Article
Mutations and polymorphisms in hemoglobin genes and the risk of pulmonary hypertension and death in sickle cell disease.
American journal of hematology - 1 Jan 2008
Taylor James G, Ackah Diana, Cobb Crystal, Orr Nick, Percy Melanie J, Sachdev Vandana, Machado Roberto, Castro Oswaldo, Kato Gregory J, Chanock Stephen J, Gladwin Mark T
Abstract excerpt
Pulmonary hypertension is a common complication of sickle cell disease (SCD) and a risk factor for early death. Hemolysis may participate in its pathogenesis by limiting nitric oxide (NO) bioavailability and producing vasculopathy. We hypothesized that hemoglobin mutations that diminish hemolysis in SCD would influence pulmonary hypertension susceptibility. Surprisingly, coincident alpha-thalassemia (Odds Ratio...
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