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输血依赖型重型地中海贫血患者的心脏铁与心脏疾病:一项 T2*磁共振成像研究。

Cardiac iron and cardiac disease in males and females with transfusion-dependent thalassemia major: a T2* magnetic resonance imaging study.

机构信息

1Department of Clinical and Experimental Medicine Pediatrics, Ferrara, Italy.

出版信息

Haematologica. 2011 Apr;96(4):515-20. doi: 10.3324/haematol.2010.025510. Epub 2011 Jan 12.

DOI:10.3324/haematol.2010.025510
PMID:21228034
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3069227/
Abstract

BACKGROUND

It has been repeatedly reported that female patients with thalassemia major survive longer than males and that the difference is due to a lower rate of cardiac disease in females.

DESIGN AND METHODS

We compared the cardiac iron load as measured by T2* magnetic resonance imaging in 776 patients (370 males) examined at the National Research Council as part of an Italian cooperative study. We also established normal left ventricular ejection fraction values for our population.

RESULTS

The prevalence of cardiac disease was higher in males than in females (105 males versus 69 females; P < 0.0001). Cardiac T2* was significantly lower in patients with heart dysfunction (P < 0.0001), but no difference was observed according to sex. Twenty males and five females had a history of cardiac arrhythmias. Their cardiac T2* was not significantly lower than that of patients without arrhythmias (24 ms versus 26 ms; P = 0.381), nor was there a difference between sexes. Liver T2* was significantly lower in males and females with heart dysfunction compared to those without. Ferritin levels were higher in patients of both sexes with heart dysfunction without significant differences between males and females. Conclusions Males and females are at the same risk of accumulating iron in their hearts, but females tolerate iron toxicity better, possibly as an effect of reduced sensitivity to chronic oxidative stress.

摘要

背景

有报道称,女性重型地中海贫血患者的存活时间长于男性,这种差异是由于女性患心脏病的比率较低。

设计和方法

我们比较了在国家研究委员会作为意大利合作研究的一部分接受检查的 776 名患者(370 名男性)的心脏铁负荷,这些患者通过 T2*磁共振成像进行了检查。我们还为我们的人群建立了正常的左心室射血分数值。

结果

男性的心脏疾病患病率高于女性(105 名男性对 69 名女性;P<0.0001)。心脏 T2在心脏功能障碍患者中显著降低(P<0.0001),但与性别无关。20 名男性和 5 名女性有心脏心律失常病史。他们的心脏 T2并不明显低于无心律失常患者(24 毫秒对 26 毫秒;P=0.381),性别之间也没有差异。肝功能障碍的男性和女性的肝脏 T2*明显低于无心脏功能障碍的患者。心脏功能障碍的男性和女性患者的铁蛋白水平均较高,但男女之间无显著差异。结论:男性和女性患心脏铁蓄积的风险相同,但女性对铁毒性的耐受性更好,这可能是由于对慢性氧化应激的敏感性降低所致。

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