TY - JOUR
TI - Aneurysmatic dilatation of ascending aorta in a patient with
beta-thalassemia and a pseudoxanthoma elasticum-like syndrome
AU - Farmakis, D
AU - Vesleme, V
AU - Papadogianni, A
AU - Tsaftaridis, P and
AU - Kapralos, P
AU - Aessopos, A
JO - Annals of Hematology
PY - 2004
VL - 83
TODO - 9
SP - 596-599
PB - Springer-Verlag
SN - 0939-5555, 1432-0584
TODO - 10.1007/s00277-004-0859-6
TODO - thalassemia; pseudoxanthoma elasticum; aortic aneurysm
TODO - 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.
ER -