Liddle, syndrome

[MIM 177 200]

(pseudohypoaldosteronism type 1)

Rare. Autosomal dominant transmission with variable penetrance. Inherited dysfunction of the sodium channels of the epithelium (ENac) at the level of the distal nephron due to a mutation in the C-terminal part of subunits , ß, and γ encoded by SCNN1B and SCNN1G genes on 16p13-p12. This causes excessive reabsorption of water and Na. 

Clinical picture: hypertension with hypokalemia and metabolic acidosis. Frequently asymptomatic.

Treatment: triamterene or amiloride


Anesthetic implications: 

check blood electrolytes; hypertension and vascular consequences with regard to heart, brain and kidney.


References : 

-         Hayes NE, Aslani A, McCaul CL. 
Anaesthetic management of a patient with Liddle’s syndrome for emergency caesarean hysterectomy. 
Int J Obstetr Anesth 2011; 20: 178-80.


Updated: January 2019