SciELO - Scientific Electronic Library Online

 
vol.107 issue5Comparing the clinical-functional state in children with cystic fibrosis detected by neonatal screening or by clinical symptomsJuvenil idiopathic arthritis: Part 1: Diagnosis, pathogenesis and clinical manifestations author indexsubject indexarticles search
Home Pagealphabetic serial listing  

Services on Demand

Journal

Article

Indicators

  • Have no cited articlesCited by SciELO

Related links

  • Have no similar articlesSimilars in SciELO

Share


Archivos argentinos de pediatría

Print version ISSN 0325-0075On-line version ISSN 1668-3501

Abstract

DI PINTO, Diana; BALESTRACCI, Alejandro  and  DELGADO, Norma. Renal manifestations of tuberous sclerosis in children. Arch. argent. pediatr. [online]. 2009, vol.107, n.5, pp.436-440. ISSN 0325-0075.

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.

Keywords : Tuberous sclerosis; Renal manifestations.

        · abstract in Spanish     · text in Spanish     · Spanish ( pdf )

 

Creative Commons License All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License