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Idiopathic collapsing glomerulopathy in children.
H K Singh1, L A Baldree, D W McKenney
1Department of Pathology and Laboratory Medicine, East Carolina University School of Medicine, Greenville, NC 27858, USA. ssingh@pcmh.com
Pediatric Nephrology (Berlin, Germany)
|February 23, 2000
Summary
Idiopathic collapsing glomerulopathy (ICG) is a rare, aggressive kidney disease. This study highlights ICG in children, showing similar clinical and pathological features to adults, often presenting as steroid-resistant nephrotic syndrome.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Glomerular Diseases
Background:
- Idiopathic collapsing glomerulopathy (ICG) is a distinct variant of focal segmental glomerulosclerosis.
- ICG typically presents with rapid renal insufficiency, predominantly in males and African-Americans.
- Similar lesions are seen in HIV-infected or IV drug-abusing patients, but most reported ICG cases are in adults.
Purpose of the Study:
- To describe the clinical and pathological characteristics of idiopathic collapsing glomerulopathy in a pediatric cohort.
- To compare pediatric ICG with previously reported adult cases.
Main Methods:
- Retrospective review of clinical data and renal biopsy findings.
- Identification of six pediatric patients diagnosed with ICG.
- Analysis of patient demographics, clinical presentation, and disease progression.
Main Results:
- Six pediatric patients (all male, 5 African-American, 1 Hispanic; ages 2-17) were identified.
- All presented with steroid-resistant nephrotic syndrome and significant proteinuria (average 6.3 g/24h).
- Five patients were HIV-negative, and none had IV drug abuse history; two progressed to end-stage renal disease within a year.
Conclusions:
- Pediatric ICG is an aggressive focal segmental glomerulosclerosis variant.
- Clinical and pathological features in children mirror those observed in adult ICG.
- Early diagnosis and management are crucial due to rapid progression to end-stage renal disease.