Document Detail


Longitudinal study of survival and causes of death in patients with thalassemia major in Greece.
MedLine Citation:
PMID:  16339695     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Iron-induced organ degeneration is the main factor of mortality in patients with thalassemia major. Since chelation therapy is at a turning point, from the laborious parenteral route to the use of new promising oral agents, we investigated the current status of survival of these patients to present reliable data that will be useful in future comparative studies. Survival probabilities were estimated by the Kaplan-Meier method, and results were compared by the log-rank test in a total of 647 thalassemic patients (pts) (52% males) born between 1/1/58 and 1/2/04. Terminal follow-up was 1/12/04. All transfusion-dependent pts monitored in our center, or in frequent contact if they had moved elsewhere, were strictly selected, excluding all rarely transfused or intermediate cases. Pts born before 1/1/75 were classified in group A (n = 366), while pts born later were included in group B (n = 281). According to the last 5 years' mean serum ferritin level, pts were divided into three hemosiderosis groups: (1) mild (<2000 microg/L) 49%, (2) moderate (2000-4000 microg/L) 28%, and (3) severe (>4000 microg/L) 23%. Of the 647 pts, 115 died (mean age: 22.6 +/- 6.2 years), most frequently by heart failure (71.3%) followed by sepsis (7.8%). Life expectancy in the entire population was up to 59% at 46 years. Survival was higher for pts born after 1975 than those before (P < .001). Statistically significantly different survival probabilities were found between groups with mild, moderate, or severe hemosiderosis (P < .001). Effective management with improved chelation therapy could lead to better results.
Authors:
Vassilis Ladis; George Chouliaras; Helen Berdousi; Emmanuel Kanavakis; Christos Kattamis
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Annals of the New York Academy of Sciences     Volume:  1054     ISSN:  0077-8923     ISO Abbreviation:  Ann. N. Y. Acad. Sci.     Publication Date:  2005  
Date Detail:
Created Date:  2005-12-12     Completed Date:  2006-07-13     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  7506858     Medline TA:  Ann N Y Acad Sci     Country:  United States    
Other Details:
Languages:  eng     Pagination:  445-50     Citation Subset:  IM    
Affiliation:
Thalassemia Unit, First Department of Pediatrics, University of Athens, Athens, Greece. thalas@paidon-agiasofia.gr
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MeSH Terms
Descriptor/Qualifier:
Acquired Immunodeficiency Syndrome / mortality
Adolescent
Adult
Age Factors
Blood Transfusion
Bone Marrow Transplantation / mortality
Cause of Death*
Chelation Therapy
Child
Child, Preschool
Cohort Studies
Combined Modality Therapy
Female
Ferritins / blood
Greece / epidemiology
Heart Failure / mortality
Hemosiderosis / etiology,  mortality
Humans
Life Expectancy
Life Tables
Liver Diseases / mortality
Longitudinal Studies
Male
Middle Aged
Sepsis / mortality
Survival Analysis
Thromboembolism / mortality
beta-Thalassemia / drug therapy,  epidemiology*,  mortality,  surgery,  therapy
Chemical
Reg. No./Substance:
9007-73-2/Ferritins

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


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