The DOI matches its Crossref record; 7 of the 7 references it cites were found in a registry or an index.
Pulmonary Alveolar Proteinosis: Progress in the First 44 Years
Abstract
Pulmonary alveolar proteinosis is a rare clinical syndrome that was first described in 1958. Subsequently, over 240 case reports and small series have described at least 410 cases in the literature. Characterized by the alveolar accumulation of surfactant components with minimal interstitial inflammation or fibrosis, pulmonary alveolar proteinosis has a variable clinical course ranging from spontaneous resolution to death with pneumonia or respiratory failure. The most effective proven treatment--whole lung lavage--was described soon after the first recognition of this disease. In the last 8 years, there has been rapid progress toward elucidation of the molecular mechanisms underlying both the congenital and acquired forms of pulmonary alveolar proteinosis, following serendipitous discoveries in gene-targeted mice lacking granulocyte-macrophage colony-stimulating factor (GM-CSF). Impairment of surfactant clearance by alveolar macrophages as a result of inhibition of the action of GM-CSF by blocking autoantibodies may underlie many acquired cases, whereas congenital disease is most commonly attributable to mutations in surfactant protein genes but may also be caused by GM-CSF receptor defects. Therapy with GM-CSF has shown promise in approximately half of those acquired cases treated, but it is unsuccessful in congenital forms of the disease, consistent with the known differences in disease pathogenesis.
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Citations
Cites
Regulation of B lymphocyte and macrophage development by graded expression of PU.1
DeKoter, R. P., & Singh, H. (2000) · Science · doi:10.1126/science.288.5470.1439 · Cited by 749
Transcription Factors, Normal Myeloid Development, and Leukemia
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Hematopoietic lineage- and stage-restricted expression of the ETS oncogene family member PU.1
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Neutrophils and monocytes express high levels of PU.1 (Spi-1) but not Spi-B
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GM-CSF Regulates Bleomycin-Induced Pulmonary Fibrosis Via a Prostaglandin-Dependent Mechanism
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The transcription factor PU.1 is involved in macrophage proliferation.
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Cited by
Inoue, Y., Trapnell, B. C., Tazawa, R., Arai, T., Takada, T., Hizawa, N., Kasahara, Y., Tatsumi, K., Hojo, M., Ichiwata, T., et al. (2008) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200708-1271oc · Cited by 490
Schneider, C., Nobs, S. P., Heer, A. K., Kurrer, M., Klinke, G., van Rooijen, N., Vogel, J., & Kopf, M. (2014) · PLoS Pathogens · doi:10.1371/journal.ppat.1004053 · Cited by 334
Sinclair, C., Bommakanti, G., Gardinassi, L., Loebbermann, J., Johnson, M. J., Hakimpour, P., Hagan, T., Benitez, L., Todor, A., Machiah, D., et al. (2017) · Science · doi:10.1126/science.aaj2155 · Cited by 126
Distinct Responses of Human Monocyte Subsets to Aspergillus fumigatus Conidia
Serbina, N. V., Cherny, M., Shi, C., Bleau, S. A., Collins, N. H., Young, J. W., & Pamer, E. G. (2009) · The Journal of Immunology · doi:10.4049/jimmunol.0803398 · Cited by 70
Souza, C. A., Marchiori, E., Gonçalves, L. P., Meirelles, G. D. S. P., Zanetti, G., Escuissato, D. L., Capobianco, J., & Souza, A. S. (2012) · European Journal of Radiology · doi:10.1016/j.ejrad.2010.12.012 · Cited by 30
Metadata
- Authors
- Seymour, J. F., & Presneill, J. J.
- Venue
- American Journal of Respiratory and Critical Care Medicine
- Publisher
- Oxford University Press (OUP)
- Published
- 2002-07-15
- Volume
- 166
- Issue
- 2
- Pages
- 215-235
- ISSN
- 1073-449X (ISSN-L)
- 1535-4970
- OpenAlex
- W2108046487
- Cited by
- 697
- References
- 7
- FWCI
- 15.65
- Type
- journal article
- Language
- en
- Open access
- closed
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- CoChat. (2026). Citation check: Pulmonary Alveolar Proteinosis: Progress in the First 44 Years. https://app.cochat.ai/papers/10.1164/rccm.2109105
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- Seymour, J. F., & Presneill, J. J. (2002). Pulmonary Alveolar Proteinosis: Progress in the First 44 Years. American Journal of Respiratory and Critical Care Medicine. https://doi.org/10.1164/rccm.2109105
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Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
