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Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: The optimal care study

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dc.contributor.author Taher A.T.
dc.contributor.author Musallam K.M.
dc.contributor.author Karimi M.
dc.contributor.author El-Beshlawy A.
dc.contributor.author Belhoul K.
dc.contributor.author Daar S.
dc.contributor.author Saned M.-S.
dc.contributor.author El-Chafic A.-H.
dc.contributor.author Fasulo M.R.
dc.contributor.author Cappellini M.D.
dc.contributor.editor
dc.date Mar-2010
dc.date.accessioned 2017-10-05T15:39:30Z
dc.date.available 2017-10-05T15:39:30Z
dc.date.issued 2010
dc.identifier 10.1182/blood-2009-09-243154
dc.identifier.isbn
dc.identifier.issn 00064971
dc.identifier.uri http://hdl.handle.net/10938/16910
dc.description.abstract Despite recent advances in understanding the pathophysiologic mechanisms behind the thalassemia intermedia (TI) phenotype, data on the effects of treatment are deficient. To provide such data, we evaluated 584 TI patients for the associations between patient and disease characteristics, treatment received, and the rate of complications. The most common disease-related complications were osteoporosis, extramedullary hematopoeisis (EMH), hypogonadism, and cholelithiasis, followed by thrombosis, pulmonary hypertension (PHT), abnormal liver function, and leg ulcers. Hypothyroidism, heart failure, and diabetes mellitus were less frequently observed. On multivariate analysis, older age and splenectomy were independently associated with an increased risk of most disease-related complications. Transfusion therapy was protective for thrombosis, EMH, PHT, heart failure, cholelithiasis, and leg ulcers. However, transfusion therapy was associated with an increased risk of endocrinopathy. Iron chelation therapy was in turn protective for endocrinopathy and PHT. Hydroxyurea treatment was associated with an increased risk of hypogonadism yet was protective for EMH, PHT, leg ulcers, hypothyroidism, and osteoporosis. Attention should be paid to the impact of age on complications in TI, and the beneficial role of splenectomy deserves revisiting. This study provides evidence that calls for prospective evaluation of the roles of transfusion, iron chelation, and hydroxyurea therapy in TI patients. © 2010 by The American Society of Hematology.
dc.format.extent
dc.format.extent Pages: (1886-1892)
dc.language English
dc.publisher WASHINGTON
dc.relation.ispartof Publication Name: Blood; Publication Year: 2010; Volume: 115; no. 10; Pages: (1886-1892);
dc.relation.ispartofseries
dc.relation.uri
dc.source Scopus
dc.subject.other
dc.title Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: The optimal care study
dc.type Article
dc.contributor.affiliation Taher, A.T., Department of Internal Medicine, Hematology-Oncology Division, American University of Beirut Medical Center, Beirut, Lebanon
dc.contributor.affiliation Musallam, K.M., Department of Internal Medicine, Hematology-Oncology Division, American University of Beirut Medical Center, Beirut, Lebanon
dc.contributor.affiliation Karimi, M., Department of Pediatrics, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran
dc.contributor.affiliation El-Beshlawy, A., Department of Pediatrics, Cairo University, Cairo, Egypt
dc.contributor.affiliation Belhoul, K., Genetic and Thalassemia Center, Al Wasl Hospital, Dubai, United Arab Emirates
dc.contributor.affiliation Daar, S., Sultan Qaboos University, Muscat, Oman
dc.contributor.affiliation Saned, M.-S., Genetic and Thalassemia Center, Al Wasl Hospital, Dubai, United Arab Emirates
dc.contributor.affiliation El-Chafic, A.-H., Department of Internal Medicine, Hematology-Oncology Division, American University of Beirut Medical Center, Beirut, Lebanon
dc.contributor.affiliation Fasulo, M.R., Ospedale Maggiore Policlinico, Istituto di Ricovero e Cura a Carattere Scientifico, University of Milan, Milano, Italy
dc.contributor.affiliation Cappellini, M.D., Ospedale Maggiore Policlinico, Istituto di Ricovero e Cura a Carattere Scientifico, University of Milan, Milano, Italy
dc.contributor.authorAddress Taher, A. T.; Department of Internal Medicine, Hematology-Oncology Division, American University of Beirut Medical Center, Beirut, Lebanon; email: ataher@aub.edu.lb
dc.contributor.authorCorporate University: American University of Beirut Medical Center; Faculty: Faculty of Medicine; Department: Internal Medicine; Division: Hematology-Oncology;
dc.contributor.authorDepartment Internal Medicine
dc.contributor.authorDivision Hematology-Oncology
dc.contributor.authorEmail ataher@aub.edu.lb
dc.contributor.authorFaculty Faculty of Medicine
dc.contributor.authorInitials Taher, AT
dc.contributor.authorInitials Musallam, KM
dc.contributor.authorInitials Karimi, M
dc.contributor.authorInitials El-Beshlawy, A
dc.contributor.authorInitials Belhoul, K
dc.contributor.authorInitials Daar, S
dc.contributor.authorInitials Saned, MS
dc.contributor.authorInitials El-Chafic, AH
dc.contributor.authorInitials Fasulo, MR
dc.contributor.authorInitials Cappellini, MD
dc.contributor.authorOrcidID
dc.contributor.authorReprintAddress Taher, AT (reprint author), Amer Univ Beirut, Med Ctr, Dept Internal Med, Div Hematol Oncol, POB 11-0236,Riad El Solh 1107, Beirut 2020, Lebanon.
dc.contributor.authorResearcherID
dc.contributor.authorUniversity American University of Beirut Medical Center
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dc.description.citedCount 90
dc.description.citedTotWOSCount 95
dc.description.citedWOSCount 91
dc.format.extentCount 7
dc.identifier.articleNo
dc.identifier.coden BLOOA
dc.identifier.pubmedID 20032507
dc.identifier.scopusID 77950420281
dc.identifier.url
dc.publisher.address 1900 M STREET. NW SUITE 200, WASHINGTON, DC 20036 USA
dc.relation.ispartofConference
dc.relation.ispartofConferenceCode
dc.relation.ispartofConferenceDate
dc.relation.ispartofConferenceHosting
dc.relation.ispartofConferenceLoc
dc.relation.ispartofConferenceSponsor
dc.relation.ispartofConferenceTitle
dc.relation.ispartofFundingAgency
dc.relation.ispartOfISOAbbr Blood
dc.relation.ispartOfIssue 10
dc.relation.ispartOfPart
dc.relation.ispartofPubTitle Blood
dc.relation.ispartofPubTitleAbbr Blood
dc.relation.ispartOfSpecialIssue
dc.relation.ispartOfSuppl
dc.relation.ispartOfVolume 115
dc.source.ID WOS:000275751300009
dc.type.publication Journal
dc.subject.otherAuthKeyword
dc.subject.otherChemCAS hydroxyurea, 127-07-1
dc.subject.otherIndex hydroxyurea
dc.subject.otherIndex adolescent
dc.subject.otherIndex adult
dc.subject.otherIndex age distribution
dc.subject.otherIndex aged
dc.subject.otherIndex article
dc.subject.otherIndex blood transfusion
dc.subject.otherIndex child
dc.subject.otherIndex cholelithiasis
dc.subject.otherIndex clinical evaluation
dc.subject.otherIndex diabetes mellitus
dc.subject.otherIndex endemic disease
dc.subject.otherIndex endocrine disease
dc.subject.otherIndex extramedullary hematopoiesis
dc.subject.otherIndex female
dc.subject.otherIndex heart failure
dc.subject.otherIndex human
dc.subject.otherIndex hypogonadism
dc.subject.otherIndex hypothyroidism
dc.subject.otherIndex iron chelation
dc.subject.otherIndex leg ulcer
dc.subject.otherIndex liver function test
dc.subject.otherIndex major clinical study
dc.subject.otherIndex male
dc.subject.otherIndex medical practice
dc.subject.otherIndex multivariate analysis
dc.subject.otherIndex osteoporosis
dc.subject.otherIndex preschool child
dc.subject.otherIndex priority journal
dc.subject.otherIndex prospective study
dc.subject.otherIndex pulmonary hypertension
dc.subject.otherIndex retrospective study
dc.subject.otherIndex school child
dc.subject.otherIndex splenectomy
dc.subject.otherIndex thalassemia
dc.subject.otherIndex thrombosis
dc.subject.otherIndex Adolescent
dc.subject.otherIndex Adult
dc.subject.otherIndex Aged
dc.subject.otherIndex Child
dc.subject.otherIndex Child, Preschool
dc.subject.otherIndex Endemic Diseases
dc.subject.otherIndex Endocrine System Diseases
dc.subject.otherIndex Female
dc.subject.otherIndex Hematopoiesis, Extramedullary
dc.subject.otherIndex Humans
dc.subject.otherIndex Hypertension, Pulmonary
dc.subject.otherIndex Liver Diseases
dc.subject.otherIndex Male
dc.subject.otherIndex Middle Aged
dc.subject.otherIndex Professional Practice
dc.subject.otherIndex Retrospective Studies
dc.subject.otherIndex Thalassemia
dc.subject.otherIndex Thrombosis
dc.subject.otherIndex Young Adult
dc.subject.otherKeywordPlus PULMONARY ARTERIAL-HYPERTENSION
dc.subject.otherKeywordPlus BETA-THALASSEMIA
dc.subject.otherKeywordPlus IRON-OVERLOAD
dc.subject.otherKeywordPlus SPLENECTOMIZED PATIENTS
dc.subject.otherKeywordPlus CHELATION-THERAPY
dc.subject.otherKeywordPlus HEART-DISEASE
dc.subject.otherKeywordPlus HYDROXYUREA
dc.subject.otherKeywordPlus TRANSFUSION
dc.subject.otherKeywordPlus HEMOGLOBIN
dc.subject.otherKeywordPlus SURVIVAL
dc.subject.otherWOS Hematology


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