Department of Chemical Pathology, Ladoke Akintola University of Technology, Osogbo, Osun State, Nigeria.
Department of Chemical Pathology, College of Medicine, University of Ibadan, Ibadan, Oyo State, Nigeria.
Kaohsiung J Med Sci. 2019 Jun;35(6):358-364. doi: 10.1002/kjm2.12062. Epub 2019 Mar 26.
Hemoglobin (Hb) and iron are prooxidants in nature and sources of free radicals in the biological system of all Hb phenotypes. Recent evidence linked abnormal hemoglobin S and C (HbSC) in sickle cell disease (SCD) to various complications in multiple oxidative processes. However, similar studies in relation to abnormal Hb traits are sparse. Besides, reports on activities of antioxidant enzymes and iron status in SCDs are still contradictory. This study assessed the interplay between lipid peroxidation and antioxidant defense capacity in various Hb variants. We enrolled 193 participants with different Hb phenotypes. They were consecutive patients with sickle cell anemia (HbSS, n = 32) and hemoglobin SC (HbSC) disease (n = 28) regularly followed up in a steady state. Other participants were subjects with abnormal Hb traits (HbAS, n = 50; HbAC, n = 33) and normal controls (HbAA, n = 50). The hematocrit (Hct) level, hemoglobin (Hb) concentration, iron status, and biochemical parameters including malondialdehyde (MDA), total antioxidant status (TAS), superoxide dismutase (SOD), and glutathione peroxidase (GPx) enzymes were investigated simultaneously. The MDA and SOD levels were significantly higher (P < 0.05) in Hb variants in order of HbSS>HbSC>HbAC>HbAS when compared with controls. Conversely, GPx and TAS levels showed significant reductions (P < 0.05). Similarly, Hct, Hb, and iron concentrations showed significant reductions (P < 0.05) sequentially following HbAC > HbAS > HbSC > HbSS compared with controls. The results suggest that both SCDs and the carriers were relatively more vulnerable to systemic oxidative stress against normal phenotype, and may be owing to ineffective antioxidant mechanisms needed for keeping spontaneous generations of free radicals in control without necessarily iron-mediated.
血红蛋白(Hb)和铁本质上是氧化剂,也是所有 Hb 表型生物系统中自由基的来源。最近的证据表明,镰状细胞病(SCD)中异常的血红蛋白 S 和 C(HbSC)与多种氧化过程中的各种并发症有关。然而,关于异常 Hb 特征的类似研究很少。此外,关于 SCD 中抗氧化酶活性和铁状态的报告仍然存在矛盾。本研究评估了不同 Hb 变体中脂质过氧化与抗氧化防御能力之间的相互作用。我们招募了 193 名具有不同 Hb 表型的参与者。他们是连续患有镰状细胞贫血(HbSS,n=32)和血红蛋白 SC(HbSC)疾病(n=28)的患者,在稳定状态下定期接受治疗。其他参与者是具有异常 Hb 特征的受试者(HbAS,n=50;HbAC,n=33)和正常对照者(HbAA,n=50)。同时检测了血细胞比容(Hct)水平、血红蛋白(Hb)浓度、铁状态以及包括丙二醛(MDA)、总抗氧化状态(TAS)、超氧化物歧化酶(SOD)和谷胱甘肽过氧化物酶(GPx)在内的生化参数。与对照组相比,HbSS>HbSC>HbAC>HbAS 时,Hb 变体中的 MDA 和 SOD 水平显著升高(P<0.05)。相反,GPx 和 TAS 水平显著降低(P<0.05)。同样,与对照组相比,Hct、Hb 和铁浓度也依次显著降低(P<0.05)。结果表明,SCD 和携带者相对于正常表型更容易受到全身氧化应激的影响,这可能是由于需要维持自由基的自发产生而不需要铁介导的有效抗氧化机制。