ArticlePediatric nephrology (Berlin, Germany)2020
Clinicopathological features of C3 glomerulopathy in children: a single-center experience.
Article in Pediatric nephrology (Berlin, Germany), 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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Who cites it
4 citing papers in PubMed.
- C3 glomerulopathy: a kidney disease mediated by alternative pathway deregulation.Frontiers in nephrology · 2024Review
- Long-term follow-up including extensive complement analysis of a pediatric C3 glomerulopathy cohort.Pediatric nephrology (Berlin, Germany) · 2022Article
- Treatment of C3 Glomerulopathy in Adult Kidney Transplant Recipients: A Systematic Review.Medical sciences (Basel, Switzerland) · 2020Article
- Dense deposit disease in an adolescent male mimicking acute post-streptococcal glomerulonephritis.HippokratiaArticle
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Authors and funding
5 authors.
Funding
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Abstract
backgroundC3 glomerulopathy (C3G) is defined by dominant glomerular deposition of C3 and minimal or no immunoglobulin, with two subtypes-dense deposit disease (DDD) and C3 glomerulonephritis (C3GN)-distinguished by features on electron microscopy (EM). Given that this rare disease has generally unfavorable yet highly variable outcomes, we sought out to review the histopathology, complement/genetic studies, and renal outcomes of pediatric patients with C3G at our institution.
methodsAll native kidney biopsies performed in a single pediatric hospital over a 10-year period were reviewed for features of C3G. Of 589 biopsy reports, we identified 9 patients fulfilling the diagnostic criteria for C3G and retrospectively reviewed their clinical chart and renal biopsy findings.
resultsWe identified 4 patients with DDD, 4 with C3GN, and 1 indeterminate case, with features of both C3GN and DDD. Five patients were positive for one or more nephritic factors (C3NeF, C4NeF, C5NeF) with 1 patient additionally positive for complement factor H (CFH) autoantibody. Genetic testing done in 5 of the 9 patients failed to identify any causative mutations. Three patients showed progressive renal dysfunction over a mean follow-up period of 33 months.
conclusionsComplement and genetic studies are now routinely recommended for patients with a histopathological diagnosis of C3G. Careful interpretation of these studies and their prognostic and therapeutic implications in conjunction with biopsy findings is needed to further understand the pathophysiology of this rare disease in children.
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