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Related Experiment Videos

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
PubMed
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.

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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.

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  • 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.