Abstract
No abstract available Galle Medical Journal, Vol 18: No. 2, September 2013;Page 31-32 DOI: http://dx.doi.org/10.4038/gmj.v18i2.6200
Highlights
Pulmonary alveolar proteinosis, a rare pulmonary disorder, was first described by Rosen et al in 1958 [1]
Congenital Pulmonary alveolar proteinosis (PAP) is due to a heterogeneous mutation of genes encoding surfactant proteins. [2,3]
Primary PAP is considered an autoimmune condition with excess surfactant production caused by GM CSF neutralizing antibodies, receptor deficiency or gene deficiency / mutation, which lead to lack of macrophage stimulation
Summary
A rare pulmonary disorder, was first described by Rosen et al in 1958 [1]. It is characterised by abnormal accumulation of surfactant derived substances in the alveoli, due to a defect either in the production of surfactant or the clearance by alveolar macrophages, which explains the high association with malignancies and unusual infections. Those affected could have mild symptoms, spontaneous resolution or progressive disease with respiratory failure
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