Abstract.
In evaluating hypertensive children and adolescents, the etiological considerations should include a set of inherited disorders that share very 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 Caucasian girl who presented with severe hypertension and a strong family history of early-onset hypertension. Her suppressed PRA, her family history, and her failure to respond to conventional antihypertensive therapy raised GRA as a potential etiology. The diagnosis was confirmed by an elevated ratio of urinary 18-oxotetrahydrocortisol to urinary tetrahydroaldosterone and genetic testing, which demonstrated the chimeric gene duplication. The molecular pathogenesis of GRA and the clinical implications are reviewed.
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Received May 15, 1996; received in revised form and accepted September 16, 1996
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Yiu, V., Dluhy, R., Lifton, R. et al. Low peripheral plasma renin activity as a critical marker in pediatric hypertension. Pediatr Nephrol 11, 343–346 (1997). https://doi.org/10.1007/s004670050292
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DOI: https://doi.org/10.1007/s004670050292