Document Detail


Apoptosis in thalassemia major reduced by a butyrate derivative.
MedLine Citation:
PMID:  16227679     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND AND OBJECTIVES: In cases of beta-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 88.9% of cases prior to treatment versus 16.7% 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.
Authors:
A El-Beshlawy; H Seoud; A Ibrahim; I Youssry; H Gabre; H Isma'eel; E Aoun; A Taher
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Acta haematologica     Volume:  114     ISSN:  0001-5792     ISO Abbreviation:  Acta Haematol.     Publication Date:  2005  
Date Detail:
Created Date:  2005-10-17     Completed Date:  2005-11-21     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0141053     Medline TA:  Acta Haematol     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  155-9     Citation Subset:  IM    
Copyright Information:
(c) 2005 S. Karger AG, Basel
Affiliation:
Pediatric Department, School of Medicine and Al Azhar Universities, Cairo, Egypt.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Apoptosis / drug effects*
Bone Marrow Cells / drug effects,  pathology
Carnitine / therapeutic use*
Child
Child, Preschool
DNA Fragmentation / drug effects
Erythroblasts / drug effects,  pathology
Female
Hematologic Agents / therapeutic use*
Humans
Male
Nucleosomes / drug effects,  pathology
beta-Thalassemia / drug therapy*,  pathology*
Chemical
Reg. No./Substance:
0/Hematologic Agents; 0/Nucleosomes; 541-15-1/Carnitine

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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