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Talasemi Majör ve İntermedialı Çocuklarda Serum Prohepsidin Düzeyleri

Year 2017, Volume: 18 Issue: 1, 8 - 12, 01.04.2017

Abstract

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References

  • 1. Saliba AN, Harb AR, Taher AT. Iron chelation therapy in transfusion-dependent thalassemia patients: current strategies and future directions. Blood Med 2015; 6: 197-209.
  • 2. Gardenghi S, Grady RW, Rivella S. Anemia, ineffective erythropoiesis, and hepcidin: interacting factors in abnormal iron metabolism leading to iron overload in β-thalassemia. Hematol Oncol Clin North Am 2010; 24: 1089-107.
  • 3. Taher A, Hershko C, Cappellini MD. Iron overload in thalassaemia intermedia: reassessment of iron chelation strategies. Br J Haematol 2009; 147: 634-40.
  • 4. Nemeth E. Hepcidin and β-thalassemia major. Blood 2013; 122: 3-4.
  • 5. Rishi G, Wallace DF, Subramaniam VN. Hepcidin: regulation of the master iron regulator. Biosci Rep 2015; 35: 1-12.
  • 6. Kim A, Nemeth E. New insights into iron regulation and erythropoiesis. Curr Opin Hematol 2015; 22: 199-205.
  • 7. Musallam KM, Cappellini MD, Wood JC, Taher AT. Iron overload in non-transfusion-dependent thalassemia: a clinical perspective. Blood Rev 2012; 26(Suppl 1): S16-9.
  • 8. Nicolas G, Bennoun M, Porteu A, Mativet S, Beaumont C, Grandchamp B, et al. Severe iron deficiency anemia in transgenic mice expressing liver hepcidin. Proc Natl Acad Sci USA 2002; 99: 4596-601.
  • 9. Frazer DM, Inglis HR, Wilkins SJ, Millard KN, Steele TM, McLaren GD, et al. Delayed hepcidin response explains the lag period in iron absorption following a stimulus to increase erythropoiesis. Gut 2004; 53: 1509-15.
  • 10. Uysal Z. Hepsidin ve demir metabolizması. 33. Ulusal hematoloji kongresi kitabı, Ankara, 2007; 9-15.
  • 11. Kulaksiz H, Gehrke SG, Janetzko A, Rost D, Bruckner T, Kallinowski B, et al. Pro-hepcidin: expression and cell specific localisation in the liver and its regulation in hereditary haemochromatosis, chronic renal insufficiency, and renal anaemia. Gut 2004; 53: 735-43.
  • 12. Kattamis A, Papassotiriou I, Palaiologou D, Galani FAA, Ladis V, Sakellaropoulos N, et al. The effects of erythropoetic activity and iron burden on hepcidin expression in patients with thalassemia major. Haematologica 2006; 91: 809-12.
  • 13. Adamsky K, Weizer O, Amariglio N, Breda L, Harmelin A, Rivella S, et al. Decreased hepcidin mRNA expression in thalassemic mice. Br J Haematol 2004; 124: 123-4.
  • 14. Franceschi LD, Daraio F, Filippini A, Carturan S, Muchitsch EM, Roetto A, et al. Liver expression of hepcidin and other iron genes in two mouse models of beta thalassemia. Haematolgica 2006; 91: 336-42.
  • 15. Vokurka M, Krijt J, Sulc K, Necas E. Hepcidin mRNA levels in mouse liver respond to inhibition of erythropoiesis. Physiol Res 2006; 55: 667-74.
  • 16. Camberlein E, Zanninelli G, Detivaud L, Lizzi AR, Sorrentino F, Vacquer S, et al. Anemia in beta-thalassemia patients targets hepatic hepcidin transcript levels independently of iron metabolism genes controlling hepcidin expression. Haematologica 2008; 93: 111-5.
  • 17. Weizer-Stern O, Adamsky A, Amariglio N, Rachmilewitz E, Breda L, Rivella S, et al. mRNA expression of iron regulatory genes in beta-thalassemia intermedia and beta-thalassemia major mouse models. Am J Hematol 2006; 81: 479-83.
  • 18. Papanikolaou G, Tzilianos M, Christakis JI, Bogdanos D, Tsimirika K, MacFarlane J, et al. Hepcidin in iron overload disorders. Blood 2005; 105: 4103-5.
  • 19. Roe MA, Spinks C, Heath AL, Harvey LJ, Foxall R, Wimperis J, et al. Serum prohepcidin concentration: no association with iron absorption in healthy men; and no relationship with ironstatus in men carrying HFE mutations, hereditary haemochromatosis patients undergoing phlebotomy treatment, or pregnant women. Br J Nutr 2007; 97: 544-9.
  • 20. Ezeh C, Ugochukwu CC, Weinstein J, Okpala I. Hepcidin, haemoglobin and ferritin levels in sickle cell anaemia. Eur J Haematol 2005; 74: 86-8.
  • 21. Ulukol B, Orhon FS, Hanoluk A, Akar N. Serum pro-hepcidin levels and relationship with ferritin in healthy non-anaemic infants. Acta Haematol 2007; 118: 70-2. Epub 2007 May 24.
  • 22. Tsuchihashi D, Abe T, Kobama H, Fujii H, Hamada Y, Nii-Kono T, et al. Serum prohepcidin as an indicator of iron status in dialysis patients. Ther Apher Dial 2008; 12: 226-31.
  • 23. Kijima H, Sawada T, Tomosugi N, Kubota K. Expression of hepcidin mRNA is uniformly suppressed in hepatocellular carcinoma. BMC Cancer 2008; 8: 67.

Serum Prohepcidin Levels in Children with Thalassemia Major and Intermedia

Year 2017, Volume: 18 Issue: 1, 8 - 12, 01.04.2017

Abstract

Objective: To determine the role of hepcidin hormone levels in iron accumulation in patients with thalassemia major (TM) and thalassemia intermedia (TI). Materials and Methods: Serum prohepcidin and ferritin levels were determined in 34 patient with TM, 10 patient with TI, who attended the Department of Pediatric Hematology Adnan Menderes University Medical Faculty and the Department of Pediatrics at Aydın Atatürk State Hospital between 1 September 2006 and 30 September 2007 and 40 control patients without infection/inflammation, hepatitis or liver failure. Serum prohepcidin levels were measured using a commercial enzyme-linked immunosorbent assay kit (DRG International, Inc. Marburg, Germany); ferritin was studied with chemiluminescence method (Immulite 2000 DPC). Results: Mean serum ferritin levels in TM, TI and control groups were 2347.97±1724.81 ng/mL (range: 144-8015 ng/mL), 1352.40±918.94 ng/mL (range: 311-3109 ng/mL), and 33.35±12.03 ng/mL (range: 20-69.1 ng/mL), respectively. Serum prohepcidin levels in the same groups were 221.78±74.38 ng/mL (range: 7l.14-446.57 ng/mL), 173.31±52.14 ng/mL (range: 100.83-267.69 ng/mL), and 218.20±50.37 ng/mL (range: 116.18-330.43 ng/mL), respectively. There was a statistically significant difference in prohepcidin levels between patients with TI and control group only (p=0.016). No correlation was found between prohepcidin and ferritin levels in all groups (r=-0.023, p=0.839). Conclusion: Low levels of prohepcidin in patients with TI may be related to increased erythropoietic activity. Prohepcidin can be an indicator of active erythropoiesis.

References

  • 1. Saliba AN, Harb AR, Taher AT. Iron chelation therapy in transfusion-dependent thalassemia patients: current strategies and future directions. Blood Med 2015; 6: 197-209.
  • 2. Gardenghi S, Grady RW, Rivella S. Anemia, ineffective erythropoiesis, and hepcidin: interacting factors in abnormal iron metabolism leading to iron overload in β-thalassemia. Hematol Oncol Clin North Am 2010; 24: 1089-107.
  • 3. Taher A, Hershko C, Cappellini MD. Iron overload in thalassaemia intermedia: reassessment of iron chelation strategies. Br J Haematol 2009; 147: 634-40.
  • 4. Nemeth E. Hepcidin and β-thalassemia major. Blood 2013; 122: 3-4.
  • 5. Rishi G, Wallace DF, Subramaniam VN. Hepcidin: regulation of the master iron regulator. Biosci Rep 2015; 35: 1-12.
  • 6. Kim A, Nemeth E. New insights into iron regulation and erythropoiesis. Curr Opin Hematol 2015; 22: 199-205.
  • 7. Musallam KM, Cappellini MD, Wood JC, Taher AT. Iron overload in non-transfusion-dependent thalassemia: a clinical perspective. Blood Rev 2012; 26(Suppl 1): S16-9.
  • 8. Nicolas G, Bennoun M, Porteu A, Mativet S, Beaumont C, Grandchamp B, et al. Severe iron deficiency anemia in transgenic mice expressing liver hepcidin. Proc Natl Acad Sci USA 2002; 99: 4596-601.
  • 9. Frazer DM, Inglis HR, Wilkins SJ, Millard KN, Steele TM, McLaren GD, et al. Delayed hepcidin response explains the lag period in iron absorption following a stimulus to increase erythropoiesis. Gut 2004; 53: 1509-15.
  • 10. Uysal Z. Hepsidin ve demir metabolizması. 33. Ulusal hematoloji kongresi kitabı, Ankara, 2007; 9-15.
  • 11. Kulaksiz H, Gehrke SG, Janetzko A, Rost D, Bruckner T, Kallinowski B, et al. Pro-hepcidin: expression and cell specific localisation in the liver and its regulation in hereditary haemochromatosis, chronic renal insufficiency, and renal anaemia. Gut 2004; 53: 735-43.
  • 12. Kattamis A, Papassotiriou I, Palaiologou D, Galani FAA, Ladis V, Sakellaropoulos N, et al. The effects of erythropoetic activity and iron burden on hepcidin expression in patients with thalassemia major. Haematologica 2006; 91: 809-12.
  • 13. Adamsky K, Weizer O, Amariglio N, Breda L, Harmelin A, Rivella S, et al. Decreased hepcidin mRNA expression in thalassemic mice. Br J Haematol 2004; 124: 123-4.
  • 14. Franceschi LD, Daraio F, Filippini A, Carturan S, Muchitsch EM, Roetto A, et al. Liver expression of hepcidin and other iron genes in two mouse models of beta thalassemia. Haematolgica 2006; 91: 336-42.
  • 15. Vokurka M, Krijt J, Sulc K, Necas E. Hepcidin mRNA levels in mouse liver respond to inhibition of erythropoiesis. Physiol Res 2006; 55: 667-74.
  • 16. Camberlein E, Zanninelli G, Detivaud L, Lizzi AR, Sorrentino F, Vacquer S, et al. Anemia in beta-thalassemia patients targets hepatic hepcidin transcript levels independently of iron metabolism genes controlling hepcidin expression. Haematologica 2008; 93: 111-5.
  • 17. Weizer-Stern O, Adamsky A, Amariglio N, Rachmilewitz E, Breda L, Rivella S, et al. mRNA expression of iron regulatory genes in beta-thalassemia intermedia and beta-thalassemia major mouse models. Am J Hematol 2006; 81: 479-83.
  • 18. Papanikolaou G, Tzilianos M, Christakis JI, Bogdanos D, Tsimirika K, MacFarlane J, et al. Hepcidin in iron overload disorders. Blood 2005; 105: 4103-5.
  • 19. Roe MA, Spinks C, Heath AL, Harvey LJ, Foxall R, Wimperis J, et al. Serum prohepcidin concentration: no association with iron absorption in healthy men; and no relationship with ironstatus in men carrying HFE mutations, hereditary haemochromatosis patients undergoing phlebotomy treatment, or pregnant women. Br J Nutr 2007; 97: 544-9.
  • 20. Ezeh C, Ugochukwu CC, Weinstein J, Okpala I. Hepcidin, haemoglobin and ferritin levels in sickle cell anaemia. Eur J Haematol 2005; 74: 86-8.
  • 21. Ulukol B, Orhon FS, Hanoluk A, Akar N. Serum pro-hepcidin levels and relationship with ferritin in healthy non-anaemic infants. Acta Haematol 2007; 118: 70-2. Epub 2007 May 24.
  • 22. Tsuchihashi D, Abe T, Kobama H, Fujii H, Hamada Y, Nii-Kono T, et al. Serum prohepcidin as an indicator of iron status in dialysis patients. Ther Apher Dial 2008; 12: 226-31.
  • 23. Kijima H, Sawada T, Tomosugi N, Kubota K. Expression of hepcidin mRNA is uniformly suppressed in hepatocellular carcinoma. BMC Cancer 2008; 8: 67.
There are 23 citations in total.

Details

Other ID JA63AY56BC
Journal Section Research Article
Authors

Celil Yılmaz This is me

Yusuf Ziya Aral This is me

Aslıhan Karul This is me

Leyla Didem Kozacı This is me

Publication Date April 1, 2017
Published in Issue Year 2017 Volume: 18 Issue: 1

Cite

EndNote Yılmaz C, Aral YZ, Karul A, Kozacı LD (April 1, 2017) Serum Prohepcidin Levels in Children with Thalassemia Major and Intermedia. Meandros Medical And Dental Journal 18 1 8–12.