Congenital Pulmonary Alveolar Proteinosis

Pulmonary alveolar proteinosis (PAP) is a rare syndrome characterized by pulmonary surfactant accumulation within the alveolar spaces. It occurs with a reported prevalence of 0.1 per 100,000 individuals. Two clinically different pediatric types have been defined as congenital PAP which is fatal and...

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Detalles Bibliográficos
Autores principales: Hammami, Saber, Harrathi, Khaled, Lajmi, Khaled, Hadded, Samir, Ben Meriem, Chebil, Guédiche, Mohamed Néji
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2013
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3655498/
https://www.ncbi.nlm.nih.gov/pubmed/23710403
http://dx.doi.org/10.1155/2013/764216
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author Hammami, Saber
Harrathi, Khaled
Lajmi, Khaled
Hadded, Samir
Ben Meriem, Chebil
Guédiche, Mohamed Néji
author_facet Hammami, Saber
Harrathi, Khaled
Lajmi, Khaled
Hadded, Samir
Ben Meriem, Chebil
Guédiche, Mohamed Néji
author_sort Hammami, Saber
collection PubMed
description Pulmonary alveolar proteinosis (PAP) is a rare syndrome characterized by pulmonary surfactant accumulation within the alveolar spaces. It occurs with a reported prevalence of 0.1 per 100,000 individuals. Two clinically different pediatric types have been defined as congenital PAP which is fatal and a late-onset PAP which is similar to the adult form and less severe. The clinical course of PAP is variable, ranging from spontaneous remission to respiratory failure. Whole-lung lavage is the current standard treatment for PAP patients. We report a new congenital case of PAP.
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spelling pubmed-36554982013-05-24 Congenital Pulmonary Alveolar Proteinosis Hammami, Saber Harrathi, Khaled Lajmi, Khaled Hadded, Samir Ben Meriem, Chebil Guédiche, Mohamed Néji Case Rep Pediatr Case Report Pulmonary alveolar proteinosis (PAP) is a rare syndrome characterized by pulmonary surfactant accumulation within the alveolar spaces. It occurs with a reported prevalence of 0.1 per 100,000 individuals. Two clinically different pediatric types have been defined as congenital PAP which is fatal and a late-onset PAP which is similar to the adult form and less severe. The clinical course of PAP is variable, ranging from spontaneous remission to respiratory failure. Whole-lung lavage is the current standard treatment for PAP patients. We report a new congenital case of PAP. Hindawi Publishing Corporation 2013 2013-04-27 /pmc/articles/PMC3655498/ /pubmed/23710403 http://dx.doi.org/10.1155/2013/764216 Text en Copyright © 2013 Saber Hammami et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Hammami, Saber
Harrathi, Khaled
Lajmi, Khaled
Hadded, Samir
Ben Meriem, Chebil
Guédiche, Mohamed Néji
Congenital Pulmonary Alveolar Proteinosis
title Congenital Pulmonary Alveolar Proteinosis
title_full Congenital Pulmonary Alveolar Proteinosis
title_fullStr Congenital Pulmonary Alveolar Proteinosis
title_full_unstemmed Congenital Pulmonary Alveolar Proteinosis
title_short Congenital Pulmonary Alveolar Proteinosis
title_sort congenital pulmonary alveolar proteinosis
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3655498/
https://www.ncbi.nlm.nih.gov/pubmed/23710403
http://dx.doi.org/10.1155/2013/764216
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AT haddedsamir congenitalpulmonaryalveolarproteinosis
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AT guedichemohamedneji congenitalpulmonaryalveolarproteinosis

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