Thrombotic microangiopathy mimicking membranoproliferative glomerulonephritis
(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)
Please use this url to cite or link to this publication:
https://lup.lub.lu.se/record/2253134
- author
- Brackman, Damien ; Sartz, Lisa LU ; Leh, Sabine ; Kristoffersson, Ann-Charlotte LU ; Bjerre, Anna ; Tati, Ramesh LU ; Fremeaux-Bacchi, Veronique and Karpman, Diana LU
- organization
- publishing date
- 2011
- 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
- pmid:21810760
- 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
- 2016-04-01 14:36:48
- date last changed
- 2022-01-28 01:35:58
@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}}, keywords = {{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 = {{https://lup.lub.lu.se/search/files/4068306/3125219.pdf}}, doi = {{10.1093/ndt/gfr422}}, volume = {{26}}, year = {{2011}}, }