Bartter's syndrome: a disorder characterized by high urinary prostaglandins and a dependence of hyperreninemia on prostaglandin synthesis.
Urinary prostaglandins E2 and F2alpha were measured by gas chromatography-mass spectrometry in three adult women and an adolescent girl with Bartter's syndrome. On a constant metabolic diet prostaglandin E2 ranged from 293 to 1,221 ng/day (mean, 640 ng/day) and exceeded the normal range for adults of 76 to 281 ng/day in all patients. Prostaglandins F2alpha ranged from 291 to 1,061 ng/day (mean, 747 ng/day) in the adult women. Only in a young girl did prostaglandins F2alpha (1,677 ng/day) clearly exceed the normal range for adults of 422 to 871 ng/day. Treatment with indomethacin, which decreased urinary prostaglandin E-like material by 69 per cent or more, did not affect blood pressure. Plasma renin activity, which ranged from 5.2 to 22.2 ng/ml/hour (patients supine) and from 23.3 to 30.4 ng/ml/hour (patients upright), and urinary aldosterone, which ranged from 14.0 to 45.6 ng/day, decreased by 79, 65 and 52 per cent, respectively. The clearance of creatinine was lower for the eight or nine days of treatment, the balances of sodium and potassium were positive, and serum potassium was higher than in control. Ibuprofen, an inhibitor of prostaglandin synthetase which differs in structure from indomethacin, produced metabolic effects which were qualitatively similar to those of indomethacin. The results indicate that the renal synthesis of prostaglandins is increased in Bartter's syndrome and that prostaglandins mediate the hyperreninemia and hyperaldosteronism which characterize the disorder. The over-production of prostaglandins by the kidney could be proximal cause of the syndrome, or secondary to intrarenal changes of an unknown nature. This study provides additional evidence for an important role for prostaglandins in the release of renin.
D000293:Adolescent; D000328:Adult; D000450:Aldosterone / Q000652:urine; D001477:Bartter Syndrome / Q000188:drug therapy / Q000378:metabolism*; D001794:Blood Pressure / Q000187:drug effects; D005260:Female; D006801:Humans; D006929:Hyperaldosteronism / Q000378:metabolism*; D007052:Ibuprofen / Q000627:therapeutic use; D007213:Indomethacin / Q000627:therapeutic use; D007668:Kidney / Q000378:metabolism; D008875:Middle Aged; D011188:Potassium / Q000378:metabolism; D011451:Prostaglandin-Endoperoxide Synthases / Q000378:metabolism; D011453:Prostaglandins / Q000652:urine*; D011458:Prostaglandins E / Q000096:biosynthesis / Q000652:urine; D011460:Prostaglandins F / Q000096:biosynthesis / Q000652:urine; D012083:Renin / Q000097:blood*; D012964:Sodium / Q000378:metabolism
D011453:Prostaglandins; D011458:Prostaglandins E; D011460:Prostaglandins F; D000450:Aldosterone; D012964:Sodium; D011451:Prostaglandin-Endoperoxide Synthases; D012083:Renin; D011188:Potassium; D007052:Ibuprofen; D007213:Indomethacin