Childhood Pulmonary Alveolar Proteinosis (PAP)

Abstract

Pulmonary alveolar proteinosis is a rare disease in childhood, generally found in infants less than 1 year old. Survival time averages less than one year after the onset of symptoms, which consist mainly of cough and dyspnea. Of the 23 patients with pulmonary alveolar proteinosis, seven had thymic alymphoplasia (30%); this association leads to speculation that the immunologic system may be incompetent or contribute to the etiology of the disease. The diagnosis of pulmonary alveolar proteinosis is confirmed by biopsy, although chest roentgenograms and sputum staining may be helpful. Therapy consists of the use of steroids, antibiotics, and inhalation therapy; bronchopulmonary lavage has been unsuccessful in children.