Pulmonary alveolar proteinosis in children: a case series

Pulmonary alveolar proteinosis, (pap) is a rare disease of unknown etiology, characterized by accumulation of intraalveolar proteinaceous material which is rich in lipid and positive on periodic acid-schiff stain. two clinically different pediatric types have been defined as congenital pap which is fulminant and fatal, and a late-onset pap which is similar to the adult form and less severe. eight children with late-onset pap were hospitalized from 1998 to 2005 in mofid children hospital. characteristics of these patients and the methods of diagnosis and treatment are presented in this case series.