Severe Hypertension with Hypokalemia and Nephroangiosclerosis Due to Liddle Syndrome’s Mutation —Liddle Syndrome Nephroangiosclerosis

Abstract

Background: Liddle syndrome (LS) is a rare autosomal-dominant cause of early-life hypertension. It is associated with hypokalemic metabolic alkalosis, hyporeninemia, and suppressed aldosterone secretion. Its morbidity and mortality are associated with hypertension and hypokalemia. The Case : An 18-year-old man with severe hypertension, hypokalemia and renal failure for years. He was treated with multiple antihypertensive drugs yet without adequate control. In this patient; LS was confirmed by biochemical and hormonal profiles as well as genetic testing. His close family-members were clinically normal and by genetic testing indicating a new mutation in our patient. He was subjected to kidney biopsy since he had; high serum creatinine at 140 umol/L and bilateral small kidneys at 9 cm, in longitudinal diameter, with thin and echogenic cortex. It showed moderate nephroangiosclerosis. Initially, he was treated with low-salt diet, Amiloride 20 mg daily, slow K and antihypertensives (Amlodipine 10 mg daily and alpha methyl dopa 250 mg twice daily). Subsequently, antihypertensives were reduced to only Amlodipine 5 mg daily. He remained stabilized her disease up to 2 years of follow-up. Conclusion: LS should be considered in hypertensive children with hypokalemia since it requires special management to avoid its hypokalemic and cardiovascular complications as well as nephroangiosclerosis.