Pediatric pulmonary alveolar proteinosis
Pulmonary alveolar proteinosis (PAP) is a subacute, progressive disease of respiratory dysfunction, accumulation of mucin-rich substances and lipids in the alveoli. Also known as pulmonary alveolar phospholipidosis, it is a rare chronic lung disease of unknown cause, characterized by the deposition of lipid-rich glycogen (PAS)-positive protein substances in the alveoli. These substances are called surfactants, which are a mixture of phospholipids and various surfactant proteins. Due to the accumulation of excessive surfactant in the alveolar cavity and airway, the ventilation and gas exchange functions of the lungs are seriously affected, resulting in dyspnea. Dyspnea is the most prominent clinical manifestation of pulmonary alveolar proteinosis. There are two types of PAP in children: fatal congenital PAP and acquired PAP.
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Site of disease:
lung -
Infectious :
Not contagious -
Frequent population:
Children -
Related symptoms:
Secondary infection chest pain dyspnea pleural friction sound reduced exercise tolerance
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