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Pediatric C3 glomerulopathy: a 12-year single-center experience

Zafirah Zahir1,2, Asif Sadiq Wani3,4, Amit Gupta5

  • 1Department of Pathology, Sanjay Gandhi Post-Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

Pediatric Nephrology (Berlin, Germany)
|October 1, 2020
PubMed

Insights

Pediatric complement component 3 glomerulopathy (C3G) differs from adult C3G in presentation and outcomes. Understanding these differences is crucial for diagnosing and managing C3G in children.

Area of Science:

  • Nephrology
  • Pediatric Nephrology
  • Glomerular Diseases

Background:

  • Complement component 3 glomerulopathy (C3G) is a rare kidney disease with limited pediatric data.
  • This study addresses the scarcity of information on childhood C3G by comparing it with adult cases.
  • It also investigates pediatric C3G subgroups and factors predicting poor outcomes.

Purpose of the Study:

  • To compare childhood C3G cases with adult C3G.
  • To analyze subgroups within pediatric C3G, specifically C3 glomerulonephritis (C3GN) and dense deposit disease (DDD).
  • To identify predictors of poor kidney outcomes in pediatric C3G.

Main Methods:

  • A 12-year retrospective, single-center cohort observational study.
  • Diagnosis of C3G followed the 2013 consensus guidelines.
  • Electron microscopy was used to categorize pediatric C3G into C3GN and DDD.

Main Results:

  • 162 C3G patients diagnosed (43 pediatric, 119 adult), predominantly male.
  • Pediatric DDD cases were younger, presented with more severe disease (crescents), but had less chronicity than pediatric C3GN.
  • Adults had lower eGFR and more biopsy chronicity; pediatric C3G showed a better prognosis. Low eGFR and interstitial fibrosis/tubular atrophy predicted kidney failure in children.

Conclusions:

  • Pediatric C3G subgroups (C3GN, DDD) show distinct clinical presentations and disease courses but similar long-term outcomes.
  • Pediatric C3G is a distinct entity from adult C3G, differing in presentation, lab results, biopsy findings, treatment, and outcomes.
  • Childhood C3G requires consideration as a separate condition, not merely a pediatric variant of adult C3G.
Abstract

Keywords:
C3 glomerulonephritisDense deposit diseaseOutcomePediatric C3GPrognosis

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