title: Aneurysmatic dilatation of ascending aorta in a patient with beta-thalassemia and a pseudoxanthoma elasticum-like syndrome.
abstract: 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.
authors.affiliation: First Department of Internal Medicine, University of Athens, Medical School, Laiko General Hospital, Athens, Greece. dfarm1@panafonet.gr
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Farmakis
authors.forename: Vassiliki
authors.initials: V
authors.lastname: Vesleme
authors.forename: Argiroula
authors.initials: A
authors.lastname: Papadogianni
authors.forename: Panagiotis
authors.initials: P
authors.lastname: Tsaftaridis
authors.forename: Pantelis
authors.initials: P
authors.lastname: Kapralos
authors.forename: Athanasios
authors.initials: A
authors.lastname: Aessopos
mesh_terms: 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