title: Characterization of iron metabolism and erythropoiesis in erythrocyte membrane defects and thalassemia traits.
abstract: OBJECTIVE
  Erythropoiesis is closely related to iron metabolism in a balanced homeostasis. Analyses of diverse erythroid and iron metabolism disorders have shown that disrupted erythropoiesis negatively affects iron homeostasis and vice versa. The aim of this study was to characterize the relationship between erythropoietic activity and iron homeostasis in pediatric patients with erythrocyte membrane defects and thalassemia traits.
  
  
  METHODS
  Selected markers of erythropoietic activity (erythropoietin, soluble transferrin receptor - sTfR and growth differentiation factor 15) and iron status parameters (serum iron, ferritin and hepcidin) were evaluated in pediatric patients with erythrocyte membrane defects and thalassemia traits.
  
  
  RESULTS
  The patients with erythrocyte membrane defects and thalassemia traits had altered iron homeostasis due to disturbed erythropoiesis. In comparison with healthy controls, they had a normal to low hepcidin/ferritin ratio and concomitantly elevated sTfR.
  
  
  CONCLUSIONS
  The findings suggest that pediatric patients with erythrocyte membrane defects and thalassemia traits are more susceptible to iron overload than the general population and that the (hepcidin/ferritin)/sTfR ratio can be used to monitor any worsening of the disease.
authors.affiliation: Department of Pediatrics, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech Republic.
authors.forename: Lucie
authors.initials: L
authors.lastname: Sulovska
authors.affiliation: Institute of Molecular and Translational Medicine, Faculty of Medicine and Dentistry, Palacky University Olomouc, Czech Republic.
authors.forename: Dusan
authors.initials: D
authors.lastname: Holub
authors.affiliation: Department of Biology, Faculty of Medicine and Dentistry, Palacky University Olomouc, Czech Republic.
authors.forename: Zuzana
authors.initials: Z
authors.lastname: Zidova
authors.affiliation: Department of Hemato-oncology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech Republic.
authors.forename: Martina
authors.initials: M
authors.lastname: Divoka
authors.affiliation: Institute of Molecular and Translational Medicine, Faculty of Medicine and Dentistry, Palacky University Olomouc, Czech Republic.
authors.forename: Marian
authors.initials: M
authors.lastname: Hajduch
authors.affiliation: Department of Pediatrics, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech Republic.
authors.forename: Vladimir
authors.initials: V
authors.lastname: Mihal
authors.affiliation: Institute of Molecular and Translational Medicine, Faculty of Medicine and Dentistry, Palacky University Olomouc, Czech Republic.
authors.forename: Jana
authors.initials: J
authors.lastname: Vrbkova
authors.affiliation: Department of Biology, Faculty of Medicine and Dentistry, Palacky University Olomouc, Czech Republic.
authors.forename: Monika
authors.initials: M
authors.lastname: Horvathova
authors.affiliation: Department of Pediatrics, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech Republic.
authors.forename: Dagmar
authors.initials: D
authors.lastname: Pospisilova
mesh_terms: D000293:Adolescent; D000704:Analysis of Variance; D002648:Child; D002675:Child, Preschool; D004612:Elliptocytosis, Hereditary / Q000097:blood*; D004910:Erythrocyte Membrane / Q000378:metabolism*; D004920:Erythropoiesis / Q000502:physiology*; D006487:Hemostasis / Q000502:physiology; D064451:Hepcidins / Q000378:metabolism; D006801:Humans; D007501:Iron / Q000378:metabolism*; D013103:Spherocytosis, Hereditary / Q000097:blood*; D013789:Thalassemia / Q000378:metabolism*
chemical_list: D064451:Hepcidins; D007501:Iron
keywords: erythrocyte membrane defects; erythropoietic activity; hepcidin; thalassemia trait