Hepcidin is suppressed by erythropoiesis in hemoglobin E b-thalassemia and b-thalassemia trait
نویسندگان
چکیده
Medical Research Council, Weatherall Institute of Molecular Medicine, University of Oxford, Oxford, United Kingdom; College of Medicine, Swansea University, Swansea, United Kingdom; University College of London Cancer Institute, Department of Haematology, University College London, United Kingdom; University of Kelaniya, Colombo, Sri Lanka; National Thalassaemia Centre, District Hospital, Kurunegala, Sri Lanka; Department of Biochemistry and Immunology, Birmingham Heartlands Hospital, Birmingham, United Kingdom; University Health Network, University of Toronto, ON, Canada; and School of Physics, University of Western Australia, Crawley, Australia
منابع مشابه
Hepcidin is suppressed by erythropoiesis in hemoglobin E β-thalassemia and β-thalassemia trait.
Hemoglobin E (HbE) β-thalassemia is the most common severe thalassemia syndrome across Asia, and millions of people are carriers. Clinical heterogeneity in HbE β-thalassemia is incompletely explained by genotype, and the interaction of phenotypic variation with hepcidin is unknown. The effect of thalassemia carriage on hepcidin is also unknown, but it could be relevant for iron supplementation ...
متن کاملTransfusion suppresses erythropoiesis and increases hepcidin in adult patients with β-thalassemia major: a longitudinal study.
β-Thalassemia major causes ineffective erythropoiesis and chronic anemia and is associated with iron overload due to both transfused iron and increased iron absorption, the latter mediated by suppression of the iron-regulatory hormone hepcidin. We sought to determine whether, in β-thalassemia major, transfusion-mediated inhibition of erythropoiesis dynamically affects hepcidin. We recruited 31 ...
متن کاملβ-thalassemia: a model for elucidating the dynamic regulation of ineffective erythropoiesis and iron metabolism.
β-thalassemia is a disease characterized by anemia and is associated with ineffective erythropoiesis and iron dysregulation resulting in iron overload. The peptide hormone hepcidin regulates iron metabolism, and insufficient hepcidin synthesis is responsible for iron overload in minimally transfused patients with this disease. Understanding the crosstalk between erythropoiesis and iron metaboli...
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Background: Beta thalassemia gene mutations are among common mutations in southwest Iran. However, Hemoglobin E (Hb E) and Hb E/β⁰ thalassemia account for a small number of hemoglobinopathies in Iran. This is the first study to directly address the existence of Hb E and consequently Hb E/β⁰ thalassemia in southwest Iran. Methods: This retrospective study discovered seven cases of Hb E/β⁰ thal...
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