American Journal of Nephrology
Clinical Study
Distal Nephron Function in Bartter’s Syndrome: Abnormal Conductance to Chloride in the Cortical Collecting Tubule?Colussi G.a · Rombolà G.a · Verde G.b · Airaghi C.a · Loli P.b · Minetti L.aaDivisions of Nephrology and Dialysis and of bEndocrinology, Niguarda-Ca’Granda Hospital, Milan, Italy
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Article / Publication Details
Received: January 16, 1992
Accepted: June 10, 1992
Published online: October 28, 2008
Issue release date: 1992
Number of Print Pages: 11
Number of Figures: 0
Number of Tables: 0
ISSN: 0250-8095 (Print)
eISSN: 1421-9670 (Online)
For additional information: https://www.karger.com/AJN
Abstract
Five patients with the clinical patterns of Bartter’s syndrome underwent a series of clearance studies in order to characterize the underlying tubule defect. Free water generation during maximal water diuresis (Ch2o), expressed as percentage of the distal delivery (Ch2o + Ccl), was lower in the patients (72.5 ± 3.2%) than in controls (84.4 ± 5.5, p < 0.0001). During maximal water diuresis and furosemide administration (40 mg i.v. as bolus), NaCl reabsorption along the diluting nephron segments could be separated into 2 components, that occurring in the loop of Henle (DRNaHL) and that occurring in tubule segments beyond the macula densa (DRNaDT): DRnahl was normal, while DRNaDT was reduced (3.1 ± 0.8 vs. 6.2 ± 2.5 ml/min in controls, p < 0.015). Thus, according to this furosemide protocol, our patients had normal solute reabsorption in the loop of Henle but reduced NaCl reabsorption in tubule segments beyond the macula densa. During 0.9% saline infusion (2 liters in 2 h, after stimulation of distal Na reabsorption with fludrocortisone) fractional excretion (FE) of K showed a linear rise with the increase of FEcI.FEK, however, was much higher in the patients than in controls for every FEci level. In contrast, the infusion of Na2SO4, after fludrocortisone administration, induced similar FEK increases in patients and in controls. Thus, in these patients Na reabsorption in the distal nephron (possibly the cortical collecting tubule) was associated with the generation of a higher than normal electric potential gradient in the presence of CI but not of another poorly reabsorbable anion, such as SO42-. These observations indicate that, in our patients, Henle’s loop function is normal, while the collecting tubule function is abnormal. We suggest that NaCl wasting and enhanced tubular secretion of H+ and K in our patients might result from an abnormally low conductance to CI in distal nephron site(s) where Na reabsorption is electrogenic, possibly the cortical collecting tubule. A larger than normal transtubular electric gradient would be generated by Na reabsorption, causing: (1) a direct stimulation of tubular secretion of K and H+ (leading to hypokalemia and alkalosis) and (2) inhibition of the reabsorption of Na (‘trapped’ into the tubular lumen by electric forces), with consequent extracellular volume contraction, hyperreninemia and hyperaldosteronism.
© 1992 S. Karger AG, Basel
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Article / Publication Details
Received: January 16, 1992
Accepted: June 10, 1992
Published online: October 28, 2008
Issue release date: 1992
Number of Print Pages: 11
Number of Figures: 0
Number of Tables: 0
ISSN: 0250-8095 (Print)
eISSN: 1421-9670 (Online)
For additional information: https://www.karger.com/AJN
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