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Klinische Wochenschrift

, Volume 60, Issue 19, pp 1212–1214 | Cite as

The role of chloride transport in the thick ascending limb in the pathogenesis of Bartter's syndrome

  • J. R. GillJr.
Article

Summary

Fractional chloride reabsorption in the thick ascending limb of the loop of Henle, measured by clearance techniques, is subnormal in patients with Bartter's syndrome. This defect is a marker for the syndrome and, presumably, is the cause of the supranormal tubular secretion of potassium that characterizes the disorder. The potassium depletion that results from excessive potassium excretion is probably the stimulus for the increased synthesis of prostacyclin by blood vessels and prostaglandin E2 by kidneys that occurs in Bartter's syndrome. The overproduction of prostaglandins mediates hyperreninemia, supranormal plasma bradykinin, supranormal plasma norepinephrine and vascular resistance to the pressor effects of angiotensin II and norepinephrine; treatment with a prostaglandin synthetase inhibitor corrects these abnormalities. Increases in angiotensin II and in norepinephrine appear to be compensatory changes, occurring in response to vasodilatation induced by vascular prostaglandins to maintain blood pressure. The hyperreninemia also stimulates production of aldosterone with aggravation of potassium loss.

Key words

Bartter's syndrome Tubular chloride transport Potassium depletion Prostaglandins Renin Norepinephrine Bradykinin Blood vessels Aldosterone 

Renaler Chloridtransport beim Bartter Syndrom

Zusammenfassung

Bei Patienten mit Bartter Syndrom ist die mittels Clearance-Technik gemessene fraktionelle Chloridresorption im dicken aufsteigenden Schenkel der Henleschen Schleife vermindert. Diese Störung ist kennzeichnend für das Syndrom und stellt wahrscheinlich die Ursache der erhöhten Kaliumsekretion bei dieser Erkrankung dar. Der Kaliummangel als Folge exzessiver Kaliumverluste dürfte der Stimulus für die beim Bartter Syndrom beobachtete Steigerung der vaskulären Prostacyclin- und renalen Prostaglandin E2-Synthese sein. Die gesteigerte Prostaglandin-Produktion führt zu Hyperreninämie, erhöhtem Plasma Bradykinin und Noradrenalin sowie zur Gefäßresistenz gegenüber der pressorischen Wirkung von Angiotensin II und Noradrenalin; diese Veränderungen werden durch Behandlung mit Prostaglandin-Synthesehemmern korrigiert. Die Zunahme von Angiotensin II und Noradrenalin erfolgt kompensatorisch zur Aufrechterhaltung des Blutdrucks als Antwort auf die durch vaskuläre Prostaglandine induzierte Gefäßerweiterung. Die Hyperreninämie stimuliert schließlich die Produktion von Aldosteron und führt damit zur Verschlimmerung des Kaliumverlusts.

Schlüsselwörter

Bartter Syndrom tubulärer Chloridtransport Kaliummangel Prostaglandine Renin Noradrenalin Bradykinin Blutgefäße Aldosteron 

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Copyright information

© Springer-Verlag 1982

Authors and Affiliations

  • J. R. GillJr.
    • 1
    • 2
  1. 1.Hypertension-Endocrine BranchNational HeartLung
  2. 2.Blood InstituteNational Institutes of HealthBethesdaUSA

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