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Damien Brackman, Lisa Sartz, Sabine Leh, Ann-Charlotte Kristoffersson, Anna Bjerre, Ramesh Tati, Veronique Frémeaux-Bacchi, Diana Karpman, Thrombotic microangiopathy mimicking membranoproliferative glomerulonephritis, Nephrology Dialysis Transplantation, Volume 26, Issue 10, October 2011, Pages 3399–3403, https://doi.org/10.1093/ndt/gfr422
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Abstract
A 4-year-old boy presented with proteinuria and developed progressive renal failure over 6 years. In the patient’s family, five individuals were affected with atypical haemolytic uraemic syndrome (aHUS) but not the patient. Renal biopsies (n = 3) showed glomerular basement membrane thickening with double contours, endothelial swelling and deposits of C3 and C1q. Electron microscopy revealed mesangial and subendothelial electron-dense deposits. Complement mutations in membrane cofactor protein (Y155D) and C3 (R713W and G1094R) were detected in all affected family members. The patient also had transient autoantibodies to factor H. The findings suggest that aHUS and glomerulopathy resembling membranoproliferative glomerulonephritis may have a common molecular background.
- proteinuria
- edema
- mutation
- kidney failure
- autoantibodies
- complement system proteins
- complement factor h
- endothelium
- membranoproliferative glomerulonephritis
- renal glomerular disease
- renal biopsy
- glomerular basement membrane
- atypical hemolytic-uremic syndrome
- cd46 antigen
- thrombotic microangiopathies
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