الفهرس 
Thalassemia SyndromesOnly 14 pages are availabe for public view
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Abstract
Background: Hepcidin, a main regulator of iron hemostasis, can potentially be used as a marker for iron overload in patients with thalassemia; hence can be used to evaluate the efficacy of chelation therapy.
Aim: We hypothesize that serum hepcidin and hepcidin/ ferritin ratio might be better markers for iron load and iron load distribution characteristics in different thalassemia syndromes. Subsequently we aimed to estimate serum hepcidin, ferritin, and hepcidin: ferritin ratio in patients with beta-thalassemia syndromes.
Patients and Methods: A prospective study was conducted on 42 patients with beta-thalassemia major and 40 patients with beta-thalassemia intermedia compared to 20 age and gender-matched healthy controls. For all patients, serum ferritin, and hepcidin were measured by ELISA assay then the hepcidin /ferritin ratio was calculated.
Results: All patients (100%) with beta-thalassemia major and 26 patients (65%) with beta-thalassemia intermedia were transfusion dependent. Sixty-one patients (74%) were on regular iron chelators, 49 of them (80%) were on desafirox with a median dose o f 20 mg/kg, 40 patients (100%) of beta thalassemia intermedia were on hydroxyurea. Patients with thalassemia syndromes had higher mean serum ferritin (3000 ng/ml), median serum hepcidin of 162 ng/ml, lower mean hemoglobin (8.2 gm/dl) and hepcidin: ferritin ratio of 0.15 compared to control (P<0.001) who had median serum ferritin (73 ng/ml), median serum hepcidin of (62.8 ng/ml), mean hemoglobin (11.03 gm/dl) and hepcidin: ferritin ratio of 0.71. Beta thalassemia major patients had a higher median serum hepcidin level of 256 ng/ml, and a lower hepcidin / ferritin ratio of 0.18 compared to patients with beta-thalassemia intermedia (p<0.001, p<0.001 respectively). Hepcidin level was negatively correlated with the age of disease onset, frequency of trans fusion.
Conclusion: Serum hepcidin level was elevated in beta-thalassemia children with more evident elevation in beta-thalassemia major patients. The younger the age of onset of Thalassemia, the higher the need for transfusion, and the dose of desafirox, the higher was the hepcidin level.