The glomerulopathy of sickle cell disease

• Type of Material: Article
• Authors: Kenneth I. Ataga, University of North Carolina; Vimal K. Derebail, University of North Carolina; David Archer, Emory University
• Language: English
• Date: 2014-06-18
• Publisher: Wiley Periodicals Inc.
• Publication Version: Final Published Version
• Copyright Statement: © 2014 The Authors American Journal of Hematology Published by Wiley Periodicals, Inc.
• License: Creative Commons Attribution-Noncommercial 3.0 Unported
• Final Published Version (URL): http://dx.doi.org/10.1002/ajh.23762
• Title of Journal or Parent Work: American Journal of Hematology
• ISSN: 0361-8609
• Volume: 89
• Issue: 9
• Start Page: 907
• End Page: 914
• Grant/Funding Information: Support for this work was also provided by an award from the North Carolina State Sickle Cell Program (KIA).; NIH; Contract grant number: R01 HL111659 (to KIA, VKD, DRA).
• Keywords: Science & Technology; ANEMIA; PREVALENCE; CHRONIC KIDNEY-DISEASE; STAGE RENAL-DISEASE; MICROALBUMINURIA; Life Sciences & Biomedicine; RISK-FACTORS; RELATIVE SYSTEMIC HYPERTENSION; URINARY ALBUMIN; CHILDREN; HEMATOLOGY; PULMONARY-HYPERTENSION; Hematology
• Research Categories: Health Sciences, General; Health Sciences, Oncology

Abstract

Sickle cell disease (SCD) produces many structural and functional abnormalities in the kidney, including glomerular abnormalities. Albuminuria is the most common manifestation of glomerular damage, with a prevalence between 26 and 68% in adult patients. The pathophysiology of albuminuria in SCD is likely multifactorial, with contributions from hyperfiltration, glomerular hypertension, ischemia-reperfusion injury, oxidative stress, decreased nitric oxide (NO) bioavailability, and endothelial dysfunction. Although its natural history in SCD remains inadequately defined, albuminuria is associated with increased echocardiography-derived tricuspid regurgitant jet velocity, systemic blood pressure, and hypertension, as well as history of stroke, suggesting a shared vasculopathic pathophysiology. While most patients with albuminuria are treated with angiotensin converting enzyme inhibitors/angiotensin receptor blockers, there are no published long-term data on the efficacy of these agents. With the improved patient survival following kidney transplantation, SCD patients with end-stage renal disease should be considered for this treatment modality. Given the high prevalence of albuminuria and its association with multiple SCD-related clinical complications, additional studies are needed to answer several clinically important questions in a bid to adequately elucidate its pathophysiology, natural history, and treatment.