Pulmonary alveolar proteinosis: report of two cases and update

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 respons...

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Autores principales: Cambursano, VH, Langer, MD, Cazaux, A, Fossati, F, Gonzalez Achaval, CJ, Azcurra, S, Mendoza, C, Schiarolli , H, Marino, M
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Lenguaje:Español
Publicado: Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología 2020
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Acceso en línea:https://revistas.unc.edu.ar/index.php/med/article/view/27687
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spelling I10-R10-article-276872020-03-05T18:50:29Z Pulmonary alveolar proteinosis: report of two cases and update Proteinosis alveolar pulmomar: Presentación de dos casos y revisión de bibliografía Cambursano, VH Langer, MD Cazaux, A Fossati, F Gonzalez Achaval, CJ Azcurra, S Mendoza, C Schiarolli , H Marino, M pulmonary alveolar proteinosis lung diseases therapy proteinosis alveolar pulmonar enfermedades pulmonares terapia 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. La Proteinosis Alveolar Pulmonar es una enfermedad infrecuente, caracterizada por lesiones radiológicas y tomográficas típicas y severas, con un cuadro clínico variable desde la insuficiencia respiratoria a la resolución espontánea. Entre sus tres variedades, la llamada forma idiopática o del adulto, representa más del 80% de los casos publicados. La fisiopatología esta dada por un proceso autoinmune dirigido contra el factor estimulante de colonias de granulocitos y macrófagos (GM-CSF), que condiciona un defecto funcional en los macrófagos que conduce a la acumulación intraalveolar de surfactante y manifestaciones infecciosas, pulmonares y extrapulmonares, a menudo por gérmenes no usuales. El conjunto de datos aportados por las imágenes, la disociación clínico-radiológica y material de lavado broncoalveolar, en general son suficientes para el diagnóstico, aunque en ocasiones se requiere de la histopatología para su confirmación. La terapéutica recomendada es el lavado pulmonar total. La mortalidad es baja y se relaciona fundamentalmente con las complicaciones infecciosas. Describimos en este trabajo nuestra experiencia diagnóstica y terapéutica con dos pacientes Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología 2020-02-14 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion application/pdf https://revistas.unc.edu.ar/index.php/med/article/view/27687 Revista de la Facultad de Ciencias Médicas de Córdoba.; Vol. 65 No. 1 (2008); 22 - 29 Revista de la Facultad de Ciencias Médicas de Córdoba; Vol. 65 Núm. 1 (2008); 22 - 29 Revista da Faculdade de Ciências Médicas de Córdoba; v. 65 n. 1 (2008); 22 - 29 1853-0605 0014-6722 spa https://revistas.unc.edu.ar/index.php/med/article/view/27687/29114 Derechos de autor 2020 Universidad Nacional de Córdoba
institution Universidad Nacional de Córdoba
institution_str I-10
repository_str R-10
container_title_str Revistas de la UNC
language Español
format Artículo revista
topic pulmonary alveolar proteinosis
lung diseases
therapy
proteinosis alveolar pulmonar
enfermedades pulmonares
terapia
spellingShingle pulmonary alveolar proteinosis
lung diseases
therapy
proteinosis alveolar pulmonar
enfermedades pulmonares
terapia
Cambursano, VH
Langer, MD
Cazaux, A
Fossati, F
Gonzalez Achaval, CJ
Azcurra, S
Mendoza, C
Schiarolli , H
Marino, M
Pulmonary alveolar proteinosis: report of two cases and update
topic_facet pulmonary alveolar proteinosis
lung diseases
therapy
proteinosis alveolar pulmonar
enfermedades pulmonares
terapia
author Cambursano, VH
Langer, MD
Cazaux, A
Fossati, F
Gonzalez Achaval, CJ
Azcurra, S
Mendoza, C
Schiarolli , H
Marino, M
author_facet Cambursano, VH
Langer, MD
Cazaux, A
Fossati, F
Gonzalez Achaval, CJ
Azcurra, S
Mendoza, C
Schiarolli , H
Marino, M
author_sort Cambursano, VH
title Pulmonary alveolar proteinosis: report of two cases and update
title_short Pulmonary alveolar proteinosis: report of two cases and update
title_full Pulmonary alveolar proteinosis: report of two cases and update
title_fullStr Pulmonary alveolar proteinosis: report of two cases and update
title_full_unstemmed Pulmonary alveolar proteinosis: report of two cases and update
title_sort pulmonary alveolar proteinosis: report of two cases and update
description 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.
publisher Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología
publishDate 2020
url https://revistas.unc.edu.ar/index.php/med/article/view/27687
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last_indexed 2022-08-20T01:27:16Z
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