Liddle's syndrome is a rare familial (autosomal dominant) disease characterized by hypertension, hypokalemic metabolic alkalosis, renal K + wasting, and suppressed renin and aldosterone secretion. Increased distal delivery of Na+ with a nonreabsorbable anion (not Cl–) enhances K+ secretion. Classically, this is seen with proximal (type 2)renal tubular acidosis (RTA) and vomiting, associated with bicarbonaturia. Diabetic ketoacidosis and toluene abuse (glue sniffing) can lead to increased delivery of β-hydroxybutyrate and hippurate, respectively, to the CCD and to renal K+ loss. High doses of penicillin derivatives administered to volume-depleted patients may likewise promote renal K+ secretion as well as an. | Chapter 046. Sodium and Water Part 14 Liddle s syndrome is a rare familial autosomal dominant disease characterized by hypertension hypokalemic metabolic alkalosis renal K wasting and suppressed renin and aldosterone secretion. Increased distal delivery of Na with a nonreabsorbable anion not Cl- enhances K secretion. Classically this is seen with proximal type 2 renal tubular acidosis RTA and vomiting associated with bicarbonaturia. Diabetic ketoacidosis and toluene abuse glue sniffing can lead to increased delivery of 0-hydroxybutyrate and hippurate respectively to the CCD and to renal K loss. High doses of penicillin derivatives administered to volume-depleted patients may likewise promote renal K secretion as well as an osmotic diuresis. Classic distal type 1 RTA is associated with hypokalemia due to increased renal K loss the mechanism of which is uncertain. Amphotericin B causes hypokalemia due to increased distal nephron permeability to Na and K and to renal K wasting. Bartter s syndrome is a disorder characterized by hypokalemia metabolic alkalosis hyperreninemic hyperaldosteronism secondary to ECF volume contraction and juxtaglomerular apparatus hyperplasia. Finally diuretic use and abuse are common causes of K depletion. Carbonic anhydrase inhibitors loop diuretics and thiazides are all kaliuretic. The degree of hypokalemia tends to be greater with long-acting agents and is dose-dependent. Increased renal K excretion is due primarily to increased distal solute delivery and secondary hyperaldosteronism due to volume depletion . See also Chap. 278. Clinical Features The clinical manifestations of K depletion vary greatly between individual patients and their severity depends on the degree of hypokalemia. Symptoms seldom occur unless the plasma K concentration is 3 mmol L. Fatigue myalgia and muscular weakness of the lower extremities are common complaints and are due to a lower more negative resting membrane potential. More severe hypokalemia may lead to .
