Abstract
Background: Endothelial activation, which often occurs in individuals with sickle cell disease (SCD) patients as a result of oxidative stress, may lead to endothelial dysfunction and acute inflammation. Evaluation of endothelin-1 levels in vaso-occlusive crisis (VOC) may help identify therapeutic targets in the management of SCD.
 Materials and Methods: This study aims to assess the role of endothelial activation in the pathophysiology of VOC using endothelin-1 in patients with sickle cell disease managed at the University of Benin Teaching Hospital.
 Materials and Methods: This was a longitudinal study conducted at the University of Benin Teaching Hospital, Benin City, Edo State between April 2018 and August 2019. Thirty-five patients with sickle cell anaemia (SCA) were evaluated during VOC and later re-evaluated in steady-state. Thirty-five HbAA subjects, matched for age and sex with the SCA population were recruited as controls. Endothelin -1 (ET1) concentrations were determined using an enzyme-linked immunosorbent assay. Data were analyzed using SPSS version 21.
 Results: The age range of the SCA patients was 18 – 45 years with a mean SD of 27.7 + 6.7 years. The mean endothelin-1 levels in SCA patients in steady state, VOC and control subjects were 9.35mM, 13.79mM and 4.80mM respectively. The mean ET-1 levels in SCA patients in steady-state and VOC was significantly increased compared to the control subjects (p < 0.001). There was no significant correlation between ET-1 and number of VOCs per year (p = 0.108) and the number of admissions per year (p < 0.005).
 Conclusion: SCA is associated with increased endothelial activation which may contribute to the pathogenesis of VOC. This finding may be important in determining the role of the use of antiinflammatory therapies in the management of SCD.
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