The DOI matches its Crossref record; 62 of the 62 references it cites were found in a registry or an index.
Rare diseases bullet 6: Pulmonary alveolar proteinosis: clinical aspects and current concepts on pathogenesis
Open accessAbstract
Pulmonary alveolar proteinosis was first described by Rosen et al in 1958. 1 It is an unusual diVuse lung disease characterised by the accumulation of large amounts of a phospholipoproteinaceous material in the alveoli.It has a variable clinical presentation and course.Most cases are primary but occasionally the condition is secondary to other conditions or inhalation of chemicals.Whole lung lavage remains the most eVective treatment and the overall prognosis is good.Surfactant homeostasis is abnormal and animal experiments suggest that this may relate, in some instances at least, to defects in GM-CSF signalling.There are at least two congenital forms of the disease and several diVerent animal models suggesting that pulmonary alveolar proteinosis is unlikely to be a single disease entity and more likely to represent a clinical syndrome. EpidemiologyPulmonary alveolar proteinosis is a rare lung disease and accurate estimates of incidence are not available.Current estimates suggest an incidence of one in two million people.The series reported in the literature suggest a male preponderance (male:female ratio 3:1).[1][2][3][4][5][6][7][8][9][10][11] Peak onset is in the third or fourth decade of life with over 80% of reported cases occurring in this age group.[1][2][3][4][5][6][7][8][9] However, there are reports of the disease occurring in neonates, 12 children, [13][14][15] and the elderly.
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Cites
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Report of a case and review of the literature
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Macrophage deactivation by interleukin 10.
Bogdan, C., Vodovotz, Y., & Nathan, C. (1991) · The Journal of experimental medicine · doi:10.1084/jem.174.6.1549 · Cited by 1,238
Pulmonary Alveolar Proteinosis
Rosen, S. H., Castleman, B., Liebow, A. A., Enzinger, F. M., & Hunt, R. T. N. (1958) · New England Journal of Medicine · doi:10.1056/nejm195806052582301 · Cited by 1,150
Stanley, E., Lieschke, G. J., Grail, D., Metcalf, D., Hodgson, G., Gall, J. A., Maher, D. W., Cebon, J., Sinickas, V, & Dunn, A. R. (1994) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.91.12.5592 · Cited by 882
Involvement of Granulocyte-Macrophage Colony-Stimulating Factor in Pulmonary Homeostasis
Dranoff, G., Crawford, A. D., Sadelain, M., Ream, B., Rashid, A., Bronson, R. T., Dickersin, G. R., Bachurski, C. J., Mark, E. L., Whitsett, J. A., et al. (1994) · Science · doi:10.1126/science.8171324 · Cited by 879
Deficiency of Pulmonary Surfactant Protein B in Congenital Alveolar Proteinosis
Nogee, L. M., deMello, D. E., Dehner, L. P., & Colten, H. R. (1993) · New England Journal of Medicine · doi:10.1056/nejm199302113280606 · Cited by 627
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Bogdan, C., Paik, J., Vodovotz, Y., & Nathan, C. (1992) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(18)50091-0 · Cited by 512
Altered surfactant function and structure in SP-A gene targeted mice.
Korfhagen, T. R., Bruno, M. D., Ross, G. F., Huelsman, K. M., Ikegami, M., Jobe, A. H., Wert, S. E., Stripp, B. R., Morris, R. E., Glasser, S. W., et al. (1996) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.93.18.9594 · Cited by 405
Cited by
Pulmonary Alveolar Proteinosis: Progress in the First 44 Years
Seymour, J. F., & Presneill, J. J. (2002) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.2109105 · Cited by 697
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
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
Shimizu, Y., Matsuzaki, S., Dobashi, K., Yanagitani, N., Satoh, T., Koka, M., Yokoyama, A., Ohkubo, T., Ishii, Y., Kamiya, T., et al. (2011) · Respiratory Research · doi:10.1186/1465-9921-12-88 · Cited by 18
Metadata
- Authors
- Shah, P. L.
- Venue
- Thorax
- Publisher
- BMJ
- Published
- 2000-01-01
- Volume
- 55
- Issue
- 1
- Pages
- 67-77
- ISSN
- 0040-6376 (ISSN-L)
- OpenAlex
- W2138512930
- Cited by
- 263
- References
- 62
- FWCI
- 10.30
- Type
- journal article
- Language
- en
- Open access
- bronze
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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