Aneurysmatic dilatation of ascending aorta in a patient with β-thalassemia and a pseudoxanthoma elasticum-like syndrome

摘要: The development of a diffuse elastic tissue defect resembling pseudoxanthoma elasticum (PXE) is recently established and frequently encountered clinical entity in β-thalassemia. spectrum this disorder not yet completely understood as it continuously being enriched with novel complications that are often serious. We present here thalassemia intermedia patient typical PXE manifestations fusiform aneurysmatic dilatation the ascending thoracic aorta, previously unknown complication these patients. An aortic aneurysm has itself notable morbidity, while may also impair particularly susceptible cardiac function β-thalassemia patients, hence affecting overall prognosis main disease.