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Pulmonary alveolar proteinosis.
Trapnell BC, Nakata K, Bonella F, Campo I, Griese M, Hamilton J, Wang T, Morgan C, Cottin V, McCarthy C. Trapnell BC, et al. Nat Rev Dis Primers. 2019 Mar 7;5(1):16. doi: 10.1038/s41572-019-0066-3. Nat Rev Dis Primers. 2019. PMID: 30846703 Review.
Pulmonary alveolar proteinosis (PAP) is a syndrome characterized by the accumulation of alveolar surfactant and dysfunction of alveolar macrophages. ...In most patients, pathogenesis is driven by reduced GM-CSF-dependent cholesterol clearance in …
Pulmonary alveolar proteinosis (PAP) is a syndrome characterized by the accumulation of alveolar surfactant and …
Pulmonary alveolar proteinosis - current and future therapeutical strategies.
Jehn LB, Bonella F. Jehn LB, et al. Curr Opin Pulm Med. 2023 Sep 1;29(5):465-474. doi: 10.1097/MCP.0000000000000982. Epub 2023 Jun 28. Curr Opin Pulm Med. 2023. PMID: 37395514 Review.
PURPOSE OF REVIEW: We discuss the most recent advances in the treatment of pulmonary alveolar proteinosis (PAP), an ultra-rare syndrome. RECENT FINDINGS: Whole lung lavage (WLL) remains the gold standard of treatment for PAP syndrome. ...SUMMARY: There are no …
PURPOSE OF REVIEW: We discuss the most recent advances in the treatment of pulmonary alveolar proteinosis (PAP), an ult …
Pharmacotherapy options in pulmonary alveolar proteinosis.
Antoniu SA, Rajnoveanu R, Grigore M, Antohe I. Antoniu SA, et al. Expert Opin Pharmacother. 2020 Aug;21(11):1359-1366. doi: 10.1080/14656566.2020.1757650. Epub 2020 Jun 8. Expert Opin Pharmacother. 2020. PMID: 32511020 Review.
INTRODUCTION: Pulmonary alveolar proteinosis (PAP) is a heterogeneous group of rare diseases characterized by the abnormal production and impaired degradation of pulmonary surfactant as a result of malfunctioning of alveolar macrophages. This is …
INTRODUCTION: Pulmonary alveolar proteinosis (PAP) is a heterogeneous group of rare diseases characterized by the abnor …
Pulmonary alveolar proteinosis: time to shift?
Papiris SA, Tsirigotis P, Kolilekas L, Papadaki G, Papaioannou AI, Triantafillidou C, Papaporfyriou A, Karakatsani A, Kagouridis K, Griese M, Manali ED. Papiris SA, et al. Expert Rev Respir Med. 2015 Jun;9(3):337-49. doi: 10.1586/17476348.2015.1035259. Epub 2015 Apr 12. Expert Rev Respir Med. 2015. PMID: 25864717 Review.
Pulmonary alveolar proteinosis (PAP) is categorized into hereditary, secondary and autoimmune PAP (aPAP) types. The common pathogenesis is the ability of the alveolar macrophages to catabolize phagocytized surfactant is affected. Hereditary …
Pulmonary alveolar proteinosis (PAP) is categorized into hereditary, secondary and autoimmune PAP (aPAP) types. …
[Lung involvement in autoinflammatory diseases].
Frémond ML, Berteloot L, Hadchouel A. Frémond ML, et al. Rev Mal Respir. 2024 Jan;41(1):18-28. doi: 10.1016/j.rmr.2023.10.009. Epub 2023 Nov 30. Rev Mal Respir. 2024. PMID: 38040588 Review. French.
Most patients with these diseases usually develop early progression to pulmonary fibrosis, which is responsible for high rates of morbidity and mortality. Other rare autoinflammatory diseases are associated with alveolar proteinosis, particularly when related …
Most patients with these diseases usually develop early progression to pulmonary fibrosis, which is responsible for high rates of mor …
The molecular basis of pulmonary alveolar proteinosis.
Carey B, Trapnell BC. Carey B, et al. Clin Immunol. 2010 May;135(2):223-35. doi: 10.1016/j.clim.2010.02.017. Epub 2010 Mar 25. Clin Immunol. 2010. PMID: 20338813 Free PMC article. Review.
Pulmonary alveolar proteinosis (PAP) comprises a heterogenous group of diseases characterized by abnormal surfactant accumulation resulting in respiratory insufficiency, and defects in alveolar macrophage- and neutrophil-mediated host defense. ...Secon …
Pulmonary alveolar proteinosis (PAP) comprises a heterogenous group of diseases characterized by abnormal surfactant ac …
Pathogenesis-driven treatment of primary pulmonary alveolar proteinosis.
Lettieri S, Bonella F, Marando VA, Franciosi AN, Corsico AG, Campo I. Lettieri S, et al. Eur Respir Rev. 2024 Aug 14;33(173):240064. doi: 10.1183/16000617.0064-2024. Print 2024 Jul. Eur Respir Rev. 2024. PMID: 39142709 Free PMC article. Review.
Pulmonary alveolar proteinosis (PAP) is a syndrome that results from the accumulation of lipoproteinaceous material in the alveolar space. ...Primary PAP is caused by disruption of granulocyte-macrophage colony-stimulating factor (GM-CSF) signalling du …
Pulmonary alveolar proteinosis (PAP) is a syndrome that results from the accumulation of lipoproteinaceous material in …
Immune dysregulation in the pathogenesis of pulmonary alveolar proteinosis.
Martinez-Moczygemba M, Huston DP. Martinez-Moczygemba M, et al. Curr Allergy Asthma Rep. 2010 Sep;10(5):320-5. doi: 10.1007/s11882-010-0134-y. Curr Allergy Asthma Rep. 2010. PMID: 20623372 Review.
Pulmonary alveolar proteinosis (PAP) is a rare disease of the lung characterized by the accumulation of surfactant-derived lipoproteins within pulmonary alveolar macrophages and alveoli, resulting in respiratory insufficiency and increased infec …
Pulmonary alveolar proteinosis (PAP) is a rare disease of the lung characterized by the accumulation of surfactant-deri …
The influence of genetics on therapeutic developments in pulmonary alveolar proteinosis.
Campo I. Campo I. Curr Opin Pulm Med. 2019 May;25(3):294-299. doi: 10.1097/MCP.0000000000000576. Curr Opin Pulm Med. 2019. PMID: 30865035 Review.
PURPOSE OF REVIEW: Pulmonary alveolar proteinosis (PAP) is characterized by the massive accumulation of lipoproteinaceous material within alveoli, which results in progressive respiratory failure. The abnormalities in surfactant clearance are caused by defect …
PURPOSE OF REVIEW: Pulmonary alveolar proteinosis (PAP) is characterized by the massive accumulation of lipoproteinaceo …
Shaping the future of an ultra-rare disease: unmet needs in the diagnosis and treatment of pulmonary alveolar proteinosis.
McCarthy C, Kokosi M, Bonella F. McCarthy C, et al. Curr Opin Pulm Med. 2019 Sep;25(5):450-458. doi: 10.1097/MCP.0000000000000601. Curr Opin Pulm Med. 2019. PMID: 31365379 Review.
PURPOSE OF REVIEW: Pulmonary alveolar proteinosis (PAP) can be considered the archetype of ultra-rare diseases with a prevalence of under 10 cases per million. We discuss the classification of PAP, the current diagnostic practice and the supplementary role of …
PURPOSE OF REVIEW: Pulmonary alveolar proteinosis (PAP) can be considered the archetype of ultra-rare diseases with a p …
14 results