Hypercalciuria with Bartter syndrome: evidence for an abnormality of vitamin D metabolism.
Some children with Bartter syndrome have hypercalciuria. To determine the mechanism for this phenomenon, we studied tubular function and calcium metabolism in six such children. All patients had hypokalemic alkalosis, normotension, hyperreninemia, growth retardation, low fractional distal chloride reabsorption (4/5), and elevated urinary prostaglandin E2 excretion (5/6). In addition, all had hypercalciuria (urinary calcium 6.5 to 25.0 mg/kg/day), with evidence of nephrocalcinosis in five. None, however, had evidence of rickets or hyperparathyroidism. There was a marked elevation in the serum concentration of 1,25-dihydroxyvitamin D in all, and four patients had a response to oral calcium loading suggestive of absorptive hypercalciuria. Five children have had long-term therapy with indomethacin. They have had improvement in hypokalemia and reduced urinary prostaglandin E2 excretion as well as reductions in the serum concentration of 1,25-dihydroxyvitamin D and in urinary calcium excretion. These data suggest that hypercalciuria in some children with Bartter syndrome is associated with an excess of 1,25-dihydroxyvitamin D. The improvement in hypercalciuria with prostaglandin synthesis inhibition may result in part from correction of this vitamin D abnormality.
Restrepo de Rovetto
C
C
Children's Hospital Medical Center, Cincinnati, Ohio.
Welch
T R
TR
Hug
G
G
Clark
K E
KE
Bergstrom
W
W
D001477:Bartter Syndrome / Q000150:complications*; D002128:Calcium Metabolism Disorders / Q000209:etiology* / Q000503:physiopathology; D002675:Child, Preschool; D002712:Chlorides / Q000493:pharmacokinetics; D015232:Dinoprostone / Q000378:metabolism; D006801:Humans; D006929:Hyperaldosteronism / Q000150:complications* / Q000453:epidemiology; D007213:Indomethacin / Q000627:therapeutic use; D007223:Infant; D007684:Kidney Tubules / Q000503:physiopathology; D008297:Male; D011188:Potassium / Q000493:pharmacokinetics; D012083:Renin / Q000378:metabolism; D014807:Vitamin D / Q000378:metabolism*
D002712:Chlorides; D014807:Vitamin D; D012083:Renin; D015232:Dinoprostone; D011188:Potassium; D007213:Indomethacin