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Role of Iron Metabolism Genetic Determinants in Response to Chelation Therapy in a Cohort of β-Thalassemia and Sickle Cell Syndromes Italian Patients
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| Zeitschriftentitel: | Thalassemia Reports |
|---|---|
| Personen und Körperschaften: | , , , , , , |
| In: | Thalassemia Reports, 4, 2014, 2, S. 2729 |
| Format: | E-Article |
| Sprache: | Englisch |
| veröffentlicht: |
MDPI AG
|
| author_facet |
Renda, Maria Concetta Renda, Disma Piazza, Angela Calvaruso, Giuseppina Fecarotta, Emanuela Giangreco, Antonino Maggio, Aurelio Renda, Maria Concetta Renda, Disma Piazza, Angela Calvaruso, Giuseppina Fecarotta, Emanuela Giangreco, Antonino Maggio, Aurelio |
|---|---|
| author |
Renda, Maria Concetta Renda, Disma Piazza, Angela Calvaruso, Giuseppina Fecarotta, Emanuela Giangreco, Antonino Maggio, Aurelio |
| spellingShingle |
Renda, Maria Concetta Renda, Disma Piazza, Angela Calvaruso, Giuseppina Fecarotta, Emanuela Giangreco, Antonino Maggio, Aurelio Thalassemia Reports Role of Iron Metabolism Genetic Determinants in Response to Chelation Therapy in a Cohort of β-Thalassemia and Sickle Cell Syndromes Italian Patients |
| author_sort |
renda, maria concetta |
| spelling |
Renda, Maria Concetta Renda, Disma Piazza, Angela Calvaruso, Giuseppina Fecarotta, Emanuela Giangreco, Antonino Maggio, Aurelio 2039-4365 MDPI AG http://dx.doi.org/10.4081/thal.2014.2729 <jats:p>In patients with β-thalassemia and sickle cell syndromes there is an important secondary iron overload due to regular blood transfusions and increased duodenal iron absorption. As in genetic hemochromatosis, also the secondary iron storage leads to tissue injury that involves all the major organs: liver, heart, kidney, endocrine glands. At present, in patients with β-thalassemia and sickle cell syndrome, iron chelation therapy is widely used for the treatment of secondary hemochromatosis, to limit the toxic effects of iron overload. In order to maintain the correct homeostasis, several genes are involved in the metabolic pathways of iron, including HFE, FPN (ferroportin) and TF (transferrin). In this study we analyzed the genes HFE, FPN and TF, to assess their possible effects on response to therapy with deferasirox and deferiprone, either as monotherapy or in combination therapy in a cohort of patients with β-thalassemia and sickle cell syndromes.</jats:p> Role of Iron Metabolism Genetic Determinants in Response to Chelation Therapy in a Cohort of β-Thalassemia and Sickle Cell Syndromes Italian Patients Thalassemia Reports |
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| title |
Role of Iron Metabolism Genetic Determinants in Response to Chelation Therapy in a Cohort of β-Thalassemia and Sickle Cell Syndromes Italian Patients |
| title_unstemmed |
Role of Iron Metabolism Genetic Determinants in Response to Chelation Therapy in a Cohort of β-Thalassemia and Sickle Cell Syndromes Italian Patients |
| title_full |
Role of Iron Metabolism Genetic Determinants in Response to Chelation Therapy in a Cohort of β-Thalassemia and Sickle Cell Syndromes Italian Patients |
| title_fullStr |
Role of Iron Metabolism Genetic Determinants in Response to Chelation Therapy in a Cohort of β-Thalassemia and Sickle Cell Syndromes Italian Patients |
| title_full_unstemmed |
Role of Iron Metabolism Genetic Determinants in Response to Chelation Therapy in a Cohort of β-Thalassemia and Sickle Cell Syndromes Italian Patients |
| title_short |
Role of Iron Metabolism Genetic Determinants in Response to Chelation Therapy in a Cohort of β-Thalassemia and Sickle Cell Syndromes Italian Patients |
| title_sort |
role of iron metabolism genetic determinants in response to chelation therapy in a cohort of β-thalassemia and sickle cell syndromes italian patients |
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http://dx.doi.org/10.4081/thal.2014.2729 |
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2014 |
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2729 |
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<jats:p>In patients with β-thalassemia and sickle cell syndromes there is an important secondary iron overload due to regular blood transfusions and increased duodenal iron absorption. As in genetic hemochromatosis, also the secondary iron storage leads to tissue injury that involves all the major organs: liver, heart, kidney, endocrine glands. At present, in patients with β-thalassemia and sickle cell syndrome, iron chelation therapy is widely used for the treatment of secondary hemochromatosis, to limit the toxic effects of iron overload. In order to maintain the correct homeostasis, several genes are involved in the metabolic pathways of iron, including HFE, FPN (ferroportin) and TF (transferrin). In this study we analyzed the genes HFE, FPN and TF, to assess their possible effects on response to therapy with deferasirox and deferiprone, either as monotherapy or in combination therapy in a cohort of patients with β-thalassemia and sickle cell syndromes.</jats:p> |
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| author | Renda, Maria Concetta, Renda, Disma, Piazza, Angela, Calvaruso, Giuseppina, Fecarotta, Emanuela, Giangreco, Antonino, Maggio, Aurelio |
| author_facet | Renda, Maria Concetta, Renda, Disma, Piazza, Angela, Calvaruso, Giuseppina, Fecarotta, Emanuela, Giangreco, Antonino, Maggio, Aurelio, Renda, Maria Concetta, Renda, Disma, Piazza, Angela, Calvaruso, Giuseppina, Fecarotta, Emanuela, Giangreco, Antonino, Maggio, Aurelio |
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| description | <jats:p>In patients with β-thalassemia and sickle cell syndromes there is an important secondary iron overload due to regular blood transfusions and increased duodenal iron absorption. As in genetic hemochromatosis, also the secondary iron storage leads to tissue injury that involves all the major organs: liver, heart, kidney, endocrine glands. At present, in patients with β-thalassemia and sickle cell syndrome, iron chelation therapy is widely used for the treatment of secondary hemochromatosis, to limit the toxic effects of iron overload. In order to maintain the correct homeostasis, several genes are involved in the metabolic pathways of iron, including HFE, FPN (ferroportin) and TF (transferrin). In this study we analyzed the genes HFE, FPN and TF, to assess their possible effects on response to therapy with deferasirox and deferiprone, either as monotherapy or in combination therapy in a cohort of patients with β-thalassemia and sickle cell syndromes.</jats:p> |
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| spelling | Renda, Maria Concetta Renda, Disma Piazza, Angela Calvaruso, Giuseppina Fecarotta, Emanuela Giangreco, Antonino Maggio, Aurelio 2039-4365 MDPI AG http://dx.doi.org/10.4081/thal.2014.2729 <jats:p>In patients with β-thalassemia and sickle cell syndromes there is an important secondary iron overload due to regular blood transfusions and increased duodenal iron absorption. As in genetic hemochromatosis, also the secondary iron storage leads to tissue injury that involves all the major organs: liver, heart, kidney, endocrine glands. At present, in patients with β-thalassemia and sickle cell syndrome, iron chelation therapy is widely used for the treatment of secondary hemochromatosis, to limit the toxic effects of iron overload. In order to maintain the correct homeostasis, several genes are involved in the metabolic pathways of iron, including HFE, FPN (ferroportin) and TF (transferrin). In this study we analyzed the genes HFE, FPN and TF, to assess their possible effects on response to therapy with deferasirox and deferiprone, either as monotherapy or in combination therapy in a cohort of patients with β-thalassemia and sickle cell syndromes.</jats:p> Role of Iron Metabolism Genetic Determinants in Response to Chelation Therapy in a Cohort of β-Thalassemia and Sickle Cell Syndromes Italian Patients Thalassemia Reports |
| spellingShingle | Renda, Maria Concetta, Renda, Disma, Piazza, Angela, Calvaruso, Giuseppina, Fecarotta, Emanuela, Giangreco, Antonino, Maggio, Aurelio, Thalassemia Reports, Role of Iron Metabolism Genetic Determinants in Response to Chelation Therapy in a Cohort of β-Thalassemia and Sickle Cell Syndromes Italian Patients |
| title | Role of Iron Metabolism Genetic Determinants in Response to Chelation Therapy in a Cohort of β-Thalassemia and Sickle Cell Syndromes Italian Patients |
| title_full | Role of Iron Metabolism Genetic Determinants in Response to Chelation Therapy in a Cohort of β-Thalassemia and Sickle Cell Syndromes Italian Patients |
| title_fullStr | Role of Iron Metabolism Genetic Determinants in Response to Chelation Therapy in a Cohort of β-Thalassemia and Sickle Cell Syndromes Italian Patients |
| title_full_unstemmed | Role of Iron Metabolism Genetic Determinants in Response to Chelation Therapy in a Cohort of β-Thalassemia and Sickle Cell Syndromes Italian Patients |
| title_short | Role of Iron Metabolism Genetic Determinants in Response to Chelation Therapy in a Cohort of β-Thalassemia and Sickle Cell Syndromes Italian Patients |
| title_sort | role of iron metabolism genetic determinants in response to chelation therapy in a cohort of β-thalassemia and sickle cell syndromes italian patients |
| title_unstemmed | Role of Iron Metabolism Genetic Determinants in Response to Chelation Therapy in a Cohort of β-Thalassemia and Sickle Cell Syndromes Italian Patients |
| url | http://dx.doi.org/10.4081/thal.2014.2729 |