Pediatric Nephrology

, Volume 24, Issue 1, pp 77–82 | Cite as

A single-center study of C1q nephropathy in children

  • Isabel Roberti
  • Noosha Baqi
  • Shefali Vyas
  • Dae Un Kim
Original Article

Abstract

C1q nephropathy (C1qN) is a rare idiopathic glomerulopathy typically seen in adolescents and young adults. All kidney biopsies done from 2002 to 2007 were analyzed (264). Thirteen cases of C1qN from 212 (6.6%) native biopsies and one case out of 52 (1.9%) transplant biopsies were reviewed regarding demographic features, clinical presentation, histopathology, treatment, and outcome. Age varied from 1 to 18 years; half were boys. Ten children (71.4%) presented with nephrotic syndrome (NS). The most common histopathology found was diffuse mesangial proliferative glomerulonephritis (DMP) by light microscopy (LM), with diffuse granular staining for C1q predominantly in the mesangium. Children with either NS or persistent gross hematuria received prednisone and angiotensin-converting enzyme inhibitors (ACEi) (11). Median follow-up was 36 months. Steroid response was complete in 6 patients (54.5%). Those with steroid resistance (5) or steroid dependence (2) received further immunosuppression with mycophenolate mofetil (MMF) or tacrolimus (Tac). Three children achieved complete remission and four partial remission. Frequent relapses were seen in 4/14 patients. Renal survival was 100%. Our report reveals a high incidence of C1qN in pediatric patients, with variable clinical presentation. Despite a high incidence of steroid resistance among those with NS, an excellent response was observed with the addition of further immunosuppression.

Keywords

C1q nephropathy Children Glomerulonephritis Nephrotic syndrome Proteinuria Hematuria Tacrolimus Mycophenolate mofetil 

References

  1. 1.
    Jennette JC, Hipp CG (1985) C1q nephropathy: A distinct pathologic entity usually causing nephrotic syndrome. Am J Kidney Dis 6:103–110PubMedGoogle Scholar
  2. 2.
    Jennette JC, Hipp CG (1985) Immunopathologic evaluation of C1q in 800 renal biopsy specimens. Am J Clin Pathol 83:415–420PubMedGoogle Scholar
  3. 3.
    Nishida M, Kawakatsu H, Komatsu H, Ishiwari K, Tamai M, Sawada T (2000) Spontaneous improvement in a case of C1q Nephropathy. Am J Kidney Dis 35:1–6CrossRefGoogle Scholar
  4. 4.
    Iskandar SS, Browning MC, Lorentz WB (1991) C1q nephropathy: A pediatric clinicopathologic study. Am J Kidney Dis 18:459–465PubMedGoogle Scholar
  5. 5.
    Fukuma Y, Hisano S, Segawa Y, Niimi K, Tsuru N, Kaku Y, Hatae K, Kiyoshi Y, Mitsudome A, Iwasaki H (2006) Clinicopathologic correlation of C1q nephropathy in children. Am J Kidney Dis 47:412–418PubMedCrossRefGoogle Scholar
  6. 6.
    Kersnik Levart T, Kenda RB, Avgustin Cavić M, Ferluga D, Hvala A, Vizjak A (2005) C1Q nephropathy in children. Pediatr Nephrol 20:1756–1761PubMedCrossRefGoogle Scholar
  7. 7.
    Lau KK, Gaber LW, Delos Santos NM, Wyatt RJ (2005) C1q nephropathy: features at presentation and outcome. Pediatr Nephrol 20:744–749PubMedCrossRefGoogle Scholar
  8. 8.
    Jennette JC, Falk RJ (2004) C1q nephropathy. In: Massry SG, Glassock RJ (eds) Textbook of nephrology, 4th edn. Lippincott, Williams & Wilkins, Philadelphia, pp 730–733Google Scholar
  9. 9.
    Markowitz GS, Schwimmer JA, Stokes MB, Nasr S, Seigle RL, Valeri AM, D’Agati VD (2003) C1q nephropathy: A variant of focal segmental glomerulosclerosis. Kidney Int 64:1232–1240PubMedCrossRefGoogle Scholar
  10. 10.
    National High Blood Pressure Education Program Working Group on Hypertension Control in Children and Adolescents (1996) Update on the 1987 task force report on high blood pressure in children and adolescents; a working group report from the national high blood pressure education program. Pediatrics 98:649–658Google Scholar
  11. 11.
    Schwartz GJ, Brion LP, Spitzer A (1987) The use of plasma creatinine concentration for estimating glomerular filtration rate in infants, children and adolescents. Pediatr Clin North Am 34:571–590PubMedGoogle Scholar
  12. 12.
    Calcott MA, Müller-Eberhard HJ (1972) C1q protein of human complement. Biochemistry 11:3443–3446PubMedCrossRefGoogle Scholar
  13. 13.
    Bao L, Quigg RJ (2007) Complement in lupus nephritis: the good, the bad, and the unknown. Semin Nephrol 27:69–80PubMedCrossRefGoogle Scholar
  14. 14.
    Turnberg D, Cook HT (2005) Complement and glomerulonephritis: new insights. Curr Opin Nephrol Hypertens 14:223–228PubMedCrossRefGoogle Scholar
  15. 15.
    Beaufils H, Alphonse JC, Guedon J, Legrain M (1978) Focal glomerulosclerosis: natural history and treatment. A report of 70 cases. Nephron 21:75–85PubMedCrossRefGoogle Scholar
  16. 16.
    Davenport A, Maciver AG, Mackenzie JC (1992) C1q nephropathy: do C1q deposits have any prognostic significance in the nephrotic syndrome? Nephrol Dial Transplant 7:391–396PubMedGoogle Scholar
  17. 17.
    Jones E, Magil A (1982) Nonsystemic mesangiopathic glomerulonephritis with “full house” immunofluorescence. Pathological and clinical observations in five patients. Am J Clin Pathol 78:29–34PubMedGoogle Scholar
  18. 18.
    Sharman A, Furness P, Feehally J (2004) Distinguishing C1Q nephropathy from lupus nephritis. Nephrol Dial Transplant 19:1420–1426PubMedCrossRefGoogle Scholar
  19. 19.
    Srivastava T, Chadha V, Taboada EM, Alon US (2000) C1q nephropathy presenting as rapidly progressive crescentic glomerulonephritis. Pediatr Nephrol 14:976–979PubMedCrossRefGoogle Scholar
  20. 20.
    Shappell SB, Myrthil G, Fogo A (1997) An adolescent with relapsing nephrotic syndrome: minimal changes versus focal-segmental glomerulosclerosis versus C1q nephropathy. Am J Kidney Dis 29:966–970PubMedCrossRefGoogle Scholar
  21. 21.
    Kuwano M, Ito Y, Amamoto Y, Aida K (1993) A case of congenital nephrotic syndrome associated with positive C1q immunofluorescence. Pediatr Nephrol 7:452–454PubMedCrossRefGoogle Scholar
  22. 22.
    Isaac J, Shihab FS (2002) De novo C1q nephropathy in the renal allograft of a kidney pancreas transplant recipient: BK induced nephropathy? Nephron 92:431–436PubMedCrossRefGoogle Scholar

Copyright information

© IPNA 2008

Authors and Affiliations

  • Isabel Roberti
    • 1
  • Noosha Baqi
    • 1
  • Shefali Vyas
    • 1
  • Dae Un Kim
    • 2
  1. 1.Department of Pediatrics, Pediatric Nephrology and Transplantation DivisionSaint Barnabas Medical CenterLivingstonUSA
  2. 2.Department of PathologySaint Barnabas Medical CenterLivingstonUSA

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