Pulmonary alveolar proteinosis [PDF]
Chronic Respiratory Disease, 2006 Pulmonary alveolar proteinosis is a rare syndrome characterized by intra-alveolar accumulation of surfactant components and cellular debris, with minimal interstitial inflammation or fibrosis. The condition has a variable clinical course, from spontaneous resolution to respiratory failure and death due to disease progression or superimposed infections.
O C, Ioachimescu, M S, Kavuru
openaire +2 more sources
Interstitial lung disease as a late complication of pulmonary alveolar proteinosis
Radiology Case Reports, 2019 Pulmonary alveolar proteinosis is a rare condition characterized by accumulation of intra-alveolar surfactant. Here, we report a case of interstitial lung disease which developed over the years in a patient with pulmonary alveolar proteinosis.
Alexandre Semionov, MD, PhD +1 more
doaj +1 more source
Clinical biological and genetic heterogeneity of the inborn errors of pulmonary surfactant metabolism [PDF]
, 2001 Pulmonary surfactant is a multimolecular complex located at the air-water interface within the alveolus to which a range of physical (surface-active properties) and immune functions has been assigned. This complex consists of a surface-active lipid layer
Clements JA +29 more
core +1 more source
Journal of Medical Case Reports, 2017 Background The congenital form of pulmonary alveolar proteinosis due to colony stimulating factor 2 receptor alpha gene mutations is a rare disease with only a few cases reported worldwide.
Adel S. Al-Haidary +3 more
doaj +1 more source
Whole lung lavage in pulmonary alveolar proteinosis: anesthetic management and challenges
Ain Shams Journal of Anesthesiology, 2022 Background Pulmonary alveolar proteinosis is a rare disorder characterized by alveolar obstruction secondary to the collection of lipoproteinaceous material in the alveoli leading to a spectrum of respiratory illness ranging from mild to severe ...
Manisha Manohar, Priyanka Bansal
doaj +1 more source
Mavrilimumab, a fully human granulocyte-macrophage colony-stimulating factor receptor α monoclonal antibody: long-term safety and efficacy in patients with rheumatoid arthritis [PDF]
, 2018 Objective: Mavrilimumab, a human monoclonal antibody, targets granulocyte-macrophage colony-stimulating factor receptor alpha. We report mavrilimumab long-term safety and efficacy in rheumatoid arthritis patients in two phase IIb studies (1071, 1107 ...
Achuthan +44 more
core +1 more source
Alveolar proteinosis in Behçet's disease
Multidisciplinary Respiratory Medicine, 2010 A 51-year-old man with Behçet's disease complained of fever, dry cough and dyspnea during exertion. Chest CT showed ground glass opacities with interstitial septal thickening in both lungs.
Tetikkurt Cuneyt +4 more
doaj +1 more source
Sirolimus-induced secondary pulmonary alveolar proteinosis
Respiratory Medicine Case Reports, 2022 Pulmonary alveolar proteinosis (PAP) is a rare pulmonary syndrome that is characterized by the accumulation of excess surfactant in the alveolar space, leading to impaired gas exchange. Sirolimus-induced PAP is an extremely rare entity that has only been
Stephanie Wang, MD +3 more
doaj +1 more source
Impulse oscillometry identifies peripheral airway dysfunction in children with adenosine deaminase deficiency. [PDF]
, 2015 Adenosine deaminase-deficient severe combined immunodeficiency (ADA-SCID) is characterized by impaired T-, B- and NK-cell function. Affected children, in addition to early onset of infections, manifest non-immunologic symptoms including pulmonary ...
Candotti, Fabio +6 more
core +2 more sources
Whole lung lavage therapy for pulmonary alveolar proteinosis: a global survey of current practices and procedures [PDF]
, 2016 Maija Halme on työryhmän WLL International Study Group jäsen.Peer ...
Halme, Maija +1 more
core +1 more source