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Cardiac status in well-treated patients with thalassemia major.
MedLine Citation:
PMID:  15458515     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: To assess cardiac status in a large group of patients with thalassemia major who had been treated in a standard way since their early infancy with intensive transfusions and deferoxamine chelation therapy and who had good compliance with this regimen. METHODS AND RESULTS: We assessed clinically and echocardiographically 202 thalassemia major patients aged 27.3 +/- 6.3 yr and 75 age and sex-matched healthy controls. Overt cardiac disease was encountered in 14 patients (6.9%), including 5 (2.5%) with congestive heart failure, aged 26-37 yr, and 9 with systolic left ventricular (LV) dysfunction, aged 23-37 yr. Ten patients (5.0%) had a history of pericarditis. Left atrial and LV diameters, LV mass and cardiac output were significantly higher in patients than in controls, while peripheral resistance and LV afterload were significantly lower. Relative LV wall thickness did not differ between patients and controls, but it was significantly lower in patients with overt cardiac disease compared to those without (P < 0.05). Restrictive LV filling was observed in 37.6% of patients and was significantly more frequent in cases with overt cardiac disease (P < 0.01). Pulmonary hypertension was practically absent. Hematological parameters and pulmonary artery pressure levels were not independently associated with the presence of overt cardiac disease. CONCLUSION: Strict lifelong adherence to the standard transfusion and deferoxamine therapy reduces considerably the occurrence of heart failure, LV dysfunction and pericarditis, prevents early heart failure and pulmonary hypertension, but does not eliminate completely cardiac disease in patients with thalassemia major.
Authors:
Athanasios Aessopos; Dimitrios Farmakis; Antonia Hatziliami; Christina Fragodimitri; Fotis Karabatsos; Jacqueline Joussef; Eftichia Mitilineou; Evanthia Diamanti-Kandaraki; John Meletis; Markisia Karagiorga
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  European journal of haematology     Volume:  73     ISSN:  0902-4441     ISO Abbreviation:  Eur. J. Haematol.     Publication Date:  2004 Nov 
Date Detail:
Created Date:  2004-10-01     Completed Date:  2004-11-06     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  8703985     Medline TA:  Eur J Haematol     Country:  Denmark    
Other Details:
Languages:  eng     Pagination:  359-66     Citation Subset:  IM    
Affiliation:
Cardiac Outpatient Clinic for Hemoglobinopathies, First Department of Internal Medicine, University of Athens Medical School, Laiko Hospital, Athens, Greece. dfarm1@panafonet.gr
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Blood Transfusion
Cardiac Output
Child
Deferoxamine / therapeutic use
Echocardiography
Female
Heart / physiopathology*
Heart Atria / pathology
Heart Diseases / epidemiology*,  prevention & control
Heart Failure / epidemiology
Heart Ventricles / pathology
Humans
Hypertension, Pulmonary / epidemiology
Iron Chelating Agents / therapeutic use
Male
Patient Compliance
Pericarditis / epidemiology
Ventricular Dysfunction, Left / epidemiology
beta-Thalassemia / complications*,  physiopathology,  therapy*
Chemical
Reg. No./Substance:
0/Iron Chelating Agents; 70-51-9/Deferoxamine

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


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