A Case of Acquired Pulmonary Alveolar Proteinosis Successfully Treated with Whole Lung Lavage
Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by the accumulation of surfactant within the alveoli. Surfactant phospholipids and proteins are produced by type II alveolar epithelial cells, and subsequently cleared by the alveolar macrophages. Cell signaling initiated by the g...
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Zusammenfassung: | Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by the accumulation of surfactant within the alveoli. Surfactant phospholipids and proteins are produced by type II alveolar epithelial cells, and subsequently cleared by the alveolar macrophages. Cell signaling initiated by the granulocyte-macrophage colony-stimulating factor (GM-CSF) receptor is essential to the breakdown of surfactant. The acquired form of PAP most commonly involves circulating anti-bodies to GM-CSF, with resultant accumulation of surfactant.
American Thoracic Society , 13 May 2016, 18 May 2016, |
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