Abstract
At 1 month of age, dizygotic twins presented with failure to thrive, hyponatremic dehydration, and hyperkalemia. Renal function and glucocorticoid production were normal. Routine fluid and electrolyte replacement failed to correct the dehydration, and the administration of desoxycorticosterone gave no response. Urinary aldosterone levels were consistently elevated, and urinary excretion of sodium was disproportionately high. The patients responded to a daily supplementary intake of 3 g of sodium chloride, with correction of the hyponatremia and dehydration and return of serum potassium concentrations to normal. Salt supplementation was discontinued after 15 months. The twins continued to thrive on regular diet and maintained normal levels of serum sodium and potassium, yet their urinary and plasma aldosterone levels remained high. The observations suggest that the defect (1) may be familial and genetic, (2) may be caused by failure of the renal tubules to respond to aldosterone, and (3) is correctable by increased intake of sodium chloride.
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