Abstract
PULMONARY alveolar proteinosis is characterized by the remittent or progressive accumulation of lipid-rich proteinaceous material within the alveolar sacs in the absence of inflammatory response.<sup>1</sup>The disease appears to be limited to the lung, although its association with rare and bizarre myeloproliferative syndromes suggests that its causative agent may also have a systemic effect.<sup>2-4</sup>Symptoms and findings are caused by a progressive interference with gaseous exchange or by superimposed bacterial and fungal infections.<sup>3-5</sup>The importance of alveolar cellular death in the pathogenesis of the disorder is supported by anatomical observations<sup>1</sup>and by the association of an elevated serum lactic acid dehydrogenase (SLDH) with active disease.<sup>6-9</sup> A new approach to the treatment of alveolar proteinosis was evolved in 1963, based on the concept that the disorder resulted from an abnormality of the pulmonary clearing process.<sup>6</sup>Since that time, the successful treatment of seven cases by pulmonary segmental irrigation has been
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