Pulmonary Alveolar Proteinosis in Four Siblings

Abstract

PULMONARY alveolar proteinosis (PAP) is a distinctive pathologic lesion in which alveoli are filled with periodic acid–Schiffpositive proteinaceous material. It is usually idiopathic, but it has been reported in association with a variety of other conditions.^{1 2 3 4 5 6 7 8} PAP is usually seen in adults, although the first description of cases included a young child.⁹ Since then, there have been several reports of PAP in infants and children, and the condition has been found at birth.⁸ This report describes four siblings in whom PAP developed during infancy. Our review of the literature disclosed several other affected sibships, yet the familial aspect of PAP . . .