Chronic Kidney Disease and Sickle Cell Disease

Document Type

Journal Article

Publication Date



Chronic Renal Disease




Biomarker; Chronic kidney disease; Focal segmental glomerulosclerosis; Glomerular hyperfiltration; Hematuria; Impaired potassium secretion; Impaired renal acidification; Impaired urinary concentrating ability; Proteinuria; Renal tubular disorder; Sickle cell disease


© 2015 Elsevier Inc. All rights reserved. Sickle cell nephropathy (SCN) encompasses a spectrum of renal functional and structural abnormalities that begins in childhood and may progress to advanced CKD or ESRD by early or late adulthood. Although SCN has traditionally been attributed to the consequences of a viscosity-vaso-occlusive process, it has increasingly been recognized that hemolysis-endothelial dysfunction may play an important contributory role. Regardless of the predominant pathogenic mechanisms, well-described clinicopathological manifestations of SCN include impaired urinary concentrating ability, impaired renal acidification and potassium secretion, hematuria, proteinuria, focal segmental glomerulosclerosis and progressive renal disease, among others. Progressive CKD may develop as a consequence of irreversible glomerular and tubulointerstitial damage. The range of kidney abnormalities associated with sickle cell disease (SCD) is wide, and the underlying pathophysiologic mechanisms are varied. Therapeutic measures to prevent or alleviate kidney complications associated with SCD may have value. © 2015 Copyright