Adolescents with congenital adrenal hyperplasia due to 21-hydroxylase deficiency have vascular dysfunction

Abstract

Context: Patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency have multiple vascular risk factors. Young adults with CAH have increased intima media thickness, but there have been no studies of vascular function and structure in children CAH. Objective: To establish whether children with CAH have reduced vascular function and increased carotid intima media thickness (cIMT) when compared to healthy and obese children. Design and Patients: Cross-sectional study of 14 patients (14.8 years ± 3.2, 7 males) with CAH secondary to 21-hydroxylase deficiency compared to 28 obese and 53 healthy controls. Measurements: All subjects had assessment of endothelial function (flow mediated dilatation, FMD), smooth muscle function (glyceryl tri-nitrate dilatation, GTN) and cIMT. Anthropometric data, resting blood pressure and biochemical variables were also measured. Results: CAH subjects had significantly reduced FMD (4.5 ± 3.0% vs 7.5 ± 5.2%; p=0.04) and GTN (17.2 ± 1.6% vs 28.4 ± 8.4%; p<0.001) when compared to controls and the impairment was comparable to the obese cohort. There was no significant difference in cIMT between groups. CAH subjects had increased insulin resistance (HOMA-IR 2.5 [0.2 to 2.9] vs 1.8 [0.5 to 4.2]; p=0.04), waist-to-height ratio (0.47 ± 0.05 vs 0.44 ± 0.04; p=0.02) and higher systolic blood pressure Z score (0.29 ± 0.9 vs -0.24 ± 0.64, p=0.01) compared to healthy controls but not when compared to obese controls. Conclusions: Subjects with CAH have evidence of vascular dysfunction by adolescence.