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Pulmonary hypertension in β-thalassemia major and the role of L-carnitine therapy

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dc.contributor.author El-Beshlawy A.
dc.contributor.author Youssry I.
dc.contributor.author El-Saidi S.
dc.contributor.author El Accaoui R.
dc.contributor.author Mansi Y.
dc.contributor.author Makhlouf A.
dc.contributor.author Taher A.
dc.contributor.editor
dc.date Nov-2008
dc.date.accessioned 2017-10-05T15:37:32Z
dc.date.available 2017-10-05T15:37:32Z
dc.date.issued 2008
dc.identifier 10.1080/08880010802244035
dc.identifier.isbn
dc.identifier.issn 08880018
dc.identifier.uri http://hdl.handle.net/10938/15773
dc.description.abstract Cardiac complications, such as pulmonary hypertension (PHT), are the leading cause of death in β-thalassemia patients. L-Carnitine, due to its role in fatty acid oxidation, might help control the elevation in pulmonary artery systolic pressure (PASP). The objectives of this study were to assess the prevalence of PHT in β-thalassemia major patients, identify clinical predictors for its development, and determine the potential effects of L-carnitine. In total, 32 patients with β-thalassemia major were recruited; 16 age- and sex-matched children constituted the control group. Cardiac evaluation was performed by using echocardiography. The patients with PHT received 50 mg-kg-day L-carnitine orally for 3 months and were then reevaluated. Based on PASP, the patients were divided into group A without PHT and group B with PHT. The prevalence of PHT was 37.5percent. The other echocardiographic measurements were not significantly different between groups A and B. PASP did not have any significant correlation with the following variables: age, total number of blood units received, splenic status, serum ferritin level, and ejection fraction. Following the administration of L-carnitine, there was a significant decrease in the mean PASP from 33.96 ± 7.85 to 24.11 ± 7.61. All cardiac dimensions decreased following L-carnitine, but the changes were not statistically significant. Even though β-thalassemia major resulted in an elevation in the PASP in only a fraction of the patients, it seems to have an impact on the heart dimensions and function of all patients. No clinical predictors were identified. Oral administration of L-carnitine appears to significantly improve PASP. Copyright © Informa Healthcare USA, Inc.
dc.format.extent
dc.format.extent Pages: (734-743)
dc.language English
dc.publisher PHILADELPHIA
dc.relation.ispartof Publication Name: Pediatric Hematology and Oncology; Publication Year: 2008; Volume: 25; no. 8; Pages: (734-743);
dc.relation.ispartofseries
dc.relation.uri
dc.source Scopus
dc.subject.other
dc.title Pulmonary hypertension in β-thalassemia major and the role of L-carnitine therapy
dc.type Article
dc.contributor.affiliation El-Beshlawy, A., Pediatric Department, Cairo University, Cairo, Egypt, 32, Falaky Street, Bab El-Louk, Cairo, Egypt
dc.contributor.affiliation Youssry, I., Pediatric Department, Cairo University, Cairo, Egypt
dc.contributor.affiliation El-Saidi, S., Pediatric Department, Cairo University, Cairo, Egypt
dc.contributor.affiliation El Accaoui, R., Department of Internal Medicine, American University, Beirut Medical Center, Beirut, Lebanon
dc.contributor.affiliation Mansi, Y., Pediatric Department, Cairo University, Cairo, Egypt
dc.contributor.affiliation Makhlouf, A., Pediatric Department, Cairo University, Cairo, Egypt
dc.contributor.affiliation Taher, A., Department of Internal Medicine, American University, Beirut Medical Center, Beirut, Lebanon
dc.contributor.authorAddress El-Beshlawy, A.32, Falaky Street, Bab El-Louk, Cairo, Egypt; email: amalelbeshlawy@yahoo.com
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 amalelbeshlawy@yahoo.com
dc.contributor.authorFaculty Faculty of Medicine
dc.contributor.authorInitials El-Beshlawy, A
dc.contributor.authorInitials Youssry, I
dc.contributor.authorInitials El-Saidi, S
dc.contributor.authorInitials El Accaoui, R
dc.contributor.authorInitials Mansi, Y
dc.contributor.authorInitials Makhlouf, A
dc.contributor.authorInitials Taher, A
dc.contributor.authorOrcidID
dc.contributor.authorReprintAddress El-Beshlawy, A (reprint author), 32 Falaky St, Cairo, Egypt.
dc.contributor.authorResearcherID
dc.contributor.authorUniversity American University of Beirut Medical Center
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dc.description.citedCount 17
dc.description.citedTotWOSCount 15
dc.description.citedWOSCount 15
dc.format.extentCount 10
dc.identifier.articleNo
dc.identifier.coden PHONE
dc.identifier.pubmedID 19065439
dc.identifier.scopusID 57449085977
dc.identifier.url
dc.publisher.address 325 CHESTNUT ST, SUITE 800, PHILADELPHIA, PA 19106 USA
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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 Pediatr. Hematol. Oncol.
dc.relation.ispartOfIssue 8
dc.relation.ispartOfPart
dc.relation.ispartofPubTitle Pediatric Hematology and Oncology
dc.relation.ispartofPubTitleAbbr Pediatr. Hematol. Oncol.
dc.relation.ispartOfSpecialIssue
dc.relation.ispartOfSuppl
dc.relation.ispartOfVolume 25
dc.source.ID WOS:000261504200003
dc.type.publication Journal
dc.subject.otherAuthKeyword β-thalassemia
dc.subject.otherAuthKeyword Echocardiography
dc.subject.otherAuthKeyword L-carnitine
dc.subject.otherAuthKeyword Pulmonary hypertension
dc.subject.otherChemCAS carnitine, 461-06-3, 541-15-1, 56-99-5
dc.subject.otherChemCAS ferritin, 9007-73-2
dc.subject.otherChemCAS Carnitine, 541-15-1
dc.subject.otherIndex carnitine
dc.subject.otherIndex ferritin
dc.subject.otherIndex absence of side effects
dc.subject.otherIndex adolescent
dc.subject.otherIndex article
dc.subject.otherIndex beta thalassemia
dc.subject.otherIndex child
dc.subject.otherIndex clinical article
dc.subject.otherIndex controlled study
dc.subject.otherIndex drug effect
dc.subject.otherIndex echocardiography
dc.subject.otherIndex female
dc.subject.otherIndex ferritin blood level
dc.subject.otherIndex heart ejection fraction
dc.subject.otherIndex heart function
dc.subject.otherIndex heart size
dc.subject.otherIndex human
dc.subject.otherIndex lung artery pressure
dc.subject.otherIndex male
dc.subject.otherIndex multivariate analysis
dc.subject.otherIndex prevalence
dc.subject.otherIndex protein blood level
dc.subject.otherIndex pulmonary hypertension
dc.subject.otherIndex Administration, Oral
dc.subject.otherIndex Adolescent
dc.subject.otherIndex beta-Thalassemia
dc.subject.otherIndex Carnitine
dc.subject.otherIndex Case-Control Studies
dc.subject.otherIndex Child
dc.subject.otherIndex Child, Preschool
dc.subject.otherIndex Echocardiography
dc.subject.otherIndex Female
dc.subject.otherIndex Heart Diseases
dc.subject.otherIndex Humans
dc.subject.otherIndex Hypertension, Pulmonary
dc.subject.otherIndex Male
dc.subject.otherIndex Prevalence
dc.subject.otherIndex Regression Analysis
dc.subject.otherKeywordPlus SICKLE-CELL-DISEASE
dc.subject.otherKeywordPlus DOPPLER-ECHOCARDIOGRAPHY
dc.subject.otherKeywordPlus ARTERY PRESSURE
dc.subject.otherKeywordPlus HEART-FAILURE
dc.subject.otherKeywordPlus CHILDREN
dc.subject.otherWOS Oncology
dc.subject.otherWOS Hematology
dc.subject.otherWOS Pediatrics


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