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We report about a girl, 22 months old, with pseudohypoaldosteronism. The clinical course was characterized mainly by renal salt loosing syndrome, complicated by suddenly occurring hyperkalemic phases and a complete inability of renal electrolyte and acid-base regulation. Congenital adrenal hyperplasia was excluded and the diagnosis based on renal salt wasting despite hyperaldosteronaemia and increased plasma renin activity. Diagnosis and therapy of pseudo-hypoaldosteronism are discussed as well as the problems of long-term oral sodium chloride substitution and long-term therapy with sodium-polystyrol-sulfonate, a cation-exchange resin.
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