Apparent Mineralocorticoid Excess Syndrome

A hereditary disease characterized by childhood onset HYPERTENSION, hypokalemic alkalosis, and low RENIN and ALDOSTERONE secretion. It results from a defect in the activity of the 11-BETA-HYDROXYSTEROID DEHYDROGENASE TYPE 2 enzyme which results in inadequate conversion of CORTISOL to CORTISONE. The build up of unprocessed cortisol to levels that stimulate MINERALOCORTICOID RECEPTORS creates the appearance of having excessive MINERALOCORTICOIDS.
Also Known As:
Mineralocorticoid Excess Syndrome, Apparent
Networked: 27 relevant articles (0 outcomes, 3 trials/studies)

Disease Context: Research Results

Related Diseases

1. Hyperaldosteronism (Conn Syndrome)
2. Liddle Syndrome
3. Pseudohypoaldosteronism
4. Hypertension (High Blood Pressure)
5. Hypokalemia


1. New, Maria I: 2 articles (01/2014 - 05/2004)
2. Warnock, D G: 2 articles (12/2001 - 06/2000)
3. Delitala, G: 2 articles (01/2001 - 07/2000)
4. Shackleton, C H: 2 articles (01/2001 - 07/2000)
5. Palermo, M: 2 articles (01/2001 - 07/2000)
6. Khattab, Ahmed M: 1 article (01/2014)
7. Shackleton, Cedric H L: 1 article (01/2014)
8. Bodalia, Jayesh B: 1 article (01/2014)
9. Hughes, Beverly A: 1 article (01/2014)
10. Nielsen, Mette Lundgren: 1 article (04/2012)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Apparent Mineralocorticoid Excess Syndrome:
1. Epithelial Sodium ChannelIBA
2. MineralocorticoidsIBA
3. 11-beta-Hydroxysteroid Dehydrogenase Type 2 (11 beta-Hydroxysteroid Dehydrogenase Type 2)IBA
4. CortisoneIBA
5. Hydrocortisone (Cortisol)FDA LinkGeneric
6. methylamphotericin B (AME)IBA
7. ReninIBA
05/01/2004 - "Apparent mineralocorticoid excess syndrome (AME) is an autosomal recessive disorder that results in low renin hypertension and other characteristic clinical features. "
06/01/2003 - "This mechanism is related either to a mutation of the gene, which encodes 11HSD2 (apparent mineralocorticoid excess syndrome and some cases of low renin hypertension) or to an acquired reduction of the activity of the enzyme due to glycyrrhetinic acid, carbenoxolone, and grapefruit juice. "
10/01/1991 - "Deficient or impaired 11 beta-hydroxy steroid dehydrogenase in the apparent mineralocorticoid excess syndrome or after licorice ingestion retards the conversion of cortisol to inactive cortisone in the kidney, leading to mineralocorticoid hypertension; this leads to suppression of the renin system and subsequently of aldosterone."
10/01/2011 - "Mineralcorticoid hypertension includes a spectrum of disorders ranging from renin-producing pathologies (renin-secreting tumors, malignant hypertension, coarctation of aorta), aldosterone-producing pathologies (primary aldosteronism - Conns syndrome, familial hyperaldosteronism 1, 2, and 3), non-aldosterone mineralocorticoid producing pathologies (apparent mineralocorticoid excess syndrome, Liddle syndrome, deoxycorticosterone-secreting tumors, ectopic adrenocorticotropic hormones (ACTH) syndrome, congenitalvadrenal hyperplasia), and drugs with mineraocorticoid activity (locorice, carbenoxole therapy) to glucocorticoid receptor resistance syndromes. "
8. 11-beta-Hydroxysteroid Dehydrogenases (11beta Hydroxysteroid Dehydrogenase)IBA
9. Glycyrrhizic Acid (Glycyrrhizin)IBA
10. TetrahydrocortisoneIBA

Therapies and Procedures

1. Kidney Transplantation