What regulates hepcidin in poly-transfused β-Thalassemia Major: Erythroid drive or store drive?
Background: Hepcidin, a key regulator of iron homeostasis, is increased by iron overload and inflammation 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 erythropoies...
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2014-01-01
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| Series: | Indian Journal of Pathology and Microbiology |
| Subjects: | |
| Online Access: | http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2014;volume=57;issue=1;spage=39;epage=42;aulast=Chauhan |
| _version_ | 1818254874076250112 |
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| author | Richa Chauhan Sunita Sharma Jagdish Chandra |
| author_facet | Richa Chauhan Sunita Sharma Jagdish Chandra |
| author_sort | Richa Chauhan |
| collection | DOAJ |
| description | Background: Hepcidin, a key regulator of iron homeostasis, is increased by iron overload and inflammation 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 coefficient was used to find 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 significant negative correlation with sTfR (r = -0.296, P < 0.0066). However, there was no correlation of hepcidin with ferritin. Ferritin and sTfR were significantly elevated in β-TM children compared to controls (P < 0.001). The mean serum hepcidin/ferritin index in the study group (0.00552) was significantly 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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| format | Article |
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| institution | Directory Open Access Journal |
| issn | 0377-4929 |
| language | English |
| last_indexed | 2024-12-12T17:02:54Z |
| publishDate | 2014-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Journal of Pathology and Microbiology |
| spelling | doaj.art-54cad6161e57400497066a5f0016f0242022-12-22T00:18:05ZengWolters Kluwer Medknow PublicationsIndian Journal of Pathology and Microbiology0377-49292014-01-01571394210.4103/0377-4929.130891What regulates hepcidin in poly-transfused β-Thalassemia Major: Erythroid drive or store drive?Richa ChauhanSunita SharmaJagdish ChandraBackground: Hepcidin, a key regulator of iron homeostasis, is increased by iron overload and inflammation 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 coefficient was used to find 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 significant negative correlation with sTfR (r = -0.296, P < 0.0066). However, there was no correlation of hepcidin with ferritin. Ferritin and sTfR were significantly elevated in β-TM children compared to controls (P < 0.001). The mean serum hepcidin/ferritin index in the study group (0.00552) was significantly 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.http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2014;volume=57;issue=1;spage=39;epage=42;aulast=Chauhanβ-Thalassemiahepcidiniron overload |
| spellingShingle | Richa Chauhan Sunita Sharma Jagdish Chandra What regulates hepcidin in poly-transfused β-Thalassemia Major: Erythroid drive or store drive? Indian Journal of Pathology and Microbiology β-Thalassemia hepcidin iron overload |
| title | What regulates hepcidin in poly-transfused β-Thalassemia Major: Erythroid drive or store drive? |
| title_full | What regulates hepcidin in poly-transfused β-Thalassemia Major: Erythroid drive or store drive? |
| title_fullStr | What regulates hepcidin in poly-transfused β-Thalassemia Major: Erythroid drive or store drive? |
| title_full_unstemmed | What regulates hepcidin in poly-transfused β-Thalassemia Major: Erythroid drive or store drive? |
| title_short | What regulates hepcidin in poly-transfused β-Thalassemia Major: Erythroid drive or store drive? |
| title_sort | what regulates hepcidin in poly transfused β thalassemia major erythroid drive or store drive |
| topic | β-Thalassemia hepcidin iron overload |
| url | http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2014;volume=57;issue=1;spage=39;epage=42;aulast=Chauhan |
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