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BackgroundRenal involvement is very common in tuberous sclerosis complex (TSC) and is characterised by the development of angiomyolipoma and cysts. The aims of the present study were to assess kidney function and clinical features of renal involvement in TSC, including kidney function, and blood pressure (BP) levels in children, adolescents and young adults. MethodsNon-selected patients with a de nite diagnosis of TSC attending the paediatric neurology outpatient department of a tertiary hospital were included in a cross-sectional study. All participants had a renal imaging study within 6 months of ambulatory blood pressure (BP) and glomerular ltration rate (GFR) assessment. Data on demographics, history, genotype, kidney function at diagnosis and last imaging were collected. ResultsThe median age of the patients was 15 years (IQR range 9 to18). 23.5% of the participants had ambulatory BP hypertension. Systolic BP levels correlated signi cantly with GFR DTPA values despite the absence of hyper ltration. Greater increase in GFR from initial TSC diagnosis till the age of the assessment, resulted in higher GFR levels in childhood and adolescence in those that developed hypertension and possibly in those with angiomyolipoma or cysts. All patients with ambulatory BP hypertension had angiomyolipomas or cysts on renal imaging studies. ConclusionsHypertension may present with increased frequency in young patients with kidney disease associated with TSC. Routine ambulatory BP measurement could be part of the annual clinical assessment in patients with TSC. What Is KnownNearly half of the patients with TSC have a premature decline in their renal function in their fth decade of life Hypertension and hyper ltration have been proposed as modi able factors of progression of renal decline

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Background Renal involvement is very common in tuberous sclerosis complex (TSC) and is characterised by the development of angiomyolipoma and cysts. The aims of the present study were to assess kidney function and clinical features of renal involvement in TSC, including kidney function, and blood pressure (BP) levels in children, adolescents and young adults. Methods Non-selected patients with a definite diagnosis of TSC attending the paediatric neurology outpatient department of a tertiary hospital were included in a cross-sectional study. All participants had a renal imaging study within 6 months of ambulatory blood pressure (BP) and glomerular filtration rate (GFR) assessment. Data on demographics, history, genotype, kidney function at diagnosis and last imaging were collected. Results The median age of the patients was 15 years (IQR range 9 to18). 23.5% of the participants had ambulatory BP hypertension. Systolic BP levels correlated significantly with GFRDTPA values despite the absence of hyperfiltration. Greater increase in GFR from initial TSC diagnosis till the age of the assessment, resulted in higher GFR levels in childhood and adolescence in those that developed hypertension and possibly in those with angiomyolipoma or cysts. All patients with ambulatory BP hypertension had angiomyolipomas or cysts on renal imaging studies. Conclusions Hypertension may present with increased frequency in young patients with kidney disease associated with TSC. Routine ambulatory BP measurement could be part of the annual clinical assessment in patients with TSC.