Renal manifestations of tuberous sclerosis among children: An Indian experience and review of the literature

Objective: The objective of this study was to describe the renal manifestations in children 0-18 years of age diagnosed with tuberous sclerosis complex (TSC) at a tertiary hospital in South India.

Methods: Data of children with TSC, who presented to Christian Medical College Vellore Hospital from January 2008 to January 2013, were analysed by a retrospective chart review. The cases were identified from outpatient records and underwent ultrasonography, urine analysis and examination of serum creatinine to recognize renal involvement.

Results: Twenty-five children with TSC were identified. Two children did not have imaging studies available and were excluded from the analysis. The age of the included children ranged from 5 days to 15 years with a median age of 8 years. Seventy-four per cent (17/23) were males. Ten of the 23 children had evidence of renal involvement (43.5%). Of the 10 children with renal involvement, 6 had angiomyolipoma (60%), 5 had renal cysts (50%) and 1 had suspected renal cell carcinoma. In two children both angiomyolipoma (AML) and cysts were noted. One child was found to have proteinuria. The rest of the children had no evidence of proteinuria and had normal creatinine clearance.

Conclusion: We conclude that all children with TSC should be screened for renal involvement and regular follow-up should be arranged.