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性别差异在心脏并发症的发展中的作用:一项在大型地中海贫血症患者队列中进行的多中心研究,旨在优化心脏随访的时机。

Gender differences in the development of cardiac complications: a multicentre study in a large cohort of thalassaemia major patients to optimize the timing of cardiac follow-up.

机构信息

Magnetic Resonance Imaging Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.

Unità Operativa di Day Hospital della Talassemia e delle Emoglobinopatie, Azienda Ospedaliero-Universitaria "S. Anna", Cona (FE), Italy.

出版信息

Br J Haematol. 2018 Mar;180(6):879-888. doi: 10.1111/bjh.15125. Epub 2018 Feb 7.

DOI:10.1111/bjh.15125
PMID:29504129
Abstract

We assessed whether male gender was associated with a higher risk of cardiac iron accumulation and fibrosis, heart dysfunction and complications in a large, multicentre cohort of thalassaemia major (TM) patients, in order to optimize the timing in cardiac follow-up. We considered 1711 TM patients (899 females, 31·09 ± 9·08 years), enrolled in the Myocardial Iron Overload in Thalassaemia Network. Clinical/instrumental data are recorded from birth to the first Cardiovascular Magnetic Resonance Imaging scan. Although having a similar risk of accumulating iron, males showed a significantly higher risk of developing cardiac dysfunction, heart failure, arrhythmias and cardiac complications overall, when compared to females (P < 0·0001). Up to 20-30 years of follow-up, the Kaplan-Meier curves for the outcomes for which the male sex was a significant prognosticator almost overlapped, whereas they clearly diverged after this period. In patients with follow-up longer than 20 years, males exhibited a significantly higher risk of ventricular dysfunction, heart failure, arrhythmias, and cardiac complications. Female patients may have an intrinsically better tolerance for iron toxicity. International guidelines suggest annual cardiac evaluation for thalassaemia patients. It is possible that female patients can be evaluated at longer intervals, thus reducing health costs.

摘要

我们评估了男性性别是否与地中海贫血症患者心脏铁积累和纤维化、心脏功能障碍和并发症的风险增加相关,以便优化心脏随访的时间。我们考虑了 1711 名地中海贫血症患者(899 名女性,31.09±9.08 岁),他们参加了地中海贫血症网络中的心肌铁超负荷研究。临床/仪器数据从出生记录到第一次心血管磁共振成像扫描。尽管男性和女性积累铁的风险相似,但与女性相比,男性发生心脏功能障碍、心力衰竭、心律失常和心脏并发症的风险显著更高(P<0.0001)。在随访长达 20-30 年期间,对于男性是显著预后预测因子的结果,其 Kaplan-Meier 曲线几乎重叠,而在此期间之后,它们明显分开。在随访时间超过 20 年的患者中,男性发生心室功能障碍、心力衰竭、心律失常和心脏并发症的风险显著更高。女性患者可能对铁毒性具有内在的更好耐受性。国际指南建议对地中海贫血症患者进行年度心脏评估。女性患者可能可以更长时间评估,从而降低医疗成本。

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