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首页> 外文期刊>Journal of Inborn Errors of Metabolism & Screening >Update on Coronary Involvement in Fabry Disease
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Update on Coronary Involvement in Fabry Disease

机译：法布里病冠状动脉介入的最新进展

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摘要

Fabry disease is a multisystemic disorder with consequent morbidity and mortality at an early age in patients of both genders. Although renal failure has been previously described as the cause of death with the highest prevalence, recent studies, based on the analysis of the population recorded in the Fabry Registry, reported that 40% of deaths had a cardiovascular origin. Data from the same registry emphasize the high risk for these patients suffering cardiovascular events?￠????particularly acute myocardial infarction?￠????and to the fact that this risk is higher for those patients who need dialysis. Microvascular dysfunction is a constant in patients with Fabry disease, which affects young patients, independently from other cardiac involvement manifestations?￠????such as left ventricular hypertrophy?￠????and from the patient?￠????s gender.
机译：法布里病是一种多系统性疾病，在男女患者的早期均会导致发病和死亡。尽管先前已将肾功能衰竭描述为患病率最高的死亡原因，但根据法布里注册表中记录的人群分析，最近的研究表明，有40％的死亡源于心血管。来自同一个登记处的数据强调了这些患者发生心血管事件的风险很高，尤其是急性心肌梗塞，而且对于那些需要透析的患者来说，这种风险更高。法布里病患者的微血管功能障碍是一个常数，它独立于其他心脏受累表现（如左心室肥大）和患者的其他影响，独立地影响年轻患者。性别。

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