There is a theoretical advantage provided by heterozygous SCD inheritance, as carriers of SCD are 90% less likely to suffer from severe malaria (Allison, In 2006, the World Health Organization recognized SCD as a global problem with significant economic implications with the incidence expected to rise above 400,000 births per year in 2050 (Piel et al., Pathophysiology SCD and its complications are a result of erythrocyte and reticulocyte morphology changes, seen in Figure 1 (Inusa et al., HbS polymerisation results in rigid, fragile sickle cells with up to a 75% reduction in lifespan (Kaul et al., Oxidative stress is both cause and effect of increased erythrocyte haemolysis (Kato et al., Haemolysis releases hemoglobin and haem into the bloodstream (Kato et al., SCD erythrocytes contain dysfunctional membrane channels, such as K-Cl cotransporters or Gardos channels, which contribute to cell dehydration, further increasing the HbS concentration and polymerisation (Frenette and Atweh, Complications SCD causes a wide range of complications in various of the body's systems, shown in Figure 2, including the cardiovascular, reproductive, pulmonary, renal, hepatobiliary, and can even cause neurocognitive dysfunction (Kato et al., Figure 2 The clinical hallmark and primary cause of SCD hospitalisations are vaso-occlusive crises (VOCs) (Rees et al., Disease-Modifying Drugs Hydroxyurea Hydroxyurea acts as an inhibitor of ribonucleotide reductase, thereby inactivating enzymes involved in cellular division (Inusa et al., Hydroxyurea has been shown to improve both clinical and hematological complications, with particular benefit to high-risk children (Platt et al., Although 63% of SCA patients in high-income countries receive hydroxyurea, due to lack of treatment guidelines, reduced clinical infrastructure and medication inaccessibility, this percentage can be as low as zero in under-resourced settings (McGann et al., L-Glutamine Glutamine, in addition to 5 other amino acids, is a conditionally essential amino acid for the body, meaning its synthesis is limited under specific physiological conditions (Inusa et al., New Therapeutic Approaches Increased knowledge regarding SCD pathophysiology has led to new therapeutic strategies
PMID 12709588
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Current data suggests that the relevance of targeting MC1R mediated pathways may depend on receptor expression levels and genetic background, emphasizing the need for continued research to clarify the receptors protective roles within epidermal layer biology
