April 15, 2021
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Sickle cell disease is an autosomal recessive disorder classically seen in African-Americans which is caused by a single nucleotide change on chromosome 11p15.5 resulting in an amino acid change from glutamic acid to valine. In the kidney, the disease manifests as sickle cell nephropathy. Histologic features seen in this disease include focal segmental glomerulosclerosis, glomerular enlargement, double contour formation, and hemosiderin deposition within tubular epithelial cells to name a few. Crescent formation is not typically seen in sickle cell nephropathy and if seen would be worrisome for a concomitant process (i.e. ANCA-associated glomerulonephritis).