORAX |
| thumbnail |
https://nuevo.metarevistas.org/ASOCIACIONCOLOMBIANADENEUMOLOGIAYCIRUGIADETORAX/logo.png |
| country_str |
Colombia |
| collection |
Revista Colombiana de Neumología |
| description_eng |
Pulmonary alveolar proteinosis is a rare disease characterized by accumulation of surfactant within the alveolar space, which leads to a deranged gas exchange. Its clinical course is variable, ranging from spontaneous resolution to respiratory failure and death. Three forms have been described: genetic, secondary, and primary or idiopathic. The latter is the most common form in the adult population. The pathophysiology is given by an autoimmune process against the granulocyte-macrophage colony-stimulating factor (GM-CSF); this process conditions a functional impairment of macrophages that leads to accumulation of surfactant within the alveoli, as well as pulmonary and extrapulmonary infectious manifestations, often due to unusual germs. The bulk of data yielded by image studies, clinical-radiological dissociation, and bronchoalveolar lavage material is usually enough for reaching diagnosis, although histopathology is sometimes required for confirmation. Recommended treatment is total pulmonary lavage. The mortality is low and is fundamentally related to infectious complications. We describe a case of pulmonary alveolar proteinosis that required confirmation by histopathologic study of thoracoscopic lung biopsy. We also offer a review of the literature.
|
| author |
Bula G., Carlos Santiago H., Ernesto Martínez O., Brenda Reyes, Melisa Pabón R., Ricardo |
| spellingShingle |
Bula G., Carlos Santiago H., Ernesto Martínez O., Brenda Reyes, Melisa Pabón R., Ricardo alveolar proteinosis dyspnea total pulmonary lavage proteinosis alveolar disnea lavado pulmonar total |
| author_facet |
Bula G., Carlos Santiago H., Ernesto Martínez O., Brenda Reyes, Melisa Pabón R., Ricardo |
| topic |
alveolar proteinosis dyspnea total pulmonary lavage proteinosis alveolar disnea lavado pulmonar total |
| topic_facet |
alveolar proteinosis dyspnea total pulmonary lavage proteinosis alveolar disnea lavado pulmonar total |
| topicspa_str_mv |
proteinosis alveolar disnea lavado pulmonar total |
| citationvolume |
29 |
| citationissue |
1 |
| publisher |
Asociación Colombiana de Neumología y Cirugía de Tórax |
| ispartofjournal |
Revista Colombiana de Neumología |
| source |
https://revistas.asoneumocito.org/index.php/rcneumologia/article/view/239 |
| language |
spa |
| format |
Article |
| rights |
https://creativecommons.org/licenses/by-nc-sa/4.0/ Revista Colombiana de Neumología - 2017 info:eu-repo/semantics/openAccess http://purl.org/coar/access_right/c_abf2 |
| type_driver |
info:eu-repo/semantics/article |
| type_coar |
http://purl.org/coar/resource_type/c_6501 |
| type_version |
info:eu-repo/semantics/publishedVersion |
| type_coarversion |
http://purl.org/coar/version/c_970fb48d4fbd8a85 |
| type_content |
Text |
| publishDate |
2017-08-01 |
| date_accessioned |
2017-08-01T00:00:00Z |
| date_available |
2017-08-01T00:00:00Z |
| url |
https://revistas.asoneumocito.org/index.php/rcneumologia/article/view/239 |
| url_doi |
https://doi.org/10.30789/rcneumologia.v29.n1.2017.239 |
| issn |
0121-5426 |
| eissn |
2538-9513 |
| doi |
10.30789/rcneumologia.v29.n1.2017.239 |
| citationstartpage |
55 |
| citationendpage |
62 |
| url2_str_mv |
https://revistas.asoneumocito.org/index.php/rcneumologia/article/download/239/224 |
| _version_ |
1811200705493467136 |