Congenital Pulmonary Alveolar Proteinosis

Saber Hammami; Khaled Harrathi; Khaled Lajmi; Samir Hadded; Chebil Ben Meriem; Mohamed Néji Guédiche

doi:10.1155/2013/764216

Case Reports in Pediatrics (Jan 2013)

Congenital Pulmonary Alveolar Proteinosis

Saber Hammami,
Khaled Harrathi,
Khaled Lajmi,
Samir Hadded,
Chebil Ben Meriem,
Mohamed Néji Guédiche

Affiliations

Saber Hammami: Pediatrics Department, University of Monastir, Faculty of Medicine, Monastir 5000, Tunisia
Khaled Harrathi: ORL Department, University of Monastir, Faculty of Medicine, Monastir 5000, Tunisia
Khaled Lajmi: Pediatrics Department, University of Monastir, Faculty of Medicine, Monastir 5000, Tunisia
Samir Hadded: Pediatrics Department, University of Monastir, Faculty of Medicine, Monastir 5000, Tunisia
Chebil Ben Meriem: Pediatrics Department, University of Monastir, Faculty of Medicine, Monastir 5000, Tunisia
Mohamed Néji Guédiche: Pediatrics Department, University of Monastir, Faculty of Medicine, Monastir 5000, Tunisia

DOI: https://doi.org/10.1155/2013/764216
Journal volume & issue: Vol. 2013

Abstract

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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.

Published in Case Reports in Pediatrics

ISSN: 2090-6803 (Print); 2090-6811 (Online)
Publisher: Wiley
Country of publisher: United Kingdom
LCC subjects: Medicine: Pediatrics
Website: https://onlinelibrary.wiley.com/journal/2920

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