Uoc Centro Of Prevenzione Diagnosi E Cura Della Talassemia

Palermo, Italy

Uoc Centro Of Prevenzione Diagnosi E Cura Della Talassemia

Palermo, Italy
SEARCH FILTERS
Time filter
Source Type

Maggio A.,Cervello | Vitrano A.,University of Palermo | Lucania G.,Cervello | Capra M.,Uoc Centro Of Prevenzione Diagnosi E Cura Della Talassemia | And 16 more authors.
American Journal of Hematology | Year: 2012

A multicenter randomized open-label long-term sequential deferiprone-deferoxamine (DFP-DFO) versus DFP alone trial (sequential DFP-DFO) performed in patients with thalassemia major (TM) was retrospectively reanalyzed to assess the variation in the left ventricular ejection fraction (LVEF) [1].


Filosa A.,Uod Centro Per Le Microcitemie | Vitrano A.,University of Palermo | Rigano P.,Cervello | Calvaruso G.,Cervello | And 15 more authors.
Blood Cells, Molecules, and Diseases | Year: 2013

Transfusion and iron chelation treatment have significantly reduced morbidity and improved survival of patients with thalassemia major. However, cardiac disease continues to be the most common cause of death. We report the left-ventricular ejection fraction, determined by echocardiography, in one hundred sixty-eight patients with thalassemia major followed for at least 5. years who received continuous monotherapy with deferoxamine (N=108) or deferiprone (N=60). The statistical analysis, using the generalized estimating equations model, indicated that the group treated with deferiprone had a significantly better left-ventricular ejection fraction than did those treated with deferoxamine (coefficient 0.97; 95% CI 0.37; 1.6, p=0.002).The heart may be particularly sensitive to iron-induced mitochondrial damage because of the large number of mitochondria and its low level of antioxidants. Deferiprone, because of its lower molecular weight, might cross into heart mitochondria more efficiently, improving their activity and, thereby, myocardial cell function. Our findings indicate that the long-term administration of deferiprone significantly enhances left-ventricular function over time in comparison with deferoxamine treatment. However, because of limitations related to the design of this study, these findings should be confirmed in a prospective, randomized clinical trial. © 2013 Elsevier Inc.


Maggio A.,Cervello | Vitrano A.,University of Palermo | Vitrano A.,Cervello | Calvaruso G.,Cervello | And 30 more authors.
Blood Cells, Molecules, and Diseases | Year: 2013

Cardiac damage remains a major cause of mortality among patients with thalassemia major. The detection of a lower cardiac magnetic resonance T2* (CMR-T2*) signal has been suggested as a powerful predictor of the subsequent development of heart failure. However, the lack of worldwide availability of CMR-T2* facilities prevents its widespread use for follow-up evaluations of cardiac function in thalassemia major patients, warranting the need to assess the utility of other possible procedures. In this setting, the determination of left ventricular ejection fraction (LVEF) offers an accurate and reproducible method for heart function evaluation. These findings suggest a reduction in LVEF ≥ 7%, over time, determined by 2-D echocardiography, may be considered a strong predictive tool for the detection of thalassemia major patients with increased risk of cardiac death. The reduction of LVEF ≥ 7% had higher (84.76%) predictive value. Finally, Kaplan-Meier survival curves of thalassemia major patients with LVEF ≥ 7% showed a statistically significant decreased probability of survival for heart disease (p = 0.0022). However, because of limitations related to the study design, such findings should be confirmed in a large long-term prospective clinical trial. © 2012 Elsevier Inc.

Loading Uoc Centro Of Prevenzione Diagnosi E Cura Della Talassemia collaborators
Loading Uoc Centro Of Prevenzione Diagnosi E Cura Della Talassemia collaborators