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.

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 ?
Pulmonary alveolar proteinosis associated with the rare autosomal recessive disorder lysinuric protein intolerance, in which there is defective cationic amino acid transport, (32) may be due to common transporter defects resulting in imbalances of arginine and nitric oxide.
Crazy paving is not specific for alveolar proteinosis. (79) The differential diagnosis includes Pneumocystis pneumonia, a cause of secondary alveolar proteinosis.
Cholesterol granulomas in pulmonary disorders, unrelated to endogenous lipoid pneumonia, pulmonary alveolar proteinosis, and cholesterol pneumonia, are a rare finding during pneumectomy or autopsy.
Savara's pipeline comprises Molgradex, an inhaled granulocyte-macrophage colony-stimulating factor (GM-CSF) in Phase 3 development for autoimmune pulmonary alveolar proteinosis (aPAP), in Phase 2a development for nontuberculous mycobacterial (NTM) lung infection in both non-cystic fibrosis (CF) and CF-affected individuals with chronic NTM lung infection and AeroVanc, a Phase 3-stage inhaled vancomycin for treatment of persistent methicillin-resistant Staphylococcus aureus (MRSA) lung infection in CF.
Pulmonary alveolar proteinosis. Eur Respir Rev 2011;20(120):98-107.
Citation: Adele Mucci et al., iPSC-Derived Macrophages Effectively Treat Pulmonary Alveolar Proteinosis in Csf2rb -Deficient Mice.
High-affinity autoantibodies specifically eliminate granulocyte-macrophage colony-stimulating factor activity in the lungs of patients with idiopathic pulmonary alveolar proteinosis. Blood 2004;103:1089-98.
Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by the accumulation of proteinaceous material in the alveoli due to decreased clearance of surfactant by alveolar macrophages.
Pulmonary alveolar proteinosis (PAP) is a rare pathology in children, and the ideal treatment is sequential unilateral or bilateral lung lavages (1).
Pulmonary alveolar proteinosis (PAP): the presence of ground glass and/or consolidative infiltrates in patchy or diffuse distributions, reticular opacities or interlobular septal thickening present within the airspace infiltrates, creating a "crazy-paving" pattern on HRCT.16
The combined company pipeline will include AeroVanc, an inhaled dry-powder vancomycin to treat chronic methicillin-resistant Staphylococcus aureus (MRSA) pulmonary infection in cystic fibrosis (CF) in preparation for a pivotal Phase 3 study, Molgradex, an inhaled nebulized GM-CSF to treat pulmonary alveolar proteinosis (PAP) currently in Phase 2/3 development and AIR001, an inhaled nebulised sodium nitrite solution to treat heart failure with preserved ejection fraction (HFpEF) currently in Phase two development.
Detailed investigation of these patients revealed that 64% (n = 16) of them had pulmonary arterial hypertension (PAH), 28% (n = 7) had interstitial lung disease (IPL), and 20% (n = 5) had alveolar proteinosis (AP).