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Physiopathology of Bone Modifications in β-Thalassemia
β-thalassemia major (βTM) or Cooley anemia is characterized by significantly reduced or absent synthesis of β-globin chains, which induces important pathologic consequences including hemolytic anemia, altered erythropoiesis, and bone marrow overstimulation. The pathogenesis of bone changes in patien...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi Publishing Corporation
2012
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3369393/ https://www.ncbi.nlm.nih.gov/pubmed/22693660 http://dx.doi.org/10.1155/2012/320737 |
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author | Perisano, Carlo Marzetti, Emanuele Spinelli, Maria Silvia Callà, Cinzia Anna Maria Graci, Calogero Maccauro, Giulio |
author_facet | Perisano, Carlo Marzetti, Emanuele Spinelli, Maria Silvia Callà, Cinzia Anna Maria Graci, Calogero Maccauro, Giulio |
author_sort | Perisano, Carlo |
collection | PubMed |
description | β-thalassemia major (βTM) or Cooley anemia is characterized by significantly reduced or absent synthesis of β-globin chains, which induces important pathologic consequences including hemolytic anemia, altered erythropoiesis, and bone marrow overstimulation. The pathogenesis of bone changes in patients with βTM is not yet completely understood. However, an unbalance in bone mineral turnover resulting from increased resorption and suppression of osteoblast activity has been detected in βTM patients. The abnormal regulation of bone metabolism may be related to hormonal and genetic factors, iron overload and iron chelation therapy, nutritional deficits, and decreased levels of physical activity. Here, we review the most recent findings on the physiopathology of bone abnormalities in βTM. Clinical presentation and radiological features of βTM-related bone changes are also discussed. |
format | Online Article Text |
id | pubmed-3369393 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2012 |
publisher | Hindawi Publishing Corporation |
record_format | MEDLINE/PubMed |
spelling | pubmed-33693932012-06-12 Physiopathology of Bone Modifications in β-Thalassemia Perisano, Carlo Marzetti, Emanuele Spinelli, Maria Silvia Callà, Cinzia Anna Maria Graci, Calogero Maccauro, Giulio Anemia Review Article β-thalassemia major (βTM) or Cooley anemia is characterized by significantly reduced or absent synthesis of β-globin chains, which induces important pathologic consequences including hemolytic anemia, altered erythropoiesis, and bone marrow overstimulation. The pathogenesis of bone changes in patients with βTM is not yet completely understood. However, an unbalance in bone mineral turnover resulting from increased resorption and suppression of osteoblast activity has been detected in βTM patients. The abnormal regulation of bone metabolism may be related to hormonal and genetic factors, iron overload and iron chelation therapy, nutritional deficits, and decreased levels of physical activity. Here, we review the most recent findings on the physiopathology of bone abnormalities in βTM. Clinical presentation and radiological features of βTM-related bone changes are also discussed. Hindawi Publishing Corporation 2012 2012-05-30 /pmc/articles/PMC3369393/ /pubmed/22693660 http://dx.doi.org/10.1155/2012/320737 Text en Copyright © 2012 Carlo Perisano et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Review Article Perisano, Carlo Marzetti, Emanuele Spinelli, Maria Silvia Callà, Cinzia Anna Maria Graci, Calogero Maccauro, Giulio Physiopathology of Bone Modifications in β-Thalassemia |
title | Physiopathology of Bone Modifications in β-Thalassemia |
title_full | Physiopathology of Bone Modifications in β-Thalassemia |
title_fullStr | Physiopathology of Bone Modifications in β-Thalassemia |
title_full_unstemmed | Physiopathology of Bone Modifications in β-Thalassemia |
title_short | Physiopathology of Bone Modifications in β-Thalassemia |
title_sort | physiopathology of bone modifications in β-thalassemia |
topic | Review Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3369393/ https://www.ncbi.nlm.nih.gov/pubmed/22693660 http://dx.doi.org/10.1155/2012/320737 |
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