title: Low peripheral plasma renin activity as a critical marker in pediatric hypertension.
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 anti-hypertensive 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.
authors.affiliation: Division of Nephrology, Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: V W
authors.initials: VW
authors.lastname: Yiu
authors.forename: R P
authors.initials: RP
authors.lastname: Dluhy
authors.forename: R P
authors.initials: RP
authors.lastname: Lifton
authors.forename: L M
authors.initials: LM
authors.lastname: Guay-Woodford
mesh_terms: D015415:Biomarkers; D002648:Child; D019405:Cytochrome P-450 CYP11B2 / Q000096:biosynthesis / Q000235:genetics; D005260:Female; D006801:Humans; D006929:Hyperaldosteronism / Q000097:blood / Q000150:complications / Q000235:genetics; D006973:Hypertension / Q000097:blood* / Q000209:etiology / Q000235:genetics; D005810:Multigene Family; D012083:Renin / Q000097:blood*
chemical_list: D015415:Biomarkers; D019405:Cytochrome P-450 CYP11B2; D012083:Renin