Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pseudoxanthoma elasticum-like diffuse elastic tissue defects and hypercoagulability are two well-established features in beta-thalassemia, which play a key role in the development of a spectrum of collateral complications in these patients. We present here a middle-aged thalassemia intermedia patient with severe aortic stenosis caused by heavy calcification of the valve, a hallmark of pseudoxanthoma elasticum. The patient underwent a successful replacement of the valve, which is the first such operation ever reported in these patients. However, rapid thrombosis leaded to failure of the mechanical valve, despite proper antithrombotic therapy.
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PMID:Aortic valve replacement in a patient with thalassemia intermedia. 1642 92

Thalassaemia is an inherited hemoglobin disorder resulting in chronic hemolytic anemia. Organ damage caused by iron overload represents the major cause of illness and the heart complications secondary to extensive myocardial iron overload are the leading cause of mortality. These patients are generally not considered as candidates for heart transplantation. We report a case of a patient with thalassemia intermedia (TI) with severe cardiac dysfunction and severe aortic stenosis who underwent percutaneous aortic valve replacement (PARV). PARV was a safe and a valid therapeutic approach dramatically to improve the clinical evolution of cardiomyopathies in our patient.
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PMID:Percutaneous aortic valve replacement in a 65-year old patient with thalassemia intermedia: A case report. 2088 99