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Liver Iron Content (LIC) in Adults with Sickle Cell Disease (SCD): Correlation with Serum Ferritin and Liver Enzymes Concentrations in Trasfusion Dependent (TD-SCD) and Non-Transfusion Dependent (NT-SCD) Patients

INTRODUCTION: Sickle cell disease (SCD) is one of the leading causes of morbidity and mortality worldwide, causing damage and dysfunction in multiple organs. The complications of this disease are numerous, affect every organ and/or tissue in the body and vary considerably among patients over the tim...

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Autores principales: Yassin, Mohamed, Soliman, Ashraf, De Sanctis, Vincenzo, Nashwan, Abdelqadir, Abusamaan, Sandra, Moustafa, Abbas, Kohla, Samah, Soliman, Dina
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Università Cattolica del Sacro Cuore 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5499497/
https://www.ncbi.nlm.nih.gov/pubmed/28698780
http://dx.doi.org/10.4084/MJHID.2017.037
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author Yassin, Mohamed
Soliman, Ashraf
De Sanctis, Vincenzo
Nashwan, Abdelqadir
Abusamaan, Sandra
Moustafa, Abbas
Kohla, Samah
Soliman, Dina
author_facet Yassin, Mohamed
Soliman, Ashraf
De Sanctis, Vincenzo
Nashwan, Abdelqadir
Abusamaan, Sandra
Moustafa, Abbas
Kohla, Samah
Soliman, Dina
author_sort Yassin, Mohamed
collection PubMed
description INTRODUCTION: Sickle cell disease (SCD) is one of the leading causes of morbidity and mortality worldwide, causing damage and dysfunction in multiple organs. The complications of this disease are numerous, affect every organ and/or tissue in the body and vary considerably among patients over the time challenging its management. THE AIM OF OUR STUDY: To determine the iron status of 17 patients with non-transfusion-dependent sickle cell disease ( NT-SCD) patients and six patients with transfusion dependent sickle cell disease (TD- SCD) using both serum ferritin level (SF) and Ferriscan® evaluation of liver iron content (LIC). We correlated the values of LIC with SF levels and some hepatic enzymes (alanine transaminase-ALT, aspartate aminotransferase -AST, alkaline phosphatase -ALP and albumin). RESULTS: 17 adults with NT-SCD (n = 17, age: 32±15 years) were studied. Seven of NT-SCD had SF > 500 μg/L, 4 out of the seven had high liver iron measured by FerriScan® (> 30 mg/g/ tissue dry weight - dw). Two patients had high LIC despite a concomitant SF concentration < 500 μg/L. Two patients had high SF (1.117 μg/L and 675 μg/L) while their LIC was normal (< 30 mg/g/dw). Five patients had elevated ALT and/or AST) concentrations. In TD-SCD (n = 6, age = 25 ± 11 years), 2 patients had SF <500 μg/L, one of them had high LIC (127 mg/g/DW). Liver enzymes were high in two patients. SF concentration correlated significantly with LIC (r = 0.85, p < 0.001). Neither SF level nor LIC was correlated significantly with hepatic enzyme levels. CONCLUSIONS: A significant number of our patients with NT-SCD had high LIC, high SF and elevated liver enzymes (ALT and AST). Despite some limitations of our study, due to the limited number of NT-SCD patients, these findings have important clinical implications. Therefore, we recommend measuring SF and LIC in NT-SCD patients to apply preventive measures with iron chelation therapy in patients with high LIC.
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spelling pubmed-54994972017-07-11 Liver Iron Content (LIC) in Adults with Sickle Cell Disease (SCD): Correlation with Serum Ferritin and Liver Enzymes Concentrations in Trasfusion Dependent (TD-SCD) and Non-Transfusion Dependent (NT-SCD) Patients Yassin, Mohamed Soliman, Ashraf De Sanctis, Vincenzo Nashwan, Abdelqadir Abusamaan, Sandra Moustafa, Abbas Kohla, Samah Soliman, Dina Mediterr J Hematol Infect Dis Original Articles INTRODUCTION: Sickle cell disease (SCD) is one of the leading causes of morbidity and mortality worldwide, causing damage and dysfunction in multiple organs. The complications of this disease are numerous, affect every organ and/or tissue in the body and vary considerably among patients over the time challenging its management. THE AIM OF OUR STUDY: To determine the iron status of 17 patients with non-transfusion-dependent sickle cell disease ( NT-SCD) patients and six patients with transfusion dependent sickle cell disease (TD- SCD) using both serum ferritin level (SF) and Ferriscan® evaluation of liver iron content (LIC). We correlated the values of LIC with SF levels and some hepatic enzymes (alanine transaminase-ALT, aspartate aminotransferase -AST, alkaline phosphatase -ALP and albumin). RESULTS: 17 adults with NT-SCD (n = 17, age: 32±15 years) were studied. Seven of NT-SCD had SF > 500 μg/L, 4 out of the seven had high liver iron measured by FerriScan® (> 30 mg/g/ tissue dry weight - dw). Two patients had high LIC despite a concomitant SF concentration < 500 μg/L. Two patients had high SF (1.117 μg/L and 675 μg/L) while their LIC was normal (< 30 mg/g/dw). Five patients had elevated ALT and/or AST) concentrations. In TD-SCD (n = 6, age = 25 ± 11 years), 2 patients had SF <500 μg/L, one of them had high LIC (127 mg/g/DW). Liver enzymes were high in two patients. SF concentration correlated significantly with LIC (r = 0.85, p < 0.001). Neither SF level nor LIC was correlated significantly with hepatic enzyme levels. CONCLUSIONS: A significant number of our patients with NT-SCD had high LIC, high SF and elevated liver enzymes (ALT and AST). Despite some limitations of our study, due to the limited number of NT-SCD patients, these findings have important clinical implications. Therefore, we recommend measuring SF and LIC in NT-SCD patients to apply preventive measures with iron chelation therapy in patients with high LIC. Università Cattolica del Sacro Cuore 2017-06-20 /pmc/articles/PMC5499497/ /pubmed/28698780 http://dx.doi.org/10.4084/MJHID.2017.037 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 Articles
Yassin, Mohamed
Soliman, Ashraf
De Sanctis, Vincenzo
Nashwan, Abdelqadir
Abusamaan, Sandra
Moustafa, Abbas
Kohla, Samah
Soliman, Dina
Liver Iron Content (LIC) in Adults with Sickle Cell Disease (SCD): Correlation with Serum Ferritin and Liver Enzymes Concentrations in Trasfusion Dependent (TD-SCD) and Non-Transfusion Dependent (NT-SCD) Patients
title Liver Iron Content (LIC) in Adults with Sickle Cell Disease (SCD): Correlation with Serum Ferritin and Liver Enzymes Concentrations in Trasfusion Dependent (TD-SCD) and Non-Transfusion Dependent (NT-SCD) Patients
title_full Liver Iron Content (LIC) in Adults with Sickle Cell Disease (SCD): Correlation with Serum Ferritin and Liver Enzymes Concentrations in Trasfusion Dependent (TD-SCD) and Non-Transfusion Dependent (NT-SCD) Patients
title_fullStr Liver Iron Content (LIC) in Adults with Sickle Cell Disease (SCD): Correlation with Serum Ferritin and Liver Enzymes Concentrations in Trasfusion Dependent (TD-SCD) and Non-Transfusion Dependent (NT-SCD) Patients
title_full_unstemmed Liver Iron Content (LIC) in Adults with Sickle Cell Disease (SCD): Correlation with Serum Ferritin and Liver Enzymes Concentrations in Trasfusion Dependent (TD-SCD) and Non-Transfusion Dependent (NT-SCD) Patients
title_short Liver Iron Content (LIC) in Adults with Sickle Cell Disease (SCD): Correlation with Serum Ferritin and Liver Enzymes Concentrations in Trasfusion Dependent (TD-SCD) and Non-Transfusion Dependent (NT-SCD) Patients
title_sort liver iron content (lic) in adults with sickle cell disease (scd): correlation with serum ferritin and liver enzymes concentrations in trasfusion dependent (td-scd) and non-transfusion dependent (nt-scd) patients
topic Original Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5499497/
https://www.ncbi.nlm.nih.gov/pubmed/28698780
http://dx.doi.org/10.4084/MJHID.2017.037
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