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Related Experiment Videos

Familial retroperitoneal fibrosis.

E J Doolin1, H Goldstein, B Kessler

  • 1Department of Surgery, Cooper Hospital/University Medical Center, Camden, NJ.

Journal of Pediatric Surgery
|December 1, 1987
PubMed
Summary
This summary is machine-generated.

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Retroperitoneal fibrosis is a rare cause of obstructive uropathy in children. This study reports the first familial cases, suggesting a link to systemic autoimmune diseases.

Area of Science:

  • Pediatric Nephrology
  • Immunology
  • Genetics

Background:

  • Retroperitoneal fibrosis (RPF) is an uncommon cause of obstructive uropathy in children.
  • The exact etiology of RPF remains largely unknown, though associations with autoimmune conditions exist.
  • Familial cases of RPF in pediatric populations have not been previously reported.

Observation:

  • This report details a family with two siblings diagnosed with idiopathic retroperitoneal fibrosis.
  • Both affected sisters and their father exhibited clinical and laboratory evidence of systemic immunologic diseases.
  • These cases represent the first documented instances of both autoimmune and familial characteristics in pediatric RPF.

Findings:

  • The presented cases suggest that idiopathic retroperitoneal fibrosis in children may have a familial component.

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  • The co-occurrence of RPF and systemic immune markers in multiple family members strengthens the autoimmune hypothesis.
  • This familial clustering indicates a potential genetic predisposition interacting with autoimmune factors.

    • Implications:

    • These findings support the concept of retroperitoneal fibrosis as a localized manifestation of a broader systemic immune disorder.
    • Further research into the genetic and immunological underpinnings of RPF is warranted.
    • Understanding the familial and autoimmune links may lead to improved diagnostic and therapeutic strategies for pediatric RPF.