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alveolar proteinosis

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alveolar proteinosis,
a very rare disease marked by the accumulation of plasma proteins, lipoproteins, and other blood components in the alveoli of the lungs, impairing the ability of the lungs to exchange oxygen and carbon dioxide. The disease tends to affect previously healthy young adults, with a higher incidence among males than females. The cause is unknown, and clinical signs vary, although only the lungs are affected. Some patients are asymptomatic, whereas others experience dyspnea and an unproductive cough. The condition may be treated with bronchopulmonary lavage. There is a risk of secondary infections.

proteinosis [pro″tēn-o´sis]
the accumulation of excess protein in the tissues.
alveolar proteinosis pulmonary alveolar proteinosis.
lipid proteinosis a hereditary defect of lipid metabolism marked by yellowish deposits of hyaline lipid carbohydrate mixture on the inner surface of the lips, under the tongue, and on the oropharynx and larynx, and in skin lesions.
pulmonary alveolar proteinosis see pulmonary alveolar proteinosis.


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Other adult respiratory disorders in which either surfactant replacement therapy or abnormal surfactant regulation may occur include pneumonia, lung transplants, sarcoidosis, hypersensitivity pneumonitis, idiopathic pulmonary fibrosis, alveolar proteinosis, obstructive lung disease including asthma, radiation pneumonitis, and drug-induced pulmonary disease.
Idiopathic pulmonary alveolar proteinosis as an autoimmune disease with neutralizing antibody against granulocyte/macrophage colony-stimulating factor.
4) This is speculated to lead to inhibition of lipid degradation and surfactant turnover, a mechanism similarly implicated in pulmonary alveolar proteinosis.
 
 
 
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