What is the role of pharmacologic therapy for familial hyperaldosteronism type I (FH-I)?

Updated: Oct 19, 2018
  • Author: George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London); Chief Editor: Robert P Hoffman, MD  more...
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Answer

In adult patients with familial hyperaldosteronism (FH) type 1 (FH-I), or glucocorticoid-remediable aldosteronism (GRA), control of hypertension can be achieved through treatment with physiologic doses of dexamethasone. In general, the lowest dose of glucocorticoid that normalizes the BP should be used (for example, 0.125–0.5 mg of dexamethasone or 2.5–5 mg of prednisolone per day), to avoid the risk the of Cushingoid side-effects. In children, however, dexamethasone is best avoided because of its adverse effects on growth and bone density. Hydrocortisone has a short half-life (a typical dose is 10-12 mg/m2) and is a better choice but is not as efficient at reducing mineralocorticoid levels. Amiloride may be a preferred option because it avoids the potential problems of growth retardation associated with the use of glucocorticoids and potential adverse effects resulting from blockade of sex steroid receptors by spironolactone.

For children receiving long-term treatment with glucocorticoids, consultation with a pediatric endocrinologist is mandatory. GRA is associated with intracranial aneurysm and hemorrhagic stroke, and screening for intracranial aneurysms in patients with proven GRA is recommended. Amiloride and spironolactone have also been used as monotherapy for treating GRA.


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