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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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Bibliographic Details
Main Authors: Saber Hammami (Author), Khaled Harrathi (Author), Khaled Lajmi (Author), Samir Hadded (Author), Chebil Ben Meriem (Author), Mohamed Néji Guédiche (Author)
Format: Book
Published: Hindawi Limited, 2013-01-01T00:00:00Z.
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Summary: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.
Item Description:2090-6803
2090-6811
10.1155/2013/764216

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