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Successful chelation in beta-thalassemia major in the 21(st) century

This century has seen a revolution the management of beta-thalassemia major. Over a 12-year period to 2016, we aimed to analyze the benefits of such advances. In 209 patients, independent of the chelation regimen, ferritin, cardiac T2* and liver iron concentration changes were evaluated. We defined...

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Autores principales: Fragodimitri, Christina, Schiza, Vasiliki, Giakoumis, Anastasios, Drakaki, Kalliopi, Salichou, Anastasia, Karampatsos, Fotis, Yousef, Jacqueline, Karageorga, Markissia, Berdoukas, Vasili, Aessopos, Athanasios
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10578721/
https://www.ncbi.nlm.nih.gov/pubmed/37832083
http://dx.doi.org/10.1097/MD.0000000000035455
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author Fragodimitri, Christina
Schiza, Vasiliki
Giakoumis, Anastasios
Drakaki, Kalliopi
Salichou, Anastasia
Karampatsos, Fotis
Yousef, Jacqueline
Karageorga, Markissia
Berdoukas, Vasili
Aessopos, Athanasios
author_facet Fragodimitri, Christina
Schiza, Vasiliki
Giakoumis, Anastasios
Drakaki, Kalliopi
Salichou, Anastasia
Karampatsos, Fotis
Yousef, Jacqueline
Karageorga, Markissia
Berdoukas, Vasili
Aessopos, Athanasios
author_sort Fragodimitri, Christina
collection PubMed
description This century has seen a revolution the management of beta-thalassemia major. Over a 12-year period to 2016, we aimed to analyze the benefits of such advances. In 209 patients, independent of the chelation regimen, ferritin, cardiac T2* and liver iron concentration changes were evaluated. We defined chelation success (ChS) as no iron load in the heart and acceptable levels in the liver. Over 3 early magnetic resonance imagings, the same parameters were assessed in 2 subgroups, the only 2 that had sufficient patients continuing on 1 regimen and for a significant period of time, 1 on deferrioxamine (low iron load patients n = 41, Group A) and 1 on deferoxamine-deferiprone (iron overloaded n = 60, Group B). Finally, 28 deaths and causes were compared to those of an earlier period. The 209 patients significantly optimized those indices, while the number of patients with chelation success, increased from 6% to 51% (P < .0001). In group A, ChS after about 8 years increased from 21 to 46% (P = .006), while in Group B, from 0% to 60% (P < .001) after about 7 years. Deaths over the 2 periods showed significant reduction. Combined clearance of cardiac and liver iron (ChS) is feasible and should become the new target for all patients. This requires, serial magnetic resonance imagings and often prolonged intensified chelation for patients.
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spelling pubmed-105787212023-10-17 Successful chelation in beta-thalassemia major in the 21(st) century Fragodimitri, Christina Schiza, Vasiliki Giakoumis, Anastasios Drakaki, Kalliopi Salichou, Anastasia Karampatsos, Fotis Yousef, Jacqueline Karageorga, Markissia Berdoukas, Vasili Aessopos, Athanasios Medicine (Baltimore) 4800 This century has seen a revolution the management of beta-thalassemia major. Over a 12-year period to 2016, we aimed to analyze the benefits of such advances. In 209 patients, independent of the chelation regimen, ferritin, cardiac T2* and liver iron concentration changes were evaluated. We defined chelation success (ChS) as no iron load in the heart and acceptable levels in the liver. Over 3 early magnetic resonance imagings, the same parameters were assessed in 2 subgroups, the only 2 that had sufficient patients continuing on 1 regimen and for a significant period of time, 1 on deferrioxamine (low iron load patients n = 41, Group A) and 1 on deferoxamine-deferiprone (iron overloaded n = 60, Group B). Finally, 28 deaths and causes were compared to those of an earlier period. The 209 patients significantly optimized those indices, while the number of patients with chelation success, increased from 6% to 51% (P < .0001). In group A, ChS after about 8 years increased from 21 to 46% (P = .006), while in Group B, from 0% to 60% (P < .001) after about 7 years. Deaths over the 2 periods showed significant reduction. Combined clearance of cardiac and liver iron (ChS) is feasible and should become the new target for all patients. This requires, serial magnetic resonance imagings and often prolonged intensified chelation for patients. Lippincott Williams & Wilkins 2023-10-13 /pmc/articles/PMC10578721/ /pubmed/37832083 http://dx.doi.org/10.1097/MD.0000000000035455 Text en Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY) (https://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle 4800
Fragodimitri, Christina
Schiza, Vasiliki
Giakoumis, Anastasios
Drakaki, Kalliopi
Salichou, Anastasia
Karampatsos, Fotis
Yousef, Jacqueline
Karageorga, Markissia
Berdoukas, Vasili
Aessopos, Athanasios
Successful chelation in beta-thalassemia major in the 21(st) century
title Successful chelation in beta-thalassemia major in the 21(st) century
title_full Successful chelation in beta-thalassemia major in the 21(st) century
title_fullStr Successful chelation in beta-thalassemia major in the 21(st) century
title_full_unstemmed Successful chelation in beta-thalassemia major in the 21(st) century
title_short Successful chelation in beta-thalassemia major in the 21(st) century
title_sort successful chelation in beta-thalassemia major in the 21(st) century
topic 4800
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10578721/
https://www.ncbi.nlm.nih.gov/pubmed/37832083
http://dx.doi.org/10.1097/MD.0000000000035455
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