• Nesma Ahmed Safwat and Mahmoud Adel Kenny.
• Clinical Pathology, Faculty of Medicine, Ain Shams University, Cairo, Egypt. drnesma@windowslive.com.
• Pediatr. Res. 2018 Dec 1; 84 (6): 869-874.

BackgroundSoluble forms of RAGE (sRAGE) have been found circulating in plasma and tissues. Evidence is accruing in human subjects linking levels of sRAGE to oxidative stress in many disorders. Because sickle cell disease (SCD) is a state of oxidative stress, we tested the hypothesis that circulating sRAGE levels may be involved in the vascular pathology of SCD.ObjectivesTo determine the sRAGE levels in children and adolescents with SCD and investigate their association with markers of hemolysis, iron overload, and SCD-related organ complications.Subjects And MethodsThe level of sRAGE was measured in 40 children and adolescent with SCD compared with 40 healthy controls using enzyme-linked immunosorbent assay (ELISA).ResultssRAGE was significantly higher in patients compared with controls (p < 0.001) and was elevated in patients with history of stroke, acute lung syndrome, and frequency of sickling crisis or serum ferritin > 2500 (p < 0.05). Patients with high sRAGE levels are candidates for chelation. sRAGE was positively correlated with HbS% (r = 0.422, p = 0.007), LDH (r = 0.329, p = 0.038), and serum ferritin levels (r = 0.516, p = 0.001). Multivariable regression analysis proved that both HbS% and serum ferritin were significant independent factors affecting sRAGE level (p < 0.05).ConclusionOur findings suggest that sRAGE may be considered as a marker for vascular dysfunction in SCD patients.

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