Beta-thalassemia

Beta-thalassemia (BT) is characterized by deficiency (Beta+) or absence (Beta0) of synthesis of the beta globin chains of hemoglobin (Hb).

Aortic aneurysm

Aortic dilatation refers to a dimension that is greater than the 95th percentile for the normal person age, sex and body size. In contrast, an aneurysm is defined as a localized dilation of the aorta that is more than 150 percent of predicted (ratio of observed to expected diameter 1.5 or more). Aneurysm should be distinguished from ectasia, which represents a diffuse dilation of the aorta less than 50 percent of normal aorta diameter.


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PMID (PMCID)
15014899
 FEMALE Adult
Aneurysmatic dilatation of ascending aorta in a patient with beta-thalassemia and a pseudoxanthoma elasticum-like syndrome.
Farmakis D, Vesleme V, Papadogianni A, Tsaftaridis P, Kapralos P, Aessopos A.
Ann Hematol. 2004;83(9):596-9.
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.