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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.

PROGNOSIS.

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.