Hypertension associated with congenital adrenal hyperplasia

Background: Congenital adrenal hyperplasia (CAH) is a group of autosomal recessive disorders resulting from the deficiency of one of the five enzymes required to synthesis cortisol, and hence, increased production of adrenocorticotrophin releasing hormone (ACTH) which leads to adrenal hyperplasia. In 11--hydroxylase and 17--hydroxylase deficiencies, the accumulation of the mineralocorticosteroids or 11-deoxy-cortisol can lead to hypertension. Design and settings: A retrospective, hospital based study, was conducted at King Khalid University Hospital (KKUH), Riyadh, Saudi Arabia, during the period January 1989 and December 2014. Methods: Medical records of children who were diagnosed to have congenital adrenal hyperplasia were retrospectively reviewed. Data included age, sex, clinical presentation and results of the relevant laboratory and radiological investigations. Results: During the period under review, 95 Saudi patients with CAH were diagnosed. Of these 76 (80%) were due to 21--hydroxylase deficiency, 15 (15.8%) patients with 11--hydroxylase deficiency, and 4 (4.2%) patients were due to 3--hydroxysteroid dehydrogenase deficiency. Six (40%) patients with 11--hydroxylase deficiency developed persistent hypertension during the course of follow-up. Conclusion: Hypertension is common occurrence in congenital adrenal hyperplasia due to 11--hydroxylase deficiency and should be considered in the differential diagnosis of hypertension.