Sickle Cell Disease in Sub-Saharan Africa: Molecular Mechanisms Underlying Episodic Crises, Current and Emerging Therapeutic Strategies in Treatment
Keywords:Sickle cell disease, Sub-Saharan Africa, emerging treatments, molecular mechanisms
Sickle cell disease (SCD) is a haematological disease that affects multipleÂ organs, thus eliciting episodes of chronic pain, acute anaemiaÂ and infection, due to a single nucleotide mutation in the Î²-globinÂ gene, which results in the substitution of a glutamic acid residue inÂ place of valine on the Î²-globin chain of the resultant haemoglobinÂ protein molecule, the sickle haemoglobin (HbS). SCD is a major causeÂ of morbidity and mortality characterized by episodes of vaso-occlusive
crises, pain syndromes and end organ dysfunctions. Its global prevalenceÂ is highest in Sub-Saharan Africa with 75% of global birthsÂ living in this region, of which Nigeria has the highest number of SCDÂ patients with about 100.000 births each year. The burden of SCD inÂ the sub-Saharan region of Africa is enormous. Emotional, financial andÂ total healthcare costs are monumental. An understanding of the mechanismÂ underlying the vaso-occlusive crises, pain syndromes, inflammatory
conditions and other sequelae of SCD appears to be essentialÂ in providing more rational treatments. The present review discuses theÂ prevalence of SCD in Africa, molecular mechanisms underlying SCDÂ episodic crises including vaso-occlusive syndrome, anaemia and infection.Â Current available treatments modalities in Sub-Saharan AfricaÂ and possible new treatment methods that cure SCD are re-examinedÂ in light of these mechanisms.
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