Carilho Rita, de Carvalho Mamede, Kuehl Ute, Pinto Susana, Pinto Anabela, Kromminga Arno, Costa Júlia
Instituto de Tecnologia Química e Biológica, Universidade Nova de Lisboa, Oeiras, Portugal.
Amyotroph Lateral Scler. 2011 Nov;12(6):439-43. doi: 10.3109/17482968.2011.584881. Epub 2011 Jul 4.
In amyotrophic lateral sclerosis (ALS), respiratory muscle weakness causes ventilatory insufficiency and tissue hypoxia, which induces a number of metabolic pathways, and in particular increases erythropoietin (EPO) synthesis. EPO is a glycoprotein with neuroprotective properties that stimulates erythropoiesis. Here, EPO plasma level in a large population of ALS patients, with and without respiratory failure, was measured. Plasma EPO level of patients with ALS (n = 98), controls with other neuromuscular diseases (n = 58) and healthy controls (n = 20) has been quantified by ELISA. No significant difference was found between ALS patients and the two control groups. EPO level was not different between bulbar- and spinal-onset patients and was not correlated with disease duration or functional impairment. However, in the ALS group EPO level was higher in females (p = 0.0006) and correlated positively with age (p = 0.006). The subgroup of ALS patients with respiratory failure had higher plasma levels of EPO compared with ALS patients with preserved respiratory function (p = 0.016), but short-term non-invasive ventilation did not change EPO level. In conclusion, EPO levels were found to be significantly higher in ALS patients with respiratory impairment representing preservation of this homeostatic mechanism.
在肌萎缩侧索硬化症(ALS)中,呼吸肌无力会导致通气不足和组织缺氧,进而引发多种代谢途径,尤其是增加促红细胞生成素(EPO)的合成。EPO是一种具有神经保护特性的糖蛋白,可刺激红细胞生成。在此,我们测定了大量有或无呼吸衰竭的ALS患者的血浆EPO水平。通过酶联免疫吸附测定法(ELISA)对98例ALS患者、58例患有其他神经肌肉疾病的对照者以及20例健康对照者的血浆EPO水平进行了定量分析。结果发现,ALS患者与两个对照组之间无显著差异。延髓起病型和脊髓起病型患者的EPO水平无差异,且与疾病持续时间或功能损害无关。然而,在ALS组中,女性的EPO水平较高(p = 0.0006),且与年龄呈正相关(p = 0.006)。与呼吸功能保留的ALS患者相比,患有呼吸衰竭的ALS患者亚组的血浆EPO水平更高(p = 0.016),但短期无创通气并未改变EPO水平。总之,我们发现呼吸功能受损的ALS患者的EPO水平显著更高,这代表了这种稳态机制的保留。