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KMID : 0359919990180061008
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Korean Journal of Nephrology
1999 Volume.18 No. 6 p.1008 ~ p.1012
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A Case of Liddle Syndrome Associated with Torsades de pointes
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윤세정/Se Jung Yoon
신재호/강태수/황학직/박홍수/강신욱/최규헌/하성규/이호영/한대석/Jae Ho Shin/Tae Soo Kang/Hak Jin Hwang/Hong Su Park/Shin Wook Kang/Kyu Hun Choi/Sung Kyu Ha/Ho Yung Lee/Dae Suk Han
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Abstract
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-Abstrat-
Liddle syndrome is a rare cause of hypokalemic hypertension and caused by renal
tubular sodium channel defect resulting in excessive sodium absorption, potassium
wasting and metabolic alkalosis. Clinically this syndrome resembles the primary
aldosteronism, however, aldosterone and renin secretion are markedly suppressed due to
chronic state of volume expansion. This syndrome is transmitted in an autosomal
dominant pattern. We have experienced a case of Liddle syndrome, a 74 years old
female accompanying severe hypokalemia, long-standing hypertension, metabolic alkalosis
and suppressed aldosterone and renin level in serum and urine. She had a history of
arrhythmia torsades de pointes, of unknown cause. We believe that the arrhythmia
resulted from severe hypokalemia secondary to this syndrome. Two of her siblings died
suddenly. probably from cardio-, cerebrovascular accidents. Five her offspring needed to
be evaluated for this syndrome due to its autosomal dominant inheritance.
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KEYWORD
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