Articles with "alveolar proteinosis" as a keyword



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Protein estimation in whole lung lavage fluid in hereditary pulmonary alveolar proteinosis due to a novel GM‐CSF receptor mutation

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Published in 2022 at "Pediatric Pulmonology"

DOI: 10.1002/ppul.25932

Abstract: Pulmonary alveolar proteinosis (PAP) is associated with abnormal surfactant accumulation in alveoli, leading to respiratory failure if untreated. This article is protected by copyright. All rights reserved. read more here.

Keywords: estimation whole; pulmonary alveolar; whole lung; alveolar proteinosis ... See more keywords
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Pediatric Lung MRI in Pulmonary Alveolar Proteinosis: An Alternative to CT as a Radiation-Free Modality

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Published in 2022 at "Indian Journal of Pediatrics"

DOI: 10.1007/s12098-022-04089-0

Abstract: A 9-y-old girl presented with lethargy, fatigue, and progressive exertional dyspnea for the last two–three years, and rapid breathing for the past six months. Chest radiograph showed confluent air-space opacities in both lungs. Chest computed… read more here.

Keywords: lung; alveolar proteinosis; modality; pulmonary alveolar ... See more keywords
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Hematopoietic stem cell transplantation for pulmonary alveolar proteinosis associated with primary immunodeficiency disease

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Published in 2017 at "International Journal of Hematology"

DOI: 10.1007/s12185-017-2375-1

Abstract: Pulmonary alveolar proteinosis (PAP) is a rare disorder that is characterized by the excessive accumulation of surfactant-like materials in the alveoli, leading to hypoxemic respiratory failure. We describe two Japanese infants with PAP associated with… read more here.

Keywords: pulmonary alveolar; cell transplantation; primary immunodeficiency; stem cell ... See more keywords
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Autoimmune pulmonary alveolar proteinosis in an adolescent successfully treated with inhaled rhGM-CSF (molgramostim)

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Published in 2018 at "Respiratory Medicine Case Reports"

DOI: 10.1016/j.rmcr.2018.02.005

Abstract: Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare parenchymal lung disease characterized by accumulation of surfactant in the airways with high levels of granulocyte-macrophage colony stimulating factor (GM-CSF) antibodies in blood. Disease leads to hypoxemic… read more here.

Keywords: pulmonary alveolar; csf; alveolar proteinosis; successfully treated ... See more keywords
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The effectiveness of N-acetylcysteine for an adult case of pulmonary alveolar proteinosis: A case report

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Published in 2019 at "Respiratory Medicine Case Reports"

DOI: 10.1016/j.rmcr.2019.100883

Abstract: Introduction Pulmonary alveolar proteinosis is a rare disease that is characterized by accumulation of surfactant and phospholipids in the pulmonary alveoli. The current mainstay of treatment is whole lung lavage. There have been rare reports… read more here.

Keywords: pulmonary alveolar; effectiveness acetylcysteine; acetylcysteine adult; case ... See more keywords
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Exacerbation of autoimmune pulmonary alveolar proteinosis that improved with lone treatment of complicating nontuberculous mycobacterial infection: A case report

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Published in 2021 at "Respiratory Medicine Case Reports"

DOI: 10.1016/j.rmcr.2021.101521

Abstract: Herein, we present the case of a 63-year-old man with autoimmune pulmonary alveolar proteinosis (APAP) complicated by Mycobacterium avium complex (MAC) infection. APAP was diagnosed based on serum anti-granulocyte-macrophage colony-stimulating factor antibody, bronchoalveolar lavage fluid… read more here.

Keywords: pulmonary alveolar; case; alveolar proteinosis; autoimmune pulmonary ... See more keywords
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iPSC-Derived Macrophages Effectively Treat Pulmonary Alveolar Proteinosis in Csf2rb-Deficient Mice

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Published in 2018 at "Stem Cell Reports"

DOI: 10.1016/j.stemcr.2018.07.006

Abstract: Summary Induced pluripotent stem cell (iPSC)-derived hematopoietic cells represent a highly attractive source for cell and gene therapy. Given the longevity, plasticity, and self-renewal potential of distinct macrophage subpopulations, iPSC-derived macrophages (iPSC-Mφ) appear of particular… read more here.

Keywords: pulmonary alveolar; alveolar proteinosis; ipsc derived; derived macrophages ... See more keywords
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Statin as a novel pharmacotherapy of pulmonary alveolar proteinosis

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Published in 2018 at "Nature Communications"

DOI: 10.1038/s41467-018-05491-z

Abstract: 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. Here, we examine the… read more here.

Keywords: pulmonary alveolar; alveolar macrophages; statin; alveolar proteinosis ... See more keywords
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Pulmonary alveolar proteinosis

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Published in 2019 at "Nature Reviews Disease Primers"

DOI: 10.1038/s41572-019-0066-3

Abstract: Pulmonary alveolar proteinosis (PAP) is a syndrome characterized by the accumulation of alveolar surfactant and dysfunction of alveolar macrophages. PAP results in progressive dyspnoea of insidious onset, hypoxaemic respiratory failure, secondary infections and pulmonary fibrosis.… read more here.

Keywords: pulmonary alveolar; alveolar macrophages; alveolar surfactant; alveolar proteinosis ... See more keywords
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135 Downregulation of ABCG1 and ABCG4 Transporters by rare Earth Oxide Nanoparticles Induces the Pulmonary Alveolar Proteinosis

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Published in 2023 at "Annals of Work Exposures and Health"

DOI: 10.1093/annweh/wxac087.172

Abstract: Pulmonary alveolar proteinosis (PAP) is rare disease that accumulates alveolar surfactant. PAP can be induced by nanoparticles (NPs), but there is little information about PAP-producing NPs and their mechanism. Here, we evaluated PAP-producing NPs including… read more here.

Keywords: pulmonary alveolar; abcg1 abcg4; alveolar proteinosis; rare earth ... See more keywords
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Serum YKL‐40 is a reliable biomarker for pulmonary alveolar proteinosis

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Published in 2017 at "Respirology"

DOI: 10.1111/resp.13082

Abstract: Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by alveolar filling. YKL‐40, a chitinase‐like protein produced by macrophages and epithelial cells, is increased in patients with interstitial lung diseases. We aimed to evaluate the… read more here.

Keywords: pulmonary alveolar; alveolar proteinosis; biomarker; serum ykl ... See more keywords