Glucocorticoid-remediable aldosteronism is associated with severe hypertension in early childhood.
OBJECTIVE
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 developed 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.
Dluhy
R G
RG
Division of Endocrinology and Hypertension, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA.
Anderson
B
B
Harlin
B
B
Ingelfinger
J
J
Lifton
R
R
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D006929:Hyperaldosteronism / Q000150:complications* / Q000188:drug therapy*; D006973:Hypertension / Q000150:complications* / Q000188:drug therapy; D007223:Infant; D008297:Male; D015995:Prevalence; D012189:Retrospective Studies; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires; D016896:Treatment Outcome
D005938:Glucocorticoids