Vnitr̆ní lékar̆ství
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Pulmonary alveolar proteinosis is a rare disease characterised by excessive accumulation of surfactant components in the alveoli and the distal airways with minimum inflammatory reaction and fibrosis of pulmonary interstitium. Three clinical forms of pulmonary alveolar proteinosis are distinguished - congenital, primary and secondary. Results of ultrastructural, biochemical and functional analyses and studies performed on genetically modified mice support the presumption that accumulation of surfactant in pulmonary alveolar proteinosis is a result of a degradation disorder and of diminished clearance of the surfactant from the alveolar space rather than of excessive synthesis of surfactant components. ⋯ In most cases, the prognosis for the disease in adult patients is good, even though there is a 10 to 15% rate of patients who develop respiratory failure. Total pulmonary lavage is considered to be the standard method of treatment. In recent years, recombinant human GM-CSF has been studied as a prospective therapy for the treatment of pulmonary alveolar proteinosis.