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01-01-2014 | Original Article

C3 nephritic factor associated with C3 glomerulopathy in children

Authors: Camille Nicolas, Vincent Vuiblet, Veronique Baudouin, Marie-Alice Macher, Isabele Vrillon, Nathalie Biebuyck-Gouge, Maud Dehennault, Sophie Gié, Denis Morin, Hubert Nivet, François Nobili, Tim Ulinski, Bruno Ranchin, Maria Chiarra Marinozzi, Stéphanie Ngo, Véronique Frémeaux-Bacchi, Christine Pietrement

Published in: Pediatric Nephrology | Issue 1/2014

Abstract

Background

C3 glomerulopathy (C3G) is characterized by predominant C3 deposits in glomeruli and dysregulation of the alternative pathway of complement. Half of C3G patients have a C3 nephritic factor (C3NeF). C3G incorporated entities with a range of features on microscopy including dense deposit diseases (DDD) and C3 glomerulonephritis (C3GN). The aim of this work was to study children cases of C3G associated with C3NeF.

Methods

We reviewed 18 cases of C3G with a childhood onset associated with C3NeF without identified mutations in CFH, CFI, and MCP genes.

Results

Clinical histories started with recurrent hematuria for seven patients, nephrotic syndrome for four, acute post-infectious glomerulonephritis for three and acute renal failure for four. Twelve patients had a low C3 at first investigation. Kidney biopsy showed ten C3GN and eight DDD. Twenty-three percent of the patients tested presented elevated sC5b9. Seven patients relapsed 3 to 6 years after the onset. At the end of follow-up, two patients were under dialysis, 11 had a persistent proteinuria, five had none; four patients did not follow any treatment. Steroids were first used in 80 % of cases.

Conclusions

C3NeF associated C3G has a heterogeneous presentation and outcome. Anti-proteinuric agents may control the disease during follow-up, even after nephrotic syndrome at the onset. The efficiency of immunosuppressive therapy remains questionable.

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Title: C3 nephritic factor associated with C3 glomerulopathy in children
Authors: Camille Nicolas
Vincent Vuiblet
Veronique Baudouin
Marie-Alice Macher
Isabele Vrillon
Nathalie Biebuyck-Gouge
Maud Dehennault
Sophie Gié
Denis Morin
Hubert Nivet
François Nobili
Tim Ulinski
Bruno Ranchin
Maria Chiarra Marinozzi
Stéphanie Ngo
Véronique Frémeaux-Bacchi
Christine Pietrement
Publication date: 01-01-2014
Publisher: Springer Berlin Heidelberg
Published in: Pediatric Nephrology / Issue 1/2014
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-013-2605-6

At a glance: The STEP trials

Springer Medicine

C3 nephritic factor associated with C3 glomerulopathy in children

Abstract

Background

Methods

Results

Conclusions

At a glance: The STEP trials

Springer Medicine

Abstract

Background

Methods

Results

Conclusions

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