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Article
April 1978

Familial Hyperkalemia, Hypertension, and Hyporeninemia With Normal Aldosterone LevelsA Tubular Defect in Potassium Handling

Author Affiliations

From the Department of Nephrology, VA Wadsworth Hospital Center, and the University of California at Los Angeles, Los Angeles (Drs Brautbar and Kleeman) and the Department of Medicine "D" and Chemical Endocrinology, Hadassah Medical Center, Jerusalem (Drs. Rosier and Leitesdorf); and the Department of Medicine A and Renal Unit, Hasharon Hospital, Petah-Tikva, Israel (Drs Levi, Djaldeti, and Epstein).

Arch Intern Med. 1978;138(4):607-610. doi:10.1001/archinte.1978.03630280069022
Abstract

A 52-year-old man had hypertension, persistent hyperkalemia, and hyperchloremic metabolic acidosis; renal and adrenal functions were normal. Four other members of the family have the same findings. The patient's plasma aldosterone (PA) level was within normal range, though plasma renin activity (PRA) was undetectable. The ability to conserve sodium with increased endogenous aldosterone levels, and the inability to increase potassium excretion while exogenous mineralocorticoid (fludrocortisone acetate) was administered, indicated a distal tubular defect in potassium handling. Effective reduction of the hyperkalemia by K+-Na+ exchange resin also corrected the acidosis and the hyperchloremia, suggesting that hyperkalemia may cause metabolic acidosis.

(Arch Intern Med 138:607-610, 1978)

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