Document Detail

Membranoproliferative glomerulonephritis with isolated C3 deposits: case report and literature review.
MedLine Citation:
PMID:  21265634     Owner:  NLM     Status:  MEDLINE    
Membranoproliferative glomerulonephritis with isolated C3 deposits (MPGNC3) is an uncommon condition characterized by overt glomerular C3 deposits in the absence of immunoglobulins and intramembranous dense deposits. Here the authors describe the clinical and morphological features of primary MPGNC3 in a 13-year-old boy and critically review the previously published cases. The patient presented with nephrotic syndrome and microscopic hematuria. Blood tests revealed very low circulating C3 levels. The renal biopsy exhibited subendothelial, subepithelial, and mesangial deposits, with C3 but not immunoglobulins seen on immunofluorescence. This case and the review of the literature indicate that the serum complement profile with decreased levels of C3 and normal levels of classical pathway components together with glomerular deposits containing exclusively complement C3 is highly suggestive of alternative pathway activation. The diagnosis of acquired and/or genetic complement abnormalities in some cases supports that complement dysregulation is implicated in the pathogenesis of MPGNC3. Such data show great promise to provide new therapy strategies based on modulation of the complement system activity.
Sihem Darouich; Rym Goucha; Mohamed Habib Jaafoura; Semy Zekri; Adel Kheder; Hédi Ben Maiz
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Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Ultrastructural pathology     Volume:  35     ISSN:  1521-0758     ISO Abbreviation:  Ultrastruct Pathol     Publication Date:  2011 Feb 
Date Detail:
Created Date:  2011-01-26     Completed Date:  2011-05-04     Revised Date:  2011-05-17    
Medline Journal Info:
Nlm Unique ID:  8002867     Medline TA:  Ultrastruct Pathol     Country:  England    
Other Details:
Languages:  eng     Pagination:  42-6     Citation Subset:  IM    
Electron Microscopy Laboratory, Faculty of Medicine of Tunis, Tunis, Tunisia.
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MeSH Terms
Complement C3 / metabolism*
Fluorescent Antibody Technique
Glomerulonephritis, Membranoproliferative / metabolism*,  pathology*
Kidney Glomerulus / metabolism,  ultrastructure
Microscopy, Electron, Transmission
Reg. No./Substance:
0/Complement C3
Erratum In:
Ultrastruct Pathol. 2011 May;35(3):151
Note: Kheder, Adel [added]; Ben Maiz, Hédi [added]

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