Aneurysmatic dilatation of ascending aorta in a patient with beta-thalassemia and a pseudoxanthoma elasticum-like syndrome.
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.
Farmakis
Dimitrios
D
First Department of Internal Medicine, University of Athens, Medical School, Laiko General Hospital, Athens, Greece. dfarm1@panafonet.gr
Vesleme
Vassiliki
V
Papadogianni
Argiroula
A
Tsaftaridis
Panagiotis
P
Kapralos
Pantelis
P
Aessopos
Athanasios
A
D000328:Adult; D001011:Aorta / Q000473:pathology*; D004108:Dilatation, Pathologic / Q000150:complications* / Q000473:pathology*; D005260:Female; D006801:Humans; D011561:Pseudoxanthoma Elasticum / Q000150:complications*; D013577:Syndrome; D014055:Tomography, Emission-Computed; D017086:beta-Thalassemia / Q000150:complications* / Q000628:therapy