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首页> 外文期刊>British Journal of Haematology >Assessment and management of iron overload in β-thalassaemia major patients during the 21st century: A real-life experience from the Italian Webthal project
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Assessment and management of iron overload in β-thalassaemia major patients during the 21st century: A real-life experience from the Italian Webthal project

机译：21世纪β地中海贫血重症患者铁超负荷的评估和管理：意大利Webthal项目的真实经验

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摘要

We conducted a cross-sectional study on 924 β-thalassaemia major patients (mean age 30·1 years) treated at nine Italian centres using the webthal software, to evaluate real-life application of iron overload assessment and management standards. Serum ferritin <2500 ng/ml was a risk factor for never having liver iron concentration (LIC) measurement, while absence of cardiac disease and siderosis were risk factors for a delay in LIC measurement >2 years. Patients who never had a cardiac MRI (CMR) T2* measurement were <18 years, had iron intake ≤0·4 mg/kg per day, or a serum ferritin <2500 ng/ml. A history of normal CMR T2* was the main risk factor for a delay in subsequent assessment of >2 years. Deferoxamine (22·8%) was more commonly used in patients with Hepatitis C Virus or high serum creatinine. Deferiprone (20·6%) was less commonly prescribed in patients with elevated alanine aminotransferase; while a deferoxamine + deferiprone combination (17·9%) was more commonly used in patients with serum ferritin >2500 ng/ml or CMR T2* <20 ms. Deferasirox (38·3%) was more commonly prescribed in patients <18 years, but less commonly used in those with heart disease or high iron intake. These observations largely echoed guidelines at the time, although some practices are expected to change in light of evolving evidence.
机译：我们使用webthal软件对9个意大利中心的924例地中海贫血重症患者（平均年龄30·1岁）进行了横断面研究，以评估其实际应用中铁超负荷评估和管理标准。血清铁蛋白<2500 ng / ml是从未进行肝铁浓度（LIC）测量的危险因素，而没有心脏病和铁屑病是LIC测量延迟> 2年的危险因素。从未进行过心脏MRI（CMR）T2 *测量的患者<18岁，铁摄入量每天≤0·4 mg / kg，或血清铁蛋白<2500 ng / ml。正常CMR T2 *的病史是随后的> 2年评估延迟的主要危险因素。丙型肝炎病毒或高血清肌酐的患者更常使用去铁胺（22·8％）。丙氨酸转氨酶升高的患者较少使用去铁酮（20·6％）。血清铁蛋白> 2500 ng / ml或CMR T2 * <20 ms的患者更常使用去铁胺+去铁酮组合（17·9％）。小于18岁的患者更常使用地拉罗司（38·3％），但心脏病或高铁摄入者则较少使用。这些观察结果在当时大体上与准则相呼应，尽管随着证据的不断发展，预计某些实践也会发生变化。

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