Page last updated: 2024-10-25

deferiprone and Cardiomyopathies

deferiprone has been researched along with Cardiomyopathies in 31 studies

Deferiprone: A pyridone derivative and iron chelator that is used in the treatment of IRON OVERLOAD in patients with THALASSEMIA.
deferiprone : A member of the class of 4-pyridones that is pyridin-4(1H)-one substituted at positions 1 and 2 by methyl groups and at position 3 by a hydroxy group. A lipid-soluble iron-chelator used for treatment of thalassaemia.

Cardiomyopathies: A group of diseases in which the dominant feature is the involvement of the CARDIAC MUSCLE itself. Cardiomyopathies are classified according to their predominant pathophysiological features (DILATED CARDIOMYOPATHY; HYPERTROPHIC CARDIOMYOPATHY; RESTRICTIVE CARDIOMYOPATHY) or their etiological/pathological factors (CARDIOMYOPATHY, ALCOHOLIC; ENDOCARDIAL FIBROELASTOSIS).

Research Excerpts

ExcerptRelevanceReference
"Deferiprone monotherapy was significantly more effective than deferoxamine over 1 year in improving asymptomatic myocardial siderosis in beta-thalassemia major."6.72Randomized controlled trial of deferiprone or deferoxamine in beta-thalassemia major patients with asymptomatic myocardial siderosis. ( Aessopos, A; Berdoukas, V; Galanello, R; Gotsis, ED; Karagiorga, M; Ladis, V; Pennell, DJ; Piga, A; Smith, GC; Tanner, MA; Westwood, MA; Wonke, B, 2006)
"We concluded that pregnancy is not a contraindication in beta-thalassemia major; complex individual care is needed to achieve a safe outcome for the mother."5.51A case of post-splenectomy transfusion-dependent homozygous beta-thalassemia major complicated with myocardial siderosis and osteoporosis and usage of iron-chelating therapy with deferiprone in pregnancy. ( Abu, MA; Ahmad, MF; Arsad, N; Chew, KT; Omar, MH; Shafiee, MN; Zakaria, NZ, 2019)
"Deferiprone has been reported to be superior to deferoxamine for the removal of cardiac iron and improvement in left ventricular (LV) function but little is known of their relative effects on the right ventricle (RV), which is being increasingly recognised as an important prognostic factor in cardiomyopathy."5.37Effect of deferiprone or deferoxamine on right ventricular function in thalassemia major patients with myocardial iron overload. ( Aessopos, A; Alam, MH; Alpendurada, F; Berdoukas, V; Carpenter, JP; Galanello, R; Gotsis, ED; Karagiorga, M; Ladis, V; Pennell, DJ; Piga, A; Roughton, M; Smith, GC; Tanner, MA; Westwood, MA, 2011)
"We enrolled nine patients with thalassemia major complicated by some degree of myocardial dysfunction."5.33Regression of myocardial dysfunction after switching from desferrioxamine to deferiprone therapy in beta-thalassemia major patients. ( Chang, JS; Huang, YC; Peng, CT; Wu, KH, 2006)
" In 18 requiring thyroxine supplementation for hypothyroidism, 10 were able to discontinue, and four reduced their thyroxine dose."5.14Normalisation of total body iron load with very intensive combined chelation reverses cardiac and endocrine complications of thalassaemia major. ( Berdoukas, V; Chouliaras, G; Farmaki, K; Pappa, C; Tzoumari, I, 2010)
"In patients with severe myocardial siderosis and impaired LV function, combined chelation therapy with subcutaneous deferoxamine and oral deferiprone reduces myocardial iron and improves cardiac function."5.13Combined chelation therapy in thalassemia major for the treatment of severe myocardial siderosis with left ventricular dysfunction. ( Agus, A; Dessi, C; Galanello, R; Nair, SV; Pennell, DJ; Pibiri, M; Smith, GC; Tanner, MA; Walker, JM; Westwood, MA, 2008)
" Despite that many chelators inhibit FRD in vitro and in vivo, only Deferiprone (L1) has been shown to be effective and safe in the reversal of oxidative stress related tissue damage in iron overload and other conditions such as cardiomyopathy, acute kidney disease, Friedreich ataxia etc."4.90Antioxidant targeting by deferiprone in diseases related to oxidative damage. ( Kolnagou, A; Kontoghiorghe, CN; Kontoghiorghes, GJ, 2014)
"The iron chelators deferoxamine (DFO) and deferiprone (L1) have demonstrated their ability to normalize cardiac function in patients with iron overload-induced cardiac disease."4.84Effects of chelation therapy on cardiac function improvement in thalassemia patients: literature review and the Taiwanese experience. ( Peng, CT; Tsai, CH; Wu, KH, 2008)
"Therapy with either deferiprone (DFP) or deferoxamine (DFO) is inadequate in achieving negative iron balance in many patients with thalassemia."4.82Combined therapy with deferoxamine and deferiprone. ( Kattamis, A, 2005)
"The incidence of cardiomyopathy was monitored in a 6-year follow-up study involving 56 transfused thalassemia patients treated with deferoxamine (DFO), deferiprone (L1) or their combination."3.73Low serum ferritin levels are misleading for detecting cardiac iron overload and increase the risk of cardiomyopathy in thalassemia patients. The importance of cardiac iron overload monitoring using magnetic resonance imaging T2 and T2*. ( Economides, C; Eracleous, E; Kolnagou, A; Kontoghiorghes, GJ, 2006)
"Deferiprone monotherapy was significantly more effective than deferoxamine over 1 year in improving asymptomatic myocardial siderosis in beta-thalassemia major."2.72Randomized controlled trial of deferiprone or deferoxamine in beta-thalassemia major patients with asymptomatic myocardial siderosis. ( Aessopos, A; Berdoukas, V; Galanello, R; Gotsis, ED; Karagiorga, M; Ladis, V; Pennell, DJ; Piga, A; Smith, GC; Tanner, MA; Westwood, MA; Wonke, B, 2006)
"We concluded that pregnancy is not a contraindication in beta-thalassemia major; complex individual care is needed to achieve a safe outcome for the mother."1.51A case of post-splenectomy transfusion-dependent homozygous beta-thalassemia major complicated with myocardial siderosis and osteoporosis and usage of iron-chelating therapy with deferiprone in pregnancy. ( Abu, MA; Ahmad, MF; Arsad, N; Chew, KT; Omar, MH; Shafiee, MN; Zakaria, NZ, 2019)
"Deferasirox monotherapy was less effective than deferiprone in improving myocardial siderosis and biventricular function and less effective than desferrioxamine in improving the LVEF."1.48MRI multicentre prospective survey in thalassaemia major patients treated with deferasirox versus deferiprone and desferrioxamine. ( Allò, M; Cuccia, L; D'Ascola, DG; Gamberini, MR; Lisi, R; Mangione, M; Meloni, A; Pepe, A; Pistoia, L; Positano, V; Restaino, G; Ricchi, P; Righi, R; Rosso, R; Spasiano, A, 2018)
"Iron chelators are used to treat iron overload cardiomyopathy patients."1.43Combined Iron Chelator and Antioxidant Exerted Greater Efficacy on Cardioprotection Than Monotherapy in Iron-Overloaded Rats. ( Chattipakorn, N; Chattipakorn, SC; Fucharoen, S; Khamseekaew, J; Kumfu, S; Srichairatanakool, S; Sripetchwandee, J; Wongjaikam, S, 2016)
"Deferiprone has been reported to be superior to deferoxamine for the removal of cardiac iron and improvement in left ventricular (LV) function but little is known of their relative effects on the right ventricle (RV), which is being increasingly recognised as an important prognostic factor in cardiomyopathy."1.37Effect of deferiprone or deferoxamine on right ventricular function in thalassemia major patients with myocardial iron overload. ( Aessopos, A; Alam, MH; Alpendurada, F; Berdoukas, V; Carpenter, JP; Galanello, R; Gotsis, ED; Karagiorga, M; Ladis, V; Pennell, DJ; Piga, A; Roughton, M; Smith, GC; Tanner, MA; Westwood, MA, 2011)
"Myocardial iron overload is the leading cause of death in patients with beta-thalassemia major."1.36Increased survival and reversion of iron-induced cardiac disease in patients with thalassemia major receiving intensive combined chelation therapy as compared to desferoxamine alone. ( Bina, P; Carta, MP; Cianciulli, P; Farci, P; Galanello, R; Grady, RW; Lai, ME; Maggio, A; Pepe, A; Sau, F; Vacquer, S, 2010)
"We enrolled nine patients with thalassemia major complicated by some degree of myocardial dysfunction."1.33Regression of myocardial dysfunction after switching from desferrioxamine to deferiprone therapy in beta-thalassemia major patients. ( Chang, JS; Huang, YC; Peng, CT; Wu, KH, 2006)

Research

Studies (31)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's20 (64.52)29.6817
2010's10 (32.26)24.3611
2020's1 (3.23)2.80

Authors

AuthorsStudies
Pepe, A4
Meloni, A3
Filosa, A2
Pistoia, L2
Borsellino, Z1
D'Ascola, DG2
Lisi, R2
Putti, MC2
Allò, M2
Gamberini, MR2
Quarta, A1
Fidone, C1
Casini, T1
Restaino, G2
Midiri, M2
Mangione, M2
Positano, V3
Casale, M1
Lee, JA1
Tchantchaleishvili, V1
Vidula, H1
Cuccia, L2
Rosso, R1
Spasiano, A1
Righi, R1
Ricchi, P2
Ahmad, MF1
Zakaria, NZ1
Arsad, N1
Chew, KT1
Abu, MA1
Shafiee, MN1
Omar, MH1
Kontoghiorghe, CN1
Kolnagou, A5
Kontoghiorghes, GJ5
Wongjaikam, S1
Kumfu, S1
Khamseekaew, J1
Sripetchwandee, J1
Srichairatanakool, S1
Fucharoen, S1
Chattipakorn, SC1
Chattipakorn, N1
Tauchenová, L1
Křížová, B1
Kubánek, M1
Fraňková, S1
Melenovský, V1
Tintěra, J1
Kautznerová, D1
Malušková, J1
Jirsa, M1
Kautzner, J1
Modell, B2
Khan, M1
Darlison, M1
Westwood, MA4
Ingram, D1
Pennell, DJ6
Peng, CT4
Chang, JS2
Wang, LY1
Chiou, SS1
Hsiao, CC1
Wang, SC1
Hung, GY1
Wu, KH4
Farmaki, K1
Tzoumari, I1
Pappa, C1
Chouliaras, G1
Berdoukas, V4
Lai, ME1
Grady, RW1
Vacquer, S1
Carta, MP1
Bina, P1
Sau, F1
Cianciulli, P2
Maggio, A1
Galanello, R5
Farci, P1
Smith, GC3
Alpendurada, F1
Carpenter, JP1
Alam, MH1
Karagiorga, M2
Ladis, V2
Piga, A4
Aessopos, A2
Gotsis, ED2
Tanner, MA3
Roughton, M1
Rossi, G1
D'Ascola, GD1
Santodirocco, M1
Caruso, V1
Romeo, MA1
Pitrolo, L1
Peluso, A1
Campisi, S1
Missere, M1
Gulino, L1
Lombardi, M1
Brittenham, GM1
Nathan, DG1
Olivieri, NF1
Pippard, MJ1
Weatherall, DJ1
Westwood, M1
Anderson, LJ1
Hershko, C2
Cappellini, MD1
Tognoni, G1
Masera, G1
Fessas, C1
Papatryphonas, A1
Economides, C2
Link, G1
Konijn, AM1
Cabantchik, ZI1
Kattamis, A1
Roggero, S1
Vinciguerra, T1
Sacchetti, L1
Gallo, V1
Longo, F1
Wonke, B1
Eracleous, E1
Huang, YC1
Chang, JG1
Ho, YJ1
Wu, SF1
Kidson-Gerber, GL1
Francis, S1
Lindeman, R1
Michaelides, Y1
Kontos, C1
Kyriacou, K1
Tsai, CH1
Dessi, C1
Agus, A1
Pibiri, M1
Nair, SV1
Walker, JM1

Clinical Trials (1)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
A Randomized, Placebo Controlled, Double Blind Trial of the Effect of Combined Therapy With Deferoxamine and Deferiprone on Myocardial Iron in Thalassemia Major Using Cardiovascular Magnetic Resonance[NCT00103753]Phase 465 participants Interventional2004-05-31Active, not recruiting
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Reviews

7 reviews available for deferiprone and Cardiomyopathies

ArticleYear
Antioxidant targeting by deferiprone in diseases related to oxidative damage.
    Frontiers in bioscience (Landmark edition), 2014, 06-01, Volume: 19, Issue:6

    Topics: Acute Kidney Injury; Antioxidants; Cardiomyopathies; Deferiprone; Free Radicals; Friedreich Ataxia;

2014
Purging iron from the heart.
    British journal of haematology, 2004, Volume: 125, Issue:5

    Topics: Cardiomyopathies; Cardiotonic Agents; Deferiprone; Deferoxamine; Humans; Iron Chelating Agents; Iron

2004
Objectives and mechanism of iron chelation therapy.
    Annals of the New York Academy of Sciences, 2005, Volume: 1054

    Topics: Animals; Benzoates; Cardiomyopathies; Cells, Cultured; Chelation Therapy; Clinical Trials as Topic;

2005
Combined therapy with deferoxamine and deferiprone.
    Annals of the New York Academy of Sciences, 2005, Volume: 1054

    Topics: Agranulocytosis; Biological Transport; Cardiomyopathies; Chelation Therapy; Clinical Trials as Topic

2005
Deferiprone: New insight.
    Annals of the New York Academy of Sciences, 2005, Volume: 1054

    Topics: Agranulocytosis; Cardiomyopathies; Clinical Trials as Topic; Deferiprone; Deferoxamine; Follow-Up St

2005
T2* magnetic resonance and myocardial iron in thalassemia.
    Annals of the New York Academy of Sciences, 2005, Volume: 1054

    Topics: Adult; Calibration; Cardiomyopathies; Chelation Therapy; Child; Cohort Studies; Combined Modality Th

2005
Effects of chelation therapy on cardiac function improvement in thalassemia patients: literature review and the Taiwanese experience.
    Hemoglobin, 2008, Volume: 32, Issue:1-2

    Topics: Cardiomyopathies; Chelation Therapy; Deferiprone; Deferoxamine; Ferritins; Humans; Iron; Iron Chelat

2008

Trials

4 trials available for deferiprone and Cardiomyopathies

ArticleYear
Normalisation of total body iron load with very intensive combined chelation reverses cardiac and endocrine complications of thalassaemia major.
    British journal of haematology, 2010, Volume: 148, Issue:3

    Topics: Adolescent; Adult; beta-Thalassemia; Blood Glucose; Cardiomyopathies; Child; Deferiprone; Deferoxami

2010
Randomized controlled trial of deferiprone or deferoxamine in beta-thalassemia major patients with asymptomatic myocardial siderosis.
    Blood, 2006, May-01, Volume: 107, Issue:9

    Topics: Adult; beta-Thalassemia; Cardiomyopathies; Deferiprone; Deferoxamine; Female; Humans; Iron; Iron Che

2006
Effective combination therapy of deferiprone and deferoxamine for the rapid clearance of excess cardiac IRON and the prevention of heart disease in thalassemia. The Protocol of the International Committee on Oral Chelators.
    Hemoglobin, 2006, Volume: 30, Issue:2

    Topics: Administration, Oral; beta-Thalassemia; Cardiomyopathies; Chelation Therapy; Clinical Protocols; Com

2006
Combined chelation therapy in thalassemia major for the treatment of severe myocardial siderosis with left ventricular dysfunction.
    Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance, 2008, Feb-25, Volume: 10

    Topics: Administration, Oral; Adult; beta-Thalassemia; Cardiomyopathies; Deferiprone; Deferoxamine; Drug The

2008

Other Studies

20 other studies available for deferiprone and Cardiomyopathies

ArticleYear
Prospective CMR Survey in Children With Thalassemia Major: Insights From a National Network.
    JACC. Cardiovascular imaging, 2020, Volume: 13, Issue:5

    Topics: Adolescent; Age Factors; beta-Thalassemia; Cardiomyopathies; Child; Deferiprone; Deferoxamine; Femal

2020
Cardiac Hemochromatosis Treated With Prolonged Extracorporeal Membrane Oxygenation Support and Chelation Therapy.
    Artificial organs, 2018, Volume: 42, Issue:5

    Topics: Adult; Cardiomyopathies; Chelation Therapy; Deferasirox; Deferiprone; Deferoxamine; Extracorporeal M

2018
MRI multicentre prospective survey in thalassaemia major patients treated with deferasirox versus deferiprone and desferrioxamine.
    British journal of haematology, 2018, Volume: 183, Issue:5

    Topics: Adult; beta-Thalassemia; Cardiomyopathies; Deferasirox; Deferiprone; Deferoxamine; Drug Substitution

2018
A case of post-splenectomy transfusion-dependent homozygous beta-thalassemia major complicated with myocardial siderosis and osteoporosis and usage of iron-chelating therapy with deferiprone in pregnancy.
    Hormone molecular biology and clinical investigation, 2019, Jul-13, Volume: 39, Issue:2

    Topics: beta-Globins; beta-Thalassemia; Blood Transfusion; Cardiomyopathies; Deferiprone; Female; Homozygote

2019
Combined Iron Chelator and Antioxidant Exerted Greater Efficacy on Cardioprotection Than Monotherapy in Iron-Overloaded Rats.
    PloS one, 2016, Volume: 11, Issue:7

    Topics: Acetylcysteine; Animals; Benzoates; Cardiomyopathies; Cardiotonic Agents; Deferasirox; Deferiprone;

2016
Successful Treatment of Iron-Overload Cardiomyopathy in Hereditary Hemochromatosis With Deferoxamine and Deferiprone.
    The Canadian journal of cardiology, 2016, Volume: 32, Issue:12

    Topics: Cardiomyopathies; Deferiprone; Deferoxamine; Ferritins; Heart Failure; Hemochromatosis; Hemochromato

2016
Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance.
    Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance, 2008, Sep-25, Volume: 10

    Topics: Adolescent; Adult; Anemia; beta-Thalassemia; Bone Marrow Transplantation; Cardiomyopathies; Cause of

2008
Update on thalassemia treatment in Taiwan, including bone marrow transplantation, chelation therapy, and cardiomyopathy treatment effects.
    Hemoglobin, 2009, Volume: 33, Issue:5

    Topics: Benzoates; beta-Thalassemia; Blood Transfusion; Bone Marrow Transplantation; Cardiomyopathies; Chela

2009
Increased survival and reversion of iron-induced cardiac disease in patients with thalassemia major receiving intensive combined chelation therapy as compared to desferoxamine alone.
    Blood cells, molecules & diseases, 2010, Aug-15, Volume: 45, Issue:2

    Topics: Adult; beta-Thalassemia; Cardiomyopathies; Chelation Therapy; Deferiprone; Deferoxamine; Drug Therap

2010
Effect of deferiprone or deferoxamine on right ventricular function in thalassemia major patients with myocardial iron overload.
    Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance, 2011, Jul-06, Volume: 13

    Topics: Adult; Analysis of Variance; beta-Thalassemia; Cardiomyopathies; Deferiprone; Deferoxamine; Female;

2011
Cardiac and hepatic iron and ejection fraction in thalassemia major: multicentre prospective comparison of combined deferiprone and deferoxamine therapy against deferiprone or deferoxamine monotherapy.
    Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance, 2013, Jan-16, Volume: 15

    Topics: Adult; Analysis of Variance; beta-Thalassemia; Cardiomyopathies; Chi-Square Distribution; Deferipron

2013
Deferiprone versus desferrioxamine in thalassaemia, and T2* validation and utility.
    Lancet (London, England), 2003, Jan-11, Volume: 361, Issue:9352

    Topics: beta-Thalassemia; Cardiomyopathies; Deferiprone; Deferoxamine; Humans; Iron Chelating Agents; Pyrido

2003
Treatment of cardiac iron overload in thalassemia major.
    Haematologica, 2003, Volume: 88, Issue:5

    Topics: beta-Thalassemia; Cardiomyopathies; Deferiprone; Deferoxamine; Drug Therapy, Combination; Heart Dise

2003
Prophylactic use of deferiprone (L1) and magnetic resonance imaging T2* or T2 for preventing heart disease in thalassaemia.
    British journal of haematology, 2004, Volume: 127, Issue:3

    Topics: Cardiomyopathies; Deferiprone; Humans; Iron Chelating Agents; Magnetic Resonance Imaging; Myocardium

2004
Low serum ferritin levels are misleading for detecting cardiac iron overload and increase the risk of cardiomyopathy in thalassemia patients. The importance of cardiac iron overload monitoring using magnetic resonance imaging T2 and T2*.
    Hemoglobin, 2006, Volume: 30, Issue:2

    Topics: Administration, Oral; Adult; Arrhythmias, Cardiac; Cardiomyopathies; Chelation Therapy; Combined Mod

2006
Regression of myocardial dysfunction after switching from desferrioxamine to deferiprone therapy in beta-thalassemia major patients.
    Hemoglobin, 2006, Volume: 30, Issue:2

    Topics: Adolescent; Adult; beta-Thalassemia; Cardiomegaly; Cardiomyopathies; Chelation Therapy; Cohort Studi

2006
Glutathione S-transferase M1 gene polymorphisms are associated with cardiac iron deposition in patients with beta-thalassemia major.
    Hemoglobin, 2006, Volume: 30, Issue:2

    Topics: Adolescent; Adult; beta-Thalassemia; Cardiomyopathies; Chelation Therapy; Child; Combined Modality T

2006
Transfusion-dependent thalassaemia: a new era.
    The Medical journal of Australia, 2008, Jan-21, Volume: 188, Issue:2

    Topics: Benzoates; Cardiomyopathies; Deferasirox; Deferiprone; Deferoxamine; Drug Prescriptions; Erythrocyte

2008
Management and clinical outcomes of transfusion-dependent thalassaemia major in an Australian tertiary referral clinic.
    The Medical journal of Australia, 2008, Jan-21, Volume: 188, Issue:2

    Topics: Adult; Age Factors; Australia; Cardiomyopathies; Deferiprone; Deferoxamine; Diabetes Mellitus; Drug

2008
Myocyte damage and loss of myofibers is the potential mechanism of iron overload toxicity in congestive cardiac failure in thalassemia. Complete reversal of the cardiomyopathy and normalization of iron load by deferiprone.
    Hemoglobin, 2008, Volume: 32, Issue:1-2

    Topics: Cardiomyopathies; Chelation Therapy; Deferiprone; Deferoxamine; Female; Ferritins; Humans; Iron; Iro

2008