|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
We discuss the classification of PAP, the current diagnostic practice and the supplementary role of genetic testing and granulocyte-macrophage colony-stimulating factor (GM-CSF) signalling in the diagnosis of congenital and hereditary PAP.
|
31365379 |
2019 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
This study aimed to investigate the clinical efficacy of GM-CSF inhalation combined with WLL in Chinese patients with PAP.
|
31513019 |
2019 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
PAP can be classified into different types on the basis of the pathogenetic mechanism: primary PAP is characterized by the disruption of granulocyte-macrophage colony-stimulating factor (GM-CSF) signalling and can be autoimmune (caused by elevated levels of GM-CSF autoantibodies) or hereditary (due to mutations in CSF2RA or CSF2RB, encoding GM-CSF receptor subunits); secondary PAP results from various underlying conditions; and congenital PAP is caused by mutations in genes involved in surfactant production.
|
30846703 |
2019 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
<b>Rationale:</b> Pulmonary alveolar proteinosis (PAP) is characterized by filling of the alveolar spaces by lipoprotein-rich material of ill-defined composition, and is caused by molecularly different and often rare diseases that occur from birth to old age.<b>Objectives:</b> To perform a quantitative lipidomic analysis of lipids and the surfactant proteins A, B, and C in lavage fluids from patients with proteinosis of different causes in comparison with healthy control subjects.<b>Methods:</b> During the last two decades, we have collected BAL samples from patients with PAP due to autoantibodies against granulocyte-macrophage colony-stimulating factor; genetic mutations in CSF2RA (colony-stimulating factor 2 receptor α-subunit), MARS (methionyl aminoacyl-tRNA synthetase), FARSB (phenylalanine-tRNA synthetase, β-subunit), and NPC2 (Niemann-Pick disease type C2); and secondary to myeloid leukemia.
|
31002528 |
2019 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
The levels of HDL-C and DLCO significantly increased while carcinoembryonic antigen (CEA), CYFRA21-1, neuron-specific enolase (NSE), and lactic dehydrogenase (LDH) levels decreased six months after granulocyte-macrophage colony-stimulating factor (GM-CSF) inhalation therapy between 14 patients with PAP.
|
31827650 |
2019 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Granulocyte macrophage colony-stimulating factor (GM-CSF) plays a role in the pathophysiology of PAP.
|
30866900 |
2019 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Evidence is required regarding treatment with exogenous anti-granulocyte/macrophage colony-stimulating factor.Here, we present a 13-year-old male patient with hereditary PAP and a 15-year-old female patient with autoimmune PAP who presented with complaints of easy fatigability and weakness to emphasize the importance of keeping in mind PAP as a differential diagnosis in patients with respiratory failure findings.
|
30113436 |
2018 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Pulmonary alveolar proteinosis (PAP) is a syndrome of reduced GM-CSF-dependent, macrophage-mediated surfactant clearance, dysfunctional foamy alveolar macrophages, alveolar surfactant accumulation, and hypoxemic respiratory failure for which the pathogenetic mechanism is unknown.
|
30087322 |
2018 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
In fact, blockade of GM-CSF-initiated signaling or deletion of the PPARγ-encoding gene <i>PPARG</i> leads to functionally defective A-MØ and the onset of pulmonary alveolar proteinosis.
|
29434585 |
2018 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Understanding the mechanism of action for neutralizing antibodies that target GM-CSF is important for improving their specificity and affinity as therapeutics and, conversely, in devising strategies to reduce the effects of GM-CSF auto-antibodies in PAP.
|
29969365 |
2018 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Impaired GM-CSF bioavailability due to anti-GM-CSF autoimmunity is the cause of approximately 90% of adult PAP cases.
|
29397349 |
2018 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease in which the abnormalities in alveolar surfactant accumulation are caused by impairments of GM-CSF pathway attributing to defects in a variety of genes.
|
28233860 |
2017 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Disseminated Cryptococcosis Due to Anti-Granulocyte-Macrophage Colony-Stimulating Factor Autoantibodies in the Absence of Pulmonary Alveolar Proteinosis.
|
28013480 |
2017 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Using static and dynamic lipidomics techniques, we compared (1) tissue PC compositions and contents, and (2) in lungs, the absolute rates of synthesis in both control mice and the granulocyte-macrophage colony-stimulating factor knockout model of PAP.
|
28489415 |
2017 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
Abnormalities in CSF2 receptor alpha (CSF2RA) were reported to cause pediatric hereditary PAP.
|
28212655 |
2017 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
CT scans were obtained within 1 month before and after therapy from 32 patients with PAP who participated in a multicenter phase II trial of granulocyte-macrophage colony-stimulating factor inhalation therapy.
|
28489417 |
2017 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Sequential Granulocyte-Macrophage Colony-Stimulating Factor Inhalation after Whole-Lung Lavage for Pulmonary Alveolar Proteinosis. A Report of Five Intractable Cases.
|
28421817 |
2017 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
To determine mechanisms by which granulocyte/macrophage-colony stimulating factor (GM-CSF) signaling normally maintains surfactant homeostasis and how its disruption causes pulmonary alveolar proteinosis (PAP), we evaluated lipid composition in alveolar macrophages and lung surfactant, macrophage-mediated surfactant clearance kinetics/dynamics, and cholesterol-targeted pharmacotherapy of PAP in vitro and in vivo.
|
28860566 |
2017 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Neutralization and clearance of GM-CSF by autoantibodies in pulmonary alveolar proteinosis.
|
26077231 |
2015 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Of 21 patients with GM-CSF-autoantibody negative PAP, 13 had no other organ involvement and 8 had some form of hematologic disorder.The latter were sequenced for GATA2.
|
26264606 |
2015 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Restoration of GM-CSF receptor signaling corrected the surfactant clearance abnormality in hPAP-iPS-Mφs.
|
24279752 |
2014 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Both human PAP and mouse GM-CSF knockout AMs are deficient in the transcription factor, peroxisome proliferator activated receptor gamma (PPARγ).
|
24044676 |
2013 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Disruption of granulocyte/macrophage colony-stimulating factor (GM-CSF) signalling causes pulmonary alveolar proteinosis (PAP).
|
21075760 |
2011 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Pulmonary alveolar proteinosis (PAP) is a lung disease characterized by a deficiency of functional granulocyte macrophage colony-stimulating factor (GM-CSF) resulting in surfactant accumulation and lipid-engorged alveolar macrophages.
|
21036914 |
2011 |
|
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
In particular, hIL-3/GM-CSF KI mice supported the development of human alveolar macrophages that partially rescued the pulmonary alveolar proteinosis syndrome.
|
21262803 |
2011 |