الفهرس 
BETA-THALASSEMIA MAJOROnly 14 pages are availabe for public view
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Abstract
Background: Beta thalassemia major is a common chronic hemolytic anemia. Cardiopulmonary compromisations are attributed to iron overload and anemia .
Aim: To compare cardiopulmonary functions in beta thalassemia major patients on different iron chelators.
Patients and methods: Sixty patients were recruited from Pediatric Hematology Clinic, Ain Shams University. They were subclassified according to their received iron chelator into: Group 1(on Desferrioxamine), group 2 (on Deferiprone) and group 3(on Deferasirox). All patients were subjected to full history, thorough clinical examination and routine laboratory investigations. ECG, echocardiography, MRI-T2*, CT chest and spirometric pulmonary functions (FEV 1, FVC, FEV 1/ FVC ratio and FEF25-75%) were performed initially and followed up after one year.
Results: Compliance was significantly higher in deferasirox group than in deferiprone and desferrioxamine groups. FEV 1, FVC , FEV 1/FVC ratio and FEF25-75% did not show significant difference between the studied groups.During the study entry, mean FEV 1 was 1.670.56, 1.7±0.57 and 1.72±0.57 liters in DFO, DFP and DFX groups respectively. Mean FVC was 2±0.67, 2.01±0.67 and 2.03±0.68 liters in DFO, DFP and DFX groups respectively. Mean FEV 1/FVC ratio was83±27.67, 84.50±28.17 and 84.70±28.23 % in DFO, DFP and DFX groups respectively. Mean FEF25-75% was 1.70±0.57, 1.82±0.61 and 1.78±0.59 liters/sec. in DFO, DFP and DFX respectively. During the study end, mean FEV 1 was 1.65±0.55, 1.76±0.59 and 1.85±0.62 liters in DFO, DFP and DFX groups respectively. Mean FVC was 1.99±0.66, 2.04±0.68 and 2.10±0.70 liters in DFO, DFP and . DFX groups respectively.. Mean FEV 1/ FVC ratio was 82±27.33, 85.2±28.40 and87.3±29.10 liters in DFO, DFP and DFX groups respectively. Mean FEF25-75% was 1.68±0.56, 1.84±0.61 and 1.86±0.62 liters in DFO, DFP and DFX groups respectively.
Mean serum ferritin levels were initially 3726.8±2079.8 (range, 1118.9-10119.7 ng/ml), 2971.7±2271 (range, 912-11170 ng/ml ) and 3242± 2323.1 (range, 421-9000 ng/ml) ng/ml in desferrioxamine, deferiprone and deferasirox groups, respectively. After one year follow up, mean ferritin levels were 2992.5±1873.3 (range, 690.5-7500 ng/ml), 2825. ±2478.8 (range, 2321-13881.5 ng/ml) and 2283.8±1502 (range, 740-6076.8 ng/ml) in DFO, DFP and DFX groups respectively, denoting the significant decrease of ferritin levels in DFX group. Mean T2* levels were initially 24.8±5 (range, 12-33 mesc), 22.5±7.1 (range,12-40 msec) and 21.5±2.6 (range, 16-29 msec) in DFO, DFP and DFX groups respectively. After one year follow up, they became 25±5.7 (range, 15-33 msec), 23±6.8 9 (range, 11-33msec) and 24.4±4.4 (range, 20-38 msec) in DFO. DFP and DFX groups respectively, which indicates the significant improvement of T2* levels in DFX group. Mean study-end ferritin level was not significantly correlated with neither mean T2* level, nor pulmonary function tests. Mean LVEF was 4 (range, 51-70%) in DFO group; mean LVEF was (range, 53-73%) in DFP group and mean LVEF was 8 (range, 52-76%) in DFX group. Meanwhile, at study-end, mean LVEF was (range, 50-68%) in DFO group; mean LVEF was (range, 54-75%) in DFP group and mean LVEF was 22.7 (range, 57-77%) in DFX group. No significant correlation was found between cardiac T2* and mean LVEF at study end.
Conclusion: Deferasirox was the iron chelator of the best compliance. FVC, FEV 1, FEV 1/FVC ratio and FEF25-75% did not differ among the 3 groups. Deferasirox was the most effective in improvement of mean serum ferritin and mean T2* values in thalassemics.