Catalog

Congenital pulmonary alveolar proteinosis

No MMS code

Link to WHO Definition

Congenital pulmonary alveolar proteinosis – http://id.who.int/icd/entity/1603032478

This is a primary, rare lung disease in which abnormal accumulation of surfactant occurs within the alveoli, interfering with gas exchange. PAP can occur in a primary form or secondarily in the settings of malignancy (especially in myeloid leukemia), pulmonary infection, or environmental exposure to dusts or chemicals.