Abnormalities of Erythrocyte Sodium Transport Systems in Bartter’s Syndrome

Abstract

The basic tubular alteration present in Bartter’s syndrome is still a subject of controversy. The possibility that a generalized defect in the transmembrane ion transport underlies the disease has been extensively investigated. Previous evaluations of cellular sodium metabolism in Bartter patients showed extremely variable findings. We have examined in the red blood cells of two patients with Bartter’s syndrome the intracellular Na⁺ and K⁺ concentrations, the activity of the ouabain-sensitive Na⁺/K⁺ pump, furosemide-sensitive Na⁺/K⁺ cotransport, Na⁺/Li⁺ countertransport and the rate constant of Na⁺ and K⁺ passive permeability. We have compared these values with those of healthy subjects and patients with chronic hypokalemia produced by conditions other than Bartter’s syndrome. Ouabain-sensitive Na⁺/K⁺ pump activity was decreased in both patients, whereas Na⁺/Li⁺ countertransport was activated. One of the patients also exhibited markedly decreased intraerythrocyte K⁺ concentration and decreased furosemide-sensitive Na⁺/K⁺ cotransport. The other had increased Na⁺/K⁺ cotransport activity and Na⁺ passive permeability. Intracellular Na⁺ and passive permeability to K⁺ were normal in both subjects. Neither oral potassium supplementation (100 mEq/day) nor indo-methacin treatment (150 mg/day) could correct these abnormalities. Our results are partially consistent with previous observations and indicate the existence of heterogenous abnormalities of erythrocyte sodium transport systems in patients with Bartter’s syndrome which are not a consequence of chronic hypokalemia.

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