Aneurysmatic dilatation of ascending aorta in a patient with beta-thalassemia and a pseudoxanthoma elasticum-like syndrome
| Autor: | Athanasios Aessopos, Argiroula Papadogianni, Dimitrios Farmakis, Vassiliki Vesleme, Panagiotis Tsaftaridis, Pantelis Kapralos |
|---|---|
| Rok vydání: | 2003 |
| Předmět: |
Cardiac function curve
Adult medicine.medical_specialty Thalassemia Aortic aneurysm hemic and lymphatic diseases medicine.artery Internal medicine Ascending aorta medicine Thoracic aorta Humans Pseudoxanthoma Elasticum Aorta Hematology business.industry beta-Thalassemia General Medicine Syndrome Pseudoxanthoma elasticum medicine.disease Cardiology Female Complication business Dilatation Pathologic Tomography Emission-Computed |
| Zdroj: | Annals of hematology. 83(9) |
| ISSN: | 0939-5555 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Full text from SpringerLink