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Rapidly progressive glomerulonephritis in Thai children
W Tapaneya-Olarn1, C Tapaneya-Olarn, V Boonpucknavig
1Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Bangkok, Thailand.
Insights
Children with extensive crescentic glomerulonephritis often experience rapid kidney decline. Over 80% crescent involvement indicates a poor prognosis for pediatric kidney disease patients.
Area of Science:
- Pediatric Nephrology
- Renal Pathology
- Glomerular Diseases
Background:
- Crescentic glomerulonephritis (CrGN) is a severe kidney disease characterized by rapid renal deterioration.
- Pediatric CrGN presents unique challenges in diagnosis and management.
- Understanding prognostic factors is crucial for guiding treatment in children.
Purpose of the Study:
- To investigate the clinical characteristics and outcomes of children diagnosed with extensive crescentic glomerulonephritis.
- To identify factors associated with poor prognosis in pediatric CrGN.
- To analyze the underlying causes of rapid progressive glomerulonephritis (RPGN) in this cohort.
Main Methods:
- Retrospective analysis of 476 patients with glomerulopathy to identify 16 children with extensive CrGN (defined as >80% crescents).
- Clinical data collection including presenting symptoms, laboratory values (serum creatinine, BUN, urinalysis), and serological markers (C3).
- Evaluation of renal biopsy findings and patient outcomes (renal recovery vs. end-stage renal disease).
Main Results:
- The study included 16 children (1-14 years) with extensive CrGN, presenting with edema, oligoanuria, hypertension, hematuria, and uremic symptoms.
- Common laboratory findings included anemia (100%), hematuria (100%), heavy proteinuria (75%), elevated creatinine (mean 804 µmol/L), and BUN (mean 38 mmol/L).
- Idiopathic RPGN (9 cases) and post-infectious glomerulonephritis (PSAGN, 6 cases) were the main causes. Eight patients progressed to end-stage renal disease (ESRD), while eight recovered with improved renal function. Extensive crescent formation (>80%) and idiopathic RPGN correlated with poor prognosis.
Conclusions:
- Extensive crescentic glomerulonephritis in children is associated with significant renal morbidity and a high risk of progression to ESRD.
- Early identification of prognostic indicators, such as the extent of crescent formation, is vital for managing pediatric CrGN.
- Further research into the specific mechanisms and targeted therapies for pediatric RPGN is warranted.
Abstract:
Sixteen children with extensive crescentic glomerulonephritis and rapid renal deterioration were selected from 476 patients with glomerulopathy for study. The patients (1-14 yr, M:F = 5:11) presented with edema, oligoanuria, hypertension, gross hematuria and uremic symptoms in 81, 62, 62, 56 and 50 per cent, respectively. The mean Scr was 804 (+/- 436) micromole/L and BUN 38 (+/- 13.4) mmole/L. Anemia was found in 100 per cent, hematuria in 100 per cent, heavy proteinuria 75 per cent, hypoalbuminemia 40 per cent, hypercholesterolemia 38 per cent and low C3 40 per cent. The underlying causes of RPGN included idiopathic 9, PSAGN 6 and LE 1. Eight patients recovered with normal or slightly elevated Scr while the diseases progressed to ESRD in 8 patients. Idiopathic RPGN and extensive (greater than 80%) crescentic glomerulonephritis correlated with a poor prognosis.