Observation of hypertension in children with 21-hydroxylase deficiency: a preliminary report.

The most common cause of congenital adrenal hyperplasia is 21-hydroxylase deficiency (21OHD). The prevalence of hypertension (HTN) in children with 21OHD is unknown, and HTN has not been reported to be a component of this disorder. As children with 21OHD are at risk of developing obesity, we hypothesized that an elevated body mass index (BMI) would be a predictive factor in the development of HTN. A retrospective chart review of children with 21OHD seen in our pediatric endocrine clinics for the past 21 yr was performed. Ninety-one children with 21OHD were identified (54% female). Of these children, six (6.6%) had HTN, and five (5.6%) had essential HTN, which was defined as HTN of unknown etiology. Elevated body mass index was not a determining factor in the development of HTN. Children with 21OHD do appear to have a higher prevalence of HTN when compared to historical reports of pediatric populations. The coexistence of HTN with a salt-wasting state and mineralocorticoid deficiency in some children with 21OHD is paradoxical and of unclear etiology.