Pulmonary alveolar proteinosis

Definition

Pulmonary alveolar proteinosis (PAP) is a rare but potentially treatable disease, characterized by impaired surfactant metabolism that leads to accumulation in the alveoli of proteinaceous material rich in surfactant protein and its component. PAP is classified into 2 main types, congenital and acquired. The acquired form is subdivided into the autoimmune form and the secondary form. The vast majority of PAP occurs as an autoimmune PAP. In autoimmune PAP, patients generate antibodies against the granulocyte macrophage colony stimulating factor (GM-CSF) protein. Whole-lung lavage is the most widely accepted therapy for symptomatic PAP. Recent data suggest that exogenous GM-CSF therapy has potential in the treatment of autoimmune PAP. Congenital PAP is also known as pulmonary surfactant metabolism dysfunction (SMDP).

References:

[1]. Aaron Hamvas, et al. Genetic disorders of surfactant proteins. Neonatology. 2007;91(4):311-7. [Content Brief]

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

[3]. Jeffrey A Whitsett, et al. Genetic disorders of surfactant homeostasis. Paediatr Respir Rev. 2006;7 Suppl 1:S240-2. [Content Brief]

[4]. Lawrence M Nogee, et al. Genetic mechanisms of surfactant deficiency. Biol Neonate. 2004;85(4):314-8. [Content Brief]

[5]. 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]

生物医学辞典

生物医学辞典は、生物学の学術用語と専門データの包括的で専門的なデータベースです。すべての説明は、権威ある書籍や高いＩＦの文献に基づいており、知りたい生物医学用語の正確な説明を得ることができます。

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