Restrepo de Rovetto C, Welch T R, Hug G, Clark K E, Bergstrom W
Children's Hospital Medical Center, Cincinnati, Ohio.
J Pediatr. 1989 Sep;115(3):397-404. doi: 10.1016/s0022-3476(89)80838-8.
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.
一些患有巴特综合征的儿童存在高钙尿症。为了确定这一现象的机制，我们对6名此类儿童的肾小管功能和钙代谢进行了研究。所有患者均有低钾性碱中毒、血压正常、高肾素血症、生长发育迟缓、远端肾小管氯重吸收分数降低（4/5）以及尿前列腺素E2排泄增加（5/6）。此外，所有患者均有高钙尿症（尿钙6.5至25.0mg/kg/天），其中5例有肾钙质沉着症的证据。然而，无一例有佝偻病或甲状旁腺功能亢进的证据。所有患者的血清1,25 - 二羟维生素D浓度均显著升高，4例患者对口服钙负荷有反应，提示吸收性高钙尿症。5名儿童长期接受吲哚美辛治疗。他们的低钾血症有所改善，尿前列腺素E2排泄减少，血清1,25 - 二羟维生素D浓度和尿钙排泄也有所降低。这些数据表明，一些患有巴特综合征的儿童的高钙尿症与1,25 - 二羟维生素D过量有关。抑制前列腺素合成后高钙尿症的改善可能部分是由于这种维生素D异常的纠正。
