eRAM
encyclopedia of Rare Disease Annotation for Precision Medicine
| Disease | thalassemia |
| Comorbidity | C0037061|siderosis |
| Sentences | 8 |
| PubMedID- 26177199 | Myocardial siderosis in beta-thalassemia major (beta-tm) remains the leading cause of death. |
| PubMedID- 21772954 | Clinical data support the efficacy of dfx in removing cardiac iron and preventing myocardial siderosis in patients with thalassemia major.67,115,116 in a one year prospective multicenter trial (epic cardiac substudy), dfx significantly improved geometric mean myocardial t2* from baseline 11.2 to 12.9 ms (+16%, p<0.0001), with significant concomitant reduction in both serum ferritin and lic.111 the two years extension of the same study showed continued improvement in myocardial t2* from the baseline 11.2 to 14.8 ms (p<0.001). |
| PubMedID- 25295007 | Randomized controlled trials of iron chelators for the treatment of cardiac siderosis in thalassaemia major. |
| PubMedID- 21282714 | Deferiprone or deferasirox for cardiac siderosis in beta-thalassemia major. |
| PubMedID- 24385534 | Cordelia was a prospective, randomized comparison of deferasirox (target dose 40 mg/kg per day) vs subcutaneous deferoxamine (50-60 mg/kg per day for 5-7 days/week) for myocardial iron removal in 197 beta-thalassemia major patients with myocardial siderosis (t2* 6-20 milliseconds) and no signs of cardiac dysfunction (mean age, 19.8 years). |
| PubMedID- 21647857 | thalassemia major patients with myocardial siderosis may have lvef values within normal limits, but this result must be interpreted cautiously. |
| PubMedID- 24976388 | Deferasirox for cardiac siderosis in beta-thalassaemia major: a multicentre, open label, prospective study. |
| PubMedID- 23949317 | Myocardial siderosis in thalassemia major remains the leading cause of death in developing countries. |
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