Alveolar proteinosis in children

Pulmonary alveolar proteinosis (PAP) is a subacute, progressive respiratory dysfunction with accumulation of mucin and lipids in the alveoli. Also known as pulmonary alveolar phospholipidosis, it is a rare chronic lung disease of unknown origin. It is characterized by the deposition of lipid-rich glycogen (PAS) -positive protein substances in the alveoli. Surface-active substances are a mixture of phospholipids and various surface-active proteins. Due to the accumulation of surface active substances in the alveolar cavity and airways, the ventilation and ventilation functions of the lungs are seriously affected, resulting in difficulty in breathing. Dyspnea is the most prominent clinical manifestation of alveolar proteinosis. There are two types of PAP in childhood: lethal congenital PAP and acquired PAP.