Metabolic and blood pressure responses to hydrocortisone in the syndrome of apparent mineralocorticoid excess.
A syndrome of low renin hypertension in childhood with apparent mineralocorticoid excess associated with a defect in the peripheral metabolism of cortisol has been described previously in 2 patients. In these patients, decreased secretion rates of glucocorticoids, mineralocorticoids, and sex steroids have been demonstrated. In a 10(10/12)-yr-old girl with this disorder, continuous iv administration of hydrocortisone in doses of 5, 10, 15, and 20 mg/day resulted in an increase in blood pressure and a decrease in serum potassium concentration. The addition of spironolactone during the continued administration of 20 mg/day hydrocortisone did not result in a decrease in blood pressure. Withdrawal of hydrocortisone and continued administration of spironolactone alone resulted in a decrease in blood pressure, a rise in serum potassium concentration, and a fall in serum sodium concentrations. These studies suggest that an abnormality in cortisol action or metabolism causing cortisol to behave as a potent mineralocorticoid may account for this syndrome of apparent mineralocorticoid excess.
D000324:Adrenocorticotropic Hormone; D000450:Aldosterone; D001794:Blood Pressure; D002648:Child; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000378:metabolism; D006914:Hydroxysteroids / Q000652:urine; D006973:Hypertension / Q000503:physiopathology*; D008797:Metyrapone; D008901:Mineralocorticoids / Q000378:metabolism*; D011188:Potassium / Q000097:blood; D012083:Renin / Q000097:blood; D013148:Spironolactone; D013577:Syndrome
D006914:Hydroxysteroids; D008901:Mineralocorticoids; D013148:Spironolactone; D000450:Aldosterone; D000324:Adrenocorticotropic Hormone; D012083:Renin; D011188:Potassium; D006854:Hydrocortisone; D008797:Metyrapone