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PedAM

Pediatric Disease Annotations & Medicines




Disease thalassemia
Symptom C0037061|siderosis
Sentences 6
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- 21282714 Deferiprone or deferasirox for cardiac siderosis in beta-thalassemia major.
PubMedID- 23949317 Myocardial siderosis in thalassemia major remains the leading cause of death in developing countries.
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- 26177199 Myocardial siderosis in beta-thalassemia major (beta-tm) remains the leading cause of death.

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