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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.
Summary
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.