Hypertension, hypokalemia and hypoaldosteronism with suppressed renin: a clinical study of a patient with Liddle's syndrome.

Abstract

A 24 yr old woman with hypertension, hypokalemic alkalosis, low plasma renin and hypoaldosteronism was studied. Plasma aldosterone, renin and K returned to normal and blood pressure fell after Na restriction of the administration of triamterene. Thiazide therapy also normalized her blood pressure while dexamethasone, spironolactone and furosemide did not improve her symptoms. Plasma aldosterone levels were low and responded poorly to a short-term ACTH injection, but responded well to the maximal adrenal stimulation by ACTH-Z. Plasma levels of cortisol, corticosterone and deoxycorticosterone were within normal range. Adrenal scintigram with 131I-aldosterol and abdominal computed axial tomography did not reveal the presence of a sizeable adrenal tumor. Urinary kallikrein excretion was low after Na restriction and showed no response to saline infusion. Excessive secretion of unusual mineralocorticoids may not exist in this case. From these observations and the results of the therapeutic responses to the diuretic agents, the primary cause of the disorder of this patient seems to be a renal defect in the distal tubule in handling Na and K which is similar to that in Liddle''s syndrome.