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Hemoglobins S and C in Upper Volta.

D Labie, C Richin, J Pagnier

    Human Genetics
    |January 1, 1984
    PubMed
    Summary

    Hemoglobinopathies like sickle cell disease (HbS) and HbC are common in Upper Volta. Environmental factors significantly impact the survival rates of individuals with these genetic blood disorders.

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    Area of Science:

    • Hematology
    • Human Genetics
    • Population Health

    Background:

    • Hemoglobinopathies, including sickle cell disease (HbS) and hemoglobin C (HbC), are significant public health concerns.
    • Upper Volta (now Burkina Faso) presents a unique demographic for studying the prevalence and impact of these genetic blood disorders.

    Purpose of the Study:

    • To investigate the incidence and distribution of hemoglobinopathies (HbS, HbC, alpha-thalassemia) in Upper Volta.
    • To analyze the relationship between gene frequencies, geographic regions, and age.
    • To assess the survival impact of environmental and medical conditions on individuals with specific hemoglobinopathy genotypes.

    Main Methods:

    • Population-based study involving 1059 individuals in Upper Volta.
    • Analysis of hemoglobin variants (HbS, HbC) and alpha-thalassemia.
    • Comparison of gene frequencies across different geographic regions (Sahel vs. Savanna).
    • Age-specific analysis of genotype frequencies and survival.

    Main Results:

    • High frequencies of HbS and HbC, along with alpha-thalassemia, were observed.
    • HbS gene frequency was higher in the arid Sahel (0.1) compared to the humid Savanna (0.03).
    • HbC gene frequency was higher in the humid Savanna (0.14) compared to the arid Sahel (0.05).
    • Alpha-thalassemia frequency decreased significantly with age.
    • No sickle cell disease (SS) homozygotes survived past one year; SC and CC genotypes had lower than expected incidence, suggesting reduced survival.

    Conclusions:

    • Upper Volta exhibits a high prevalence of HbS and HbC hemoglobinopathies.
    • Geographic and environmental factors play a role in the differential distribution of HbS and HbC genes.
    • Adverse environmental and medical conditions in Upper Volta severely limit the survival of individuals with sickle cell disease and related genotypes.

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