[Renal manifestations of tuberous sclerosis in children].

INTRODUCTION

Tuberous sclerosis is a neurocutaneous disorder characterized by the growth of hamartomas in multiple organs. Renal involvement is frequent and is the second cause of morbidity and mortality in these patients.

OBJECTIVES

To describe de prevalence, type and long-term outcome of renal involvement in children with tuberous sclerosis.

PATIENTS AND METHODS

We retrospectively reviewed clinical notes and renal ultrasounds of 38 children followed between 1989 and 2008 who met diagnostic criteria for tuberous sclerosis.

RESULTS

Median patient age was 10 years and median follow-up was 6.7 years; 44.7% (17 patients, 11 females) showed renal manifestations diagnosed by ultrasound screening in 13, in 2 by palpable mass and in another 2 by hypertension. On initial evaluation, renal ultrasound was abnormal in 7 children (18.4%); during followup another 10 patients (26.3%) developed renal lesions. Renal involvement included: multiple angiomyolipomas in 18.4%, cysts in 18.4% and angiomyolipomas plus cysts in 7.9%. Renal cysts were detected at a median age of 3.1 years and angiomyolipomas at 10.7 years. Two patients have required invasive procedures due to symptomatic large cysts, 5 developed proteinuria and 3 arterial hypertension. None presented renal carcinoma neither evolved to chronic renal failure. CONCLUSIONS. Our findings confirm a high rate of renal involvement in children with tuberous sclerosis, thus we emphasize periodic renal surveillance of these patients in order to diminish the morbidity of this disease.