Background: Sickle cell anemia (SCA) is an inherited blood disease with known complications as a result of certain pathophysiological dysfunctions. It has been suggested that an increase in oxidative stress contributes to the incidence of these changes. Objectives: This study investigated the oxidant/antioxidant status of patients with SCA, and evaluated the effect of SCA on antioxidant enzymes and their cofactors. Methods: The study included 42 patients with SCA (in steady state), and a control group of 50 age-matched individuals without SCA. Serum malondialdehyde (MDA), copper, zinc, ferritin and iron levels, red blood cell (RBC) superoxide dismutase (SOD) and catalase levels were measured for the SCA and control groups. Results: Significantly lower levels of antioxidant enzymes (RBC SOD and catalase) and higher serum MDA levels (biomarker of oxidative stress) were found in SCA patients compared to the control group (all p < 0.001). Increased levels of serum ferritin, iron and copper and decreased zinc concentrations were also found in the SCA patients compared to the control group (all p < 0.001). In the SCA group, there were significant negative correlations between MDA levels and RBC SOD, RBC catalase, and serum zinc levels (p < 0.01), while a significant positive correlation between MDA with serum copper and iron levels (p < 0.01) was observed. Conclusion: SCA is associated with alterations in markers of oxidative stress including an increased MDA level, decreased antioxidant enzyme levels, and altered levels of enzyme cofactors (zinc, copper, and iron). This suggests that these antioxidant enzymes could be used as effective therapeutic targets for the treatment of this disease and supplementation of patients with substances with antioxidant properties may reduce the complications of this disease.

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Patients with sickle cell anemia (SCA) can acquire many biochemical abnormalities, including altered magnesium levels. However, the roles of magnesium in the pathogenesis and management of SCA need to be determined. The aim of this work was to evaluate magnesium levels among pediatric patients with SCA in Basra, Iraq. The study employed a case-control design and examined 87 patients with SCA (3-15 years old) who had attended the Basra Center for Hereditary Blood Diseases while in a steady state and 90 apparently healthy control subjects. Complete blood count, red blood cell (RBC), and serum magnesium, calcium, potassium, sodium, zinc, and copper levels were measured in all subjects. The results revealed significantly lower RBC and serum magnesium levels among the patients with SCA (3.62 ± 0.42 and 1.35 ± 0.19 mg/dL, respectively) than those among the control subjects (4.47 ± 0.55 and 1.87 ± 0.27 mg/dL, respectively). In addition, compared to the control subjects, the patients with SCA had significantly lower serum levels of potassium, sodium, and zinc; significantly higher serum levels of copper; and significantly higher Ca/Mg and Na/Mg ratios. Among the SCA patients, the RBC magnesium level was significantly negatively associated with the frequencies of vaso-occlusive crises (r = - 0.423, P < 0.001) and disease-related hospitalization (r = - 0.225, P < 0.05). To conclude, the RBC magnesium level, but not the serum magnesium level, is significantly associated with vaso-occlusive crises and hospitalization. Therefore, screening and management of low RBC magnesium levels in SCA patients are required.

In a study on a group of 186 newborn babies presenting with jaundice, erythrocyte glucose-6-phosphate dehydrogenase (G6PD) deficiency was detected in 95 (51%) of the patients. The incidence of severe hyperbilirubinaemia appeared to be much greater in G6PD-deficient infants (46%) than in infants who did not have the red cell defect (15%). No change was found in this association when ABO incompatibility was excluded. Phototherapy did not reduce the need for exchange transfusion, which was necessary in 27 babies. Eight babies developed kernicterus and one died. Early detection of G6PD deficiency and close surveillance of the affected newborns may be important in reducing the risk of severe neonatal jaundice and kernicterus associated with G6PD deficiency in Basrah.

Basra, southern Iraq, was mapped for haemoglobinopathies and glucose-6-phosphate dehydrogenase [G6PD] deficiency. Of 1064 couples aged 14-60 years recruited from the Public Health Laboratory, 49 had beta-thalassaemia trait, 69 had sickle-cell trait, 2 had haemoglobin D trait, 2 had haemoglobin C trait and 1 had high persistent fetal haemoglobin. Carriers of major beta-globin disorders comprised 11.48%. G6PD deficiency was detected in 133 individuals [12.5%]. Only 10 couples [0.94%] were at risk of having children affected with either sickle-cell disease or beta-thalassaemia major. These defects constitute a real health problem and necessitate a management plan and public health education for early diagnosis and therapy