Respiratory Burst Enzymes and Oxidant-antioxidant Status in Nigerian Children with Sickle Cell Disease

Aim: To measure respiratory burst enzymes, pro-oxidants, antioxidants and red cell indices in Nigerian children with sickle cell disease (HbSS) below five years of age and compared with apparently healthy children with normal haemoglobin (HbAA).

Method: A total of 45 subjects were recruited which included 23 children (age range 10 – 48 months) with HbSS and 22 children (age- and sex- matched) with HbAA. Blood samples were collected and red cell indices were determined using automated haematology analyser while serum superoxide dismutase (SOD), glutathione peroxidise (GSH-Px) and myeloperoxidase (MPO) activities were measured using ELISA kits. Serum malondialdehyde (MDA), hydrogen peroxide (H2O2), glutathione S transferase (GST), catalase (Cat), xanthine oxidase (XO) and glutathione (GSH) were measured with colorimetric techniques. MPO, SOD and Cat represented respiratory burst enzymes; MDA, H2O2 and XO were measured as pro-oxidants while GSH, GST and GSH-Px were the measured antioxidants.

Results: Mean concentration of malondialdehyde was significantly reduced (5.56±1.12nmol/L vs. 6.46±1.11nmol/L, P=.04) in HbSS children compared with HbAA children. Similarly, mean serum activity of myeloperoxidase in HbSS children was significantly reduced compared with HbAA children (66.12±13.34U/mL vs 77.02±13.54U/mL, P=.03). However, there were no significant differences in mean concentration of serum glutathione, hydrogen peroxide; serum activities of glutathione peroxidase, catalase, superoxide dismutase, xanthine oxidase and glutathione S transferase in HbSS children compared with HbAA children