SAT-469 - Early Oxidative and Inflammatory Activation Is Associated with Atherogenic Risk and Hypertension in Children with 11Β-Hydroxylase Deficiency

Background
Children with 11β-hydroxylase deficiency (11β-OHD) are exposed to chronic excess of steroid precursors and adrenal androgens, which may predispose them to cardiometabolic and vascular complications. However, oxidative stress and inflammatory pathways have not been systematically investigated in this population.
Methods
Nine children with genetically 11β-OHD and 20 age- and sex-matched healthy controls were evaluated. Oxidative stress and inflammatory biomarkers, including malondialdehyde (MDA), reactive oxygen species (ROS), neopterin, and total antioxidant capacity (FRAP), were measured. Atherogenic lipid indices (TG/HDL ratio and Castelli index1) were calculated. Nailfold capillaroscopy and 24-hour ambulatory blood pressure monitoring were performed.
Results
Patients (M/F: 3/6) and controls (M/F: 9/11) were comparable in age and BMI SDS. Within the patient group, three children had normal BMI and six were obese. No significant differences were observed in oxidative stress markers or atherogenic lipid indices between normal-weight and obese patients. Compared with controls, patients exhibited significantly higher levels of neopterin (p = 0.009), ROS (p= 0.021), and MDA (p < 0.001), along with altered total antioxidant capacity assessed by FRAP (p = 0.040), indicating an imbalance between oxidative and antioxidative pathways. TG/HDL ratio and Castelli index1 showed non-significant trends toward higher values in patients. Correlation analysis revealed significant positive associations between MDA levels and TG/HDL ratio (ρ = 0.49, p = 0.007) as well as Castelli index1 (ρ = 0.40, p = 0.032). MDA was strongly correlated with neopterin (ρ = 0.57, p = 0.001). ROS levels were positively correlated with FRAP (ρ = 0.38, p = 0.041), consistent with a compensatory antioxidant response. Hypertension was common among patients, and oxidative stress markers particularly MDA and neopterin were independently associated with hypertensive status. No significant associations were observed between nailfold capillaroscopy findings and either biomarker levels or hypertension.
Conclusion
Children with 11β-OHD exhibit increased oxidative stress and inflammatory activation associated with atherogenic lipid indices and hypertension, even in clinically well-controlled patients. Despite the small cohort inherent to this rare condition, these findings support oxidative stress and inflammation as early drivers of hypertension and vascular dysfunction in children with 11β-OHD. Importantly, standard clinical and biochemical control may not fully reflect early vascular risk, highlighting a critical early-life window for targeted cardiometabolic surveillance. Oxidative and inflammatory biomarkers may represent early indicators of vascular risk before overt microvascular structural changes become detectable.

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