Wood John C
Division of Cardiology, Children's Heart Center, Children's Hospital of Los Angeles, Los Angeles, CA 90027-0034, USA.
Hemoglobin. 2009;33 Suppl 1(Suppl 1):S81-6. doi: 10.3109/03630260903347526.
Thalassemia major is characterized by chronic ineffective erythropoiesis and anemia as its primary problems. These, in turn, produce physiologic adaptations in the cardiovascular system as well as pathologic/iatrogenic processes such as iron overload, splenectomy, nutritional deficiencies, chronic oxidative stress, and lung disease. This article discusses the pathophysiology of thalassemia as it relates to the cardiovascular system, the mechanisms and monitoring of iron cardiomyopathy, pulmonary hypertension, and vascular aging in thalassemia patients.
重型地中海贫血的主要特征是慢性无效红细胞生成和贫血。这些问题进而会在心血管系统中产生生理适应性变化,以及引发诸如铁过载、脾切除术、营养缺乏、慢性氧化应激和肺部疾病等病理/医源性过程。本文讨论了地中海贫血与心血管系统相关的病理生理学、铁性心肌病的机制和监测、地中海贫血患者的肺动脉高压和血管老化。