What regulates hepcidin in poly-transfused β-Thalassemia Major: Erythroid drive or store drive.

Loading...
Thumbnail Image
Date
2014-01
Journal Title
Journal ISSN
Volume Title
Publisher
Abstract
Background: Hepcidin, a key regulator of iron homeostasis, is increased by iron overload and infl ammation while suppressed by hypoxia. In spite of iron overload in β-Thalassemia Major (β-TM), a paradoxical decrease in hepcidin is observed. Aim: To assess the opposing effects of enhanced erythropoiesis due to anemia and iron overloading on hepcidin in β-TM patients. Setting and Design: This prospective observational study was done at our tertiary care hospital. Materials and Methods: Eighty-three pediatric polytransfused (> 20 transfusions) patients of β-TM were compared with 70 children who served as controls. Serum assays for ferritin, transferrin receptors (sTfR) and hepcidin were performed. Statistical analysis: Independent Student t test was used to compare variables between both the groups. A Pearson correlation coeffi cient was used to fi nd any correlation between ferritin, sTfR and hepcidin. Results: The mean value of hepcidin in β-TM children was 13.88±10.68 ng/ml (range, 0.9-60 ng/ml) and showed signifi cant negative correlation with sTfR (r = –0.296, P < 0.0066). However, there was no correlation of hepcidin with ferritin. Ferritin and sTfR were signifi cantly elevated in β-TM children compared to controls (P < 0.001). The mean serum hepcidin/ferritin index in the study group (0.00552) was signifi cantly lower (P value < 0.001) than the controls (0.378) thus indicating inappropriate levels of hepcidin to iron overload. Conclusion: In polytransfused β-TM children increased iron demand dominates over iron overload in regulating hepcidin. In spite of excessive iron load, the inappropriate hepcidin levels may further contribute to iron overload enhancing iron toxicity.
Description
Keywords
β-Thalassemia, hepcidin, iron overload
Citation
Chauhan Richa, Sharma Sunita, Chandra Jagdish. What regulates hepcidin in poly-transfused β-Thalassemia Major: Erythroid drive or store drive. Indian Journal of Pathology & Microbiology. 2014 Jan-Mar 57 (1): 39-42.