| Level of fetal hemoglobin in children with sickle cell anemia: influence of gender, haplotype and alpha-thalassemia-2 trait. | |
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MedLine Citation:
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PMID: 7694436 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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We have evaluated hematological data for numerous blood samples collected from 51 sickle cell anemia (SS) patients during their first 10 years of life. Haplotypes and alpha-globin gene status were determined in all patients. A total of 482 hematological evaluations and 317 fetal hemoglobin (Hb F) determinations are presented. The data show that the Hb F levels are the highest in patients with haplotypes 3/3 and the lowest in those with haplotypes 20/20. This is reflected in differences in total hemoglobin, PCV value, and RBC counts, which are the lowest in the 20/20 group and the highest in the 3/3 group. Female SS patients with haplotypes 19/19 have higher Hb F levels than their male counterparts; the same was observed for the patients with the 19/3 haplotype combination but not for those with the 20/3 haplotype combination. A concomitant alpha-thalassemia-2 heterozygosity had little or no effect on the hemoglobin level. The data confirm that SS children, aged 1-10 years, with a homozygosity for haplotype No. 20 are most severely affected by their disease. |
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Authors:
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A D Adekile; T H Huisman |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
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Title: Acta haematologica Volume: 90 ISSN: 0001-5792 ISO Abbreviation: Acta Haematol. Publication Date: 1993 |
Date Detail:
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Created Date: 1993-12-15 Completed Date: 1993-12-15 Revised Date: 2007-11-14 |
Medline Journal Info:
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Nlm Unique ID: 0141053 Medline TA: Acta Haematol Country: SWITZERLAND |
Other Details:
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Languages: eng Pagination: 34-8 Citation Subset: IM |
Affiliation:
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Department of Biochemistry and Molecular Biology, Medical College of Georgia, Augusta 30912-2100. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Anemia, Sickle Cell
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blood*,
genetics Child Child, Preschool Female Fetal Hemoglobin / analysis* Globins / genetics Haplotypes* Heterozygote Homozygote Humans Infant Male Sex Characteristics* alpha-Thalassemia / blood*, genetics |
| Grant Support | |
ID/Acronym/Agency:
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HLB-41544/HL/NHLBI NIH HHS |
| Chemical | |
Reg. No./Substance:
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9004-22-2/Globins; 9034-63-3/Fetal Hemoglobin |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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