lipid proteinosis

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Related to proteinosis: lipoid proteinosis

lipid proteinosis

[′lip·əd ‚prō·dē·ə′nō·səs]
(medicine)
A hereditary disorder characterized by extracellular deposits of phospholipid-protein conjugate involving various areas of the body, including the skin and air passages.
References in periodicals archive ?
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.
Savara's pipeline comprises Molgradex, an inhaled granulocyte-macrophage colony-stimulating factor (GM-CSF) in Phase 3 development for autoimmune pulmonary alveolar proteinosis, in Phase 2a development for nontuberculous mycobacterial lung infection in both non-cystic fibrosis 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.
A novel missense mutation in exon 7 of the ECM1 gene in an Iranian lipoid proteinosis patient.
Citation: Adele Mucci et al., iPSC-Derived Macrophages Effectively Treat Pulmonary Alveolar Proteinosis in Csf2rb -Deficient Mice.
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.
Colten, "Deficiency of pulmonary surfactant protein B in congenital alveolar proteinosis," New England Journal of Medicine, vol.
Two patients with alveolar proteinosis had ECMO for inability to tolerate single lung ventilation for whole lung lavage, one patient had Pneumocystis carinii pneumonia and another patient had H1N1 pneumonia with refractory hypoxaemia and protracted runs of 48 and 30 days, respectively.
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
Lipoid proteinosis is also known as hyalinosis cutis et mucosae, Urbach-Wiethe disease.
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.