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Congenital pulmonary alveolar proteinosis

Orpha number ORPHA264675
Synonym(s) Congenital PAP
Prevalence Unknown
Inheritance Autosomal dominant
Autosomal recessive
Age of onset Infancy
Neonatal
ICD-10
  • J84.0
ICD-O -
OMIM
UMLS
  • C2931035
MeSH -
MedDRA -

Summary

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.


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