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C1q nephropathy in children: clinical characteristics and outcome

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Abstract

Background

C1q nephropathy has been suggested as a separate disease entity. C1q positivity has also been described in association with nephrotic syndrome (NS) as a potential marker for worse outcome. The aims of this study were to describe the clinical characteristics, laboratory parameters and outcomes of 35 children whose renal histology revealed predominant mesangial C1q deposition and to investigate if the experience at our institution supports the above hypothesis.

Method

Clinical and pathological characteristics of all children whose kidney biopsies showed positive C1q staining were retrospectively recorded. The outcome of children with minimal change nephrotic syndrome (MCNS) and predominant mesangial C1q deposition based on C1q staining was compared with that of a concurrent group of children with MCNS with no such immune staining.

Results

The median age of the patient cohort was 4.5 years (range 6 months to 16 years), 69 % were boys and 88 % presented with nephrotic syndrome (NS). Children with C1q staining and MCNS had more relapses (p = 0.001) and shorter relapse-free periods (p = 0.033) than those with negative immunostaining, but the long-term outcomes were similar in both groups.

Conclusion

Our data do not support C1q nephropathy as a separate diagnostic category. Children with MCNS and mesangial C1q deposition (staining) showed more relapses but no difference in long term renal outcome.

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Acknowledgments

NJS is part supported by the NIHR GOSH BRC and GOSHCC. The results presented in this study have not been published previously in whole or part. The authors would like to thank Mr. Sarath Gamage for his support with statistical analysis.

Conflict of interest

None.

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Authors and Affiliations

  1. Nephrology Unit, Great Ormond Street Hospital for Children, Great Ormond Street, London, WC1N 3JH, UK

    Vindya N. Gunasekara & Kjell Tullus

  2. Nephrology Unit, Lady Ridgeway Hospital for Children, Colombo, Sri Lanka

    Vindya N. Gunasekara

  3. Department of Paediatric Pathology, Great Ormond Street Hospital for Children, Great Ormond Street, London, WC1N 3JH, UK

    Neil J. Sebire

  4. Institute of Child Health, Faculty of Biomedical Sciences, University College London (UCL), London, UK

    Neil J. Sebire

Authors
  1. Vindya N. Gunasekara
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  2. Neil J. Sebire
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  3. Kjell Tullus
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Corresponding author

Correspondence to Kjell Tullus.

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Gunasekara, V.N., Sebire, N.J. & Tullus, K. C1q nephropathy in children: clinical characteristics and outcome. Pediatr Nephrol 29, 407–413 (2014). https://doi.org/10.1007/s00467-013-2692-4

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  • DOI: https://doi.org/10.1007/s00467-013-2692-4

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