Successful management of an extreme example of neonatal hyperprostaglandin-E syndrome (Bartter's syndrome) with the new cyclooxygenase-2 inhibitor rofecoxib.

OBJECTIVE

To describe the successful treatment of an unusual case of severe neonatal Bartter's syndrome refractory to treatment with indomethacin.

DESIGN

Case report, clinical.

SETTING

Tertiary care intensive care unit.

PATIENTS: A patient with neonatal hyperprostaglandin-E syndrome and excessive requirements of intravenous (via central venous catheter) water and salt supplementation, failure to thrive, vomiting, and massive growth retardation, despite adequate treatment with indomethacin.

MAIN RESULT: Four weeks after induction of the new cyclooxygenase-2 inhibitor rofecoxib, the patient was well, on full enteral feeds, thriving, and had gained 600 g in weight. A lower supplementary potassium, magnesium, and sodium intake was required. Reinstitution of indomethacin therapy resulted in severe deterioration, despite high indomethacin doses; symptoms improved again after rofecoxib administration. No side effects have been seen thus far.

CONCLUSION

This report shows that in selected patients with a severe form of neonatal Bartter's syndrome, the new cyclooxygenase-2 inhibitor rofecoxib may control the clinical symptoms of hyperprostaglandin-E syndrome after ineffective indomethacin therapy.