Congenital pulmonary alveolar proteinosis

Definition:

Congenital pulmonary alveolar proteinosis is also known as pulmonary surfactant metabolism dysfunction (SMDP). It is caused by one of the three different genes associated with surfactant metabolism in type II epithelial cells and characterized by respiratory distress after birth. Congenital PAP is also associated with mutations in CSF2RA and CSF2RB genes, encoding alpha and beta chains of the GM-CSF receptor.

References:

[1]. Ajmal Khan, et al. Pulmonary alveolar proteinosis. Respir Care. 2011 Jul;56(7):1016-28. [Content Brief]

[2]. Margarita Martinez-Moczygemba, et al. Immune dysregulation in the pathogenesis of pulmonary alveolar proteinosis. Curr Allergy Asthma Rep. 2010 Sep;10(5):320-5. [Content Brief]

[3]. R Borie, et al. Pulmonary alveolar proteinosis. Eur Respir Rev. 2011 Jun;20(120):98-107. [Content Brief]

[4]. Takeshi Tanaka, et al. Adult-onset hereditary pulmonary alveolar proteinosis caused by a single-base deletion in CSF2RB. J Med Genet. 2011 Mar;48(3):205-9. [Content Brief]

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