What regulates hepcidin in poly-transfused β-Thalassemia Major: Erythroid drive or store drive.
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Date
2014-01
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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.
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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.