LOW PERIPHERAL PLASMA-RENIN ACTIVITY AS A CRITICAL MARKER IN PEDIATRIC HYPERTENSION

In evaluating hypertensive children and adolescents, the etiological c onsiderations should include a set of inherited disorders that share v ery low plasma renin activity (PRA) as a common feature. In particular among these disorders, glucocorticoid remediable aldosteronism (GRA) appears to be emerging as an important etiology of hypertension in the pediatric population. We report the evaluation of a 9-year-old Caucas ian girl who presented with severe hypertension and a strong family hi story of early-onset hypertension. Her suppressed PRA, her family hist ory, and her failure to respond to conventional anti-hypertensive ther apy raised GRA as a potential etiology. The diagnosis was confirmed by an elevated ratio of urinary 18-oxotetrahydrocortisol to urinary tetr ahydro-aldosterone and genetic testing, which demonstrated the chimeri c gene duplication. The molecular pathogenesis of GRA and the clinical implications are reviewed.