Evidence for a novel mechanism independent of myocardial iron in β-thalassemia cardiac pathogenesis.

ABSTRACT: Human β-thalassemia major is one of the most prevalent genetic diseases characterized by decrease/absence of β-globin chain production with reduction of erythrocyte number. The main cause of death of treated β-thalassemia major patients with chronic blood transfusion is early cardiac complications that have been attributed to secondary iron overload despite optimal chelation. Herein, we investigated pathophysiological mechanisms of cardiovascular dysfunction in a severe murine model of β-thalassemia from 6 to 15-months of age in the absence of confounding effects related to transfusion. Our longitudinal echocardiography analysis showed that β-thalassemic mice first display a significant increase of cardiac output in response to limited oxygen-carrying erythrocytes that progressed rapidly t

SUBMITTER: Stoyanova E 

PROVIDER: S-EPMC3524169 | biostudies-literature | 2012

REPOSITORIES: biostudies-literature