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Endomyocardial fibrosis: A form of endemic restrictive cardiomyopathy

机译:心肌内膜纤维化:一种地方性限制性心肌病

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摘要

Endomyocardial fibrosis is a form of endemic restrictive cardiomyopathy that affects mainly children and adolescents, and is geographically restricted to some poor areas of Africa, Latin America and Asia. It is a condition with high morbidity and mortality, for which no effective therapy is available. Although several hypotheses have been proposed as triggers or causal factors for the disease, none are able to explain the occurrence of the disease worldwide.In endemic areas of Africa endomyocardial fibrosis is as common a cause of heart failure as rheumatic heart disease, accounting for up to 20% of cases of heart failure and imposes a considerable burden to the communities and the health systems. However, due to lack of resources for research in these areas, the exact epidemiology, etiology and pathogenesis remain unknown, and the natural history is incompletely understood.We here review the main aspects of epidemiology, natural history, clinical picture and management of endomyocardial fibrosis, proposing new ways to increase research into this challenging and neglected cardiovascular disease.
机译:心内膜纤维化是一种地方性限制性心肌病,主要影响儿童和青少年,并且在地理上局限于非洲,拉丁美洲和亚洲的一些贫困地区。这是一种高发病率和高死亡率的疾病,目前尚无有效的治疗方法。尽管已经提出了几种假设来引发疾病或引起疾病的原因,但没有一个能够解释该疾病的全球发生范围。在非洲的流行地区,心内膜纤维化与风湿性心脏病一样是导致心力衰竭的常见原因,占了占20%的心力衰竭病例,给社区和卫生系统带来相当大的负担。然而,由于缺乏这些领域的研究资源,确切的流行病学,病因和发病机理仍然未知,并且对自然病史的理解还不完整。 ,提出了新的方法来加强对这一具有挑战性和被忽视的心血管疾病的研究。

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