Sickle Cell Nephropathy in Children

Abstract

Sickle cell disease (SCD) is one of the most common genetic disorders in the United States, affecting over 100, 000 Americans and millions of people worldwide, primarily of African or Mediterranean descent. SCD is a group of hemoglobinopathies inherited in an autosomal recessive fashion. Nearly all organ systems are affected by SCD, including the kidneys. The renal manifestations of SCD are known as sickle cell nephropathy (SCN). Multiple pathogenic processes contribute to development of SCN, notably renal ischemia and hemolysis, which result in both glomerular and tubulointerstitial disease. Clinically significant complications of SCD include hyperfiltration, albuminuria, hyposthenuria, hematuria, hypertension, acute kidney injury (AKI), and chronic kidney disease (CKD). Despite these complications, occurrence of end-stage kidney disease (ESKD) is relatively uncommon, particularly in children. Of those who progress to ESKD, both dialysis and transplantation are viable treatments. While transplantation in SCN is associated with increased complications compared to other causes of ESKD, it is preferred over dialysis due to more favorable outcomes.