Primary interstitial lung disease specific to childhood due to pulmonary surfactant protein anomalies Congenital pulmonary alveolar proteinosis UMLS:C2931035 OMIM:614370 OMIM:265120 Congenital PAP OMIM:300770 Congenital pulmonary alveolar proteinosis is a very rare primary interstitial lung disease due to pulmonary surfactant accumulation within the alveolar macrophages and alveoli, characterized by a variable clinical course ranging from an asymptomatic clinical presentation and spontaneous remission, to symptoms such as dyspnea and cough, or to severe respiratory failure. ICD10:J84.0 OMIM:610913 OMIM:610921 orphanet