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Renal manifestations of sickle cell disease

Author
Edgar V Lerma, MD, FACP, FASN, FAHA
Section Editor
Gary C Curhan, MD, ScD
Deputy Editors
Alice M Sheridan, MD
Jennifer S Tirnauer, MD

INTRODUCTION

Vaso-occlusive phenomena and hemolysis are the clinical hallmarks of sickle cell disease. The polymerization of deoxy hemoglobin S results in distortion of the red blood cell (RBCs) into the classic sickle shape and a marked decrease in red cell deformability and is the primary cause of vaso-occlusive phenomena [1]. Subsequent changes in red cell membrane structure and function, disordered cell volume control, and increased adherence to vascular endothelium also play an important role [1,2]. (See "Sickle hemoglobin polymer: Structure and functional properties".)

Vaso-occlusion results in recurrent, painful episodes and a variety of serious organ system complications that can lead to lifelong disabilities and even death. The renal manifestations of sickle cell disease will be reviewed here. The other manifestations, diagnosis, and management of this disorder and other sickle cell syndromes are discussed separately. (See "Overview of the clinical manifestations of sickle cell disease" and "Diagnosis of sickle cell disorders" and "Overview of the management and prognosis of sickle cell disease" and "Overview of variant sickle cell syndromes".)

EPIDEMIOLOGY

Clinically significant renal involvement occurs more frequently in sickle cell disease than in sickle cell trait or in combined hemoglobinopathies, with the exception of renal medullary carcinoma, which appears to be more common among patients with sickle cell trait [3-7]. Renal infarcts and papillary necrosis occur in either sickle cell disease or trait, with prevalence estimates of 30 to 40 percent in radiographic studies [8,9].

Among patients with sickle cell disease, the prevalence of proteinuria has been estimated to be 20 to 25 percent, and decreased kidney function has been reported in 5 to 30 percent [10-13]. Among patients with other sickling hemoglobinopathies, albuminuria and/or proteinuria has been reported in 8 to 30 percent [12-15] (with the prevalence increasing with age [12]) and decreased kidney function in 6 percent [12].

In the United States, sickle cell disease accounts for <1 percent of all new cases of end-stage renal disease (ESRD) [16]. The incidence of renal failure in patients with sickle cell disease was best described in two related cohort studies [4,5]:

                          

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