Characterization of iron metabolism and erythropoiesis in erythrocyte membrane defects and thalassemia traits
Language English Country Czech Republic Media print-electronic
Document type Journal Article
PubMed
26592557
DOI
10.5507/bp.2015.054
Knihovny.cz E-resources
- Keywords
- erythrocyte membrane defects, erythropoietic activity, hepcidin, thalassemia trait,
- MeSH
- Analysis of Variance MeSH
- Elliptocytosis, Hereditary blood MeSH
- Spherocytosis, Hereditary blood MeSH
- Child MeSH
- Erythrocyte Membrane metabolism MeSH
- Erythropoiesis physiology MeSH
- Hemostasis physiology MeSH
- Hepcidins metabolism MeSH
- Humans MeSH
- Adolescent MeSH
- Child, Preschool MeSH
- Thalassemia metabolism MeSH
- Iron metabolism MeSH
- Check Tag
- Child MeSH
- Humans MeSH
- Adolescent MeSH
- Child, Preschool MeSH
- Publication type
- Journal Article MeSH
- Names of Substances
- Hepcidins MeSH
- Iron MeSH
BACKGROUND AND AIMS: 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. CONCLUSION: 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.
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