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Congenital pulmonary alveolar proteinosis: a case report.

Pulmonary Alveolar Proteinosis (PAP) is a rare entity characterized by the intra-alveolar accumulation of proteinaceous PAS positive and diastase resistant material. Its pathogenesis remains unclear, although a surfactant protein B deficiency and changes in the granulocyte-macrophage colony stimulating factor (GM-CSF) receptor have been proposed as involved mechanisms. Clinically two forms of PAP occur in the p...