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Bilateral lung disease, extensive and diffuse. Diagnosis of pulmonary alveolar proteinosis by bronchoscopic cryobiopsy

Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the intra-alveolar accumulation of a proteinaceous phospholipid-laden material called surfactant. Clinically, this disease should be suspected with respiratory failure in association with a crazy paving pattern on high-resolutio...

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Autores principales: Gando, Sebastián, Duré, Roberto, Violi, Damián, Vazquez, Bibiana, Labarca, Gonzalo, Fernandez-Bussy, Sebastián
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5633158/
https://www.ncbi.nlm.nih.gov/pubmed/29021953
http://dx.doi.org/10.1016/j.rmcr.2017.09.010
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author Gando, Sebastián
Duré, Roberto
Violi, Damián
Vazquez, Bibiana
Labarca, Gonzalo
Fernandez-Bussy, Sebastián
author_facet Gando, Sebastián
Duré, Roberto
Violi, Damián
Vazquez, Bibiana
Labarca, Gonzalo
Fernandez-Bussy, Sebastián
author_sort Gando, Sebastián
collection PubMed
description Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the intra-alveolar accumulation of a proteinaceous phospholipid-laden material called surfactant. Clinically, this disease should be suspected with respiratory failure in association with a crazy paving pattern on high-resolution chest computed tomography. We report a 24-year-old gentleman who was referred to us for a history of respiratory failure, treatment with invasive ventilation and tracheostomy. His blood exams and biochemistry were normal. His infectious and rheumatological panel was negative for a secondary disease. A flexible bronchoscopy with a transbronchial biopsy through a CryoProbe was performed. An anatomopathological analysis was periodic acid-Schiff positive for PAP. A CryoProbe is a recently developed diagnostic tool that improves the diagnostic yield in diffuse lung diseases compared to bronchoscopy with transbronchial biopsy. This method should be considered for patients with diffuse lung disease and PAP.
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spelling pubmed-56331582017-10-11 Bilateral lung disease, extensive and diffuse. Diagnosis of pulmonary alveolar proteinosis by bronchoscopic cryobiopsy Gando, Sebastián Duré, Roberto Violi, Damián Vazquez, Bibiana Labarca, Gonzalo Fernandez-Bussy, Sebastián Respir Med Case Rep Case Report Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the intra-alveolar accumulation of a proteinaceous phospholipid-laden material called surfactant. Clinically, this disease should be suspected with respiratory failure in association with a crazy paving pattern on high-resolution chest computed tomography. We report a 24-year-old gentleman who was referred to us for a history of respiratory failure, treatment with invasive ventilation and tracheostomy. His blood exams and biochemistry were normal. His infectious and rheumatological panel was negative for a secondary disease. A flexible bronchoscopy with a transbronchial biopsy through a CryoProbe was performed. An anatomopathological analysis was periodic acid-Schiff positive for PAP. A CryoProbe is a recently developed diagnostic tool that improves the diagnostic yield in diffuse lung diseases compared to bronchoscopy with transbronchial biopsy. This method should be considered for patients with diffuse lung disease and PAP. Elsevier 2017-09-28 /pmc/articles/PMC5633158/ /pubmed/29021953 http://dx.doi.org/10.1016/j.rmcr.2017.09.010 Text en © 2017 The Authors http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
Gando, Sebastián
Duré, Roberto
Violi, Damián
Vazquez, Bibiana
Labarca, Gonzalo
Fernandez-Bussy, Sebastián
Bilateral lung disease, extensive and diffuse. Diagnosis of pulmonary alveolar proteinosis by bronchoscopic cryobiopsy
title Bilateral lung disease, extensive and diffuse. Diagnosis of pulmonary alveolar proteinosis by bronchoscopic cryobiopsy
title_full Bilateral lung disease, extensive and diffuse. Diagnosis of pulmonary alveolar proteinosis by bronchoscopic cryobiopsy
title_fullStr Bilateral lung disease, extensive and diffuse. Diagnosis of pulmonary alveolar proteinosis by bronchoscopic cryobiopsy
title_full_unstemmed Bilateral lung disease, extensive and diffuse. Diagnosis of pulmonary alveolar proteinosis by bronchoscopic cryobiopsy
title_short Bilateral lung disease, extensive and diffuse. Diagnosis of pulmonary alveolar proteinosis by bronchoscopic cryobiopsy
title_sort bilateral lung disease, extensive and diffuse. diagnosis of pulmonary alveolar proteinosis by bronchoscopic cryobiopsy
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5633158/
https://www.ncbi.nlm.nih.gov/pubmed/29021953
http://dx.doi.org/10.1016/j.rmcr.2017.09.010
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