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Published on: June 7, 2014
[Renal lesions in tuberous sclerosis]
Francis Schillinger1, Richard Montagnac
1Service de néphrologie-hémodialyse, centre hospitalier général, 101, avenue Anatole-France, 10003 Troyes, France. francis.schillinger@libertysurf.fr
Nephrologie & Therapeutique
|March 22, 2007
Summary
Tuberous sclerosis often involves benign kidney tumors like angiomyolipomas and cysts. Chronic kidney disease in these patients is rare but can lead to end-stage renal failure requiring dialysis or transplantation.
Area of Science:
- Nephrology
- Oncology
- Genetics
Context:
- Tuberous sclerosis complex (TSC) is a genetic disorder.
- Renal manifestations are common in TSC, including benign tumors and cysts.
- Chronic renal failure is an infrequent but serious complication.
Purpose:
- To analyze the characteristics and outcomes of chronic renal failure in patients with tuberous sclerosis.
- To investigate the prevalence of different renal pathologies in TSC patients with renal failure.
Summary:
- A national survey identified 65 patients with TSC and chronic renal failure.
- Renal lesions included angiomyolipomas (23.1%), cysts (18.5%), both (53.8%), and malignancies (12.3%).
- Most patients with end-stage renal failure (48) required dialysis, with 20 successfully transplanted.
Impact:
- Highlights the importance of monitoring renal function in TSC patients.
- Provides data on the management and prognosis of renal failure in this population.
- Informs clinical practice regarding the renal complications of tuberous sclerosis.
