| Thalassemia intermedia is associated with a proatherogenic biochemical phenotype. | |
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MedLine Citation:
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PMID: 21402483 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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OBJECTIVE: Unlike beta thalassemia major (β-TM) in which cardiac siderosis represents the leading cause of mortality and morbidity, in beta thalassemia intermedia (β-TI), pulmonary hypertension (PHT) and thrombosis seems to be the major cardiovascular complications. However, the mechanism underlying these complications in β-TI is still unclear. Endothelial dysfunction, the key early event in atherogenesis, is now emerging as an important cardiovascular risk determiner in β-TI patients. Among the factors known to affect endothelial function, iron and cholesterol merit particular consideration in β-TI patients. Therefore, with the aim to extend our knowledge on the mechanisms connecting atherosclerosis to β-TI, in this study, we compared lipid and iron metabolism in serum and in peripheral blood mononuclear cells (PBMCs) from β-TI and β-TM patients and controls. METHODS AND RESULTS: In this study the iron status and the lipid profile in serum and in peripheral blood mononuclear cells (PBMCs) of 22 adult β-TI patients were examined, and compared with 70 adult β-TM, and 50 age-matched controls. Despite the great variability, levels of serum iron and transferrin saturation were significantly higher in β-TI compared to both controls and β-TM. By contrast, transferrin and hepcidin levels were lower in β-TI patients. Changes in serum indicators in β-TI patients were associated with altered expressions in PBMCs of hepcidin and IL-1α, involved in some way in the regulation of iron homeostasis. In addition β-TI exhibited a reduction of total and high density lipoprotein cholesterol in serum and of neutral cholesterol ester hydrolase in PBMCs, and an increase of cytoplasmic neutral lipids and mRNA levels of acetyl-coenzymeA:cholesterol acyltransferase. CONCLUSIONS: Taken together, these findings provide experimental support for the idea that β-TI patients exhibit a proatherogenic biochemical phenotype which may contribute to enhance cardiovascular risk in these subjects. |
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Authors:
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Maria Eliana Lai; Stefania Vacquer; Maria Paola Carta; Alessandra Spiga; Pierluigi Cocco; Fabrizio Angius; Antonella Mandas; Sandra Dessì |
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Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-3-12 |
Journal Detail:
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Title: Blood cells, molecules & diseases Volume: - ISSN: 1096-0961 ISO Abbreviation: - Publication Date: 2011 Mar |
Date Detail:
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Created Date: 2011-3-15 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9509932 Medline TA: Blood Cells Mol Dis Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Copyright Information:
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Copyright © 2011 Elsevier Inc. All rights reserved. |
Affiliation:
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Department of Internal Medical Sciences, University of Cagliari, SS 554 Km 4.5, 09042 Monserrato, Cagliari, Italy. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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