Pulmonary Alveolar Proteinosis in Children: A case series

Pulmonary Alveolar Proteinosis(PAP) is a rare disease of unknown etiology, characterized by the accumulation of proteinoceous material in the alveoli that is rich in lipid and is positive on periodic acid-Schiff(PAS) stain. PAP is rare in children. Two forms are encountered in pediatric practice: congenital alveolar proteinosis (CAP) and a later–onset form that is generally less severe. Broncho-alveolar lavage is the key to diagnosis. Therapeutic lung lavages are the only effective treatment for severe cases. Other therapeutic trials have been proposed such as: lung transplantation, adminstration of GM- CSF and gene-therapy. This case series presents six cases of PAP which were diagnosed by open lung biopsy in Mofid children hospital in Tehran capital city over a three-year period (2003–2006) and one in 1998. The aim of the present study was to provide detailed data on the characteristics of these patients and the methods of diagnosis and treatment.