Pulmonary Alveolar Proteinosis in Four Siblings

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 . . .