BARTTERS-SYNDROME - OBSERVATIONS ON THE PATHO-PHYSIOLOGY

  • 1 January 1982
    • journal article
    • research article
    • Vol. 51  (203) , 251-270
Abstract
The pathophysiology of Bartter''s syndrome affecting 7 adults was investigated. Saralasin infusion caused a fall in blood pressure in all patients, suggesting that angiotensin was contributing to the maintenance of blood pressure. Following a water load, urinary Cl- concentrations and osmolality were both low. No positive evidence for a defect in Cl- reabsorption in the ascending limb of the loop of Henle was obtained. The effect of high and low dietary Na on plasma Na, K, Cl-, Mg, renin activity, aldosterone, 6-keto-PG[prostaglandin]F1a, thromboxane B2, urinary kallikrein, platelet function and erythrocyte membrane cation transport were studied. A variety of responses was observed. Na restriction increased (or Na loading decreased) plasma renin activity, aldosterone, 6-keto-PGF1a, urinary kallikrein and the platelet aggregation abnormality in some but not all individuals. Treatment with indomethacin was undertaken in all patients and studied in detail in 1 patient. There was weight gain, increase in plasma Na and K, decrease in capillary pH, positive Na and K balance, and decrease in plasma renin activity, 6-keto-PGF1a, thromboxane B2 and urinary kallikrein. Hypomagnesemia and excessive urinary Mg loss persisted unchanged. A variety of abnormalities of erythrocyte membrane cation transport was found and these persisted during high- and low-Na and high-K intakes, and during treatment with indomethacin, despite correction of intracellular Na and K concentrations. Bartter''s syndrome is associated with an abnormality of erythrocyte membane Na and K transport. Many of the other metabolic abnormalities may be the consequence of K and Na depletion.