AME, syndrome

[MIM 218 030]

(deficiency of 11-β-hydroxysteroid dehydrogenase, apparent mineralocorticoid excess, Ulick syndrome)

Acronym in English of Apparent Mineralocorticoid Excess.


Extremely rare. Autosomal dominant transmission of a mutation of the HSD11B2 gene (16q22.1).

The deficiency in 11-β-hydroxysteroid dehydrogenase (or cortisol 11-β-cetoreductase type II) decreases the peripheral degradation of cortisol in cortisone (inactive), thus allowing the excess cortisol to bind to  mineralocorticoid receptors. The clinical presentation is similar to that of an excess in aldosterone but with low aldosterone blood levels:


-         hypertension with low renin

-         metabolic alkalosis

-         hypokalemia and hypernatremia.


The clinical presentation depends on the residual activity of 11-β- hydroxysteroid dehydrogenase: the cases with  hypercalciuria and nephrocalcinosis could be due to a total lack of activity. It is possible that the cases of hypertension with low renin levels are minor forms of this disease.

There is in general a good therapeutic response to spironolactone whether or not associated with amiloride. In some cases, it is necessary to administer exogenous corticosteroids to reduce endogenous steroid production.


Anesthetic implications:

arterial hypertension and its possible complications: renal function, LVH. check blood electrolytes


References : 

-        Morineau G, Sulmont V, Salomon R, Fiquet-Kempf B, Jeunemaître X, Nicod J, Ferrari P. 
Apparent mineralocorticoid excess : report of six cases and extensive personal experience. 
J Am Soc Nephrol 2006 ; 17 : 3176-84.

-        Parvez Y, El sayed O. 
Apparent mineralocorticoid excess (AME) syndrome. 
Indian Pediatr 2013 ; 50 : 416-8


Updated: November 2019