Author(s)

Kamel El-Reshaid^1*, Shaikha Al-Bader²

¹Department of Medicine, Faculty of Medicine, Kuwait University, Kuwait City, Kuwait.
²Department of Medicine, Nephrology Unit, Jaber Al-Ahmad Hospital, Ministry of Health, Kuwait City, Kuwait.

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.

Amiloride, Genetic Testing, Hypertension, Hypokalemia, Liddle Syndrome, Nephroangiosclerosis

El-Reshaid, K. and Al-Bader, S. (2025) Severe Hypertension with Hypokalemia and Nephroangiosclerosis Due to Liddle Syndrome’s Mutation
—Liddle Syndrome Nephroangiosclerosis. Open Journal of Nephrology, 15, 152-159. doi: 10.4236/ojneph.2025.152014.