C3 nephritic factor associated with C3 glomerulopathy in children

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• 作者：Camille Nicolas (1)
  Vincent Vuiblet (2)
  Veronique Baudouin (4)
  Marie-Alice Macher (4)
  Isabele Vrillon (5)
  Nathalie Biebuyck-Gouge (6)
  Maud Dehennault (7)
  Sophie Gi(8)
  Denis Morin (9)
  Hubert Nivet (10)
  Franois Nobili (11)
  Tim Ulinski (12)
  Bruno Ranchin (13)
  Maria Chiarra Marinozzi (14)
  Sthanie Ngo (3)
  Vonique Freaux-Bacchi (16) (3)
  Christine Pietrement (1) (15)
  
• 关键词：Complement alternative pathway ; C3 glomerulonephritis ; Dense deposit disease ; Membranoproliferative glomerulonephritis ; C3 glomerulopathy
• 刊名：Pediatric Nephrology
• 出版年：2014
• 出版时间：January 2014
• 年：2014
• 卷：29
• 期：1
• 页码：85-94
• 全文大小：
• 作者单位：Camille Nicolas (1)
  Vincent Vuiblet (2)
  Veronique Baudouin (4)
  Marie-Alice Macher (4)
  Isabele Vrillon (5)
  Nathalie Biebuyck-Gouge (6)
  Maud Dehennault (7)
  Sophie Gi(8)
  Denis Morin (9)
  Hubert Nivet (10)
  Franois Nobili (11)
  Tim Ulinski (12)
  Bruno Ranchin (13)
  Maria Chiarra Marinozzi (14)
  Sthanie Ngo (3)
  Vonique Freaux-Bacchi (16) (3)
  Christine Pietrement (1) (15)
  
  1. Department of Pediatrics, Nephrology unit, CHU de Reims, Reims, France
  2. Department of Pathology, Department of Nephrology, CHU de Reims, Reims, France
  4. Assistance Publique-Hopitaux de Paris, Department of Pediatric Nephrology, Hpital Robert Debr Paris, France
  5. Department of Pediatrics, Nephrology Unit, CHU Nancy, Nancy, France
  6. Assistance Publique-Hpitaux de Paris, Department of Pediatric Nephrology, Hpital Necker Enfants-Malades, Paris, France
  7. Department of Pediatrics, Nephrology Unit, CHRU Lille Jeanne de Flandre, Lille, France
  8. Department of Pediatrics, Nephrology Unit, CHU Rennes, Rennes, France
  9. Department of Pediatrics, Nephrology Unit, CHU Montpellier, Montpellier, France
  10. Department of Pediatrics, Nephrology Unit, CHU Tours, Tours, France
  11. Department of Pediatrics, Nephrology Unit, CHU Besanon, Besanon, France
  12. Department of Pediatric Nephrology, Hpital Trousseau, Assistance Publique-Hpitaux de Paris, Paris, France
  13. Department of Pediatric Nephrology, Hpital Femme Me-Enfant, Bron, France
  14. INSERM Umrs872, CRC Paris, Paris, France
  3. Assistance Publique-Hopitaux de Paris, Department of Immunology, Hpital Europn Georges Pompidou, Paris, France
  16. Service dmmunologie Biologique, Hpital Europn Georges Pompidou, 20-40 rue Leblanc, 75908, Paris cedex 15, France
  15. Piatrie A, CHU de Reims, 45 rue Cognacq Jay, 51092, Reims cedex, France
  
• ISSN：1432-198X

文摘

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 6years 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.