Abstract

Objective: Congenital adrenal hyperplasia (CAH) is a group of inherited disorders of adrenal steroidogenesis that may predispose affected patients to hypertension through both disease- and treatment-related mechanisms. Although hypertension represents an important comorbidity in CAH, its prevalence and contributing factors in the pediatric population, particularly across different CAH subtypes, remain incompletely defined. The aim of this study was to evaluate the prevalence and determinants of hypertension in patients with CAH.

Material and Methods: This retrospective, single-center study included 143 children and adolescents aged 1–21 years with a confirmed diagnosis of CAH who were followed at a tertiary pediatric center. Blood pressure measurements obtained during routine outpatient visits were evaluated according to age-, gender-, and height-specific pediatric reference values. Patients were classified by CAH subtype, and demographic, clinical, and treatment-related characteristics were compared between patients with and without hypertension. Correlations between blood pressure levels and clinical parameters were analyzed.

Results: A total of 143 patients with CAH (female-to-male ratio, 1.38) were included, with a median follow-up duration of 111.33 months (IQR; 144.70). Hypertension was diagnosed in 15 patients (10.5%). The prevalence of hypertension differed significantly according to CAH subtype (p<0.001), with the highest rate observed in patients with 11β-hydroxylase deficiency (88.8%), followed by those with 21-hydroxylase deficiency (8.4%). No cases of hypertension were observed in other CAH subtypes. Patients with hypertension received significantly higher hydrocortisone doses compared with normotensive patients (p=0.012), while fludrocortisone dose did not differ between groups. Blood pressure levels showed strong correlations with age at last visit and moderate correlations with follow-up duration and body mass index. Hypertensive cardiomyopathy was detected in 40% of hypertensive patients and was more frequent in those with 11β-hydroxylase deficiency.

Conclusion: Hypertension is a relevant clinical finding in children with CAH and is closely associated with disease subtype, particularly 11β-hydroxylase deficiency. Long-term follow-up, regular blood pressure monitoring and careful evaluation of glucocorticoid exposure may be important for cardiovascular risk assessment in this population.

Keywords: Congenital adrenal hyperplasia, hypertension, 11-beta-hydroxylase deficiency, 21-hydroxylase deficiency, glucocorticoids

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How to cite

1.
İnözü M, Avcı B, Büyükyılmaz G, Uçaktürk SA, Aydın Z, Çaycı FŞ, et al. Hypertension in children with congenital adrenal hyperplasia: Prevalence and associated factors. Turk J Pediatr Dis. 2026;20(2):139-145. https://doi.org/10.12956/TJPD.2025.1296