Glucocorticoid-responsive hypertension in early childhood followed up for five years: A case of 11-beta-hydroxylase deficiency (pp. 359-363)
Authors: Alphonsus N Onyiriuka and Nosakhare J Iduoriyekemwen
Abstract: Background: 11-beta-hydroxlase deficient congenital adrenal hyperplasia (CAH) is a rare cause of hypertension in infancy. Such hypertension is usually noted in later childhood and adolescence. Objective: To describe a case of an 18-month old girl with glucocorticoid-responsive hypertension followed up for five years. Methods: In this paper we described the case of a seven-year old Nigerian girl whose hypertension secondary to 11-beta-hydroxylase-deficient congenital adrenal hyperplasia was diagnosed at the age of 18 months and followed up for five years. Results: The hypertension responded to glucocorticoid therapy, reoccurred on default and resolved on re-starting glucocorticoid. The blood pressure has remained normal over a period of five-and-half years on maintenance prednisolone. Anthropometry at the age of seven years showed weight 24kg (75th percentile), height (50th percentile), body mass index (50 -75th percentile). Sexual maturity rating showed breast Tanner stage 3 and pubic hair stage 2. No further increase in size of the stump of the clitoris. Some of the management challenges encountered in the care of this patient included lack of laboratory facilities for diagnosis and monitoring of therapy, brief period of default, and irregular supply of oral hydrocortisone. Conclusion: Although 11-beta-hydroxylase deficient CAH is a rare cause of hypertension in early childhood, it should be considered in the differential diagnosis of hypertension with virilisation in any prepubercent female.