Since McQuarrie1 in 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,2,3 with excessive secretion of the adrenal steroids,1,4 and after the administration of various diuretics,5 adrenocorticotropic hormone, or adrenal steroids.6Studies 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
CAMACHO AM, BLIZZARD RM. Congenital Hypokalemia of Probable Renal Origin: A Newly Described Entity Due to an Inherited Metabolic Defect. Am J Dis Child. 1962;103(4):535–555. doi:10.1001/archpedi.1962.02080020548003
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