Year: 2017
Type: conference-abstract
Abstract: INTRODUCTION AND AIMS: C3 glomerulopathy is a histopathologically defined entity with isolated or predominant deposits of C3 on immunofluorescence studies of the renal biopsy tissue. Aim To describe the clinicopathological profile of patients presenting with C3 glomerulopathy and their renal outcome. Study setting Pediatric Nephrology Division in a teaching Hospital Study design Retrospective Cohort Study METHODS: Retrospective analysis of renal biopsies (2006-2016; n=298) which showed C3 dominant deposits on Immunofluorescence, in isolation or with trace amounts of immunoglobulin were identified. Cases were classified as Dense Deposit Disease (DDD) or C3 glomerulonephritis (C3GN) based on the Electron Microscopy morphology. The clinical, biochemical, serological profile, eGFR and need for renal replacement therapy was noted. The data was expressed as frequencies and the renal survival expressed as cumulative probability of survival by Kaplan Meir analysis. RESULTS: Thirteen patients had C3 glomerulopathy accounting for 4% of all renal biopsies done during this period. 4 with Dense Deposit Disease and 9 with C3 Glomerulonephritis. Isolated C3 deposits were seen in 60%. The median age of presentation was 8 years (4yrs- 13.5yrs) with 7 boys and 7 girls. There were 4 categories of presentation: atypical nephrotic syndrome with microscopic hematuria and low C3 (54%), PIGN like presentation with nephritic-nephrotic syndrome (15%), Isolated hematuria (15%) and Nephritic-nephrotic progressed rapidly to acute renal failure (15%). The eGFR at presentation was <60ml/min/1.73m2 in 50% of the patients, with median eGFR = 68ml/min/1.73m2 (8-107 ml/min/1.73m2). The median follow up was 26 months (range: 2 months - 131 months). All patients had hematuria and low C3 at presentation. Nephrotic range proteinuria was seen in 92%. Hypocomplementemia persisted in 50% of the patients. Kaplan Meir renal survival analyses curve showed median survival without deranged renal functions was 60 months, but cumulative survival without progression to ESRD at 1 year was 83%, 66.67% at 5 year and also at the end of 10 years. MP836 Figure
Institution: Bai Jerbai Wadia Hospital for Children
Language: English
Subfield: Nephrology
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W2619863377