alveolar proteinosis


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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 ?
From the archives of the AFIP: pulmonary alveolar proteinosis.
The various aetiologies evolved from our study were ABPA (5 cases), BOOP (2 cases), Pulmonary alveolar proteinosis (1 case), Strongyloides (1 case), Amiodarone pneumonitis (1 case), Wegener's granulomatosis (1 case), Acute myeloid leukaemia (1 case) and so on.
Nakata, "Pulmonary alveolar proteinosis," The New England Journal of Medicine, vol.
Familial CFA/IPF) CPI DLPD related with known causes (for example: drug, aspiration, Other interstitial connective tissue penumonia forms diseases, infections, environement) Hypersensitivity pneumonia LAM Interstitial pneumonia types LCG (NSIP, DIP, LIP, UIP, DAD, CP) Alveolar proteinosis Sarcoidosis Eosinophilic pneumonia Idiopatic/infantile pulmonary hemosiderosis Persistent tachypnea of infancy Pulmonary interstitial glycogenosis DLPD related with known causes (for example: drug, aspiration, Congenital disorders connective tissue diseases, infections, environement) DIP Hypersensitivity pneumonia (DMB) Interstitial pneumonia types LIP (NSIP, DIP, LIP, UIP, DAD, CP) (IYS) Lipoid pneumonia (CMD) ?
They address diseases like Marfan and Goodpasture's syndromes, sarcoidosis, autoimmune pulmonary alveolar proteinosis, hereditary haemorrhagic telangiectasia, Hermansky-Pudlak syndrome, primary ciliary dyskinesia, cystic fibrosis, scleroderma lung disease, and alpha-1 antitrypsin deficiency, and their epidemiology, genetic basis and molecular pathogenesis, animal models, clinical presentation, diagnosis, conventional management and treatment strategies, and future therapies and directions.
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.
Whole lung lavage in the treatment of pulmonary alveolar proteinosis.
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.
A 12-year-old Cambodian girl diagnosed with pulmonary alveolar proteinosis (PAP) was referred from a large urban hospital to a community visiting nurse agency for continued management within the home.
One of the workers, a 34-year-old man, subsequently died with acute silicotic alveolar proteinosis.
Pulmonary alveolar proteinosis (PAP) is a rare lung disease where periodic acid Schiff (PAS)-positive eosinophilic material accumulates in the alveoli of the lungs.