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- Title
C3 Glomerulopathy and post-infectious glomerulonephritis define a disease spectrum.
- Authors
Al-Ghaithi, Badria; Chanchlani, Rahul; Riedl, Magdalena; Thorner, Paul; Licht, Christoph
- Abstract
Background: Post-infectious glomerulonephritis (PIGN) usually follows a benign course, but few children have an atypical, severe presentation, and these exceptional cases have been linked to the dysregulation of the complement alternative pathway (CAP). There is a considerable overlap in the histopathological features of PIGN and C3 glomerulopathy (C3G), which is also associated with CAP dysregulation but has a poorer outcome. We hypothesized that PIGN and C3G define a disease spectrum, and that in the past there may be some children with C3G who were misclassified with PIGN before C3G was described as a separate disease entity. Methods: Children with PIGN ( n = 33) diagnosed between 1985 and 2010 who underwent a renal biopsy due to their unusual course were reviewed and of them, 8 were reclassified into C3G based on the current classification criteria. Outcome was based on the degree of proteinuria, C3 level, and renal function at follow-up. Results: Sixteen (72.7%) children with typical PIGN recovered completely as compared to only 2 (25%) with C3G. Of note, children with 'typical' PIGN had a more severe disease course at onset; however, the outcome at last follow up was favorable. Conclusions: Our results support the hypothesis that PIGN and C3G form a disease spectrum and have different long-term clinical implications and management strategies.
- Subjects
ONTARIO; TREATMENT of glomerulonephritis; BIOPSY; CHI-squared test; COMPLEMENT (Immunology); CONFIDENCE intervals; ELECTRON microscopy; FISHER exact test; FLUORESCENT antibody technique; GLOMERULAR filtration rate; GLOMERULONEPHRITIS; IMMUNOGLOBULINS; INFECTION; LONGITUDINAL method; MICROSCOPY; PROBABILITY theory; PROTEINURIA; DATA analysis software; DESCRIPTIVE statistics; MANN Whitney U Test; DISEASE complications; DIAGNOSIS
- Publication
Pediatric Nephrology, 2016, Vol 31, Issue 11, p2079
- ISSN
0931-041X
- Publication type
Article
- DOI
10.1007/s00467-015-3311-3