Low peripheral plasma renin activity as a critical marker in pediatric hypertension |
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| Authors: | Verna W Y Yiu Robert P Dluhy Richard P Lifton Lisa M Guay-Woodford |
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| Institution: | (1) Division of Nephrology, Children’s Hospital, Harvard Medical School, Boston, Massachusetts, USA, US;(2) Division of Endocrinology-Hypertension, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts, USA, US;(3) Howard Hughes Medical Institute, Departments of Medicine and Genetics, Yale University School of Medicine, New Haven, Connecticut, USA, US |
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| 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.
Received May 15, 1996; received in revised form and accepted September 16, 1996 |
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| Keywords: | : Hypertension Plasma renin activity Glucocorticoid remediable aldosteronism |
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