Τίτλος:
Aneurysmatic dilatation of ascending aorta in a patient with
beta-thalassemia and a pseudoxanthoma elasticum-like syndrome
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
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.
Συγγραφείς:
Farmakis, D
Vesleme, V
Papadogianni, A
Tsaftaridis, P and
Kapralos, P
Aessopos, A
Περιοδικό:
Annals of Hematology
Λέξεις-κλειδιά:
thalassemia; pseudoxanthoma elasticum; aortic aneurysm
DOI:
10.1007/s00277-004-0859-6
