首页> 外文期刊>Acta Haematologica >Survival and complications of beta-thalassemia in Lebanon: a decade's experience of centralized care.
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Survival and complications of beta-thalassemia in Lebanon: a decade's experience of centralized care.

机译:黎巴嫩β地中海贫血的生存和并发症:集中护理的十年经验。

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摘要

beta-Thalassemia major is a debilitating disease with a considerable incidence in Lebanon (around 2-3% carriership). The present article describes our experience to this day with 214 patients, emphasizing the survival of beta-thalassemia major and development of complications among patients with different parameters. Fifteen deaths were reported. The most common cause of death was heart failure (60%). Patients with a ferritin level of 3,000 ng/ml showed better survival than those with a level >3,000 ng/ml (p < 0.006). In addition, patients with a ferritin level of 1,500 ng/ml showed less complication-free survival than those with a level >1,500 ng/ml (p < 0.024). High level of ferritin (1,500 ng/ml) is associated with increased risk of heart failure. Overall and complication-free survival were statistically different among patients classified according to birth cohort or ferritin level. The Chronic Care Center, a multidisciplinary center located in the suburbs of Beirut, led to an increase in complication-free as well as overall survival. Although patients are being diagnosed earlier and chelation therapy is being initiated at an earlier age, complications due to iron overload still persist. The introduction of new oral iron chelators and better iron overload quantitation methods will most likely modify this picture, and a follow-up study will examine their impact.
机译:严重的β-地中海贫血是一种使人衰弱的疾病,在黎巴嫩发病率很高(约占2-3%的携带者)。本文介绍了我们迄今为止对214例患者的经验,强调了重度β地中海贫血的生存率以及具有不同参数的患者中并发症的发生。据报道有十五人死亡。最常见的死亡原因是心力衰竭(60%)。铁蛋白水平为3,000 ng / ml的患者比水平> 3,000 ng / ml的患者表现出更好的生存率(p <0.006)。此外,铁蛋白水平为1,500 ng / ml的患者显示的无并发症生存率低于水平> 1,500 ng / ml的患者(p <0.024)。高水平的铁蛋白(1,500 ng / ml)与心力衰竭的风险增加有关。根据出生队列或铁蛋白水平分类的患者的总体生存率和无并发症生存率在统计学上不同。慢性护理中心是位于贝鲁特郊区的一个多学科中心,导致无并发症以及整体生存率的提高。尽管较早地诊断出患者,并在较早的年龄开始了螯合疗法,但由于铁超载而引起的并发症仍然存在。引入新的口服铁螯合剂和更好的铁过载定量方法将最有可能改变这种状况,后续研究将检查其影响。

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