MO1036: 24-H Central Blood Pressure is Related to Renal Lesions in Children with Tuberous Sclerosis Complex

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Abstract BACKGROUND AND AIMS Adult data show that in people with tuberous sclerosis complex (TSC), blood pressure is related to the extent of renal involvement. Central blood pressure and 24-h ambulatory blood pressure are stronger predictors of a cardiovascular burden compared to office peripheral blood pressure. The study aimed to analyse 24-h central blood pressure and its relation with renal involvement in paediatric patients with TSC. METHOD In a group of 32 children with TSC (9.6 ± 4.4 years, 16 boys, 16 girls), we evaluated peripheral and central 24-h ambulatory blood pressure, office peripheral blood pressure, medications used, renal lesions in magnetic resonance and ultrasonography, including the presence of angiomyolipoma (AML), atypical AML, cysts and their maximal diameter (mm) and laboratory parameters. RESULTS In the studied group, 20 (62.5%) children had renal angiomyolipomas, including 5 (15.6%) with atypical angiomyolipomas, 22 (68.8%) with renal cysts and 4 children (12.5%) were treated with mTOR inhibitors (2 patients with everolimus and 2 patients with sirolimus) at the moment of evaluation, glomerular filtration rate (GFR) was 127.4 ± 36.7, one patient had GFR 80.5, 22 patients had GFR between 90 and 139 and 9 patients had hyperfiltration defined as GFR > 140 mL/min/1.73 m2. A total of 4 (12.5%) children had elevated blood pressure in ABPM. The 24-h central systolic and diastolic blood pressure correlated with maximal diameter of renal cysts (mm) (r = 0.359, P = .044; r = 0.466, P = .029) and urinary albumin loss (mg/24-h) (r = 0.511, P = .004; r = 0.478, P = .021), 24-h central systolic, mean blood pressure and pulse pressure with serum cystatin-C (ng/mL) (r = 0.523, P = .005; r = 0.464, P = .015; r = 0.419, P = .029); 24-h peripheral MAP Z-score correlated with cyst size (mm) (r = 0.571, P = .021). No significant correlations between presence and AML size and peripheral and central blood pressure were found in the study group. There were following positive correlations of arterial stiffness parameters: 24-h central augmentation index (24hcAI x 75HR) with total cholesterol (r = 0.357, P = .045) and ambulatory arterial stiffness index (AASI) with uric acid (r = 0.520, P = .003). Size of AML correlated with age (r = 0.468, P = .007), whereas there was no correlation between cyst size and age (r = 0.145, P = .416). CONCLUSION