- Poulami Maitra1,
- Melissa Caughey2,
- Laura Robinson3,
- Payal C. Desai4,
- Susan Jones5,
- Mehdi Nouraie6,
- Mark T. Gladwin7,
- Alan Hinderliter3,
- Jianwen Cai1 and
- Kenneth I. Ataga5,*
- 1 Department of Biostatistics, University of North Carolina, Chapel Hill, USA;
- 2 Division of Cardiology, University of North Carolina, Chapel Hill, USA;
- 3 Childrens Hospital of Philadelphia, Philadelphia, USA;
- 4 Division of Hematology, The Ohio State University, Columbus, USA;
- 5 Division of Hematology/Oncology, University of North Carolina, Chapel Hill, USA;
- 6 Department of Medicine, Howard University, Washington, USA;
- 7 Department of Medicine, University of Pittsburgh, Pittsburgh, USA
- ↵* Corresponding author; email:
Although recent studies show an improved survival of children with sickle cell disease in the US and Europe, mortality remains high for adult patients. This study was conducted to evaluate the factors associated with mortality in adult patients following the approval of hydroxyurea. We first evaluated the association between selected variables and mortality at an academic center (UNC cohort). Data sources were then searched for publications from 1998 to June, 2016, with meta-analysis of eligible studies conducted in North America and Europe to evaluate the associations of selected variables with mortality in adult patients. Nine studies, combined with the UNC cohort (total: 3257 patients) met the eligibility criteria. Mortality was significantly associated with age (per 10-year increase in age) (7 studies, 2,306 participants; hazard ratio: 1.28; 95% confidence interval: 1.10-1.50), tricuspid regurgitant jet velocity ≥ 2.5 m/s (5 studies, 1,577 participants; hazard ratio: 3.03; 95% confidence interval: 2.0 – 4.60), reticulocyte count (3 studies, 1,050 participants; hazard ratio: 1.05; 95% confidence interval: 1.01-1.10), log(N-terminal-pro-brain natriuretic peptide) (3 studies, 800 participants; hazard ratio: 1.68; 95% confidence interval: 1.48 - 1.90), and fetal hemoglobin (7 studies, 2,477 participants; hazard ratio: 0.97; 95% confidence interval: 0.94-1.0). This study identifies variables associated with mortality in adult patients with sickle cell disease in the hydroxyurea era.
- Received August 10, 2016.
- Accepted January 12, 2017.
- Copyright © 2017, Ferrata Storti Foundation