title: What regulates hepcidin in poly-transfused β-Thalassemia Major: erythroid drive or store drive?
abstract: 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.
  
  
  OBJECTIVE
  To assess the opposing effects of enhanced erythropoiesis due to anemia and iron overloading on hepcidin in β-TM patients.
  
  
  METHODS
  This prospective observational study was done at our tertiary care hospital.
  
  
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pathology, Lady Hardinge Medical College and Kalawati Saran Children's Hospital,New Delhi, India.
authors.forename: Richa
authors.initials: R
authors.lastname: Chauhan
authors.forename: Sunita
authors.initials: S
authors.lastname: Sharma
authors.forename: Jagdish
authors.initials: J
authors.lastname: Chandra
mesh_terms: D000293:Adolescent; D001803:Blood Transfusion; D002648:Child; D002675:Child, Preschool; D005260:Female; D005786:Gene Expression Regulation; D064451:Hepcidins / Q000097:blood*; D006801:Humans; D007501:Iron / Q000378:metabolism*; D008297:Male; D017086:beta-Thalassemia / Q000628:therapy*
chemical_list: D064451:Hepcidins; D007501:Iron