Departamento de Biomédica, Universidad Especializada de las Américas, Panamá, República de Panamá.
Eur J Haematol. 2010 Dec;85(6):529-37. doi: 10.1111/j.1600-0609.2010.01528.x.
To correlate the clinical and hematological features of β-globin gene haplotypes with the oxidative stress status in pediatric patients with sickle cell disease (SCD).
A total of 95 patients with SCD and 40 healthy children were studied. The β-globin cluster, plasma lipid peroxidation (LPO) and plasma nitrite plus nitrate (NOx), and erythrocyte content of glutathione (GSH) and glutathione disulfide (GSSG), and glutathione peroxidase (GPx), reductase (GRd), and superoxide dismutase (SOD) activities were measured.
Plasma LPO (P < 0.001) and NOx (P < 0.05) were significantly higher in patients than in controls. In erythrocytes of patients with SCD, the activities of GRd (P < 0.001) and SOD (P < 0.05) were lower, and the GSSG/GSH ratio (P < 0.001) and GPx activity (P < 0.001) were higher than in controls. High LPO levels and low SOD plus GRd activities were associated with increased severity of clinical manifestations, which correspond mainly to patients with Bantu and Benin haplotypes. LPO levels were reduced in patients with high fetal hemoglobin (HbF) levels, whereas the NOx levels and GRd activity tended to increase in this group.
Our results detected an important oxidative stress in patients with SCD and suggest that at least three redox markers, i.e., LPO, GRd, and SOD, were related with their clinical outcomes. Moreover, a relationship between high HbF and low LPO, and high HbF and high GRd activity and NOx levels were found.
分析β珠蛋白基因单倍型与小儿镰状细胞病（SCD）患者氧化应激状态的临床和血液学特征。
研究共纳入 95 例 SCD 患儿和 40 例健康儿童。测定β珠蛋白簇、血浆脂质过氧化（LPO）和血浆亚硝酸盐/硝酸盐（NOx）、红细胞谷胱甘肽（GSH）和谷胱甘肽二硫化物（GSSG）含量以及谷胱甘肽过氧化物酶（GPx）、还原酶（GRd）和超氧化物歧化酶（SOD）活性。
患儿的血浆 LPO（P＜0.001）和 NOx（P＜0.05）水平显著高于对照组。SCD 患儿红细胞的 GRd（P＜0.001）和 SOD（P＜0.05）活性较低，GSSG/GSH 比值（P＜0.001）和 GPx 活性（P＜0.001）较高。高 LPO 水平和低 SOD+GRd 活性与临床表现严重程度增加有关，主要与班图和贝宁单倍型患者相关。LPO 水平在高胎儿血红蛋白（HbF）水平的患者中降低，而该组的 NOx 水平和 GRd 活性趋于增加。
我们的研究结果表明 SCD 患者存在严重的氧化应激，至少有 3 种氧化还原标志物，即 LPO、GRd 和 SOD，与患者的临床结局相关。此外，还发现 HbF 水平较高与 LPO 水平较低、HbF 水平较高与 GRd 活性和 NOx 水平较高相关。
