An elevated estimated pulmonary arterial systolic pressure, whenever measured, is associated with excess mortality in adults with sickle cell disease

摘要

Sickle cell disease (SCD) has a complex clinical pheno-type that belies the simple underlying molecular defect, in which a single mutation in the beta-globin chain causes abnormal hemoglobin polymerization. The pleiotropic clinical features of SCD include cardiac, cerebral, and renal vasculopathy, osteonecrosis, and recurrent painful vaso-occlusive crises.