Bartter's syndrome: 10 cases in childhood. Results of long-term indomethacin therapy

Abstract

Ten children with Bartter's syndrome are described. Their ages at diagnosis ranged from three months to 15 years and there was an equal sex distribution. A wide spectrum of severity of clinical and biochemical features was found. Hypercalcaemia, hypophosphataemia, hypercalcuria, nephrocalcinosis, rickets and urine acidification defects were seen in some patients. Two affected children were siblings. Six children were treated over periods of six to 24 months with indomethacin with remarkable clinical and biochemical improvement. Catch-up growth was demonstrated in all treated cases. Tolerance to indomethacin appeared to develop in some children. Only one serious complication was seen with this therapy, a duodenal ulcer in a child on high dosage. Of those children not treated with indomethacin, one died, one is now on indomethacin elsewhere and two are well without therapy.