The kidney in tuberous sclerosis: manifestations and molecular genetic mechanisms

Nephrol Dial Transplant. 1996;11 Suppl 6:34-7. doi: 10.1093/ndt/11.supp6.34.


Renal involvement is common in tuberous sclerosis. Angiomyolipomas and cysts are found in approximately 50 and 30% of patients respectively, and often occur together. Tuberous sclerosis also appears to be associated with a small but increased risk of renal cell carcinoma. Recent studies have begun to elucidate the molecular genetic mechanisms underlying the renal manifestations of this systemic autosomal dominant disorder.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Angiomyolipoma / etiology
  • Carcinoma, Renal Cell / etiology
  • Cysts / etiology
  • Humans
  • Kidney Diseases / etiology*
  • Kidney Neoplasms / etiology
  • Molecular Biology
  • Tuberous Sclerosis / complications*
  • Tuberous Sclerosis / genetics