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Apoptosis in thalassemia major reduced by a butyrate derivative

Show simple item record El-Beshlawy A. Seoud H. Ibrahim A. Youssry I. Gabre H. Isma'eel H. Aoun E. Taher A.
dc.contributor.editor Oct-2005 2017-10-05T15:40:19Z 2017-10-05T15:40:19Z 2005
dc.identifier 10.1159/000087890
dc.identifier.issn 00015792
dc.description.abstract Background and Objectives: In cases of β-thalassemia major, apoptosis appears to be greatly enhanced in the early-stage erythroid precursors in the bone marrow leading to ineffective erythropoiesis. L-Carnitine is found to strongly reduce apoptosis in different diseases. We investigated the effect of oral L-carnitine therapy on apoptosis in thalassemia major patients. Methods: Eighteen thalassemia major patients with a mean age of 12.2 ± 6.6 years were included. Detection of apoptosis was done by photometric enzyme immunoassay (ELISA) and agarose gel electrophoresis before and after 6 months of oral therapy with L-carnitine (50 mg-kg-day). Results: A significant decrease of apoptosis frequency in the erythroid precursors in the bone marrow of studied cases was noted after therapy. The quantity of nucleosomes measured by ELISA dropped from 3.65 ± 1.338 to 1.60 ± 0.65 after therapy (p = 0.005). A positive ladder pattern reflecting apoptosis on agarose gel electrophoresis was detected in 88.9percent of cases prior to treatment versus 16.7percent after therapy (p = 0.006). Patients also had a significant decrease in the frequency of transfusions and increase in the pre-transfusion hemoglobin levels after therapy. Conclusion: L-Carnitine seems to be a good modulator of apoptotic processes in thalassemic patients leading to a decreased frequency of programmed erythroblast death and general improvement of the disease condition. Copyright © 2005 S. Karger AG.
dc.format.extent Pages: (155-159)
dc.language English
dc.publisher BASEL
dc.relation.ispartof Publication Name: Acta Haematologica; Publication Year: 2005; Volume: 114; no. 3; Pages: (155-159);
dc.source Scopus
dc.title Apoptosis in thalassemia major reduced by a butyrate derivative
dc.type Article
dc.contributor.affiliation El-Beshlawy, A., Pediatric Department, School of Medicine, Al Azhar Universities, Cairo, Egypt
dc.contributor.affiliation Seoud, H., Pediatric Department, School of Medicine, Al Azhar Universities, Cairo, Egypt
dc.contributor.affiliation Ibrahim, A., Clinical Pathology Department, School of Medicine, Al Azhar Universities, Cairo, Egypt
dc.contributor.affiliation Youssry, I., Pediatric Department, School of Medicine, Al Azhar Universities, Cairo, Egypt, 1 Ben Kutiba st., Nasr City, Cairo, Egypt
dc.contributor.affiliation Gabre, H., Clinical Pathology Department, School of Medicine, Al Azhar Universities, Cairo, Egypt
dc.contributor.affiliation Isma'eel, H., Department of Internal Medicine, American University of Beirut, Medical Center, Beirut, Lebanon
dc.contributor.affiliation Aoun, E., Department of Internal Medicine, American University of Beirut, Medical Center, Beirut, Lebanon
dc.contributor.affiliation Taher, A., Department of Internal Medicine, American University of Beirut, Medical Center, Beirut, Lebanon
dc.contributor.authorAddress Youssry, I.1 Ben Kutiba st., Nasr City, Cairo, Egypt; email:
dc.contributor.authorCorporate University: American University of Beirut Medical Center; Faculty: Faculty of Medicine; Department: Internal Medicine;
dc.contributor.authorDepartment Internal Medicine
dc.contributor.authorFaculty Faculty of Medicine
dc.contributor.authorInitials El-Beshlawy, A
dc.contributor.authorInitials Seoud, H
dc.contributor.authorInitials Ibrahim, A
dc.contributor.authorInitials Youssry, I
dc.contributor.authorInitials Gabre, H
dc.contributor.authorInitials Isma'eel, H
dc.contributor.authorInitials Aoun, E
dc.contributor.authorInitials Taher, A
dc.contributor.authorReprintAddress Youssry, I (reprint author), 1 Ben Kutiba St, Nasr City, Cairo, Egypt.
dc.contributor.authorUniversity American University of Beirut Medical Center
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dc.description.citedCount 9
dc.description.citedTotWOSCount 5
dc.description.citedWOSCount 5
dc.format.extentCount 5
dc.identifier.coden ACHAA
dc.identifier.pubmedID 16227679
dc.identifier.scopusID 27544462407
dc.relation.ispartOfISOAbbr Acta Haematol.
dc.relation.ispartOfIssue 3
dc.relation.ispartofPubTitle Acta Haematologica
dc.relation.ispartofPubTitleAbbr Acta Haematol.
dc.relation.ispartOfVolume 114
dc.source.ID WOS:000232664900006
dc.type.publication Journal
dc.subject.otherAuthKeyword Apoptosis
dc.subject.otherAuthKeyword L-Carnitine
dc.subject.otherAuthKeyword Thalassemia
dc.subject.otherChemCAS carnitine, 461-06-3, 541-15-1, 56-99-5
dc.subject.otherChemCAS hemoglobin, 9008-02-0
dc.subject.otherChemCAS Carnitine, 541-15-1
dc.subject.otherChemCAS Hematologic Agents
dc.subject.otherChemCAS Nucleosomes
dc.subject.otherIndex butyric acid derivative
dc.subject.otherIndex carnitine
dc.subject.otherIndex hemoglobin
dc.subject.otherIndex adolescent
dc.subject.otherIndex adult
dc.subject.otherIndex agar gel electrophoresis
dc.subject.otherIndex apoptosis
dc.subject.otherIndex article
dc.subject.otherIndex blood transfusion
dc.subject.otherIndex bone marrow
dc.subject.otherIndex child
dc.subject.otherIndex clinical article
dc.subject.otherIndex drug efficacy
dc.subject.otherIndex enzyme linked immunosorbent assay
dc.subject.otherIndex erythroblast
dc.subject.otherIndex erythroid precursor cell
dc.subject.otherIndex female
dc.subject.otherIndex human
dc.subject.otherIndex human cell
dc.subject.otherIndex male
dc.subject.otherIndex nucleosome
dc.subject.otherIndex photometry
dc.subject.otherIndex priority journal
dc.subject.otherIndex thalassemia major
dc.subject.otherIndex time series analysis
dc.subject.otherIndex Adolescent
dc.subject.otherIndex Apoptosis
dc.subject.otherIndex beta-Thalassemia
dc.subject.otherIndex Bone Marrow Cells
dc.subject.otherIndex Carnitine
dc.subject.otherIndex Child
dc.subject.otherIndex Child, Preschool
dc.subject.otherIndex DNA Fragmentation
dc.subject.otherIndex Erythroblasts
dc.subject.otherIndex Female
dc.subject.otherIndex Hematologic Agents
dc.subject.otherIndex Humans
dc.subject.otherIndex Male
dc.subject.otherIndex Nucleosomes
dc.subject.otherKeywordPlus PROGRAMMED CELL-DEATH
dc.subject.otherKeywordPlus L-CARNITINE TREATMENT
dc.subject.otherKeywordPlus CERAMIDE GENERATION
dc.subject.otherKeywordPlus BETA-THALASSEMIA
dc.subject.otherKeywordPlus PROPIONYL CARNITINE
dc.subject.otherKeywordPlus DISEASE
dc.subject.otherKeywordPlus ERYTHROPOIESIS
dc.subject.otherKeywordPlus ERYTHROCYTES
dc.subject.otherKeywordPlus ANEMIA
dc.subject.otherKeywordPlus SYSTEM
dc.subject.otherWOS Hematology

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