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Relationship of foetal haemoglobin levels and β s haplotypes in homozygous sickle cell disease
Author(s) -
Falusi A. G.,
Kulozik A. E.
Publication year - 1990
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/j.1600-0609.1990.tb00405.x
Subject(s) - haplotype , fetal hemoglobin , biology , genetics , sickle cell anemia , population , disease , cell , globin , immunology , gene , genotype , medicine , pregnancy , fetus , environmental health
The foetal haemoglobin (HbF) levels and the haplotypes of β s chromosomes in sickle cell anaemia patients in Nigeria were evaluated. The mean HbF level was 5.9 ± 3.8% with a range of 0.9–16.7%. 80% of the patients had HbF values below 8% and 94% had HbF levels below 10%. No significant difference in haematological parameters was seen between those with less than 2% HbF and those with greater than 8% HbF. The presence (+) or absence (−) of eight restriction endonuclease enzyme sites within the β s globin gene cluster (haplotype) on chromosome 11 were mapped. The common haplotype (− − − − + + − +) in 97% of the chromosomes examined closely correlates with the low levels of foetal haemoglobin generally observed in sickle cell patients in the same population.