Treatment of Hypertension in a Child With 11Beta-Hydroxylase Deficiency: A Case Report

Background: 11beta-hydroxylase deficiency (11βohd) is clinically presented with external genitaliavirilization in girls and precocious puberty in boys. low renin hypertension occurs in both sexes. earlydiagnosis and treatment of hypertension can prevent complications.objectives: to provide a practical management for controlling hypertension in patients with 11-betahydroxylasedeficiency.methods: we described a 4.5 years old girl of 46.xx, who presented with ambiguous genitaliaat birth and hypertension later in follow-up. the patient had received the appropriate dosage ofhydrocortisone and the level of 17-hydroxy progesterone was within the acceptable range but thehypokalemia persisted. both hypertension and hypokalemia were normalized when spironolactonewas added.results: blocking mineralocorticoid receptor treat hypertension in 11-beta-hydroxylase deficiency.conclusions: intermittent measurement of blood pressure is necessary for patients with 11βohd. inthese patients, spironolactone is effective in treating mineralocorticoid-mediated hypertension andhypokalemia by blocking mineralocorticoid receptors.