Pulmonary Alveolar Proteinosis in Four Siblings

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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 . . .