title: [Renal manifestations in tuberous sclerosis].
abstract: BACKGROUND
  Tuberous sclerosis is a rare inherited disease which can involve several organs. Renal involvement is one of the most severe manifestations of the disease. We analysed in this study renal involvement of tuberous sclerosis.
  
  
  METHODS
  We studied retrospectively nine cases of tuberous sclerosis with renal involvement diagnosed between 1994 and 2005. The diagnosis was based on the presence of typical extra-renal manifestations and familial history of tuberous sclerosis.
  
  
  RESULTS
  There were eight females and one male. Their mean age was 40.8+/-16.4 years (range: 15-62). At first presentation, we noted hypertension in three cases, proteinuria in seven cases, hematuria in eight cases and renal failure in five cases (end stage renal failure in four cases). By ultrasonography, we found angiomyolipoma in one case, cysts in four cases and angiomyolipoma associated to cysts in four cases. Nephrectomy was performed in two patients, in one case for hydronephrosis secondary to urolithiasis and in the other for hemorrhagic complication. In the later, pathological examination revealed renal carcinoma. At last evaluation, two patients were lost to follow-up, one had normal serum creatinine, five were on renal replacement therapy and the latter died from sepsis following kidney removal.
  
  
  CONCLUSIONS
  In our patients, renal disease was diagnosed late at chronic renal failure stage. Patients with end-stage renal failure require dialysis and renal transplantation, but we recommend binephrectomy after starting dialysis and before transplantation due to the risk of cancer and bleeding related to angiomyolipomas.
authors.affiliation: Service de médecine interne A (M8), hôpital Charles-Nicolle, boulevard 9-avril, BS 1006 Tunis, Tunisie. fethi.hamida@rns.tn
authors.forename: F
authors.initials: F
authors.lastname: Ben Hamida
authors.forename: I
authors.initials: I
authors.lastname: Gorsane
authors.forename: C
authors.initials: C
authors.lastname: Gharbi
authors.forename: H
authors.initials: H
authors.lastname: Kaaroud
authors.forename: S
authors.initials: S
authors.lastname: Barbouch
authors.forename: W
authors.initials: W
authors.lastname: Smaoui
authors.forename: I
authors.initials: I
authors.lastname: Helal
authors.forename: S
authors.initials: S
authors.lastname: Beji
authors.forename: R
authors.initials: R
authors.lastname: Goucha
authors.forename: H
authors.initials: H
authors.lastname: Hedri
authors.forename: F
authors.initials: F
authors.lastname: Ben Moussa
authors.forename: T
authors.initials: T
authors.lastname: Ben Abdallah
authors.forename: H
authors.initials: H
authors.lastname: Ben Maiz
authors.forename: A
authors.initials: A
authors.lastname: Kheder
mesh_terms: D000293:Adolescent; D000328:Adult; D018207:Angiomyolipoma / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006417:Hematuria / Q000209:etiology; D006801:Humans; D052177:Kidney Diseases, Cystic / Q000209:etiology; D007676:Kidney Failure, Chronic / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D007680:Kidney Neoplasms / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009392:Nephrectomy; D011507:Proteinuria / Q000209:etiology; D012189:Retrospective Studies; D014402:Tuberous Sclerosis / Q000150:complications* / Q000175:diagnosis / Q000601:surgery