Author/Authors :
Tabatabaei, Ahmad shahid beheshti university of medical sciences - Mofid Children Hospital, تهران, ايران , Karimi, Abdollah shahid beheshti university of medical sciences - Mofid Children Hospital - Pediatric Infectious Research Center, تهران, ايران , Rafiee Tabatabaei, Sedigheh shahid beheshti university of medical sciences - Mofid Children Hospital - Pediatric Infectious Research Center, تهران, ايران , Radpay, Badiozzaman shahid beheshti university of medical sciences - Masih Daneshvari Hospital, تهران, ايران , Jadali, Farzaneh shahid beheshti university of medical sciences - Mofid Children Hospital - Pediatric Infections Research Center, تهران, ايران , Shiva, Farideh shahid beheshti university of medical sciences - Mofid Children Hospital - Pediatric Infectious Research Center, تهران, ايران , Hadipour Jahromy, Mana shahid beheshti university - Pediatric Infections Research Center, تهران, ايران
Abstract :
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.
Keywords :
Pulmonary Alveolar Proteinosis , Respiratory Failure , Respiratory Distress , Children
