alveolar proteinosis


Also found in: Dictionary, Wikipedia.

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.

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?te-in-o'sis) [? + osis, condition]
Accumulation of excess proteins in the tissues.

alveolar proteinosis

Pulmonary alveolar proteinosis.

lipoid proteinosis

A rare autosomal recessive condition resulting from an undefined metabolic defect. Yellow deposits of a mixture of protein and lipoid occur, esp. on the mucous surface of the mouth and tongue. Nodules may appear on the face, extremities, and epiglottis and vocal cords, the latter producing hoarseness.

pulmonary alveolar proteinosis

A disease of unknown cause in which eosinophilic material is deposited in the alveoli. The principal symptom is dyspnea. Death from pulmonary insufficiency may occur, but complete recovery has been observed. There is no specific treatment, but general supportive measures including antibiotics and bronchopulmonary lavage have helped. In about 25% of cases, the disease clears spontaneously, but in most untreated cases the disease is progressive and leads to respiratory failure.
Synonym: alveolar proteinosis See: bronchoalveolar lavage
References in periodicals archive ?
A computed tomography (CT) scan confirmed the mosaic distributions of mixed interstitial and ground-glass infiltrates consistent with pulmonary alveolar proteinosis.
Pulmonary alveolar proteinosis is a rare disease of unknown etiology and variable natural history that results in the accumulation of proteinaceous surfactant-like material within the alveoli.
Included in these patterns are (1) acute or subacute injury (infection, drug toxicity, systemic autoimmune disease); (2) chronic interstitial inflammation; (3) granulomatous inflammation and its differential diagnosis; (4) vascular diseases (including vasculitis/diffuse alveolar hemorrhage, pulmonary hypertension, and intravenous drug abuse microangiopathy); and (5) alveolar-filling processes such as alveolar proteinosis.
Pulmonary alveolar proteinosis (PAP) is not one disease but rather a manifestation of a number of disparate diseases and conditions.
Pulmonary alveolar proteinosis is an extremely rare disease in which a fluid accumulates within spaces in the lungs where oxygen and carbon dioxide are exchanged.
Cholesterol granulomas in pulmonary disorders, unrelated to endogenous lipoid pneumonia, pulmonary alveolar proteinosis, and cholesterol pneumonia, are a rare finding during pneumectomy or autopsy.
Clinical significance of anti-GM-CSF antibodies in idiopathic pulmonary alveolar proteinosis.
Bronchoalveolar lavage cytology in pulmonary alveolar proteinosis.
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.
4]) have significantly higher activity than single gene products; c) after ozone exposure, all SP-A variants showed a decreased ability to stimulate TNF-[alpha] and IL-8 production, and the level of the decrease varied among SP-A variants (26-48%); and d) human SP-A from patients with alveolar proteinosis exhibited a minimal decrease (18% and 12%, respectively) in its ability to stimulate TNF-[alpha] and IL-8 after in vitro ozone exposure.
They transplanted either normal or gene-corrected macrophages into the respiratory tracts of mice, which were bred to mimic the hereditary form of a human disease called hereditary pulmonary alveolar proteinosis (hPAP).