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“Chloride-shunt” syndrome: An overlooked cause of renal hypercalciuria

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Abstract

The case of a 7-year-old boy with the normotensive form of “chloride-shunt” syndrome is described. An unusual feature was the clinical presentation with lithiasis, caused by marked hypercalciuria of renal origin. The present studies were carried out to investigate the nature of the renal tubular defect. Indices for proximal and distal sodium chloride reabsorption were increased during hypotonic saline diuresis. Baseline sodium chloride excretion was low but increased above the range of control values after acute furosemide administration. Baseline potassium excretion was low, was not modified by the infusion of sodium chloride and increased significantly during infusions of sodium sulphate or sodium bicarbonate. Calcium excretion remained unchanged during sodium chloride, sodium sulphate or sodium bicarbonate infusions, but increased after furosemide administration. Nasal insufflation of 1-desamino-8-d-arginine-vasopressin induced both an increase in potassium excretion and a decrease in calcium and magnesium excretion. Plasma atrial natriuretic peptide was increased and was not significantly modified by infusion of hypertonic saline or acute administration of furosemide. These findings indicate that the primary renal abnormality appears to be an enhanced tubular reabsorption of sodium chloride, apparently present in the proximal tubule and the ascending loop of Henle. The associated presence of hypercalciuria also suggests a transport defect in the distal tubule. Decreased potassium excretion probably depends on a voltage-shunting defect in the cortical collecting tubule, which can be reversed by increasing the delivery of non-reabsobable anions or by enhancing the conductance of the luminal membrane. Treatment with hydrochlorothiazide was successful in correcting most biochemical abnormalities but did not result in catch-up growth.

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Correspondence to Juan Rodríguez-Soriano.

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Rodríguez-Soriano, J., Vallo, A. & Domínguez, M.J. “Chloride-shunt” syndrome: An overlooked cause of renal hypercalciuria. Pediatr Nephrol 3, 113–121 (1989). https://doi.org/10.1007/BF00852890

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Key words

  • Renal tubular disorders
  • “Chlorideshunt” syndrome
  • Hyperkalaemia
  • Hypercalciuria
  • Atrial natriuretic peptide
  • Antidiuretic hormone