Congenital Hypokalemia of Probable Renal Origin

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Abstract
Introduction Since McQuarrie1in 1937 described the association of low serum potassium and hypochloremic alkalosis in a patient with Cushing's syndrome, multiple causes of potassium loss and consequent hypochloremic alkalosis have been documented. These electrolyte alterations frequently occur with chronic vomiting and/or diarrhea,2,3with excessive secretion of the adrenal steroids,1,4 and after the administration of various diuretics,5adrenocorticotropic hormone, or adrenal steroids.6 Studies pertaining to another cause of hypokalemic hypochloremic alkalosis are the basis of this report. This electrolyte alteration has probably been present from birth in 2 cousins whose primary renal dysfunction appeared to be an inability to conserve potassium. The signs and symptoms of this inborn error of metabolism closely simulate many of the signs and symptoms of hyperaldosteronism. However, the studies to be presented demonstrate that this congenital hypokalemia is probably of primary renal origin and differs from reported cases of hyperaldosteronism in

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