A pediatric case of kidney transplantation in Bartter's syndrome with end-stage renal failure

Abstract

We report here on a pediatric case of preemptive kidney transplantation in type IV Bartter's syndrome with end-stage renal failure. The patient was a 15-year-old boy. Oral intake and weight gain in infancy were poor and, at 10 months old, polyposia and polyuria were evident. Laboratory data documented hypokalemia, a high level of plasma renin activity, and moderate renal insufficiency at 1 year 9 months old, which established the diagnosis of Bartter's syndrome with sensorineural deafness (type IV Bartter's syndrome). He was treated with indomethacin, spironolactone and potassium chloride orally thereafter. Renal function had been deteriorating since puberty, and he was referred to our hospital for kidney transplantation in April 2004. Although we intended to perform living-related kidney transplantation for him in November 2004, blood urea nitrogen (156 mg/dL) and serum creatinine (17.2 mg/dL) levels became elevated in October. Therefore, we began hemodialysis before kidney transplantation to improve his general condition. Kidney transplantation from his mother, who was forty years old, was successfully performed. We administered enalapril maleate and losartan potassium preoperatively, although the levels of hormones involved in the renin-angiotensin-aldosterone system were normal preoperatively. After transplantation, renal function improved and there were no abnormalities in the renin-angiotensin-aldosterone system. Chronic renal failure rarely develops in Bartter's syndrome. Theadministration of NSAIDs, which is often recommended to treat Bartter's syndrome, may promote renal dysfunction, as does the disease itself. Moreover, we consider kidney transplantation a useful treatment for Bartter's syndrome patients with chronic renal failure because not only renal function but also endocrine abnormalities caused by Bartter's syndrome improve after transplantation.