Glucocorticoid-remediable aldosteronism is associated with severe hypertension in early childhood

Objectives: To review the childhood course of glucocorticoid-remediable aldosteronism (GRA) in order to provide management guidelines for hypertension in children. Methods: Records for 20 children with GRA (aged 1 month to 18 years; 16 with hypertension) were retrospectively reviewed. Results: Of the 16 children with GRA who del eloped hypertension, 50% had moderate-severe hypertension (blood pressure [BP] >99th centile for age and sex); 32% had mild hypertension (BP >95th and <99th centile), and 18% had borderline normal BP (BP >90th and <95th centile). Eight of 16 children with hypertension who received directed monotherapy (glucocorticoid suppression or aldosterone receptor/sodium epithelial channel antagonists) maintained BP below the 90th centile. Three additional subjects receiving a combination of directed therapies or a combination of directed therapies and nifedipine were unable to achieve BP control. At GRA diagnosis, 5 of 8 children were normokalemic, and plasma renin activity was suppressed in 5 of 5 subjects. Conclusions: Clinicians should have a high index of suspicion for GRA, especially in children with severe hypertension and a positive family history of early-onset hypertension and/or premature hemorrhagic stroke. Directed monotherapy is often successful in controlling BP in GRA.