Document Detail


Myocardial iron loading in transfusion-dependent thalassemia and sickle cell disease.
MedLine Citation:
PMID:  14630822     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Cardiac T2* (magnetic resonance imaging relaxation parameter) is abnormally low in approximately 40% of adults with thalassemia major (TM), suggesting myocardial iron deposition, but it is unknown at what age this occurs. To address this question, we measured cardiac T2* and function in 19 young patients (aged 7-26 years) with TM as well as 17 patients receiving long-term transfusions for sickle cell anemia (SCA) matched for age, sex, and liver iron content. Cardiac T2* was normal in all of the SCA patients but was low (high iron) in 8 of 19 TM patients. Abnormal T2* was observed only in the TM patients receiving transfusions for 13 years or longer and was correlated with ferritin but not liver iron levels. Cardiac dysfunction was present in 3 of the 8 patients with low T2*. Cardiac T2* changes have a long latency relative to liver iron accumulation. Total transfusional burden is a significant independent risk factor for low cardiac T2* and may partially account for differences observed between patients with SCA and TM.
Authors:
John C Wood; J Michael Tyszka; Susan Carson; Marvin D Nelson; Thomas D Coates
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.     Date:  2003-11-20
Journal Detail:
Title:  Blood     Volume:  103     ISSN:  0006-4971     ISO Abbreviation:  Blood     Publication Date:  2004 Mar 
Date Detail:
Created Date:  2004-02-20     Completed Date:  2004-04-20     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  7603509     Medline TA:  Blood     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1934-6     Citation Subset:  AIM; IM    
Affiliation:
Division of Pediatric Cardiology, Childrens Hospital Los Angeles, Los Angeles, CA 90027, USA. jwood@chla.usc.edu
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Age Factors
Anemia, Sickle Cell / metabolism*
Blood Transfusion
Child
Female
Hemoglobins / biosynthesis
Humans
Iron / blood*,  metabolism*
Iron Overload
Male
Myocardium / metabolism*
Sex Factors
Thalassemia / metabolism*
Time Factors
Grant Support
ID/Acronym/Agency:
RR00043-43/RR/NCRR NIH HHS
Chemical
Reg. No./Substance:
0/Hemoglobins; 7439-89-6/Iron

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


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