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Crescentic glomerulonephritis in children: a review of 43 cases
R N Srivastava1, A Moudgil, A Bagga
1Department of Pediatrics, All India Institute of Medical Sciences, New Delhi.
Insights
Crescentic glomerulonephritis (GN) in children often leads to poor kidney outcomes. Early diagnosis and treatment may improve renal function in some cases, but the prognosis remains grave.
Area of Science:
- Pediatric Nephrology
- Renal Pathology
Background:
- Crescentic glomerulonephritis (GN) is a severe kidney disease characterized by rapid loss of kidney function.
- Large crescents in over 50% of glomeruli indicate a poor prognosis.
Purpose of the Study:
- To analyze the clinical presentation, treatment, and outcomes of pediatric crescentic GN.
- To evaluate the long-term renal function in children diagnosed with this condition.
Main Methods:
- Retrospective analysis of 43 children with crescentic GN over 22 years.
- Review of clinical data, renal biopsy findings, and treatment regimens.
- Assessment of renal function and patient outcomes.
Main Results:
- 31 patients presented with acute nephritic features and renal insufficiency.
- 44% received immunosuppressive therapy; 6 patients recovered renal function.
- 7 of 11 post-streptococcal GN cases progressed to end-stage renal disease.
Conclusions:
- Pediatric crescentic GN has a generally poor prognosis, regardless of etiology.
- While some patients may experience partial improvement, progression to chronic renal insufficiency is common.
- A small subset of patients may recover renal function, particularly with aggressive therapy.
Abstract:
Forty-three children with crescentic glomerulonephritis (GN), having large crescents in more than 50% of the glomeruli, were observed during a period of 22 years. There were 17 boys and 26 girls between the ages of 3.5 and 14 years (mean 8.7 +/- 2.6). Thirty-one patients (72%) presented with acute nephritic features and increasing renal insufficiency (rapidly progressive GN) whereas 12 had an insidious onset with nephrotic syndrome, or rarely with nonspecific symptoms. Eleven patients had evidence of poststreptococcal GN and 6 an underlying systemic disorder. Renal biopsy showed large crescents in greater than 80% of the glomeruli in 38 cases (100% in 28) which were predominantly fibrocellular or fibrous in 80% of the patients. Nineteen patients (44%) were treated with prednisolone, cyclophosphamide and dipyridamole; in addition, 8 were also given anticoagulants. Six patients received pulse doses of corticosteroids. In 23 patients, there was inexorable progression of renal failure, 14 showed partial improvement but subsequently had varying degrees of renal insufficiency and in 6, there was recovery of renal function with normal levels of serum creatinine. Of the latter, 4 had received immunosuppressive anticoagulant therapy and 2 only supportive care. Of 11 patients with poststreptococcal crescentic GN, 7 progressed to end-stage renal disease and 2 developed chronic renal insufficiency. Our findings confirm the poor outcome of crescentic GN in children, irrespective of the underlying etiology. In a small proportion of cases, the disorder may have an insidious onset and a slowly progressive course, but an equally grave prognosis.