Pulmonary Alveolar Proteinosis: An Uncommon Cause of Chronic Neonatal Respiratory Distress1,2

A full-term newborn infant had the onset of respiratory distress immediately after birth. She required supplemental oxygen from birth, and had pulmonary alveolar proteinosis (PAP) documented by open lung biopsies at 6 and at 12 weeks of age. Light and electron microscopy showed dense, PAS-positive granular, homogeneous material within the alveolar spaces, diagnostic of PAP. No Pneumocystic carinii organisms were demonstrated on silver staining, and bacterial and viral cultures were negative. The infant died of progressive respiratory insufficiency at 1 yr of age. Pulmonary alveolar proteinosis should be considered a rare cause of chronic respiratory distress in the newborn infant.