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Article

Alveolar proteinosis of genetic origins

28 Oct 2020

Abstract excerpt

Pulmonary alveolar proteinosis (PAP) is a rare form of chronic interstitial lung disease, characterised by the intra-alveolar accumulation of lipoproteinaceous material. Numerous conditions can lead to its development. Whereas the autoimmune type is the main cause in adults, genetic defects account for a large part of cases in infants and children. Even if associated extra-respiratory signs may guide the...

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PMID: 33115790
DOI: 10.1183/16000617.0187-2019

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