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Transient Elastography (TE) is a Useful Tool for Assessing the Response of Liver Iron Chelation in Sickle Cell Disease Patients

Sickle cell disease patients often need regular blood transfusions to improve both the quality of life and survival from the veno-occlusive complications of the disease. Deferasirox, a convenient long acting oral agent, has recently been introduced in clinical practice with promising efficacy. This...

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Autores principales: Delicou, Sophia, Maragkos, Konstantinos, Tambaki, Maria, Kountouras, Dimitrios, Koskinas, John
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Università Cattolica del Sacro Cuore 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6131104/
https://www.ncbi.nlm.nih.gov/pubmed/30210742
http://dx.doi.org/10.4084/MJHID.2018.049
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author Delicou, Sophia
Maragkos, Konstantinos
Tambaki, Maria
Kountouras, Dimitrios
Koskinas, John
author_facet Delicou, Sophia
Maragkos, Konstantinos
Tambaki, Maria
Kountouras, Dimitrios
Koskinas, John
author_sort Delicou, Sophia
collection PubMed
description Sickle cell disease patients often need regular blood transfusions to improve both the quality of life and survival from the veno-occlusive complications of the disease. Deferasirox, a convenient long acting oral agent, has recently been introduced in clinical practice with promising efficacy. This study aims to evaluate the association of liver stiffness and possible fibrosis with iron deposition and confirm the use of elastography as a validated test of responding to chelation with low cost and easy access. 15 patients with sickle cell disease and systemic or occasional transfusions were evaluated with MRI, transient elastography and biochemistry, for liver iron(LIC) and liver stiffness(LSM) before onset and one year after taking Deferasirox. All patients completed the study. Our results showed improvement in hepatic iron and hepatic stiffness after chelation therapy; Furthermore ALT, AST, LDH and ferritin levels have improved after 12 months of therapy with deferasirox. During the study no serious adverse events were encountered indicating the safety of the drug. Transient liver elastography findings correlate with serum ferritin and LIC in patients with sickle cell disease and it is a useful tool for assessing the response of liver iron chelation therapy.
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spelling pubmed-61311042018-09-12 Transient Elastography (TE) is a Useful Tool for Assessing the Response of Liver Iron Chelation in Sickle Cell Disease Patients Delicou, Sophia Maragkos, Konstantinos Tambaki, Maria Kountouras, Dimitrios Koskinas, John Mediterr J Hematol Infect Dis Original Article Sickle cell disease patients often need regular blood transfusions to improve both the quality of life and survival from the veno-occlusive complications of the disease. Deferasirox, a convenient long acting oral agent, has recently been introduced in clinical practice with promising efficacy. This study aims to evaluate the association of liver stiffness and possible fibrosis with iron deposition and confirm the use of elastography as a validated test of responding to chelation with low cost and easy access. 15 patients with sickle cell disease and systemic or occasional transfusions were evaluated with MRI, transient elastography and biochemistry, for liver iron(LIC) and liver stiffness(LSM) before onset and one year after taking Deferasirox. All patients completed the study. Our results showed improvement in hepatic iron and hepatic stiffness after chelation therapy; Furthermore ALT, AST, LDH and ferritin levels have improved after 12 months of therapy with deferasirox. During the study no serious adverse events were encountered indicating the safety of the drug. Transient liver elastography findings correlate with serum ferritin and LIC in patients with sickle cell disease and it is a useful tool for assessing the response of liver iron chelation therapy. Università Cattolica del Sacro Cuore 2018-09-01 /pmc/articles/PMC6131104/ /pubmed/30210742 http://dx.doi.org/10.4084/MJHID.2018.049 Text en This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by-nc/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Original Article
Delicou, Sophia
Maragkos, Konstantinos
Tambaki, Maria
Kountouras, Dimitrios
Koskinas, John
Transient Elastography (TE) is a Useful Tool for Assessing the Response of Liver Iron Chelation in Sickle Cell Disease Patients
title Transient Elastography (TE) is a Useful Tool for Assessing the Response of Liver Iron Chelation in Sickle Cell Disease Patients
title_full Transient Elastography (TE) is a Useful Tool for Assessing the Response of Liver Iron Chelation in Sickle Cell Disease Patients
title_fullStr Transient Elastography (TE) is a Useful Tool for Assessing the Response of Liver Iron Chelation in Sickle Cell Disease Patients
title_full_unstemmed Transient Elastography (TE) is a Useful Tool for Assessing the Response of Liver Iron Chelation in Sickle Cell Disease Patients
title_short Transient Elastography (TE) is a Useful Tool for Assessing the Response of Liver Iron Chelation in Sickle Cell Disease Patients
title_sort transient elastography (te) is a useful tool for assessing the response of liver iron chelation in sickle cell disease patients
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6131104/
https://www.ncbi.nlm.nih.gov/pubmed/30210742
http://dx.doi.org/10.4084/MJHID.2018.049
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