A 87-year-old woman with mineralocorticoid excess due to 11ß-HSD2 deficiency

Abstract

A 87-year-old woman presented with hypretension and motor disturbance in upper and lower extremities due to severe muscle weakness. As she had a history of licorice administration, laboratory data was obtained approximately 3 months after the drug cessation, She showed hypokalemia (2.7mEq/l), metabolic alkalosis and reduced plasma renin activity (PRA). Despite the mineralocorticoid excess, plasma aldosterone concentration (PAC) and 24-hour urine aldosterone were markedly diminished (32pg/ml and 1.1μg/day, respectively). Thyroid function was normal, and plasma ACTH and serum cortisol levels were within normal limits. Serum potassium levels was elevated (3.9mEq/l) and blood pressure returned to normal by Cortisol suppression with dexamethasone, 1.5mg per day. Moreover, administration of spironolactone, 50-75mg per day, caused additional elevation of serum potassium level (4.7mEq/l) with clinical improvement. These results revealed that the mineralocorticoid excess, found in the present patient, was responsible to dexamesathone and spironolactone, suggesting 1β-HSD2 deficiency. Serum cortisol/cortisone ratio (0.95) was also elevated, as compared with age-matched female control (0.28-0.72). The active component of licorice, glycyrrhetinic acid, has a mineralocorticoid-like side effect. However, in the present patient, diminution in serum potassium level and PAC were still found approximately 1 year after stopping licorice. Recently, elderly patients with 11β-HSD2 deficiency are often reported, therefore further investigations in relation to the changes caused by aging are needed to elucidate this abnormality.