Results 21 to 30 of about 9,034 (208)

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, John Kosiuk, MD   +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, Crouch E, deMello DE, Dirksen U, Doyle IR, Fisher JH, Floros J, Glasser SW, Green D, Hawgood S, Ikegami M, Ikegami M, Ikegami M, Kitamura T, Kresch MJ, Madan T, Mahut B, Mason RJ, Myrianthopoulos NC, Nogee LM, Pilot-Matias TJ, Power JHT, Prakash UBS, Reed JA, Robertson B, Rosen SH, Stoll BJ, Tredano M, Vorbroker DK, Wang J-Y   +29 more
core   +1 more source

A newly identified novel variant in the CSF2RA gene in a child with pulmonary alveolar proteinosis: a case report

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, Wadha Alotaibi, Sami A. Alhaider, Suhail Al-Saleh   +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]

, 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, Akagawa, Aletaha, Aletaha, Antoniu, Avci, Berghen, Borg, Borie, Brown, Buch, Burmester, Burmester, Calisto, Cella, Chen, Cook, Cornish, Cottin, Crotti, Dawson, Felson, Fleetwood, Fries, Greven, Guo, Hamilton, Jaguin, Kelly, Khan, Lazaro, Listing, Minter, Prevoo, Shaw, Shibata, Singh, Tsuchiya, Van der Heijde, Van Gestel, Van Onna, Ware, Weinblatt, Yoshida, Zhan   +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, Tetikkurt Seza, Ozdemir Imran, Zuhur Cigdem, Bayar Nihal   +4 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

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, Hershfield, Michael S, Kohn, Donald B, Komarow, Hirsh D, Metcalfe, Dean D, Sokolic, Robert, Young, Michael   +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, WLL International Study Group   +1 more
core   +1 more source

Alveolar macrophages of GM-CSF knockout mice exhibit mixed M1 and M2 phenotypes [PDF]

, 2013
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, Dalrymple, Heidi, Kavuru, Mani S, Malur, Achut G, Malur, Anagha, NC DOCKS at East Carolina University, Thomassen, Mary Jane   +6 more
core   +1 more source