The goals of therapy are to supply adequate nutrition and to maintain the serum
Potassium level above 3.5 meq/L. Therapy is initiated with oral potassium
Chloride supplementation, increasing the dose until the serum potassium level
Reaches 3.5 meq/L or the dosage reaches 250 meq/24 hr. Reasonably well tolerated
Potassium preparations include K-Lyte/Cl (Mead Johnson Company, Evansville, IN),
Flavored effervescent tablets containing 25 or 50 meq of potassium chloride, and
Micro-K 10 Extencaps (A.H. Robins Company, Richmond, VA). Sodium chloride
Supplementation may also be required in small children. If the serum potassium
Level remains below 3.5 meq/L (mmol/L) after reaching a dose of 250 meq/24 hr of
Potassium chloride, then triamterene, 5-10 mg/kg/24 hr in divided doses, should
Be added. If this fails to resolve the hypokalemia, then indomethacin, 3-5
Mg/kg/24 hr divided into three doses, should be given. Patients receiving
Indomethacin should be monitored for signs of gastrointestinal irritation.
The long-term prognosis of Bartter syndrome is uncertain. Many patients remain
Well, but some cases (especially those with glomerular or interstitial
Abnormalities) progress to renal insufficiency. Despite severe growth
Retardation in infancy, normal stature is ultimately obtained. The suggestion
That mental retardation occurs in patients who have severe disease in the 1st yr
Of life remains to be confirmed.
Madrigal G, Saborio P, Mora F, et al: Bartter’s syndrome in Costa Rica: A
Description of 20 cases. Pediatr Nephrol 11:296, 1997.
Mccredie DA: Variants of Bartter’s syndrome. Pediatr Nephrol 10:419, 1996.
Pollak MR, Delaney VB, Graham RM, et al: Gitelman’s syndrome (Bartter’s variant)
Maps to the thiazide-sensitive cotransporter gene locus on chromosome 16q13 in a.