title: Urological counseling and followup in pediatric tuberous sclerosis complex.
abstract: OBJECTIVE
  We review our experience with renal manifestations in pediatric patients with the tuberous sclerosis complex, and offer recommendations for urological counseling, followup and treatment of these patients.
  
  
  METHODS
  We reviewed clinical notes on 41 patients with the tuberous sclerosis complex followed at our institution from childhood. Patient data were gathered in a database focusing on renal involvement. The latter was assessed by periodic clinical evaluations and ultrasound. The risk of renal involvement was evaluated in relation to patient age, genotypic pattern and number of extrarenal manifestations.
  
  
  RESULTS
  Overall, 15 patients (36.6%) had renal involvement. The latter increased with age and was more common in cases with TSC2 genotypic pattern or multiple extrarenal manifestations. Angiomyolipomas were the most common lesions (11 patients), followed by renal cysts (2) and polycystic kidney disease (2). Cystic lesions were the most common in patients younger than 16 years. Renal failure developed in the 2 patients with polycystic kidney disease by the 2nd decade of life. Overall, treatment was required in 2 cases of symptomatic angiomyolipoma. Both patients were female, and had multiple extrarenal manifestations and bilateral renal involvement. One patient underwent open surgery at age 21.3 years and 1 underwent radiological embolization at age 23.4 years.
  
  
  CONCLUSIONS
  Pediatric patients with the tuberous sclerosis complex should undergo urological evaluation and followup. Although most of the lesions remain silent during childhood, the incidence of renal involvement increases with age. The need for treatment is highest in females with multiple extrarenal manifestations and bilateral renal involvement.
authors.affiliation: Section of Pediatric Urology, Department of Urology, University Hospital of Padua, Padua, Italy. marcocastagnetti@hotmail.com
authors.forename: Marco
authors.initials: M
authors.lastname: Castagnetti
authors.forename: Beatrice
authors.initials: B
authors.lastname: Vezzù
authors.forename: AnnaMaria
authors.initials: A
authors.lastname: Laverda
authors.forename: Sandra
authors.initials: S
authors.lastname: Zampieri
authors.forename: Waifro
authors.initials: W
authors.lastname: Rigamonti
mesh_terms: D017677:Age Distribution; D002648:Child; D002675:Child, Preschool; D003376:Counseling / Q000379:methods*; D019468:Disease Management; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D007558:Italy / Q000453:epidemiology; D007674:Kidney Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*; D008279:Magnetic Resonance Imaging; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D017678:Sex Distribution; D013997:Time Factors; D014402:Tuberous Sclerosis / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*; D014567:Urography