Long-term treatment with deferiprone enhances left ventricular ejection functionwhen compared to deferoxamine in patients with thalassemia major

Angela Vitrano, Paolo Rigano, Luciano Prossomariti, Giuseppina Calvaruso, Calogera Gerardi, Francesco Gagliardotto, Maddalena Casale, Rita Barone, Michele Rizzo, Giuseppe D'Ascola, Liana Cuccia, Lorella Pitrolo, Paolo Cianciulli, Saveria Campisi, Angela Ciancio, Aldo Filosa, Vincenzo Caruso, Marcello Capra, Aurelio Maggio

Risultato della ricerca: Article

17 Citazioni (Scopus)

Abstract

Transfusion and iron chelation treatment have significantly reduced morbidity and improved survival of patients with thalassemia major. However, cardiac disease continues to be the most common cause of death.We report the left-ventricular ejection fraction, determined by echocardiography, in one hundred sixtyeightpatients with thalassemia major followed for at least 5 years who received continuous monotherapy withdeferoxamine (N = 108) or deferiprone (N = 60). The statistical analysis, using the generalized estimatingequations model, indicated that the group treated with deferiprone had a significantly better left-ventricularejection fraction than did those treated with deferoxamine (coefficient 0.97; 95% CI 0.37; 1.6, p = 0.002).The heart may be particularly sensitive to iron-induced mitochondrial damage because of the large number ofmitochondria and its low level of antioxidants. Deferiprone, because of its lower molecular weight, might crossinto heart mitochondria more efficiently, improving their activity and, thereby, myocardial cell function.Our findings indicate that the long-term administration of deferiprone significantly enhances left-ventricularfunction over time in comparison with deferoxamine treatment. However, because of limitations related tothe design of this study, these findings should be confirmed in a prospective, randomized clinical trial.
Lingua originaleEnglish
Numero di pagine4
RivistaBLOOD CELLS, MOLECULES, & DISEASES
Volume13
Stato di pubblicazionePublished - 2013

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Deferoxamine
beta-Thalassemia
Iron
Heart Mitochondria
Therapeutics
Stroke Volume
Echocardiography
Cause of Death
Heart Diseases
Randomized Controlled Trials
Antioxidants
Molecular Weight
Morbidity
Survival
deferiprone

All Science Journal Classification (ASJC) codes

  • Molecular Medicine
  • Molecular Biology
  • Hematology
  • Cell Biology

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Long-term treatment with deferiprone enhances left ventricular ejection functionwhen compared to deferoxamine in patients with thalassemia major. / Vitrano, Angela; Rigano, Paolo; Prossomariti, Luciano; Calvaruso, Giuseppina; Gerardi, Calogera; Gagliardotto, Francesco; Casale, Maddalena; Barone, Rita; Rizzo, Michele; D'Ascola, Giuseppe; Cuccia, Liana; Pitrolo, Lorella; Cianciulli, Paolo; Campisi, Saveria; Ciancio, Angela; Filosa, Aldo; Caruso, Vincenzo; Capra, Marcello; Maggio, Aurelio.

In: BLOOD CELLS, MOLECULES, & DISEASES, Vol. 13, 2013.

Risultato della ricerca: Article

Vitrano, A, Rigano, P, Prossomariti, L, Calvaruso, G, Gerardi, C, Gagliardotto, F, Casale, M, Barone, R, Rizzo, M, D'Ascola, G, Cuccia, L, Pitrolo, L, Cianciulli, P, Campisi, S, Ciancio, A, Filosa, A, Caruso, V, Capra, M & Maggio, A 2013, 'Long-term treatment with deferiprone enhances left ventricular ejection functionwhen compared to deferoxamine in patients with thalassemia major', BLOOD CELLS, MOLECULES, & DISEASES, vol. 13.
Vitrano, Angela ; Rigano, Paolo ; Prossomariti, Luciano ; Calvaruso, Giuseppina ; Gerardi, Calogera ; Gagliardotto, Francesco ; Casale, Maddalena ; Barone, Rita ; Rizzo, Michele ; D'Ascola, Giuseppe ; Cuccia, Liana ; Pitrolo, Lorella ; Cianciulli, Paolo ; Campisi, Saveria ; Ciancio, Angela ; Filosa, Aldo ; Caruso, Vincenzo ; Capra, Marcello ; Maggio, Aurelio. / Long-term treatment with deferiprone enhances left ventricular ejection functionwhen compared to deferoxamine in patients with thalassemia major. In: BLOOD CELLS, MOLECULES, & DISEASES. 2013 ; Vol. 13.
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title = "Long-term treatment with deferiprone enhances left ventricular ejection functionwhen compared to deferoxamine in patients with thalassemia major",
abstract = "Transfusion and iron chelation treatment have significantly reduced morbidity and improved survival of patients with thalassemia major. However, cardiac disease continues to be the most common cause of death.We report the left-ventricular ejection fraction, determined by echocardiography, in one hundred sixtyeightpatients with thalassemia major followed for at least 5 years who received continuous monotherapy withdeferoxamine (N = 108) or deferiprone (N = 60). The statistical analysis, using the generalized estimatingequations model, indicated that the group treated with deferiprone had a significantly better left-ventricularejection fraction than did those treated with deferoxamine (coefficient 0.97; 95{\%} CI 0.37; 1.6, p = 0.002).The heart may be particularly sensitive to iron-induced mitochondrial damage because of the large number ofmitochondria and its low level of antioxidants. Deferiprone, because of its lower molecular weight, might crossinto heart mitochondria more efficiently, improving their activity and, thereby, myocardial cell function.Our findings indicate that the long-term administration of deferiprone significantly enhances left-ventricularfunction over time in comparison with deferoxamine treatment. However, because of limitations related tothe design of this study, these findings should be confirmed in a prospective, randomized clinical trial.",
author = "Angela Vitrano and Paolo Rigano and Luciano Prossomariti and Giuseppina Calvaruso and Calogera Gerardi and Francesco Gagliardotto and Maddalena Casale and Rita Barone and Michele Rizzo and Giuseppe D'Ascola and Liana Cuccia and Lorella Pitrolo and Paolo Cianciulli and Saveria Campisi and Angela Ciancio and Aldo Filosa and Vincenzo Caruso and Marcello Capra and Aurelio Maggio",
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TY - JOUR

T1 - Long-term treatment with deferiprone enhances left ventricular ejection functionwhen compared to deferoxamine in patients with thalassemia major

AU - Vitrano, Angela

AU - Rigano, Paolo

AU - Prossomariti, Luciano

AU - Calvaruso, Giuseppina

AU - Gerardi, Calogera

AU - Gagliardotto, Francesco

AU - Casale, Maddalena

AU - Barone, Rita

AU - Rizzo, Michele

AU - D'Ascola, Giuseppe

AU - Cuccia, Liana

AU - Pitrolo, Lorella

AU - Cianciulli, Paolo

AU - Campisi, Saveria

AU - Ciancio, Angela

AU - Filosa, Aldo

AU - Caruso, Vincenzo

AU - Capra, Marcello

AU - Maggio, Aurelio

PY - 2013

Y1 - 2013

N2 - Transfusion and iron chelation treatment have significantly reduced morbidity and improved survival of patients with thalassemia major. However, cardiac disease continues to be the most common cause of death.We report the left-ventricular ejection fraction, determined by echocardiography, in one hundred sixtyeightpatients with thalassemia major followed for at least 5 years who received continuous monotherapy withdeferoxamine (N = 108) or deferiprone (N = 60). The statistical analysis, using the generalized estimatingequations model, indicated that the group treated with deferiprone had a significantly better left-ventricularejection fraction than did those treated with deferoxamine (coefficient 0.97; 95% CI 0.37; 1.6, p = 0.002).The heart may be particularly sensitive to iron-induced mitochondrial damage because of the large number ofmitochondria and its low level of antioxidants. Deferiprone, because of its lower molecular weight, might crossinto heart mitochondria more efficiently, improving their activity and, thereby, myocardial cell function.Our findings indicate that the long-term administration of deferiprone significantly enhances left-ventricularfunction over time in comparison with deferoxamine treatment. However, because of limitations related tothe design of this study, these findings should be confirmed in a prospective, randomized clinical trial.

AB - Transfusion and iron chelation treatment have significantly reduced morbidity and improved survival of patients with thalassemia major. However, cardiac disease continues to be the most common cause of death.We report the left-ventricular ejection fraction, determined by echocardiography, in one hundred sixtyeightpatients with thalassemia major followed for at least 5 years who received continuous monotherapy withdeferoxamine (N = 108) or deferiprone (N = 60). The statistical analysis, using the generalized estimatingequations model, indicated that the group treated with deferiprone had a significantly better left-ventricularejection fraction than did those treated with deferoxamine (coefficient 0.97; 95% CI 0.37; 1.6, p = 0.002).The heart may be particularly sensitive to iron-induced mitochondrial damage because of the large number ofmitochondria and its low level of antioxidants. Deferiprone, because of its lower molecular weight, might crossinto heart mitochondria more efficiently, improving their activity and, thereby, myocardial cell function.Our findings indicate that the long-term administration of deferiprone significantly enhances left-ventricularfunction over time in comparison with deferoxamine treatment. However, because of limitations related tothe design of this study, these findings should be confirmed in a prospective, randomized clinical trial.

UR - http://hdl.handle.net/10447/78781

M3 - Article

VL - 13

JO - BLOOD CELLS, MOLECULES, & DISEASES

JF - BLOOD CELLS, MOLECULES, & DISEASES

SN - 1079-9796

ER -