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Crescentic glomerulonephritis in children
H M Jardim1, J Leake, R A Risdon
1Renal Unit, Hospital for Sick Children, London, UK.
Insights
Crescentic glomerulonephritis in children often leads to kidney failure, with 50% progressing to end-stage renal disease. Early treatment is crucial, as fibrous crescents indicate a poorer prognosis than fibrocellular ones.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Immunology
Background:
- Crescentic glomerulonephritis (CGN) is a severe kidney disease characterized by rapid glomerular damage.
- CGN in pediatric populations presents unique challenges in diagnosis and management.
- Understanding prognostic factors is vital for improving outcomes in affected children.
Purpose of the Study:
- To review and analyze data from pediatric patients with CGN treated at a major children's hospital.
- To identify clinical features, underlying causes, and treatment strategies for CGN in children.
- To evaluate the long-term outcomes and identify prognostic indicators for renal failure in pediatric CGN.
Main Methods:
- Retrospective review of 30 pediatric patients diagnosed with CGN (over 50% glomeruli with crescents) over 13 years.
- Evaluation of initial clinical presentations, including edema, hypertension, hematuria, oliguria, and glomerular filtration rate (GFR).
- Analysis of various underlying etiologies (e.g., Henoch-Schönlein purpura, lupus, anti-GBM disease) and treatment regimens (plasma exchange, immunosuppressants).
Main Results:
- 50% of patients progressed to end-stage renal failure, requiring dialysis or transplantation.
- The time interval between disease onset and treatment initiation was a significant prognostic factor.
- Fibrous crescents were associated with significantly worse outcomes compared to fibrocellular crescents (P < 0.05).
Conclusions:
- Pediatric CGN has a high rate of progression to end-stage renal failure.
- Timely intervention is critical for improving patient prognosis.
- Histopathological features, specifically the type of crescent formation, can predict disease outcome.
Abstract:
Data on patients with crescentic glomerulonephritis (greater than 50% glomeruli with crescents), referred to the Hospital for Sick Children during the past 13 years, were reviewed. Thirty patients (13 male, 17 female) aged 3.7-15.7 years (mean 9.5) were evaluated. Initial clinical features included: oedema (24/30), hypertension (19/30), gross haematuria (15/30), oliguria (15/30) and a decreased glomerular filtration rate (GFR less than 30 ml/min per 1.73 m2) (22/30). Henoch-Schönlein purpura was present in 9 patients, microscopic polyarteritis in 3, polyarteritis nodosa in 1, Wegener's granulomatosis in 1, systemic lupus erythematosus in 1, post-streptococcal glomerulonephritis in 2, mesangiocapillary glomerulonephritis in 7, anti-glomerular basement membrane glomerulonephritis in 2, and 4 were idiopathic. In 10 patients 50%-79% of glomeruli were affected by crescentic changes (group 1) and in the remaining 20, 80% or more (group 2). The crescents were cellular, fibrocellular or fibrous, and the degree of sclerosis was assessed. Patients in both groups were treated with plasma exchange, corticosteroids, anticoagulants, cyclophosphamide and azathioprine in different combinations. On follow-up, 3 patients were dead, 1 was lost to follow-up, 12 were on dialysis/transplant programmes, 4 had a GFR of less than 30 and 10 a GFR of more than 30 ml/min per 1.73 m2. In our experience, 50% progressed to end-stage renal failure. The interval between disease onset and start of treatment was a prognostic factor for outcome. Fibrous crescents were associated with a worse outcome than fibrocellular crescents (P less than 0.05). Outcome was not, however, related to the percentage of glomeruli affected (P greater than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)