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Renal arteriography in von Hippel-Lindau Diease
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.
Abstract:
Since 1960, 17 patients with von Hippel-Lindau disease have had cerebellar hemangioblastomas removed at the Victoria General Hospital, Halifax, Nova Scotia. The 4 most recent patients have had renal arteriography as a part of their investigation. Though 3 of these had negative urography, all 4 had positive findings on arteriography. The first patient had bilateral renal cell carcinomas and cysts and failed to respond to 1 year of 'Laetrile' therapy; the second had small cysts in each kidney; the third had bilateral renal cysts, multiple left renal cell carcinomas and a clinically unsuspected right pheochromocytoma; the fourth had a small renal cell carcinoma. It is recommended that all patients with von Hippel-Lindau disease and siblings with any manifestations of the disease have renal arteriography as part of their early investigation.
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