Characterization of iron metabolism and erythropoiesis in erythrocyte membrane defects and thalassemia traits

Autor: Marian Hajduch, Zuzana Zidova, Jana Vrbkova, Lucie Sulovska, Monika Horvathova, Dusan Holub, Dagmar Pospisilova, Vladimír Mihál, Martina Divoka
Jazyk: angličtina
Rok vydání: 2016
Předmět:
0301 basic medicine
medicine.medical_specialty
Iron metabolism disorder
congenital
hereditary
and neonatal diseases and abnormalities

Adolescent
Iron
Population
lcsh:Medicine
Spherocytosis
Hereditary

General Biochemistry
Genetics and Molecular Biology

03 medical and health sciences
0302 clinical medicine
Hepcidins
Hepcidin
erythropoietic activity
Internal medicine
hemic and lymphatic diseases
medicine
Humans
Erythropoiesis
Child
education
Soluble transferrin receptor
Analysis of Variance
Hemostasis
education.field_of_study
biology
medicine.diagnostic_test
business.industry
Erythrocyte Membrane
lcsh:R
Elliptocytosis
Hereditary

Ferritin
030104 developmental biology
Endocrinology
thalassemia trait
Erythropoietin
Child
Preschool

biology.protein
Serum iron
Thalassemia
erythrocyte membrane defects
hepcidin
business
030215 immunology
medicine.drug
Zdroj: Biomedical Papers, Vol 160, Iss 2, Pp 231-237 (2016)
ISSN: 1804-7521
1213-8118
Popis: 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.
Databáze: OpenAIRE