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Combined chelation therapy in thalassemia major for the treatment of severe myocardial siderosis with left ventricular dysfunction.


ABSTRACT:

Background

In thalassemia major (TM), severe cardiac siderosis can be treated by continuous parenteral deferoxamine, but poor compliance, complications and deaths occur. Combined chelation therapy with deferiprone and deferoxamine is effective for moderate myocardial siderosis, but has not been prospectively examined in severe myocardial siderosis.

Methods

T2* cardiovascular magnetic resonance (CMR) was performed in 167 TM patients receiving standard subcutaneous deferoxamine monotherapy, and 22 had severe myocardial siderosis (T2* < 8 ms) with impaired left ventricular (LV) function. Fifteen of these patients received combination therapy with subcutaneous deferoxamine and oral deferiprone with CMR follow-up.

Results

At baseline, deferoxamine was prescribed at 38 +/-

SUBMITTER: Tanner MA 

PROVIDER: S-EPMC2289829 | biostudies-literature | 2008 Feb

REPOSITORIES: biostudies-literature