BARTER'S SYNDROME: EFFECTS OF LONG-TERM TREATMENT WITH PROPRANOLOL (P) AND IBUPROFREN (I)

Abstract

Two brothers (RB age 19, JB age 9) with a three-year history of hypokalemic alkalosis, increased peripheral renin activity (PRA), normotension, and decreased growth rate (GR) were treated with P (2 mg/kg). This resulted in a marked decrease in PRA and an 8-20% increase in plasma volume as measured by ¹²⁵I-tagged albumin. Serum K was unaffected but when spironolactone and K supplements (600 meq/day) were added, it was maintained above 3.0 meq/1. One year later I was added to this regimen resulting in a further decrease in PRA. K supplements were discontinued but serum K remained above 3.0 meq/1. Prior to therapy with P and Iw, growth rates were 3.0-5.0 cm/year. Both have grown at 7.0-12.0 cm/year since therapy was initiated. Results are shown below:

It is concluded that I is useful in the long-term therapy of Barter's syndrome and that the growth rate can be normalized if K balance is restored.

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