Proteinose alveolar congénita.
Pulmonary Alveolar Proteinosis (PAP) is a rare entity characterized by the intra-alveolar accumulation of proteinaceous PAS positive and diastase resistant material. Its pathogenesis remains unclear, although a surfactant protein B deficiency and changes in the granulocyte-macrophage colony stimulat...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Ordem dos Médicos
2005-04-01
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| Series: | Acta Médica Portuguesa |
| Online Access: | https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1009 |
| _version_ | 1797996257603485696 |
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| author | Susana Pissarra Gustavo Rocha Inês Acevedo Hercília Guimarães |
| author_facet | Susana Pissarra Gustavo Rocha Inês Acevedo Hercília Guimarães |
| author_sort | Susana Pissarra |
| collection | DOAJ |
| description | Pulmonary Alveolar Proteinosis (PAP) is a rare entity characterized by the intra-alveolar accumulation of proteinaceous PAS positive and diastase resistant material. Its pathogenesis remains unclear, although a surfactant protein B deficiency and changes in the granulocyte-macrophage colony stimulating factor (GM-CSF) receptor have been proposed as involved mechanisms. Clinically two forms of PAP occur in the pediatric age group: acquired and congenital. The congenital form, very rare, presents in the neonatal period with respiratory insufficiency that starts in the first hours of life and is rapidly progressive, despite therapeutic measures. A definitive diagnosis is based on characteristic histological and immunohistochemical findings of pulmonary samples obtained by lung biopsy. The authors report on a case of Congenital Pulmonary Alveolar Proteinosis that is not associated with SP-B deficiency. |
| first_indexed | 2024-04-11T10:14:44Z |
| format | Article |
| id | doaj.art-152d717ab5d44abfb7af76147218cf55 |
| institution | Directory Open Access Journal |
| issn | 0870-399X 1646-0758 |
| language | English |
| last_indexed | 2024-04-11T10:14:44Z |
| publishDate | 2005-04-01 |
| publisher | Ordem dos Médicos |
| record_format | Article |
| series | Acta Médica Portuguesa |
| spelling | doaj.art-152d717ab5d44abfb7af76147218cf552022-12-22T04:29:59ZengOrdem dos MédicosActa Médica Portuguesa0870-399X1646-07582005-04-0118210.20344/amp.1009Proteinose alveolar congénita.Susana Pissarra0Gustavo RochaInês AcevedoHercília GuimarãesDepartamento de Pediatria, HS João, Porto.Pulmonary Alveolar Proteinosis (PAP) is a rare entity characterized by the intra-alveolar accumulation of proteinaceous PAS positive and diastase resistant material. Its pathogenesis remains unclear, although a surfactant protein B deficiency and changes in the granulocyte-macrophage colony stimulating factor (GM-CSF) receptor have been proposed as involved mechanisms. Clinically two forms of PAP occur in the pediatric age group: acquired and congenital. The congenital form, very rare, presents in the neonatal period with respiratory insufficiency that starts in the first hours of life and is rapidly progressive, despite therapeutic measures. A definitive diagnosis is based on characteristic histological and immunohistochemical findings of pulmonary samples obtained by lung biopsy. The authors report on a case of Congenital Pulmonary Alveolar Proteinosis that is not associated with SP-B deficiency.https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1009 |
| spellingShingle | Susana Pissarra Gustavo Rocha Inês Acevedo Hercília Guimarães Proteinose alveolar congénita. Acta Médica Portuguesa |
| title | Proteinose alveolar congénita. |
| title_full | Proteinose alveolar congénita. |
| title_fullStr | Proteinose alveolar congénita. |
| title_full_unstemmed | Proteinose alveolar congénita. |
| title_short | Proteinose alveolar congénita. |
| title_sort | proteinose alveolar congenita |
| url | https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1009 |
| work_keys_str_mv | AT susanapissarra proteinosealveolarcongenita AT gustavorocha proteinosealveolarcongenita AT inesacevedo proteinosealveolarcongenita AT herciliaguimaraes proteinosealveolarcongenita |