Pulmonary Hypertension in Thalassemia: Association with Hemolysis, Arginine Metabolism Dysregulation, and a Hypercoagulable State
نویسندگان
چکیده
منابع مشابه
The hypercoagulable state in thalassemia.
Thalassemia is a congenital hemolytic disorder caused by a partial or complete deficiency of alpha- or beta-globin chain synthesis. Homozygous carriers of beta-globin gene defects suffer from severe anemia and other serious complications from early childhood. The disease is treated by chronic blood transfusion. However, this can cause severe iron overload resulting in progressive organ failure....
متن کاملThalassemia Associated Pulmonary Hypertension
Cardiac disease is the main cause of death in both forms of thalassemia; thalassemia major (TM) and thalassemia intermedia (TI). Pulmonary hypertension (PH) is one of the cardiopulmonary morbidities with high mortality that, if not treated, may trigger right-sided heart failure and premature death. PH is defined as a mean pulmonary artery pressure of ≥25 mmHg at rest or ≥30 mmHg during exercise...
متن کاملDysregulated arginine metabolism, hemolysis-associated pulmonary hypertension, and mortality in sickle cell disease.
CONTEXT Sickle cell disease is characterized by a state of nitric oxide resistance and limited bioavailability of l-arginine, the substrate for nitric oxide synthesis. We hypothesized that increased arginase activity and dysregulated arginine metabolism contribute to endothelial dysfunction, pulmonary hypertension, and patient outcomes. OBJECTIVE To explore the role of arginase in sickle cell...
متن کاملNo association of the hypercoagulable state with sickle cell disease related pulmonary hypertension.
Pulmonary hypertension (PHT) occurs in approximately 30% of adult patients with sickle cell disease (SCD) and is a risk factor for early death. Hypercoagulability has been linked to PHT in general and pulmonary artery thrombosis contributes to PHT progression regardless of its cause. Sickle cell patients are characterized by a hypercoagulable state and both autopsy and imaging studies in sickle...
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ژورنال
عنوان ژورنال: Advances in Pulmonary Hypertension
سال: 2007
ISSN: 1933-088X
DOI: 10.21693/1933-088x-6.1.31