! Not to be confused with Gordon syndrome (1) 

[MIM 145 260, 614 491, 614 492, 614 495, 614 496]

(Familial hyperkaliemic high BP, pseudo-aldosteronism type 2, PHA2, Spitzer-Weinstein syndrome, chloride shunting syndrome)

High B.P.. Autosomal dominant or recessive transmission. Four genes have been identified. A mutation of those genes can cause this syndrome by increasing the functioning of the Na-CL cotransporter which is sensitive to the thiazides at the level of the distal nephron: WNK4 (17q11-q21), WNK1 (12p13.3), KLHL3 and CUL3 (coding for cullin 3). Mutations of CUL3 cause a more severe phenotype.

In addition to the systemic arterial hypertension, other porblems can occur:

-        hyperkalemia,

-        hyperchloremic metabolic acidosis

-        hypercalciuria

-        in the absence of any renal failure

Sometimes: dental anomalies (unique incisor, etc), growth retardation can be associated.

Low plasma levels of renin and aldosterone with a very good response to thiazides. Treatment: reduce sodium intake and add thiazides.

Anesthetic implications: 

check TA and electrolytes, risk of hyperkalemia. avoid administering NaCl 0.9%

References : 

-        Power GE, Hellier C, Gordon RD.
Emergency anaesthesia in a patient with Gordon’s syndrome.
Anaesth Intensive Care 2004 ; 32 : 275-7.

-        Bruel A, Vargas-Poussou R, Jeunemaitre X, Labbe A, Merlin E, Bessenay L.
Syndrome de Gordon : de l’importance de mesurer la pression artérielle de l’enfant.
Arch Pédiatr 2016 ; 23 : 827-31

Updated: August 2016