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Continued improvement in myocardial T2* over two years of deferasirox therapy in β-thalassemia major patients with cardiac iron overload

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dc.contributor.author Pennell D.J.
dc.contributor.author Porter J.B.
dc.contributor.author Cappellini M.D.
dc.contributor.author Chan L.L.
dc.contributor.author El-Beshlawy A.
dc.contributor.author Aydinok Y.
dc.contributor.author Ibrahim H.
dc.contributor.author Li C.-K.
dc.contributor.author Viprakasit V.
dc.contributor.author Elalfy M.S.
dc.contributor.author Kattamis A.
dc.contributor.author Smith G.
dc.contributor.author Habr D.
dc.contributor.author Domokos G.
dc.contributor.author Roubert B.
dc.contributor.author Taher A.
dc.contributor.editor
dc.date Jan-2011
dc.date.accessioned 2017-10-05T15:37:55Z
dc.date.available 2017-10-05T15:37:55Z
dc.date.issued 2011
dc.identifier 10.3324/haematol.2010.031468
dc.identifier.isbn
dc.identifier.issn 03906078
dc.identifier.uri http://hdl.handle.net/10938/16025
dc.description.abstract Background The efficacy of cardiac iron chelation in transfusion-dependent patients has been demonstrated in one-year prospective trials. Since normalization of cardiac T2* takes several years, the efficacy and safety of deferasirox was assessed for two years in patients with β-thalassemia major in the cardiac sub-study of the EPIC trial. Design and Methods Eligible patients with myocardial T2* greater than 5 to less than 20 ms received deferasirox, with the primary endpoint being the change in T2* from baseline to two years. Results Baseline myocardial T2* was severe (5 to 10 ms) in 39 patients, and moderate-to-mild (10 to 20 ms) in 62 patients. Mean deferasirox dose was 33.1±3.7 mg-kg-d in the one-year core study increasing to 36.1±7.7 mg-kg-d during the second year of treatment. Geometric mean myocardial T2* increased from a baseline of 11.2 to 14.8 ms at two years (P0.001). In patients with moderate-to-mild baseline T2*, an increase was seen from 14.7 to 20.1 ms, with normalization (≥20 ms) in 56.7percent of patients. In those with severe cardiac iron overload at baseline, 42.9percent improved to the moderate-to-mild group. The incidence of drug-related adverse events did not increase during the extension relative to the core study and included (≥5percent) increased serum creatinine, rash and increased alanine aminotransferase. Conclusions Continuous treatment with deferasirox for two years with a target dose of 40 mg-kg-d continued to remove iron from the heart in patients with β-thalassemia major and mild, moderate and severe cardiac siderosis. © 2011 Ferrata Storti Foundation.
dc.format.extent
dc.format.extent Pages: (48-54)
dc.language English
dc.publisher PAVIA
dc.relation.ispartof Publication Name: Haematologica; Publication Year: 2011; Volume: 96; no. 1; Pages: (48-54);
dc.relation.ispartofseries
dc.relation.uri
dc.source Scopus
dc.subject.other
dc.title Continued improvement in myocardial T2* over two years of deferasirox therapy in β-thalassemia major patients with cardiac iron overload
dc.type Article
dc.contributor.affiliation Pennell, D.J., Royal Brompton Hospital, London, United Kingdom
dc.contributor.affiliation Porter, J.B., University College London, London, United Kingdom
dc.contributor.affiliation Cappellini, M.D., Universitá di Milano, Ca Granda Foundation IRCCS, Milan, Italy
dc.contributor.affiliation Chan, L.L., University Malaya Medical Centre, Kuala Lumpur, Malaysia
dc.contributor.affiliation El-Beshlawy, A., Cairo University, Cairo, Egypt
dc.contributor.affiliation Aydinok, Y., Ege University Medical Faculty, Izmir, Turkey
dc.contributor.affiliation Ibrahim, H., Hospital Kuala Lumpur, Kuala Lumpur, Malaysia
dc.contributor.affiliation Li, C.-K., Prince of Wales Hospital, Chinese University of Hong Kong, Hong Kong
dc.contributor.affiliation Viprakasit, V., Department of Pediatrics, Siriraj Hospital, Mahidol University Bangkoknoi, Bangkok, Thailand
dc.contributor.affiliation Elalfy, M.S., Ain Shams University, Cairo, Egypt
dc.contributor.affiliation Kattamis, A., First Dept. of Pediatrics, University of Athens, Athens, Greece
dc.contributor.affiliation Smith, G., Royal Brompton Hospital, London, United Kingdom
dc.contributor.affiliation Habr, D., Novartis Pharmaceuticals, East Hanover, NJ, United States
dc.contributor.affiliation Domokos, G., Novartis Pharma AG, Basel, Switzerland
dc.contributor.affiliation Roubert, B., Novartis Pharma AG, Basel, Switzerland
dc.contributor.affiliation Taher, A., American University of Beirut, Beirut, Lebanon
dc.contributor.authorAddress Pennell, D. J.; Royal Brompton Hospital, Sydney Street, London SW3 6NP, United Kingdom; email: d.pennell@ic.ac.uk
dc.contributor.authorCorporate University: American University of Beirut Medical Center; Faculty: Faculty of Medicine; Department: Internal Medicine;
dc.contributor.authorDepartment Internal Medicine
dc.contributor.authorDivision
dc.contributor.authorEmail d.pennell@ic.ac.uk
dc.contributor.authorFaculty Faculty of Medicine
dc.contributor.authorInitials Pennell, DJ
dc.contributor.authorInitials Porter, JB
dc.contributor.authorInitials Cappellini, MD
dc.contributor.authorInitials Chan, LL
dc.contributor.authorInitials El-Beshlawy, A
dc.contributor.authorInitials Aydinok, Y
dc.contributor.authorInitials Ibrahim, H
dc.contributor.authorInitials Li, CK
dc.contributor.authorInitials Viprakasit, V
dc.contributor.authorInitials Elalfy, MS
dc.contributor.authorInitials Kattamis, A
dc.contributor.authorInitials Smith, G
dc.contributor.authorInitials Habr, D
dc.contributor.authorInitials Domokos, G
dc.contributor.authorInitials Roubert, B
dc.contributor.authorInitials Taher, A
dc.contributor.authorOrcidID
dc.contributor.authorReprintAddress Pennell, DJ (reprint author), Royal Brompton Hosp, Cardiovasc MR Unit, Sydney St, London SW3 6NP, England.
dc.contributor.authorResearcherID Li, Chi Kong-J-8456-2013
dc.contributor.authorUniversity American University of Beirut Medical Center
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dc.description.citedCount 36
dc.description.citedTotWOSCount 37
dc.description.citedWOSCount 32
dc.format.extentCount 7
dc.identifier.articleNo
dc.identifier.coden HAEMA
dc.identifier.pubmedID 21071497
dc.identifier.scopusID 78650996705
dc.identifier.url
dc.publisher.address VIA GIUSEPPE BELLI 4, 27100 PAVIA, ITALY
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dc.relation.ispartofConferenceCode
dc.relation.ispartofConferenceDate
dc.relation.ispartofConferenceHosting
dc.relation.ispartofConferenceLoc
dc.relation.ispartofConferenceSponsor
dc.relation.ispartofConferenceTitle
dc.relation.ispartofFundingAgency
dc.relation.ispartOfISOAbbr Haematol-Hematol. J.
dc.relation.ispartOfIssue 1
dc.relation.ispartOfPart
dc.relation.ispartofPubTitle Haematologica
dc.relation.ispartofPubTitleAbbr Haematologica
dc.relation.ispartOfSpecialIssue
dc.relation.ispartOfSuppl
dc.relation.ispartOfVolume 96
dc.source.ID WOS:000286902400010
dc.type.publication Journal
dc.subject.otherAuthKeyword β-thalassemia major
dc.subject.otherAuthKeyword Cardiac iron overload
dc.subject.otherAuthKeyword Deferasirox
dc.subject.otherAuthKeyword Iron chelation
dc.subject.otherAuthKeyword Myocardial T2*
dc.subject.otherChemCAS deferasirox, 201530-41-8
dc.subject.otherChemCAS ferritin, 9007-73-2
dc.subject.otherChemCAS iron, 14093-02-8, 53858-86-9, 7439-89-6
dc.subject.otherChemCAS Benzoic Acids
dc.subject.otherChemCAS Ferritins, 9007-73-2
dc.subject.otherChemCAS Iron, 7439-89-6
dc.subject.otherChemCAS Iron Chelating Agents
dc.subject.otherChemCAS Triazoles
dc.subject.otherChemCAS deferasirox
dc.subject.otherIndex deferasirox
dc.subject.otherIndex ferritin
dc.subject.otherIndex iron
dc.subject.otherIndex adolescent
dc.subject.otherIndex alanine aminotransferase blood level
dc.subject.otherIndex article
dc.subject.otherIndex aspartate aminotransferase blood level
dc.subject.otherIndex child
dc.subject.otherIndex creatinine blood level
dc.subject.otherIndex disease severity
dc.subject.otherIndex drug efficacy
dc.subject.otherIndex drug eruption
dc.subject.otherIndex drug safety
dc.subject.otherIndex drug withdrawal
dc.subject.otherIndex female
dc.subject.otherIndex ferritin blood level
dc.subject.otherIndex heart function
dc.subject.otherIndex heart muscle
dc.subject.otherIndex human
dc.subject.otherIndex incidence
dc.subject.otherIndex iron overload
dc.subject.otherIndex kidney tubule disorder
dc.subject.otherIndex major clinical study
dc.subject.otherIndex male
dc.subject.otherIndex multicenter study
dc.subject.otherIndex nephritis
dc.subject.otherIndex prospective study
dc.subject.otherIndex school child
dc.subject.otherIndex side effect
dc.subject.otherIndex thalassemia major
dc.subject.otherIndex Adolescent
dc.subject.otherIndex Adult
dc.subject.otherIndex Benzoic Acids
dc.subject.otherIndex beta-Thalassemia
dc.subject.otherIndex Child
dc.subject.otherIndex Female
dc.subject.otherIndex Ferritins
dc.subject.otherIndex Humans
dc.subject.otherIndex Iron
dc.subject.otherIndex Iron Chelating Agents
dc.subject.otherIndex Iron Overload
dc.subject.otherIndex Magnetic Resonance Imaging
dc.subject.otherIndex Male
dc.subject.otherIndex Middle Aged
dc.subject.otherIndex Myocardium
dc.subject.otherIndex Prognosis
dc.subject.otherIndex Prospective Studies
dc.subject.otherIndex Triazoles
dc.subject.otherIndex Ventricular Function, Left
dc.subject.otherIndex Young Adult
dc.subject.otherKeywordPlus MAGNETIC-RESONANCE
dc.subject.otherKeywordPlus DEFEROXAMINE
dc.subject.otherKeywordPlus SURVIVAL
dc.subject.otherKeywordPlus DEFERIPRONE
dc.subject.otherKeywordPlus TRANSFUSION
dc.subject.otherKeywordPlus TRIAL
dc.subject.otherWOS Hematology


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