alveolar proteinosis


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alveolar proteinosis

n proteinosis f alveolar
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References in periodicals archive ?
Pulmonary alveolar proteinosis (PAP) is a rare disorder of alveolar accumulation of lipoproteinaceous surfactant components.
Shimmer said lead program, Molgradex, has shown compelling clinical results in a rare Orphan pulmonary disease know as autoimmune Pulmonary Alveolar Proteinosis, and might have strong utility in treating chronic lung infections.
Clinical Scenario.--Pulmonary alveolar proteinosis (PAP) is not one disease but rather a manifestation of a number of disparate diseases and conditions.
FDA approved its Investigational New Drug application for Molgradex, an inhaled formulation of recombinant human granulocyte-macrophage colony-stimulating factor, for the treatment of autoimmune pulmonary alveolar proteinosis, or PAP, allowing for the expansion of the company's Phase 3 IMPALA study into the U.S.
There are few reports of these lesions in pulmonary disorders unrelated to endogenous lipoid pneumonia, pulmonary alveolar proteinosis, or cholesterol pneumonia.
Molgradex for autoimmune pulmonary alveolar proteinosis (aPAP)
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.
1d), confirming the diagnosis of alveolar proteinosis. The whole hole-lung lavage was performed for both lungs sequentially.
The authors estimated the occupational burden of these lung diseases: Asthma, 16 per cent, chronic obstructive pulmonary disease (COPD) 14 per cent, chronic bronchitis 13 per cent, idiopathic pulmonary fibrosis 26 per cent, hypersensitivity pneumonitis19 per cent, sarcoidosis and other granulomatous disease 30 per cent, pulmonary alveolar proteinosis 29 per cent, community-acquired pneumonia (in working-age adults) 10 per cent, and tuberculosis (in silica dust-exposed workers), 2 per cent.
Based on the experience of the authors, the cases address non-malignant and malignant airway obstruction, then technical challenges in interventional bronchoscopy, such as fistulation, bleeding, copious secretions, critical airway obstruction, rigid tube coring and dilatation, flexible forceps dilatation, stenting, and pulmonary alveolar proteinosis. The book ends with videos of rigid bronchoscope intubation and comments based on the cases.
A specific diagnosis should be reported (eg, Langerhans cell histiocytosis, lymphangioleiomyomatosis, pulmonary alveolar proteinosis); if no specific pattern is identified (either normal lung or unclassifiable fibrosis), a descriptive diagnosis could be made.
IMPALA is a pivotal Phase 3 clinical study evaluating Molgradex, an inhaled formulation of recombinant human granulocyte-macrophage colony-stimulating factor, for the treatment of autoimmune pulmonary alveolar proteinosis, or aPAP.