CLINICAL CHARACTERISTICS OF LIDDLE-LIKE SYNDROME IN THE ELDERLY: A SINGLE-CENTER CASE SERIES

 

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https://storage.unitedwebnetwork.com/files/1099/6875bbc2b2464eb3627ce60c6a98b8f5.pdf
CLINICAL CHARACTERISTICS OF LIDDLE-LIKE SYNDROME IN THE ELDERLY: A SINGLE-CENTER CASE SERIES

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TAKAMOTO
OHSE
TAKAMOTO OHSE takamoto.sea@gmail.com JCHO Tokyo Shinjuku Medical Center Nephrology Tokyo Japan *
Ryunosuke Yoshimura rsk446ra.h@gmail.com JCHO Tokyo Shinjuku Medical Center Nephrology Tokyo Japan -
Takahiro Yoshikawa rohikatawakashiyo@yahoo.co.jp JCHO Tokyo Shinjuku Medical Center Nephrology Tokyo Japan -
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Liddle's syndrome is an early-onset hypertensive disorder inherited in an autosomal dominant manner, fundamentally characterized by increased activity of epithelial sodium channels (ENaC) in the renal collecting ducts. Hypokalemia in the elderly due to a similar mechanism—termed "Liddle's-like syndrome in the elderly"—has been sporadically reported, but its clinical characteristics remain poorly understood.

Over a 36-month period from December 2016 to November 2019, we analyzed hospitalized patients aged 80 or older admitted to our nephrology department or referred from other departments who had serum potassium levels <3.0 mEq/L and urinary potassium concentrations >20 mEq/L. Patients taking medications known to promote potassium excretion were excluded. We analyzed the frequency of such cases and their clinical and laboratory characteristics.

During the study period, 59 inpatients aged 80 or older in our department had serum potassium levels <3.0 mEq/L. Of the 37 non-dialysis patients, 11 cases (29.7%) had urinary potassium concentrations >20 mEq/L and no identifiable drug-induced cause. Including eligible referrals from other departments, 21 cases met the criteria and were analyzed. The mean age was 89.6 years, and all patients exhibited marked emaciation. Three were male. Among the 19 patients who underwent blood gas analysis, all had serum pH >7.4 and HCO₃⁻ ≥27, indicating metabolic alkalosis. None had elevated aldosterone levels, but all had a transtubular potassium gradient (TTKG) ≥5, indicating enhanced aldosterone activity. Hypoalbuminemia (albumin ≤3.0 g/dL) was seen in 86% of cases, and all 18 patients tested for free T3 had low values. While many patients recovered naturally with treatment of the underlying condition, in persistent cases, spironolactone or potassium supplements were ineffective, and potassium levels improved only with triamterene.

Although reports of elderly Liddle's-like syndrome are limited, we encountered 21 cases over a 36-month period. Nearly 30% of non-dialysis patients aged 80 or older with hypokalemia in our nephrology department appeared to have this condition, suggesting that many undiagnosed cases may exist in daily clinical practice. Our study identified three consistent and novel findings—emaciation, hypoalbuminemia, and low T3 syndrome—implying that malnutrition and overall physical decline may contribute. This syndrome should be considered in the differential diagnosis of unexplained hypokalemia, and further evaluation of these new findings in more cases is essential to establish the disease concept.

Kewords