Karakukcu Cigdem, Karakukcu Musa, Unal Ekrem, Patiroglu Turkan, Ozdemir Mehmet A, Torun Yasemin A, Tang Peter H
Department of Biochmistry, Training and Research Hospital, Ataturk Street, Kayseri, Turkey.
Hemoglobin. 2012;36(3):219-29. doi: 10.3109/03630269.2012.672507. Epub 2012 Apr 6.
The aim of this study was to evaluate the plasma coenzyme Q(10) (CoQ(10)) concentration, a vitamin-like substance found in every cell, which is also viewed as the most effective membrane antioxidant, of thalassemic patients and investigate the effect of chelating agents and ferritin levels on its concentration in patients with β-thalassemia major (β-TM). The study included 44 β-TM patients undergoing deferasirox (DFRA) or deferoxamine (DFO) chelation monotherapies or combined therapy with deferiprone (L1) and DFO, 20 patients with β-thalassemia (β-thal) traits and a control group of 22 healthy sex- and age-matched subjects. Complete blood counts, liver and renal function tests, lipid profiles, ferritin and plasma CoQ(10) [by high performance liquid chromatography (HPLC)] were analyzed. The mean age (14.7 ± 7.3 years; median 14.3 years) and sex (26 males, 18 females) of the β-TM patients were not statistically different from the β-thal trait patients and the control group. The plasma CoQ(10) concentration was 0.425 ± 0.136 μmol/L in β-TM patients, 0.508 ± 0.159 μmol/L in the β-thal trait patients and 0.534 ± 0.133 μmol/L in the control group. The difference was significant in both the β-TM (p < 0.001) and β-thal trait patients (p <0.05) compared to the control group. The CoQ(10) concentration was also associated with ferritin levels in β-TM patients; the β-TM patients with high ferritin levels had a lower CoQ(10) (p <0.05) concentration. Also, higher plasma CoQ(10) levels were detected in β-TM patients undergoing DFRA treatment, according to combined therapy administered (0.457 ± 0.115 vs. 0.382 ± 0.127 mg/dL respectively, p <0.05). In conclusion, both the β-thal trait and β-TM patients have lower antioxidant capacity as demonstrated by the lower CoQ(10) levels. The type of chelating agents and ferritin levels are factors effecting CoQ(10) concentration in β-TM patients.
本研究旨在评估地中海贫血患者血浆辅酶Q(10)(CoQ(10))的浓度,CoQ(10)是一种存在于每个细胞中的类维生素物质,也被视为最有效的膜抗氧化剂,并研究螯合剂和铁蛋白水平对重型β地中海贫血(β-TM)患者血浆CoQ(10)浓度的影响。该研究纳入了44例接受地拉罗司(DFRA)或去铁胺(DFO)螯合单药治疗或与去铁酮(L1)和DFO联合治疗的β-TM患者、20例具有β地中海贫血(β-thal)特征的患者以及22名年龄和性别匹配的健康受试者作为对照组。分析了全血细胞计数、肝肾功能检查、血脂谱、铁蛋白和血浆CoQ(10) [通过高效液相色谱法(HPLC)]。β-TM患者的平均年龄(14.7±7.3岁;中位数14.3岁)和性别(26名男性,18名女性)与β-thal特征患者和对照组相比无统计学差异。β-TM患者的血浆CoQ(10)浓度为0.425±0.136μmol/L,β-thal特征患者为0.508±0.159μmol/L,对照组为0.534±0.133μmol/L。与对照组相比,β-TM患者(p<0.001)和β-thal特征患者(p<0.05)的差异均具有统计学意义。β-TM患者的CoQ(10)浓度也与铁蛋白水平相关;铁蛋白水平高的β-TM患者CoQ(10)浓度较低(p<0.05)。此外,根据联合治疗情况,接受DFRA治疗的β-TM患者血浆CoQ(10)水平较高(分别为0.457±0.115与0.382±0.127mg/dL,p<0.05)。总之,β-thal特征患者和β-TM患者的抗氧化能力均较低,这通过较低的CoQ(10)水平得以证明。螯合剂类型和铁蛋白水平是影响β-TM患者CoQ(10)浓度的因素。