Renal arteriography in von Hippel-Lindau Diease

Journal of the Canadian Association of Radiologists
|December 1, 1978
PubMed

Insights

Von Hippel-Lindau disease patients often have kidney issues. Renal arteriography is recommended for early detection of these complications in affected individuals and their siblings.

Area of Science:

  • Nephrology
  • Oncology
  • Genetics

Background:

  • Von Hippel-Lindau (VHL) disease is a genetic disorder associated with tumor formation.
  • Cerebellar hemangioblastomas are a common manifestation of VHL disease.
  • Kidney abnormalities, including cysts and renal cell carcinoma, are also prevalent in VHL patients.

Observation:

  • This study reviewed 17 VHL patients undergoing cerebellar hemangioblastoma removal.
  • The 4 most recent patients underwent renal arteriography.
  • Urography results were negative in 3 of these 4 patients.

Findings:

  • Renal arteriography revealed abnormalities in all 4 patients.
  • Abnormalities included bilateral renal cell carcinomas, cysts, and a pheochromocytoma.
  • One patient with bilateral renal cell carcinomas and cysts did not respond to 'Laetrile' therapy.

Implications:

  • Renal arteriography is a valuable diagnostic tool for detecting kidney abnormalities in VHL disease.
  • Early investigation with renal arteriography is recommended for all VHL patients and their siblings.
  • Prompt diagnosis and management of renal manifestations are crucial for VHL patients.

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