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中间型地中海贫血中的心脏病

Heart disease in thalassemia intermedia.

作者信息

Aessopos A, Tsironi M, Andreopoulos A, Farmakis D

机构信息

First Department of Internal Medicine, University of Athens Medical School, Laiko General Hospital, Athens, Greece.

出版信息

Hemoglobin. 2009;33 Suppl 1:S170-6. doi: 10.3109/03630260903351676.

Abstract

Thalassemia intermedia (TI) presents with a clinical phenotype ranging between the severe, transfusion-dependent TM and the asymptomatic carrier state. Cardiovascular complications represent the primary cause of mortality both in TM and in TI. Several factors have been reported to interfere in the pathophysiology of cardiovascular abnormalities in TI, including high output state, vascular abnormalities, endothelial dysfunction, elastic tissue disorders, hypercoagulability, valvular abnormalities and, to a less extent, iron overload. These mechanisms end up affecting both left and right heart, hence leading to ventricular remodeling and finally heart failure.

摘要

中间型地中海贫血(TI)的临床表型介于严重的、依赖输血的重型地中海贫血(TM)和无症状携带者状态之间。心血管并发症是TM和TI患者死亡的主要原因。据报道,有几个因素会干扰TI患者心血管异常的病理生理学,包括高输出状态、血管异常、内皮功能障碍、弹性组织紊乱、高凝状态、瓣膜异常,以及程度较轻的铁过载。这些机制最终会影响左右心室,从而导致心室重塑,最终发展为心力衰竭。

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