Bartter's syndrome. New insights into pathogenesis and treatment.
Discussed here is a patient with normotension, hypokalemic alkalosis, hyperreninemia, hyperaldosteronism, juxtaglomerular cell hyperplasia and insensitivity to the pressor effects of angiotensin (Bartter's syndrome). The hyperreninemia and hyperaldosteronism were both suppressible with volume expansion. Hypokalemia was correctible both short-term with potassium chloride infusions and long-term with spironolactone. Nevertheless, the abnormal pressor response to infused angiotensin could not be corrected by these maeuvers, suggesting that this defect is likely to be of primary pathophysiologic significance. We found that potassium loading markedly stimulated aldosterone excretion. This may explain the inadequacy of potassium supplementation alone to correct the hypokalemia and the observed "escape" from the potassium conserving effects of spironolactone seen in patients with Bartter's syndrome. The administration of propranolol in large doses only partially suppressed the marked hyperreniemia of our patient and failed to prevent a subsequent rise in the renin level which was associated with spironolactone therapy. In contrast, suppression of the renin level to normal was demonstrated by sodium loading. It is suggested that patients with Bartter's syndrome be treated simultaneously with large doses of spironolactone and a high sodium intake.
D000328:Adult; D000450:Aldosterone / Q000097:blood; D000804:Angiotensin II; D001477:Bartter Syndrome / Q000097:blood / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D001810:Blood Volume; D005260:Female; D006801:Humans; D006929:Hyperaldosteronism / Q000209:etiology*; D007668:Kidney / Q000503:physiopathology; D007671:Kidney Concentrating Ability; D011189:Potassium Chloride / Q000627:therapeutic use; D011311:Pressoreceptors / Q000187:drug effects; D011433:Propranolol / Q000627:therapeutic use; D012083:Renin / Q000097:blood; D012964:Sodium / Q000378:metabolism; D013148:Spironolactone / Q000627:therapeutic use
D000804:Angiotensin II; D013148:Spironolactone; D000450:Aldosterone; D011189:Potassium Chloride; D012964:Sodium; D011433:Propranolol; D012083:Renin