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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 …
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 - 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 …
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. ...
Pulmonary alveolar proteinosis (PAP) is categorized into hereditary, secondary and autoimmune PAP (aPAP) types. …
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. ...In hereditary PAP, the rupture of GM-CSF signaling i …
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 …
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 …
Fanconi anemia and beta c deficiency-associated pulmonary alveolar proteinosis as two hereditary diseases of childhood which are potentially curable by stem cell gene therapy but require different therapeutic approaches.
Dirksen U, Moritz T, Burdach S, Flasshove M, Hanenberg H. Dirksen U, et al. Klin Padiatr. 1999 Jul-Aug;211(4):329-35. doi: 10.1055/s-2008-1043810. Klin Padiatr. 1999. PMID: 10472572 Review.
Fanconi anemia (FANC) and pulmonary alveolar proteinosis associated with deficiency of the beta-chain common to the GM-CSF/IL3/IL5 receptors (beta c-PAP) are rare inherited disorders of childhood or adolescence. Hematopoietic stem cell gene therapy aiming at …
Fanconi anemia (FANC) and pulmonary alveolar proteinosis associated with deficiency of the beta-chain common to the GM- …
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 …
[Clinical, biological and genetic heterogeneity of the inborn errors of pulmonary surfactant metabolism: SP-B deficiency and alveolar proteinosis].
Tredano M, Blic JD, Griese M, Fournet JC, Elion J, Bahuau M. Tredano M, et al. Ann Biol Clin (Paris). 2001 Mar-Apr;59(2):131-48. Ann Biol Clin (Paris). 2001. PMID: 11282516 Free article. Review. French.
Pulmonary surfactant is a multimolecular complex located at the air-water interface within the alveolus and to which a bulk of functions has been assigned, physical (surface-active properties) as well as immune or depurant. ...Beside hyaline membrane disease of the preterm …
Pulmonary surfactant is a multimolecular complex located at the air-water interface within the alveolus and to which a bulk of functi …
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