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Thrombotic microangiopathy mimicking membranoproliferative glomerulonephritis

Brackman, Damien; Sartz, Lisa LU ; Leh, Sabine; Kristoffersson, Ann-Charlotte LU ; Bjerre, Anna; Tati, Ramesh LU ; Fremeaux-Bacchi, Veronique and Karpman, Diana LU (2011) In Nephrology Dialysis Transplantation 26(10). p.3399-3403
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... (More)
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. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
complement, C3, haemolytic uraemic syndrome, membranoproliferative, glomerulonephritis, eculizumab
in
Nephrology Dialysis Transplantation
volume
26
issue
10
pages
3399 - 3403
publisher
Oxford University Press
external identifiers
  • wos:000296349200055
  • scopus:84863881370
ISSN
1460-2385
DOI
10.1093/ndt/gfr422
language
English
LU publication?
yes
id
bd69b501-77f3-471d-af53-a18ab83f1fcf (old id 2253134)
date added to LUP
2012-01-02 07:59:14
date last changed
2017-08-20 04:09:32
@article{bd69b501-77f3-471d-af53-a18ab83f1fcf,
  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.},
  author       = {Brackman, Damien and Sartz, Lisa and Leh, Sabine and Kristoffersson, Ann-Charlotte and Bjerre, Anna and Tati, Ramesh and Fremeaux-Bacchi, Veronique and Karpman, Diana},
  issn         = {1460-2385},
  keyword      = {complement,C3,haemolytic uraemic syndrome,membranoproliferative,glomerulonephritis,eculizumab},
  language     = {eng},
  number       = {10},
  pages        = {3399--3403},
  publisher    = {Oxford University Press},
  series       = {Nephrology Dialysis Transplantation},
  title        = {Thrombotic microangiopathy mimicking membranoproliferative glomerulonephritis},
  url          = {http://dx.doi.org/10.1093/ndt/gfr422},
  volume       = {26},
  year         = {2011},
}