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Primary pulmonary alveolar proteinosis [PDF]
Introduction. Pulmonary alveolar proteinosis is an uncommon disease characterized by the accumulation of surfactant proteins and phospholipids within the alveolar spaces. Acquired disease can be idiopathic (primary) and secondary.
Šarac Sanja +5 more
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Therapeutic Whole Lung Lavage for Pulmonary Alveolar Proteinosis: Technique With Continuous Video‐Enabled Double Lumen Endotracheal Tube [PDF]
Therapeutic whole lung lavage with a continuous video‐enabled double‐lumen endotracheal tube improves the safety of therapeutic whole lung lavage in autoimmune pulmonary alveolar proteinosis.
Kevin Davidson
doaj +2 more sources
Whole Lung Lavage in Autoimmune Pulmonary Alveolar Proteinosis: Unique Challenges in a Resource‐Limited Setting [PDF]
Autoimmune pulmonary alveolar proteinosis (PAP) is characterized by antibodies to granulocyte–macrophage colony‐stimulating factor (GM‐CSF), alveolar macrophage dysfunction, and surfactant accumulation.
Ashesh Dhungana +6 more
doaj +2 more sources
Background D40LG-associated X-linked hyper-IgM syndrome with pulmonary alveolar proteinosis has rarely been reported, and its genotype-phenotypic correlation remains elusive. Case presentation We describe a five-month-old boy with CD40LG mutation (c.516T
Hong-bo Xu +5 more
doaj +1 more source
Background Pulmonary alveolar proteinosis is a rare interstitial lung disease characterized by accumulating surfactant materials in the alveoli. The autoimmune form is by far the most common in adults, while in the pediatric age group, the vast majority ...
Abdalla Mohmed Alasiri +5 more
doaj +1 more source
Pulmonary Alveolar Proteinosis: Case Report
Pulmonary alveolar proteinosis is a rare disease of the lungs due to abnormal surfactant metabolism with accumulation of pathological protein lipid substance in the lumen of alveoli.Presented case of idiopathic alveolar proteinosis is characterized by ...
N. A. Karoli +2 more
doaj +1 more source
Interstitial lung disease as a late complication of pulmonary alveolar proteinosis
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]
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
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
Mavrilimumab, a fully human granulocyte-macrophage colony-stimulating factor receptor α monoclonal antibody: long-term safety and efficacy in patients with rheumatoid arthritis [PDF]
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

