The Kidney in Sickle Cell Disease: Pathophysiology and Clinical Review

Sickle cell disease is a major health problem in many countries. Sickled erythrocytes in the renal medullary vessels are the hallmark of the disease, and are associated with a variety of renal complications. Renal ischemia, microinfarcts and papillary necrosis occur, and renal tubular abnormalities follow. Glomerular disease with proteinuria may be observed. Renal failure, both acute and chronic, is the expected outcome of the disease. Hematuria, electrolyte and acid base disturbances are common throughout the course of the disease. End-stage renal disease as a complication of aggressive sickle cell disease should be treated with dialysis or kidney transplantation; both are effective. There is a tendency towards better survival in sickle cell nephropathy patients undergoing transplantation than in those treated with hemodialysis alone. Whether bone marrow transplantation in the early stage of the disease can halt the progression of sickle cell nephropathy is unknown and awaits clinical studies