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Author Notes:

Correspondence: Mehdi Nouraie, Vascular Medicine Institute, Department of Medicine, University of Pittsburgh, Pittsburgh, PA 15260. nouraies@upmc.edu

Subjects:

Research Funding:

None declared

Keywords:

  • Science & Technology
  • Life Sciences & Biomedicine
  • Hematology
  • Prevention
  • Death
  • Pain

Validation of a composite vascular high-risk profile for adult patients with sickle cell disease

Tools:

Journal Title:

American Journal of Hematology

Volume:

Volume 94, Number 12

Publisher:

, Pages E312-E314

Type of Work:

Article | Post-print: After Peer Review

Abstract:

In the US, the median survival of patients with sickle cell disease (SCD) is 50 years, which is approximately two decades less than African Americans without SCD. As patients with SCD grow older, they develop end-organ damage related to vascular complications, including pulmonary hypertension, diastolic left heart disease, and nephropathy.1 More than 50% of adult patients have dysfunction of at least one organ system, and 25% have multi-organ dysfunction. Simultaneous end-organ disease in heart, lung and kidney increased mortality by approximately 4-fold.1 Intravascular hemolysis contributes to pulmonary vasculopathy and renal insufficiency by elaboration of free hemoglobin, which produces redox stress, inflammation, hypercoagulability, and vascular injury.2 We have reported that elevated tricuspid regurgitation velocity (TRV), a non-invasive marker of elevated systolic pulmonary artery pressure, is associated with an increased risk of death in two independent cohorts.3,4 N-terminal-prohormone B-type natriuretic peptide (BNP) ≥160 pg/mL also predicts pulmonary hypertension, and is associated with high risk of mortality.5 Moreover, patients with chronic renal insufficiency are at higher risk of death

Copyright information:

© 2019 Wiley Periodicals, Inc.

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