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Evaluation of the efficacy of oral deferiprone in β-thalassemia major by multislice multiecho T2*

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Pepe A, Lombardi M, Positano V, Cracolici E, Capra M, Malizia R, Prossomariti L, De Marchi D, Midiri M, Maggio A. Evaluation of the efficacy of oral deferiprone in -thalassemia major by multislice multiecho T2*.

Eur J Haematol 2005. © Blackwell Munksgaard 2005. Abstract: 

Objectives: Oral deferiprone (L1) appears to be promising in the treatment of β-thalassemia major (TM) patients. T2* magnetic resonance imaging (MRI) with a single measurement in the mid-ventricular septum was validated as a quantitative evaluation of myocardial iron overload. Previous studies suggested a marked heterogeneity of iron distribution in the myocardium. We set up a multislice multiecho T2* MRI for the detection of this heterogeneity. The aim of our study was to investigate differences between the L1 vs. the subcutaneous desferrioxamine (DF)-treated patients using this new approach.Methods: Thirty-six β-TM patients (age 29 ± 8 yr) underwent MRI. Eighteen patients received long-term L1, and 18 other patients matched for age and sex received DF. T2* multiecho sequences on three short axis views of the left ventricle were obtained and analyzed by custom-made software. In each slice, the myocardium was automatically segmented into four segments. Cine-dynamic images were also obtained to evaluate biventricular function.Results: For multislice T2* technique, the coefficient of variation for intra- and inter-observer, and inter-study reproducibility was 3.9%, 4.7%, and 5.5%, respectively. The global heart T2* value was significantly higher in the L1 vs. DF group (35 ± 7 vs. 27 ± 2 ms; P = 0.02). The number of segments with normal T2* value (>20 ms) was significantly higher in the L1 vs. the DF group (11 ± 1 vs. 8 ± 5 segments; P = 0.03). We did not detect significant differences in biventricular function parameters.Conclusions: This new approach confirms that L1 could be more effective than DF in removal of myocardial iron.
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Keywords: chelation treatment; iron overload; magnetic resonance imaging; multislice multiecho T2*; β-thalassemia

Document Type: Research Article

Affiliations: 1: MRI Laboratory, Institute of Clinical Physiology, CNR, Pisa, Italy 2: Department of Radiology, University of Palermo, Palermo, Italy 3: Pediatria per le Emopatie Ereditarie, G. Di Cristina Hospital ARNAS, Palermo, Italy 4: Pediatria II per le Emopatie Ereditarie, Villa Sofia-CTO Hospital, Palermo, Italy 5: Centro per la Cura delle Microcitemie, Cardarelli Hospital, Napoli, Italy 6: Ematologia II con Talassemia, V. Cervello Hospital, Palermo, Italy

Publication date: March 1, 2006

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