Successful management of a patient with a C3 Glomerulonephritis and crescentic pattern: a case report

BMC Research Notes

Conceição Mota²  Graça Ferreira³  Ana Pinho¹ 

[1] Department of Nephrology, Centro Hospitalar Algarve, Faro Hospital, Leão Penedo, 8000-386 Faro, Portugal;Department of Pediatric Nephrology, Centro Hospitalar Porto, Porto, Portugal;Department of General Pediatrics, Centro Hospitalar Vila Nova de Gaia, Gaia, Portugal

关键词: Child;    Crescentic glomerulonephritis;    C3 glomerulonephritis;    Alternative pathway of complement;   

Others  :  1125660
DOI  :  10.1186/1756-0500-7-792

 received in 2014-06-20, accepted in 2014-10-22,  发布年份 2014

【 摘 要 】

Background

Crescentic glomerulonephritis is a rare condition in children and is typically associated with renal insufficiency. Dysfunction of the alternative complement pathway is an unusual aetiology with an unknown mechanism.

Case presentation

We report a case of a previously healthy 12-year-old Caucasian girl who was examined on emergency owing to an asymptomatic gross haematuria. An active urinary sediment and nephrotic-range proteinuria were identified, and serologic examination showed a decreased serum C3 concentration not associated with any immunologic or infectious cause. Oedema, hypertension, and renal insufficiency were not observed. A renal biopsy was performed, and crescentic glomerulonephritis associated with C3 glomerulonephritis was diagnosed. Prompt treatment with intravenous steroids resulted in complete resolution of the gross haematuria. Further examination did not detect any underlying acquired cause. A combination of oral steroids and cyclophosphamide, followed by mycophenolate mofetil, was maintained and resulted in clinical remission during an 8-month follow-up.

Conclusion

The presence of severe injury such as crescentic glomerulonephritis secondary to C3 glomerulonephritis is extremely unusual in children. This is the first known case of paediatric crescentic glomerulonephritis secondary to C3 glomerulonephritis that presented with gross haematuria and was treated early and effectively with immunosuppressive therapy based on its severe histologic features.

【 授权许可】

   
2014 Pinho et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

【 参考文献 】

• [1]Dewan D, Gulati S, Sharma RK, Prasad N, Jain M, Gupta A, Kumar A: Clinical spectrum and outcome of crescentic glomerulonephritis in children in developing countries. Pediatr Nephrol (Berlin, Germany) 2008, 23(3):389-394.
• [2]Sinha A, Puri K, Hari P, Dinda AK, Bagga A: Etiology and outcome of crescentic glomerulonephritis. Indian Pediatr 2013, 50(3):283-288.
• [3]Jennette JC: Rapidly progressive crescentic glomerulonephritis. Kidney Int 2003, 63(3):1164-1177.
• [4]Fervenza FC, Smith RJH, Sethi S: Association of a novel complement factor H mutation with severe crescentic and necrotizing glomerulonephritis. Am J Kidney Dis 2012, 60(1):126-132.
• [5]Sandhu G, Bansal A, Ranade A, Jones J, Cortell S, Markowitz GS: C3 glomerulopathy masquerading as acute postinfectious glomerulonephritis. Am J Kidney Dis 2012, 60(6):1039-1043.
• [6]Nicolas C, Vuiblet V, Baudouin V, Macher MA, Vrillon I, Biebuyck-Gouge N, Dehennault M, Gié S, Morin D, Nivet H, Nobili F, Ulinski T, Ranchin B, Marinozzi MC, Ngo S, Frémeaux-Bacchi V, Pietrement C: C3 nephritic factor associated with C3 glomerulopathy in children. Pediatr Nephrol (Berlin, Germany) 2014, 29(1):85-94.
• [7]Fakhouri F, Fremeaux-Bacchi V, Noel LH, Cook HT, Pickering MC: C3 glomerulopathy: a new classification. Nat Rev Nephrol 2010, 6(8):494-499.
• [8]Bomback AS, Appel GB: Pathogenesis of the C3 glomerulopathies and reclassification of MPGN. Nat Rev Nephrol 2012, 8(11):634-642.
• [9]Hawfield A, Iskandar SS, Smith RJ: Alternative pathway dysfunction in kidney disease: a case report and review of dense deposit disease and C3 glomerulopathy. Am J Kidney Dis 2013, 61(5):828-831.
• [10]Sethi S, Fervenza FC, Zhang Y, Zand L, Vrana JA, Nasr SH, Theis JD, Dogan A, Smith RJ: C3 glomerulonephritis: Clinicopathological findings, complement abnormalities, glomerular proteomic profile, treatment, and follow-up. Kidney Int 2012, 82(4):465-473.
• [11]Sethi S, Fervenza FC, Zhang Y, Zand L, Meyer NC, Borsa N, Nasr SH, Smith RJ: Atypical postinfectious glomerulonephritis is associated with abnormalities in the alternative pathway of complement. Kidney Int 2013, 83(2):293-299.
• [12]Servais A, Noel LH, Roumenina LT, Le Quintrec M, Ngo S, Dragon-Durey MA, Macher MA, Zuber J, Karras A, Provot F, Moulin B, Grünfeld JP, Niaudet P, Lesavre P, Frémeaux-Bacchi V: Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies. Kidney Int 2012, 82(4):454-464.
• [13]Bomback AS, Smith RJ, Barile GR, Zhang Y, Heher EC, Herlitz L, Stokes MB, Markowitz GS, D'Agati VD, Canetta PA, Radhakrishnan J, Appel GB: Eculizumab for dense deposit disease and C3 glomerulonephritis. Clin J Am Soc Nephrol 2012, 7(5):748-756.
• [14]Medjeral-Thomas NR, O'Shaughnessy MM, O'Regan JA, Traynor C, Flanagan M, Wong L, Teoh CW, Awan A, Waldron M, Cairns T, O'Kelly P, Dorman AM, Pickering MC, Conlon PJ, Cook HT: C3 glomerulopathy: Clinicopathologic features and predictors of outcome. Clin J Am Soc Nephrol 2014, 9(1):46-53.