Advertisement

The pathogenesis of Bartter's syndrome

Functional and histologic studies
      This paper is only available as a PDF. To read, Please Download here.

      Abstract

      We describe a patient with Bartter's syndrome. In addition to the well-known characteristic findings by light microscopy, electron micrograms confirmed the presence of juxtaglomerular cell hyperplasia with polymorphous renin secretory granules and dense multivesicular bodies. Volume expansion by albumin infusion decreased plasma renin activity and aldosterone excretion, and improved the pressor response to exogenous angiotensin, suggesting that the renin-angiotensin-aldosterone system was not autonomous but that a decreased extracellular volume might be a major defect in this patient. During hypotonic saline diuresis, moreover, fractional free water clearance per fractional distal sodium delivery, Math Eq + CNa, was markedly depressed in the patient when compared with the value in the controls. Evidence presented suggests that chronic extracellular volume depletion exists as a consequence of an impaired sodium transport in the ascending limb of Henle's loop.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to The American Journal of Medicine
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Bartter FC
        • Pronove P
        • Gill Jr, JR
        • et al.
        Hyperplasia of the juxtaglomerular complex with hyperaldosteronism and hypokalemic alkalosis, a new syndrome.
        Am J Med. 1962; 33: 811
        • Cannon PJ
        • Leeming JM
        • Sommers SC
        • et al.
        Juxtagtomerular cell hyperplasia and secondary hyperaldosteronism (Bartter's syndrome): a reevaluation of the pathophysiology.
        Medicine (Baltimore). 1968; 47: 107
        • Brackett Jr, NC
        • Koppel M
        • Randall RE
        • et al.
        Hyperplasia of the juxtaglomerular complex with secondary hyperaldosteronism without hypertension (Bartter's syndrome).
        Am J Med. 1968; 44: 803
        • Goodman AD
        • Vagnucci AH
        • Hartroft PM
        Pathogenesis of Bartter's syndrome.
        N Engl J Med. 1969; 281: 1435
        • Imai M
        • Yabuta K
        • Murata H
        • et al.
        A case of Bartter's syndrome with abnormal renin response to salt load.
        J Pediatr. 1969; 74: 738
        • White MG
        Bartter's syndrome, a manifestation of renal tubular defects.
        Arch Intern Med. 1972; 129: 41
        • Chaimovitz C
        • Levi J
        • Better OS
        • et al.
        Studies on the site of renal salt loss in a patient with Bartter's syndrome.
        Pediatr Res. 1973; 7: 89
        • Modlinger RS
        • Nicolls GL
        • Krakoff LR
        • et al.
        Some observations of the pathogenesis of Bartter's syndrome.
        N Engl J Med. 1973; 289: 1022
        • Osserman EF
        • Lawlor DP
        Serum and urinary lysozyme (muramidase) in monocytic and monomyelocytic leukemia.
        J Exp Med. 1966; 124: 921
        • Langan J
        • et al.
        A simple radioimmunoassay for urinary aldosterone.
        J Clin Endocrinol Metab. 1974; 38: 189
        • Stein RM
        • Abramson RG
        • Kahn T
        • et al.
        Effect of hypotonic saline loading in hydrated dog: evidence for a saline-induced limit on distal tubular sodium transport.
        J Clin Invest. 1967; 46: 1205
        • Catanzaro F
        • Bourgoignie J
        • Serirat P
        • et al.
        Angiotensin-infusion test. Correlation with renin activity in peripheral venous blood.
        Arch Intern Med. 1968; 122: 10
        • Dirks JH
        • Cirskena WJ
        • Berliner RW
        The effect of saline infusion on sodium reabsorption by the proximal tubule of the dog.
        J Clin Invest. 1965; 44: 1160
        • Seldin DW
        • Eknoyan G
        • Suki WN
        • et al.
        Localization of diuretic action from the pattern of water and electrolyte excretion.
        Ann NY Acad Sci. 1966; 139: 328
        • Suki WN
        • Eknoyan G
        • Reactor Jr, FC
        • et al.
        The renal diluting and concentratirg mechanism in hypercalcemia.
        Nephron. 1969; 6: 50
        • Fisher ER
        Lysosomal nature of juxtaglomerular granules.
        Science. 1966; 152: 1752
        • De Duve C
        • Wattiaux R
        Function of lysosomes.
        Ann Rev Physiol. 1966; 28: 435
        • Hatt PY
        The juxtaglomerular apparatus.
        in: Dalton AJ Haguenau F Ultrastructure of the kidney. Academic Press, New York1967: 101
        • Pasternack A
        • Perheentupa J
        • Launiala K
        • et al.
        Kidney biopsy findings in familial chloride diarrhea.
        Acta Endcrinol (Kbn). 1967; 55: 1
        • Bartter FC
        • Delea CS
        • Kawasaki T
        • et al.
        The adrenal cortex and the kidney.
        Kidney Int. 1974; 6: 272