Archivos argentinos de pediatria · 2009
[Renal manifestations of tuberous sclerosis in children].
Аннотация на английскомСтатьяНазвание и аннотация: Перевод в очереди
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Di Pinto Diana, Balestracci Alejandro, Delgado Norma
Аннотация
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.
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.