Abstract
Bartter's Syndrome: 10 cases in childhood. Results of long term Indomethacin therapy including effects on Prostaglandins in urine.
Ten children with Bartter's Syndrome were studied. Their ages at diagnosis ranged from 3 months to 15 years. There was an equal sex distribution. A wide spectrum of severity in terms of clinical and biochemical features was found. In addition to those findings considered diagnostic of the condition, some patients were shown to have hypercabaemia, hypophosphataemia, hypercalciuria, nephrocalcinosis, rickets and urine acidfioation defects. Two affected children were siblings. Six children were treated over prolonged periods (6 - 24 months) with Indomethacin. The effects of treatment were monitored in several ways, including regular measurement of PRA, plasma aldosterone, G.F.R. and PGE2, PGF2α and PGFM in urine. Remarkable clinical and biochemical improvement was documented including catch-up growth in all cases. In spite of this, PG findings in urine were inconsistent and tolerance to Indomethacin appeared to develop in some children. Although PG synthetase inhibitors have revolutionized treatment of Bartter's Syndrome it is questioned whether excess renal PG production is the primary cause of the disorder of just another epi-phenomenon.
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Dillon, M., Shah, V. & Mitchell, M. Bartter's Syndrome: 10 cases in childhoods. Results of long term Indomethacin therapy including effects on Prostaglandins in urine: 46. Pediatr Res 14, 173 (1980). https://doi.org/10.1203/00006450-198002000-00073
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DOI: https://doi.org/10.1203/00006450-198002000-00073