Renal tubular disease with muscle paralysis and hypokalemia

Abstract

Ten patients who were hospitalized because of flaccid quadriplegia and hypokalemia were found to have renal tubular disease. Nine had typical renal tubular acidosis with hyperchloremia and persistently alkaline urine. One had an as yet poorly understood renal tubular disease characterized by excessive renal sodium, potassium and chloride loss, alkalosis, hydronephrosis, nephrocalcinosis and mental deficiency. Balance studies in several patients demonstrated a large negative potassium balance with abnormal urinary potassium losses even during hypokalemic episodes. Other clinical and laboratory features included polyuria, polydipsia, nephrocalcinosis, hyposthenuria, hypouricemia and pyelonephritis, with occasional findings, during the hypokalemic phase, of normocalcemic tetany, hyperdynamic circulation, pulsus alternans and hypophosphatemia. Of possible etiologic significance was the presence or history of pyelonephritis in eight patients. Histamine achlorhydria in one patient studied suggested a possible generalized defect in carbonic anhydrase activity. This patient's red blood cells were found to contain normal carbonic anhydrase activity, and her arterial alveolar pCO 2 gradient was normal at rest and after exercise. Although these results fail to support a generalized deficiency of this enzyme, they do not exclude a localized deficiency within the renal tubular cells. Each patient responded well to potassium and alkali therapy and paralysis has not recurred. Renal function as measured by blood non-protein nitrogen, phenolsulfonphthalein excretion, urinalysis and intravenous pyelograms has remained normal in all subjects, six of whom have been under observation for five years or longer. Three patients no longer require potassium supplements, and one patient appears to have had a complete remission from her renal tubular disease.