Abstract

Background/aimOxidative stress contributes to pathophysiological dysfunction in sickle cell anemia (SCA). Copper (Cu) is a prooxidant, whereas zinc (Zn) and selenium (Se) are antioxidant trace elements. This study investigates the serum levels of Cu, Zn, and Se among children with SCA.Materials and methodsThis cross-sectional study was performed at King Khalid University Hospital, Riyadh. Thirty-three children with SCA in steady state and 33 age- and sex-matched normal healthy children were included in the study. Cu, Zn, and Se levels were measured by inductively coupled plasma-mass spectrometry (ICP-MS) instrument.ResultsThe median serum Cu levels among SCA patients (1.3 μg/mL) were higher than those of the controls (0.88 μg/mL; P < 0.0001). Zn (0.61 μg/mL) and Se (74 ng/mL) levels among SCA patients, however, were significantly lower than those of the controls (0.94 μg/mL; P < 0.0001) and (91.2 ng/mL; P < 0.0001), respectively. The Cu/Zn ratio among SCA patients (1.92) was higher than that of the controls (0.98).ConclusionDecreased blood levels of antioxidant trace elements may contribute to the pathophysiology in SCA by promoting oxidative stress. The monitoring of trace element levels in SCA appears to be vital for decreasing morbidity associated with the disorder.

Highlights

  • Sickle cell anemia (SCA) is a hemoglobinopathy that manifests in infancy and is associated with shortened life expectancy due to repeated episodes of acute illness and organ damage [1]

  • Decreased blood levels of antioxidant trace elements may contribute to the pathophysiology in sickle cell anemia (SCA) by promoting oxidative stress

  • The monitoring of trace element levels in SCA appears to be vital for decreasing morbidity associated with the disorder

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Summary

Introduction

Sickle cell anemia (SCA) is a hemoglobinopathy that manifests in infancy and is associated with shortened life expectancy due to repeated episodes of acute illness and organ damage [1]. It is characterized by a predominant presence of sickle hemoglobin (HbS) resulting from a substitution of glutamic acid to valine at sixth amino acid position in the β globin chain [2]. Low level of ROS activity has been attributed to inadequate vitamin intake or decreased production of enzymes due to cofactors deficiency [5]. Copper (Cu) and zinc (Zn) as cofactors are essential for the optimal performance of superoxide dismutase (SOD), an ROS scavenging enzyme responsible for handling superoxide radicals, preventing tissue damage [6,7]

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