PS-BPR03-5: A CASE OF LIDDLE SYNDROME-LIKE REFRACTORY HYPERTENSION WITH TREATMENT-RESISTANT HYPOKALEMIA COMPLICATED BY NEPHROTIC SYNDROME DUE TO DIABETIC NEPHROPATHY

Abstract

A 62-year-old woman had been treated for diabetic nephrotic syndrome, hypokalemia, and edema with perindopril, furosemide, potassium L-aspartate, valsartan, and spironolactone since January, X. On May 15, X, fatigue and nausea appeared, and on the morning of May 20, these symptoms increased. On the morning of May 20, she visited the hospital. She was urgently admitted to the hospital because of prominent edema in both lower limbs, elevated blood pressure (164/83 mmHg), marked hypokinemia (K 2.4 mEq/L), and metabolic alkalosis (pH 7.612, HCO3- 31.3 mmol/L) in the arterial blood gas. Post-hospitalization examination revealed hyporeninemia and hypoaldosteronemia. Since administration of spironolactone in addition to previous medications failed to improve the hypokaremia and hypertension, triamterene 100 mg was added. The treatment was resulted in rapid hypotension, accompanied by improvement of hypokalemia and metabolic alkalosis. The dose of triamterene was later reduced to 50 mg, and excess blood pressure lowering was improve. Genetic testing did not identify any mutations in the beta or gamma subunits of the ENaC gene. Discussion: In this patient, no hypokalemia was observed in previous examinations. The hypokalemia in this patient was spironolactone-resistant, and the fact that triamterene, a distal tubular epithelial Na channel (ENaC) inhibitor, was effective in this patient; furthermore, no genetic abnormality in ENaC, suggests the possibility of an acquired ENaC abnormality similar to the Liddle syndrome.