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Haemoglobin DIbadan in a haemoglobin S endemic population
African Journal of Medicine and Medical Sciences
|September 1, 1979
Summary
Haemoglobin D Ibadan (HbD) and sickle cell haemoglobin (HbS) are difficult to distinguish. New diagnostic techniques accurately identified HbD and HbS in a family, aiding screening in regions with high incidence.
Area of Science:
- Hematology
- Medical Genetics
Background:
- Haemoglobin D Ibadan (HbD) presents diagnostic challenges due to its similarity to sickle cell haemoglobin (HbS).
- High incidence of HbS in Ibadan necessitates reliable screening methods for differentiating haemoglobin variants.
Observation:
- A family with Haemoglobin D Ibadan (beta 87 threonine to lysine), sickle cell haemoglobin (HbS), and normal adult haemoglobin (HbA) was studied.
- Combined techniques including solubility, sickling rate, shaking test, and electrophoresis were employed for diagnosis.
Findings:
- Unequivocal diagnosis of haemoglobin types and quantitative estimation were achieved.
- HbD showed slightly reduced solubility compared to HbA.
- Individuals with HbS+HbD had 57-60% HbD, suggesting increased HbD synthesis relative to HbS.
- HbA+HbD heterozygotes had 41% HbD, indicating diminished HbD synthesis relative to HbA.
Implications:
- Developed diagnostic strategies enable accurate identification and quantification of HbD and HbS.
- These methods are crucial for effective screening programs in populations with co-existing HbD and HbS.
- Physicochemical and hematological characteristics of HbD heterozygotes are normal, simplifying clinical assessment.