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Sickle cell disease in Sudan.

R A Bayoumi1, Y A Abu Zeid, A Abdul Sadig

  • 1Department of Biochemistry, Faculty of Medicine, University of Khartoum, Sudan.

Transactions of the Royal Society of Tropical Medicine and Hygiene
|January 1, 1988
PubMed
Summary

Sickle cell disease (SCD) in Sudan presents severely, similar to African populations, with young patients experiencing crises and infections. Disease severity did not correlate with HbF levels or erythrocyte 2,3-DPG.

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Area of Science:

  • Hematology
  • Genetics
  • Public Health

Background:

  • Sickle cell disease (SCD) is a genetic blood disorder with varying clinical manifestations.
  • Understanding the specific SCD phenotype in different ethnic and geographic populations is crucial for targeted healthcare.
  • Sudan's location suggests a potential overlap between West African and Arabian SCD genetic influences.

Purpose of the Study:

  • To characterize the clinical, hematological, and biochemical features of sickle cell disease in Sudanese patients.
  • To compare the SCD phenotype in Sudan with those observed in African and Arabian populations.
  • To investigate potential correlations between disease severity and specific biochemical markers like HbF and erythrocyte 2,3-DPG.

Main Methods:

  • Clinical assessment of 50 Sudanese patients with sickle cell disease.

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  • Hematological and biochemical analyses including hemoglobin typing (HbSS, HbS/β+thalassemia), HbF, HbA2, reticulocyte count, and serum bilirubin.
  • Family data collection to determine SCD genotypes.
  • Correlation analysis between clinical presentation and laboratory findings.

    • Main Results:

    • The majority of patients (84%) were from the Baggara tribe, indicating a link to the West African HbS belt.
    • Common presentations in young children included vaso-occlusive crisis, severe anemia, hand-foot syndrome, infections, and malnutrition.
    • Cardiac enlargement was universal, with significant rates of splenomegaly (42%) and bone infarctive lesions (20%).
    • Mean hemoglobin was 7.3 g/dl, HbF was 7%, and HbA2 was 2.8%.
    • The SCD pattern in Sudan aligns with the severe African type, not the milder Saudi Arabian form.
    • No correlation was found between disease amelioration and HbF levels or erythrocyte 2,3-DPG.

    Conclusions:

    • Sickle cell disease in Sudan exhibits a severe phenotype comparable to that in other African populations.
    • Disease presentation in young Sudanese children is characterized by significant morbidity from vaso-occlusive events and infections.
    • Factors other than HbF levels or erythrocyte 2,3-DPG appear to influence SCD severity in this population.