title: Bartter syndrome: benefits and side effects of long-term treatment.
abstract: The present study reports clinical and laboratory data of patients with Bartter syndrome at diagnosis and follow-up with emphasis on the long-term benefits and side effects of the pharmacological therapy, which includes indomethacin and potassium supplementation. We followed 12 children, 6 boys, with a median age at diagnosis of 24.5 months (range 7-137 months) and at the end of the study 157.5 months (range 26.0-224.0 months). All children presented with polyuria and polydipsia, dehydration, and metabolic and electrolyte disturbances with failure to thrive. However, at study entry 5 of 12 patients also had hypophosphatemia, which disappeared after a mean time of 50+/-22.4 months, 3 of 12 had nephrocalcinosis, and 2 of 12 had typical renal cysts. Despite treatment, hypokalemia was persistent in some patients. During long-term follow-up we observed recovery of growth velocity and adequate metabolic and electrolyte balance. However, we noticed renal and gastrointestinal complications: 2 patients had a perforated gastric ulcer, 1 had a gastric ulcer, and gastritis was detected in 3 children. A decreased glomerular filtration rate was observed in 2 patients during follow-up. Our data emphasize the need for regular surveillance of renal function and gastrointestinal endoscopy in these patients. As an alternative to indomethacin, we present our satisfactory preliminary results with rofecoxib.
authors.affiliation: Pediatric Nephrology Unit, Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, Brazil. vaisbich@terra.com.br
authors.forename: Maria Helena
authors.initials: MH
authors.lastname: Vaisbich
authors.forename: Maria Danisi
authors.initials: MD
authors.lastname: Fujimura
authors.forename: Vera H
authors.initials: VH
authors.lastname: Koch
mesh_terms: D001477:Bartter Syndrome / Q000188:drug therapy*; D002648:Child; D002675:Child, Preschool; D016861:Cyclooxygenase Inhibitors / Q000009:adverse effects / Q000627:therapeutic use*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007213:Indomethacin / Q000009:adverse effects / Q000627:therapeutic use*; D007223:Infant; D007783:Lactones / Q000009:adverse effects / Q000627:therapeutic use*; D008297:Male; D011188:Potassium / Q000627:therapeutic use*; D013450:Sulfones / Q000009:adverse effects / Q000627:therapeutic use*; D013997:Time Factors
chemical_list: D016861:Cyclooxygenase Inhibitors; D007783:Lactones; D013450:Sulfones; C116926:rofecoxib; D011188:Potassium; D007213:Indomethacin