Cheung Y F, Chan Godfrey C F, Ha S Y
Division of Paediatric Cardiology, Grantham Hospital, The University of Hong Kong, Hong Kong, China.
Br J Haematol. 2008 May;141(5):728-33. doi: 10.1111/j.1365-2141.2008.07092.x. Epub 2008 Mar 3.
Deferasirox (ICL670) has been shown to have rapid accessibility to intracellular labile iron. We tested the hypothesis that oral deferasirox improves arterial dysfunction in patients with beta-thalassaemia major. Nineteen thalassaemia patients, aged 23 +/- 7 years, with normal left ventricular (LV) function were treated with deferasirox at 25-35 mg/kg/d for 12 months. LV function, brachial arterial flow-mediated dilation (FMD), carotid arterial stiffness, and serum ferritin levels were determined at baseline prior to initiation, after 6 months and after 12 months of therapy. The baseline cardiovascular indices were compared with those of 17 age-matched controls. Longitudinal changes in patients during the treatment period were also determined. Compared with controls, patients had similar echocardiographic indices of LV function (all P > 0.05), while their baseline brachial FMD was reduced (P < 0.001) and carotid stiffness increased (P = 0.019). An increase in FMD (P < 0.001) and a decrease in carotid stiffness (P = 0.007) were found at 6 and 12 months follow-up. The stiffness index correlated inversely with FMD (r = -0.42, P = 0.001). Although there was an increase in ferritin level at 12 months (3303 +/- 1185 ng/ml vs. 2714 +/- 780 ng/ml at baseline, P = 0.006), no significant correlation existed between ferritin level and FMD or carotid stiffness. In conclusion, deferasirox therapy in thalassaemia patients is associated with improved arterial function.
地拉罗司(ICL670)已被证明能快速作用于细胞内不稳定铁。我们检验了以下假设:口服地拉罗司可改善重型β地中海贫血患者的动脉功能障碍。19名年龄在23±7岁、左心室(LV)功能正常的地中海贫血患者接受了为期12个月的地拉罗司治疗,剂量为25 - 35 mg/kg/d。在治疗开始前的基线、治疗6个月后和12个月后,分别测定LV功能、肱动脉血流介导的舒张功能(FMD)、颈动脉僵硬度和血清铁蛋白水平。将基线心血管指标与17名年龄匹配的对照组进行比较。还确定了治疗期间患者的纵向变化。与对照组相比,患者的LV功能超声心动图指标相似(所有P>0.05),而其基线肱动脉FMD降低(P<0.001),颈动脉僵硬度增加(P = 0.019)。在随访6个月和12个月时发现FMD增加(P<0.001),颈动脉僵硬度降低(P = 0.007)。僵硬度指数与FMD呈负相关(r = -0.42,P = 0.001)。虽然12个月时铁蛋白水平有所升高(3303±1185 ng/ml vs. 基线时的2714±780 ng/ml,P = 0.006),但铁蛋白水平与FMD或颈动脉僵硬度之间无显著相关性。总之,地中海贫血患者接受地拉罗司治疗与动脉功能改善有关。