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Related Experiment Videos

C1q nephropathy: a pediatric clinicopathologic study.

S S Iskandar1, M C Browning, W B Lorentz

  • 1Department of Pathology, Bowman Gray School of Medicine, Wake Forest University, Winston-Salem, NC 27157-1072.

American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation
|October 1, 1991
PubMed
Summary

Children with nephrotic syndrome and C1q deposits often show no glomerular changes on light microscopy, mimicking minimal change disease. However, these patients respond poorly to steroids, indicating a distinct condition.

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Area of Science:

  • Nephrology
  • Immunopathology
  • Pediatric Nephrology

Background:

  • Proteinuria and nephrotic syndrome in children can have diverse underlying causes.
  • Minimal change disease (MCD) is a common cause of nephrotic syndrome in children, typically responding well to steroids.
  • Distinguishing MCD from other glomerular diseases is crucial for appropriate management.

Purpose of the Study:

  • To investigate the histological and immunopathological features of childhood nephrotic syndrome with prominent mesangial C1q deposits.
  • To determine the clinical course and response to steroid therapy in these patients.

Main Methods:

  • Analysis of kidney biopsies from 15 children with proteinuria.
  • Histological examination using light microscopy.

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  • Immunofluorescence (IF) microscopy to detect immune deposits.
  • Ultrastructural examination using electron microscopy.

    • Main Results:

    • Fifteen children presented with nephrotic-level proteinuria.
    • No glomerular histologic alterations were seen in 8 cases; focal and segmental scarring was present in 7.
    • All cases showed prominent mesangial C1q deposits on IF, with variable immunoglobulins.
    • Electron microscopy revealed conspicuous mesangial electron-dense deposits in most cases.
    • Cases without glomerular histologic alterations were indistinguishable from MCD on light microscopy but showed poor response to prednisone.
    • Cases with C1q deposits uniformly had an unsatisfactory response to oral prednisone.

    Conclusions:

    • The presence of prominent mesangial C1q deposits identifies a subset of childhood nephrotic syndrome.
    • This subset may be histologically similar to MCD but exhibits steroid resistance.
    • C1q deposition is a key marker for identifying these steroid-resistant cases, regardless of light microscopic findings.