The DOI matches its Crossref record; 2 of the 2 references it cites were found in a registry or an index.
Concurrent Sickle-Cell Anemia and α-Thalassemia: Effect on Severity of Anemia
Abstract
We studied 47 patients with sickle-cell anemia to determine the effect of alpha-thalassemia on the severity of their hemolytic anemia. We diagnosed alpha-thalassemia objectively by using alpha-globin-gene mapping to detect alpha-globin-gene deletions, studying 25 subjects with the normal four alpha-globin-genes, 18 with three, and four with two. The mean hemoglobin, hematocrit, and absolute reticulocyte levels (+/- S.D.) were 7.9 +/- 0.9 g per deciliter (4.9 +/- 0.6 mmol per liter), 22.9 +/- 2.9 per cent, and 501,000 +/- 126,000 per cubic millimeter, respectively, in the non-thalassemic group; 9.8 +/- 1.6 g per deciliter (6.1 +/- 1.0 mmol per liter), 29.0 +/- 5.0 per cent, and 361,000 +/- 51,000 per cubic millimeter in the group with three alpha-globin genes; and 9.2 +/- 1.0 g per deciliter (5.7 +/- 0.6 mmol per liter), 27.5 +/- 3.0 per cent, and 100,000 +/- 15,000 per cubic millimeter in the group with two alpha-globin genes. Deletion of alpha-globin genes was also accompanied by a decreased mean corpuscular hemoglobin concentration (MCHC) in post-reticulocyte erythrocytes and by increased hemoglobin F levels. The decreased intraerythrocytic hemoglobin S concentration and elevated hemoglobin F levels associated with alpha-thalassemia appear to diminish the degree of hemolytic anemia found in sickle-cell disease.
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Cites
Natural History of Sickle Cell Anemia in Saudi Arabs: A Study of 270 Subjects
PERRINE, R. P., PEMBREY, M. E., JOHN, P., PERRINE, S., & SHOUP, F. (1978) · Annals of Internal Medicine · doi:10.7326/0003-4819-88-1-1 · Cited by 284
Individual variation in the production and survival of F cells in sickle-cell disease
Dover, G. J., Boyer, S. H., Charache, S., & Heintzelman, K. (1978) · New England Journal of Medicine · doi:10.1056/nejm197812282992603 · Cited by 144
Cited by
Kato, G. J., Gladwin, M. T., & Steinberg, M. H. (2007) · Blood Reviews · doi:10.1016/j.blre.2006.07.001 · Cited by 876
Molecular basis of biomaterial-mediated foreign body reactions
Hu, W.-J., Eaton, J. W., Ugarova, T. P., & Tang, L. (2001) · Blood · doi:10.1182/blood.v98.4.1231 · Cited by 431
Kato, G. J., Hebbel, R. P., Steinberg, M. H., & Gladwin, M. T. (2009) · American Journal of Hematology · doi:10.1002/ajh.21475 · Cited by 349
The Interaction of Alpha-Thalassemia and Homozygous Sickle-Cell Disease
Higgs, D. R., Aldridge, B. E., Lamb, J., Clegg, J. B., Weatherall, D. J., Hayes, R. J., Grandison, Y., Lowrie, Y., Mason, K. P., Serjeant, B. E., et al. (1982) · New England Journal of Medicine · doi:10.1056/nejm198206173062402 · Cited by 313
Brittenham, G., Schechter, A., & Noguchi, C. (1985) · Blood · doi:10.1182/blood.v65.1.183.183 · Cited by 197
Metadata
- Authors
- Embury, S. H., Dozy, A. M., Miller, J., Davis, J. R., Kleman, K. M., Preisler, H., Vichinsky, E., Lande, W. N., Lubin, B. H., Kan, Y. W., & Mentzer, W. C.
- Venue
- New England Journal of Medicine
- Publisher
- Massachusetts Medical Society
- Published
- 1982-02-04
- Volume
- 306
- Issue
- 5
- Pages
- 270-274
- ISSN
- 0028-4793 (ISSN-L)
- 1533-4406
- OpenAlex
- W2024571301
- Cited by
- 237
- References
- 2
- FWCI
- 9.30
- Type
- journal article
- Language
- en
- Open access
- closed
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- Embury, S. H., Dozy, A. M., Miller, J., Davis, J. R., Kleman, K. M., Preisler, H., Vichinsky, E., Lande, W. N., Lubin, B. H., Kan, Y. W., & Mentzer, W. C. (1982). Concurrent Sickle-Cell Anemia and α-Thalassemia: Effect on Severity of Anemia. New England Journal of Medicine. https://doi.org/10.1056/nejm198202043060504
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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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