Gordon's syndrome: increased maximal rate of the Na-K-Cl cotransport and erythrocyte membrane replacement of sphingomyelin by phosphatidylethanolamine

Objective Gordon's syndrome comprises hypertension, hyperchloremic acidemia , hyperkalemia and intact renal function. We hypothesize that disturbances of one or more cell membrane ion carriers, handling sodium, chloride and po tassium, might be relevant in this disorder and, furthermore, that such dis turbances might be related to altered cell membrane composition. Design and methods: In a patient diagnosed with Gordon's syndrome, we asses sed the kinetics (K-m and maximal rate) of four membrane sodium transport s ystems in sodium-enriched erythrocytes, according to the technique of Garay . We also measured the lipid composition of erythrocyte membrane in this pa tient and 69 essential hypertensive controls, using the latroscan technique , Results: Compared to reference values of patients with essential hypertensi on, this patient exhibited a marked increase in the maximal rate of the Na-K+-2Cl(-) cotransport (964.0 mu mol/l per cell versus the 391.6 +/- 222 mu mol/l per cell in essential hypertensives). Also, there was an increased c oncentration of erythrocyte membrane phospatidylethanolamine and a reduced concentration of sphingomyelin (27.9 and 11.1% versus 17.9 +/- 3.8% and 18. 2 +/- 3.4%, respectively). Conclusions: We conclude that this abnormality in membrane Na+-K+-2Cl(-) co transport could be responsible for the hyperkalemia, hyperchloremic acidemi a and increased reabsorption of sodium observed in this condition and, furt hermore, that such disturbance in membrane cotransport might be related to altered phospholipid concentration in cell membranes, J Hypertens 2000, 18: 1327-1330 (C) Lippincott Williams & Wilkins.