Renal pathology in adults over 40 with sickle-cell disease

ABSTRACT

In a retrospective study of hospital necropsies, we examined the kidneys of 21 patients with homozygous sickle-cell disease who died at the age of 40 or over. Renal failure had caused or contributed to death in ten cases. All kidneys showed changes of papillary damage to a variable extent. In three cases, there was mild cortical irregularity, and in thirteen, there was moderate to severe cortical irregularity, including scarring in some with obvious loss of functioning tissue. Light microscopic examination revealed hypertrophic glomeruli with increased cellularity, lobulation and capilary basement membrane splitting. Glomerulosclerosis was common, and patchy deposits of fibrin were present in glomerular capilaries in complex glomerulonephritis, we suggest that sclerosis consequent upon longstanding hyperfiltration may be more important. Glomerular filtration rate is abnormally high in children with sickle-cell disease but in later childhood renal insufficiency, proteinuria and eventually death from renal failure become common