Liddle, syndrome
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(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