Two uncommon causes of mineralocorticoid excess. Syndrome of apparent mineralocorticoid excess and glucocorticoid-remediable aldosteronism.
Glucocorticoid-remediable aldosteronism is an inherited form of mineralocorticoid excess associated with moderate overproduction of aldosterone, in which biochemical and clinical remission is dramatically induced by small amounts of glucocorticoids. The disorder is associated with characteristic overproduction of 18-hydroxycortisol and 18-oxocortisol, which must now be regarded as an essential diagnostic feature and also as an aid in the detection of the disorder in the face of partial clinical expression.
Ulick
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Department of Medicine, Mt. Sinai School of Medicine, University of New York, New York.
D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D006854:Hydrocortisone / Q000031:analogs & derivatives / Q000096:biosynthesis; D006929:Hyperaldosteronism / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology*; D008901:Mineralocorticoids / Q000378:metabolism*; D013577:Syndrome
D005938:Glucocorticoids; D008901:Mineralocorticoids; C038135:18-oxocortisol; C033689:18-hydroxycortisol; D006854:Hydrocortisone