Abstract: TH-PO1101

An Unusual Case of Peripheral Edema Due to Suspected Liddle’s Syndrome

Session Information

Category: Fluid, Electrolytes, and Acid-Base

  • 704 Fluid, Electrolyte, Acid-Base Disorders

Authors

  • Ali, Sadeem, ECU, Greenville, North Carolina, United States
  • Radhakrishnan, Sri jegan, ECU, Greenville, North Carolina, United States
  • Obi, Reginald Ifeanyi, ECU Physicians Nephrology, Greenville, North Carolina, United States
Background

Liddle's syndrome is a rare autosomal dominant disease affecting epithelial sodium channels in which there is a primary increase in collecting tubule sodium reabsorption and usually associated potassium wasting. Patient usually presents with hypertension, hypokalemia, and metabolic alkalosis, with an overall clinical picture mimicking mineralocorticoid excess. However, its presentation with a chief complaint of peripheral edema has not been reported to our knowledge.

Methods

We present a 47 years old female who has been hypertensive since the mid thirties of her age. She came for management of hard to control generalized edema, mostly in her lower extremities, for the last three years. No cardiac or liver etiology identified. She had tried several diuretics including hydrochlorothiazide, furosemide, spironolactone and was then on chlorthalidone 50 milligrams daily. She had been profoundly hypokalemic while on diuretics and requiring 80 milli equivalents daily replacement of potassium chloride. Her labs also showed metabolic alkalosis and relatively high sodium levels. We suspected a potassium wasting syndrome. After holding chlorthalidone for three days, with continued potassium replacement, her trans tubular potassium gradient was calculated at 7.9 confirming urinary potassium wasting. Her plasma aldosterone to renin activity ratio was 13.2, making primary hyperaldosteronism unlikely. We started her on amiloride as 5 mg twice a day due to clinical suspicion of Liddle's syndrome.
After three weeks, her swelling has completely resolved and blood pressure also normalized . Her potassium supplementation and all antihypertensive medications were discontinued except for a reduced dose of amiloride. Her labs now show normal potassium and bicarbonate levels. Genetic testing for Liddle's syndrome has not been done in this patient because it will not change management.

Conclusion

Refractory peripheral edema could be an unusual presentation of this rare but treatable condition. Paying close attention for pathophysiologic clues of Liddle’s syndrome will result in more specific management for subsets of patients presenting with edema, refractory to the commonly used loop and thiazide diuretics.