Document Detail

Aneurysmatic dilatation of ascending aorta in a patient with beta-thalassemia and a pseudoxanthoma elasticum-like syndrome.
MedLine Citation:
PMID:  15014899     Owner:  NLM     Status:  MEDLINE    
The development of a diffuse elastic tissue defect resembling pseudoxanthoma elasticum (PXE) is a recently established and frequently encountered clinical entity in beta-thalassemia. The clinical spectrum of this disorder is not yet completely understood as it is continuously being enriched with novel complications that are often serious. We present here a thalassemia intermedia patient with typical PXE manifestations and a fusiform aneurysmatic dilatation of the ascending thoracic aorta, a previously unknown complication in these patients. An aortic aneurysm has itself a notable morbidity, while it may also impair the particularly susceptible cardiac function of beta-thalassemia patients, hence affecting the overall prognosis of the main disease.
Dimitrios Farmakis; Vassiliki Vesleme; Argiroula Papadogianni; Panagiotis Tsaftaridis; Pantelis Kapralos; Athanasios Aessopos
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2004-03-11
Journal Detail:
Title:  Annals of hematology     Volume:  83     ISSN:  0939-5555     ISO Abbreviation:  Ann. Hematol.     Publication Date:  2004 Sep 
Date Detail:
Created Date:  2004-09-27     Completed Date:  2004-10-20     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9107334     Medline TA:  Ann Hematol     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  596-9     Citation Subset:  IM    
First Department of Internal Medicine, University of Athens, Medical School, Laiko General Hospital, Athens, Greece.
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MeSH Terms
Aorta / pathology*
Dilatation, Pathologic / complications*,  pathology*
Pseudoxanthoma Elasticum / complications*
Tomography, Emission-Computed
beta-Thalassemia / complications*,  therapy

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