Document Detail

Level of fetal hemoglobin in children with sickle cell anemia: influence of gender, haplotype and alpha-thalassemia-2 trait.
MedLine Citation:
PMID:  7694436     Owner:  NLM     Status:  MEDLINE    
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.
A D Adekile; T H Huisman
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Acta haematologica     Volume:  90     ISSN:  0001-5792     ISO Abbreviation:  Acta Haematol.     Publication Date:  1993  
Date Detail:
Created Date:  1993-12-15     Completed Date:  1993-12-15     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  0141053     Medline TA:  Acta Haematol     Country:  SWITZERLAND    
Other Details:
Languages:  eng     Pagination:  34-8     Citation Subset:  IM    
Department of Biochemistry and Molecular Biology, Medical College of Georgia, Augusta 30912-2100.
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MeSH Terms
Anemia, Sickle Cell / blood*,  genetics
Child, Preschool
Fetal Hemoglobin / analysis*
Globins / genetics
Sex Characteristics*
alpha-Thalassemia / blood*,  genetics
Grant Support
Reg. No./Substance:
9004-22-2/Globins; 9034-63-3/Fetal Hemoglobin

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