A population study of renal disease in patients with tuberous sclerosis

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Abstract
Objective To establish prevalence of renal disease, asympotoamtic renal lesions and possible renal symptoms in geographically defined population of individuals with tuberous sclerosis. Patients and methods The study involved 131 patients (64 men, 67 women) with tuberous sclerosis who were resident in nine of the districts within the Wessex Region and three Bristol Health Districts and who had been identified by a prevalence study [1]. The patients' been identified by a prevalence study [1]. The patients' mean age was 22 years (range 6 months-74 years). Established renal disease was identified by history. Where possible individuals were seen were examined. Specific enquiry was made for flank pain and macroscopic haematuria. Renal ultrasound, blood pressure measurement and urine analysis was offered to all individuals with tuberous sclerosis who were resident in the Bath Health District. Results Eight patients (6%) had a history of either renal polycystic kidney disease (two patients) or haemorrhage from renal angiomyolipomas (five female patients, one male patient). A further 21% of female and 3% of male patients had a history of severe flank pain or haematuria. Renal ultrasound screening revealed abnormalities in 10 of 21 individuals; angiomyolipoma > 1 cm were found in seven and were twice as common in female patients. Conclusions Regular clinical review of individuals with tuberous sclerosis should include enquiry for renal symptoms and abdominal examination. Lesions >4cm are most likely to be symptomatic but sound screening of adolescents or adults can be recommended.

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