Results 31 to 40 of about 61,025 (285)

Pulmonary alveolar proteinosis

, 2010
Pulmonary alveolar proteinosis is a rare disease characterized by an accumulation of surfactant-like within the alveoli. Three forms are recognized: (1) primary or idiopathic, which is the most common; (2) secondary to immunodeficiency syndromes, hematologic malignancies, especially myeloid, and toxic exposure; and (3) a rare disease considered as ...
Liz Silverstone, Yuranga Weerakkody
openalex   +3 more sources

Assessment of Statin Treatment for Pulmonary Alveolar Proteinosis without Hypercholesterolemia: A 12-Month Prospective, Longitudinal, and Observational Study

BioMed Research International, 2022
Background Pulmonary alveolar proteinosis (PAP) is a rare disorder which is characterized by the accumulation of excessive surfactant lipids and proteins in alveolar macrophages and alveoli. Oral statin therapy has been reported to be a novel therapy for
Shenyun Shi, X. Gui, Jingjing Ding, Shangwen Yang, Xin Xin, Kaifeng Xu, Yonglong Xiao   +6 more
semanticscholar   +1 more source

Pathogenesis-driven treatment of primary pulmonary alveolar proteinosis. [PDF]

Eur Respir Rev
Pulmonary alveolar proteinosis (PAP) is a syndrome that results from the accumulation of lipoproteinaceous material in the alveolar space. According to the underlying pathogenetic mechanisms, three different forms have been identified, namely primary ...
Lettieri S, Bonella F, Marando VA, Franciosi AN, Corsico AG, Campo I.   +5 more
europepmc   +2 more sources

Neutralizing GM-CSF autoantibodies in pulmonary alveolar proteinosis, cryptococcal meningitis and severe nocardiosis

Respiratory Research, 2022
Background Anti GM-CSF autoantibodies (aAb) have been related to acquired pulmonary alveolar proteinosis (PAP) and described in cases of severe infections such as cryptococcosis and nocardiosis in previously healthy subjects.
H. Salvator, Aristine Cheng, Lindsey B. Rosen, P. Williamson, J. Bennett, Anuj K. Kashyap, L. Ding, K. Kwon-Chung, H. Namkoong, C. Zerbe, S. Holland   +10 more
semanticscholar   +1 more source

Autoimmune Pulmonary Alveolar Proteinosis.

American Journal of Respiratory and Critical Care Medicine, 2022
Autoimmune pulmonary alveolar proteinosis is a rare disease characterized by myeloid cell dysfunction, abnormal pulmonary surfactant accumulation, and innate immune deficiency.
Cormac McCarthy, B. Carey, B. Trapnell
semanticscholar   +1 more source

Allogeneic Hematopoietic Stem Cell Transplantation After Prior Lung Transplantation for Hereditary Pulmonary Alveolar Proteinosis: A Case Report

Frontiers in Immunology, 2022
Pulmonary alveolar proteinosis (PAP) is a rare, diffuse lung disorder characterized by surfactant accumulation in the small airways due to defective clearance by alveolar macrophages, resulting in impaired gas exchange.
H. Beeckmans, G. Ambrocio, S. Bos, A. Vermaut, V. Geudens, A. Vanstapel, B. Vanaudenaerde, F. De Baets, T. Malfait, M. Emonds, D. Van raemdonck, H. Schoemans, R. Vos   +12 more
semanticscholar   +1 more source

Pulmonary Alveolar Proteinosis and Multiple Infectious Diseases in a Child with Autosomal Recessive Complete IRF8 Deficiency

Journal of Clinical Immunology, 2022
Autosomal recessive (AR) complete IRF8 deficiency is a rare severe inborn error of immunity underlying an absence of blood myeloid mononuclear cells, intracerebral calcifications, and multiple infections. Only three unrelated patients have been reported.
J. Rosain, A. Bernasconi, E. Prieto, Lucía Caputi, Tom Le Voyer, Guadalupe Buda, M. Martí, J. Bohlen, Anna-Lena Neehus, C. Castaños, Rosario Gallagher, K. Dorgham, M. Oleastro, Laura Perez, S. Danielián, J. Dipierri, J. Casanova, J. Bustamante, M. Villa   +18 more
semanticscholar   +1 more source

A Murine Model of Hereditary Pulmonary Alveolar Proteinosis Caused by Homozygous Csf2ra Gene Disruption.

American Journal of Physiology - Lung cellular and Molecular Physiology, 2022
Hereditary pulmonary alveolar proteinosis (hPAP) is a rare disorder caused by recessive mutations in GM-CSF receptor subunit a/b genes (CSF2RA/CSF2RB, respectively) characterized by impaired GM-CSF dependent surfactant clearance by alveolar macrophages ...
K. Shima, P. Arumugam, Anthony Sallese, Yuko Horia, Yan Ma, C. Trapnell, M. Wessendarp, C. Chalk, Cormac McCarthy, B. Carey, B. Trapnell, Takuji Suzuki   +11 more
semanticscholar   +1 more source

Pulmonary Alveolar Proteinosis: Case Report

Архивъ внутренней медицины, 2020
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, E. E. Arhangelskaja, O. T. Zarmanbetova   +2 more
doaj   +1 more source

The Role of GM-CSF Autoantibodies in Infection and Autoimmune Pulmonary Alveolar Proteinosis: A Concise Review

Frontiers in Immunology, 2021
Autoantibodies to multiple cytokines have been identified and some, including antibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF), have been associated with increased susceptibility to infection.
A. Ataya, Vijaya Knight, B. Carey, Elinor Lee, E. Tarling, Tisha Wang   +5 more
semanticscholar   +1 more source