title: Clinical and molecular insights into tuberous sclerosis complex renal disease.
abstract: Patients with tuberous sclerosis complex are at great risk of developing renal lesions as part of their disease. These lesions include renal cysts and tumors. Significant advances in understanding the cell biology of these renal lesions has already led to clinical trials demonstrating that pharmacological interventions are likely possible. This review focuses on the pathology of these renal lesions, their underlying cell biology, and the possible therapeutic strategies that may prove to significantly improve care for these patients.
authors.affiliation: Division of Nephrology and Hypertension, Cincinnati Children's Hospital Medical Center, MLC 7022, 3333 Burnet Avenue, Cincinnati, OH, 45229-3039, USA.
authors.forename: Brian J
authors.initials: BJ
authors.lastname: Siroky
authors.forename: Hong
authors.initials: H
authors.lastname: Yin
authors.forename: John J
authors.initials: JJ
authors.lastname: Bissler
mesh_terms: D018207:Angiomyolipoma / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D000903:Antibiotics, Antineoplastic / Q000627:therapeutic use; D002648:Child; D006801:Humans; D052177:Kidney Diseases, Cystic / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D007680:Kidney Neoplasms / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D000076222:Mechanistic Target of Rapamycin Complex 1; D046912:Multiprotein Complexes; D011506:Proteins / Q000502:physiology; D020123:Sirolimus / Q000627:therapeutic use; D058570:TOR Serine-Threonine Kinases; D014402:Tuberous Sclerosis / Q000150:complications* / Q000188:drug therapy / Q000473:pathology
chemical_list: D000903:Antibiotics, Antineoplastic; D046912:Multiprotein Complexes; D011506:Proteins; D000076222:Mechanistic Target of Rapamycin Complex 1; D058570:TOR Serine-Threonine Kinases; D020123:Sirolimus