End-stage renal failure in adults with the tuberous sclerosis complex

Abstract

Background. Tuberous sclerosis (TSC) is a multisystem disorder encompassing a wide spectrum of pathological renal lesions. Renal involvement is commonly asymptomatic but can result in significant morbidity, and renal failure has been reported. The risk of renal failure in patients with TSC in the UK has not been defined. Methods. A survey of the 170 members of the European Dialysis and Transplant Association was carried out to identify and collect clinical information on patients with TSC in their renal replacement programme. Results. Ten patients were identified as having end-stage renal failure (ESRF) and TSC, giving an incidence of 1% in patients with TSC and normal intellect. There was a wide spectrum of underlying pathology and clinical presentation within this group. Conclusion. ESRF is rare in TSC but does contribute to significant morbidity and mortality in this group.