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- Title
C3 nephritic factor associated with C3 glomerulopathy in children.
- Authors
Nicolas, Camille; Vuiblet, Vincent; Baudouin, Veronique; Macher, Marie-Alice; Vrillon, Isabele; Biebuyck-Gouge, Nathalie; Dehennault, Maud; Gié, Sophie; Morin, Denis; Nivet, Hubert; Nobili, François; Ulinski, Tim; Ranchin, Bruno; Marinozzi, Maria Chiarra; Ngo, Stéphanie; Frémeaux-Bacchi, Véronique; Pietrement, Christine
- 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.
- Subjects
FRANCE; BIOPSY; COMPLEMENT (Immunology); GLOMERULONEPHRITIS; KIDNEY glomerulus; U-statistics; RETROSPECTIVE studies; DESCRIPTIVE statistics; GENETICS
- Publication
Pediatric Nephrology, 2014, Vol 29, Issue 1, p85
- ISSN
0931-041X
- Publication type
Article
- DOI
10.1007/s00467-013-2605-6