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
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
Impulse oscillometry identifies peripheral airway dysfunction in children with adenosine deaminase deficiency. [PDF]
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]
Maija Halme on työryhmän WLL International Study Group jäsen.Peer ...
Halme, Maija +1 more
core +1 more source
Alveolar macrophages develop from fetal monocytes that differentiate into long-lived cells in the first week of life via GM-CSF [PDF]
Tissue-resident macrophages can develop from circulating adult monocytes or from primitive yolk sac-derived macrophages. The precise ontogeny of alveolar macrophages (AMFs) is unknown. By performing BrdU labeling and parabiosis experiments in adult mice,
De Kleer, Ismé +9 more
core +2 more sources
Alveolar macrophages of GM-CSF knockout mice exhibit mixed M1 and M2 phenotypes [PDF]
Background Activin A is a pleiotrophic regulatory cytokine, the ablation of which is neonatal lethal. Healthy human alveolar macrophages (AMs) constitutively express activin A, but AMs of patients with pulmonary alveolar proteinosis (PAP) are ...
Barna, Barbara P +6 more
core +1 more source
Human Inborn Errors of Immunity: 2019 Update of the IUIS Phenotypical Classification. [PDF]
Since 2013, the International Union of Immunological Societies (IUIS) expert committee (EC) on Inborn Errors of Immunity (IEI) has published an updated phenotypic classification of IEI, which accompanies and complements their genotypic classification ...
Ailal, Fatima +18 more
core
Proceedings: Regenerative Medicine for Lung Diseases: A CIRM Workshop Report. [PDF]
The mission of the California Institute of Regenerative Medicine (CIRM) is to accelerate treatments to patients with unmet medical needs. In September 2016, CIRM sponsored a workshop held at the University of California, Los Angeles, to discuss ...
DeWitt, Natalie D +2 more
core +1 more source
Highlights of the Brazilian Thoracic Association guidelines for interstitial lung diseases [PDF]
Interstitial lung diseases (ILDs) are heterogeneous disorders, involving a large number of conditions, the approach to which continues to pose an enormous challenge for pulmonologists.
Aidé, Miguel Abidon +24 more
core +3 more sources
Autoimmune hyperphosphatemic tumoral calcinosis in a patient with FGF23 autoantibodies [PDF]
Hyperphosphatemic familial tumoral calcinosis (HFTC)/hyperostosis-hyperphosphatemia syndrome (HHS) is an autosomal recessive disorder of ectopic calcification due to deficiency of or resistance to intact fibroblast growth factor 23 (iFGF23). Inactivating
Burbelo +14 more
core +1 more source

