| Pulmonary hypertension in beta-thalassemia. | |
MedLine Citation:
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PMID: 16339682 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Cardiac involvement represents the leading cause of mortality in both forms of beta-thalassemia, namely, thalassemia major (TM) and thalassemia intermedia (TI), and pulmonary hypertension (PHT) is part of the cardiopulmonary complications of the disease. PHT was initially documented in a small group of TI patients with right heart failure. In a subsequent study of a large 110-patient series, aged 32.5 +/- 11.4 years, age-related PHT was encountered in nearly 60% of cases, having caused right heart failure in six of them; interestingly, all patients had preserved left ventricular systolic function. Conflicted evidence, however, existed with respect to the development of PHT in heterogeneously treated and young TM populations. To resolve this discrepancy, a recent study compared cardiac disease between two large aged-matched groups of TM (n = 131) and TI (n = 74) patients, both treated uniformly in the currently accepted manner (regular transfusion and chelation therapy in TM, absence of any particular treatment in TI); well-treated TM patients, in contrast to TI patients, did not develop PHT, while systolic left ventricular dysfunction was present only in TM cases. PHT in beta-thalassemia results from a rather complex pathophysiology, in which chronic tissue hypoxia seems to hold a key role. Although both forms of the disease share a common molecular background, the diverse severity of the genetic defect and of the resulting clinical phenotype require a different therapeutic approach. Regular lifelong therapy in TM patients eliminates chronic hypoxia, thereby preventing PHT, whereas the absence of systematic treatment in TI leads to a cascade of reactions that compensate for chronic anemia, but at the same time allow the development of PHT. |
Authors:
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Athanasios Aessopos; Dimitrios Farmakis |
Publication Detail:
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Type: Journal Article; Review |
Journal Detail:
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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:
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Created Date: 2005-12-12 Completed Date: 2006-07-13 Revised Date: 2007-11-15 |
Medline Journal Info:
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Nlm Unique ID: 7506858 Medline TA: Ann N Y Acad Sci Country: United States |
Other Details:
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Languages: eng Pagination: 342-9 Citation Subset: IM |
Affiliation:
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First Department of Internal Medicine, University of Athens Medical School, Laiko General Hospital, 17 Aghiou Thoma St., Athens 115 27, Greece. aaisopos@cc.uoa.gr |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Age Factors Anoxia / etiology, physiopathology Blood Transfusion Cardiac Output Chelation Therapy Heart Failure / etiology, physiopathology Humans Hypertension, Pulmonary / etiology*, physiopathology Iron Overload / etiology, physiopathology Middle Aged Thromboembolism / etiology, physiopathology Vascular Resistance Ventricular Dysfunction, Left / etiology, physiopathology beta-Thalassemia / complications*, drug therapy, physiopathology, therapy |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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