BACKGROUND A small number of studies have confirmed the presence of oxidative damage in patients with Duchenne muscular dystrophy (DMD). Nevertheless, it is unknown if there a relationship of circulating markers of oxidative stress with a muscle injury. OBJECTIVE We evaluated if oxidative damage and anti-oxidant markers are associated with muscle damage in DMD. METHODS This cross-sectional study included 24 patients with DMD classified in ambulatory and non-ambulatory. Markers of muscle damage (creatine kinase [CK]), oxidative damage (malondialdehyde [MDA], and 8-isoprostane), anti-oxidant function (Thiol and mRNA of NRF2 and NF-κB) and nitric oxide (NO) were quantified in circulation. RESULTS Total NO, MDA, and 8-isoprostane concentrations were significantly (p < 0.05) higher, and thiol concentration was lower in non-ambulatory than ambulatory patients. A significant correlation (p < 0.05) between muscle injury (evaluated by Vignos scale) with CK (r = -0.382), NO (r = 0.444), MDA (r = 0.503), 8-isoprostanes (r = 0.435) and thiol (r = -0.430) was observed. CONCLUSION These findings suggest that non-ambulatory have high oxidative damage and low anti-oxidant function than ambulatory patients with DMD. Total nitric oxide and oxidative damage plasma markers increase, but the anti-oxidant marker thiol decreases with a muscle injury in boys with DMD. The findings of this study suggest that these markers could be considered as goods indicators of oxidative damage in longitudinal studies to evaluate the muscle injury during DMD progression. Additionally, these findings add new information about the pathophysiology of DMD.

中文翻译：

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氧化应激的循环标志物与肌营养不良症患者的肌肉损伤有关

背景 少数研究证实杜氏肌营养不良症（DMD）患者存在氧化损伤。然而，尚不清楚氧化应激的循环标志物是否与肌肉损伤有关。目的 我们评估氧化损伤和抗氧化标志物是否与 DMD 中的肌肉损伤有关。方法 本横断面研究包括 24 名 DMD 患者，分为可走动和不可走动。肌肉损伤（肌酸激酶 [CK]）、氧化损伤（丙二醛 [MDA] 和 8-异前列烷）、抗氧化功能（NRF2 和 NF-κB 的硫醇和 mRNA）和一氧化氮 (NO) 的标志物在循环。结果 总 NO、MDA 和 8-异前列烷浓度显着 (p < 0.05) 更高，非卧床患者的硫醇浓度低于卧床患者。肌肉损伤（通过 Vignos 量表评估）与 CK (r = -0.382)、NO (r = 0.444)、MDA (r = 0.503)、8-异前列烷 (r = 0.435) 和硫醇之间存在显着相关性 (p < 0.05) (r = -0.430) 被观察到。结论 这些发现表明，与非卧床DMD患者相比，非卧床患者具有较高的氧化损伤和较低的抗氧化功能。在患有 DMD 的男孩中，总一氧化氮和氧化损伤血浆标志物增加，但抗氧化标志物硫醇随着肌肉损伤而减少。这项研究的结果表明，这些标志物可以被视为纵向研究中氧化损伤的良好指标，以评估 DMD 进展过程中的肌肉损伤。此外，这些发现增加了有关 DMD 病理生理学的新信息。