[HTML][HTML] Prevalence and pathologic features of sickle cell nephropathy and response to inhibition of angiotensin-converting enzyme
RJ Falk, J Scheinman, G Phillips… - … England Journal of …, 1992 - Mass Medical Soc
RJ Falk, J Scheinman, G Phillips, E Orringer, A Johnson, JC Jennette
New England Journal of Medicine, 1992•Mass Medical SocBackground. Nephropathy may develop in patients with sickle cell disease. We determined
the prevalence of proteinuria and renal insufficiency in a group of patients with sickle cell
disease and investigated the renal pathologic changes and the effects of an angiotensin-
converting—enzyme inhibitor (enalapril) on protein excretion in patients found to have
nephropathy. Methods. We prospectively screened 381 patients with sickle cell disease for
the presence of proteinuria and renal insufficiency. Renal biopsy and measurements of …
the prevalence of proteinuria and renal insufficiency in a group of patients with sickle cell
disease and investigated the renal pathologic changes and the effects of an angiotensin-
converting—enzyme inhibitor (enalapril) on protein excretion in patients found to have
nephropathy. Methods. We prospectively screened 381 patients with sickle cell disease for
the presence of proteinuria and renal insufficiency. Renal biopsy and measurements of …
Background.
Nephropathy may develop in patients with sickle cell disease. We determined the prevalence of proteinuria and renal insufficiency in a group of patients with sickle cell disease and investigated the renal pathologic changes and the effects of an angiotensin-converting—enzyme inhibitor (enalapril) on protein excretion in patients found to have nephropathy.
Methods.
We prospectively screened 381 patients with sickle cell disease for the presence of proteinuria and renal insufficiency. Renal biopsy and measurements of glomerular filtration rate, effective renal plasma flow, and urinary protein excretion were performed in 10 patients with mild nephropathy before and after the administration of enalapril, and again two to three weeks after its discontinuation.
Results.
Of the 381 patients with sickle cell disease, 26 (7 percent) had serum creatinine concentrations above the normal range and 101 (26 percent) had proteinuria of at least 1 +. The renal lesions in the 10 patients who had biopsies consisted of glomerular enlargement and perihilar focal segmental glomerulosclerosis. The mean (±SD) glomerular area in these patients was 28.7±4.1 × 103 μm2, as compared with 15.8±4.3×103 μm2 in 10 control patients without renal disease who had died of trauma (P<0.001).
During the administration of enalapril, the mean 24-hour urinary protein excretion decreased 57 percent (range, 23 to 79 percent) below the base-line value (P<0.001 ), and it increased to 25 percent below the base-line value after enalapril was discontinued. The glomerular filtration rate and effective renal plasma flow did not change significantly.
Conclusions.
Approximately 25 percent of patients with sickle cell disease have proteinuria. Treatment with enalapril reduces the degree of proteinuria in these patients, suggesting that glomerular capillary hypertension may be a pathogenic factor in sickle cell nephropathy. (N Engl J Med 1992;326:910–5.)
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