Bartter syndrome in a neonate: early treatment with indomethacin

The neonatal form of Bartter syndrome is characterized by intrauterine onse t of polyuria leading to severe polyhydramnios. We report a patient with th e early onset of the syndrome and a similar history in a previous sibling w ho died in early neonatal life. The patient is a female product of 33 weeks of gestation complicated by severe polyhydramnios. Her birth weight was 2, 100 g. Polyuria led to severe dehydration on the 3rd day of life. Laborator y studies showed hypokalemia, hyponatremia, and elevated plasma levels of r enin and aldosterone. Hy percalciuria was associated with echographic evide nce of nephrocalcinosis. Indomethacin therapy resulted in a significant red uction in urine volume and correction of biochemical abnormalities. Growth and development are satisfactory after 4 years of indomethacin therapy, but nephrocalcinosis remains unchanged.