Hepatic and Cardiac Iron-load in Children on Long-term Chelation with Deferiprone for Thalassemia Major

Objective: To evaluate the efficacy of prolonged deferiprone monotherapy in patients with ?-thalassemia major. Methods: This cross-sectional study included 40 patients (age range 9 to38 years) with thalassemia major receiving deferiprone for ?5 years. Serum ferritin, andmyocardial iron concentration (MIC) and liver iron concentration (LIC) assessed by T2*MRIwere recorded. Results: The patients were receiving deferiprone for a mean (SD) duration of12.1 (4.7) years. The median (IQR) dose of deferiprone was 85 (74.3, 95) mg/kg/day. TheMIC was normal or had a mild, moderate or severe elevation in 29 (72.5%), 3 (7.5%), 3(7.5%), and 5 (12.5%) patients. The LIC was normal or had a mild, moderate or severeelevation in 2 (5%), 4 (10%), 11 (27.5%) and 23 (57.5%) patients. Conclusions: The majorityof patients receiving deferiprone had a moderate/severe hepatic but normal cardiac iron load.Prolonged deferiprone monotherapy was suboptimal for hepatic iron load in the majority.

Keywords

Cardiac failure, Hemosiderosis, Hepatic fibrosis, Oral iron chelators

Citation

Totadri Sidharth, Bansal Deepak, Trehan Amita, Khadwal Alka, Bhatia Anmol, Sodhi Kushaljit Singh, Bhatia Prateek, Jain Richa, Das Reena, Khandelwal Niranjan. Hepatic and Cardiac Iron-load in Children on Long-term Chelation with Deferiprone for Thalassemia Major. Indian Pediatrics. 2018 Jul ; 55(7): 573-575

URI

https://imsear.searo.who.int/handle/123456789/199172

Collections

Indian Pediatrics

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