Results 41 to 50 of about 61,025 (285)

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

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.
Mani S. Kavuru, Octavian C. Ioachimescu
openaire   +2 more sources

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

Methionine supplementation for multi-organ dysfunction in MetRS-related pulmonary alveolar proteinosis

European Respiratory Journal, 2021
Introduction Pulmonary alveolar proteinosis related to mutations in the methionine tRNA synthetase (MARS1) gene is a severe, early-onset disease that results in death before the age of 2 years in one-third of patients.
A. Hadchouel, D. Drummond, C. Pontoizeau, L. Aoust, Maria Hurtado Nedelec, J. El Benna, E. Gachelin, C. Perisson, C. Vigier, M. Schiff, F. Lacaille, Thierry Molina, L. Berteloot, S. Renolleau, C. Ottolenghi, J. Treluyer, J. de Blic, C. Delacourt   +17 more
semanticscholar   +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

Pulmonary alveolar proteinosis due to heterozygous mutation in OAS1: Whole lung lavages for long‐term bridging to hematopoietic stem cell transplantation

Pediatric Pulmonology, 2021
Pulmonary alveolar proteinosis (PAP) is defined by increased accumulation of surfactant in the alveolar space. PAP has been reported to be associated with a large number of clinical conditions and diseases.
E. Seidl, D. Schramm, C. Schön, K. Reiter, I. Pawlita, M. Kappler, S. Reu‐Hofer, F. Hauck, Michael H. Albert, M. Griese   +9 more
semanticscholar   +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