Pulmonary alveolar proteinosis

Nature and origin of alveolar lipid
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      The alveolar material and the metabolism of pulmonary lipids in alveolar proteinosis was studied. We analyzed pulmonary tissue from three patients and pulmonary washings from nine patients with alveolar proteinosis in various phases of the disorder, and investigated synthesis and dissipation of pulmonary lipids. The results were compared with similar observations in patients with other pulmonary diseases.
      Phospholipid, principally palmitoyl lecithin, was the predominant lipid in tissue and washings from both groups of patients. The total lipid and phospholipid content of tissue and washings was much greater in patients with active alveolar proteinosis than in patients without this disorder, but the relative proportions of phosphatide fractions and fatty acid esters were similar in both groups of patients. The lipid normally present in lung washings and that accumulating in alveolar proteinosis is the same and it is synthesized by the lung. No enhancement of lipid synthesis could be demonstrated in alveolar proteinosis but the removal or degradation of alveolar phospholipid was found to be impaired. There was no deficiency of plasma lipoprotein lipase activity. Pulmonary washings contained increased amounts of protein, but this protein did not have electrophoretic mobility and was apparently denatured. It contained large amounts of palmitoyl lecithin but the material accumulating within alveoli did not have surface tension-lowering activity and was inhibitory to surfactant. The concept is advanced that alveolar proteinosis results from prolonged retention of the metabolic products and by-products of the alveolar lining.
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