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백운희 ( Baeg Un Hui ),김영욱 ( Kim Yeong Ug ),홍순창 ( Hong Sun Chang ),박상준 ( Park Sang Jun ),김윤권 ( Kim Yun Gwon ),김소연 ( Kim So Yeon ),김영중 ( Kim Yeong Jung ),조민구 ( Jo Min Gu ),이권전 ( Lee Gwon Jeon ) 대한신장학회 2003 Kidney Research and Clinical Practice Vol.22 No.4
Liddle`s syndrome is a rare inferited disease with characteristic clinical manifestations of hytertension an hypokalemic metabolic alkalosis. Markedly suppressed serum aldosterone and renin levels are important laboratory findings to differentiate this disorder from primary hyperaldosterionism. When Liddle et al. reported the disorder in 1963, they proposed aggressive Na+ absorption and increased excretion of K+ as the pathogenesis of the syndrome. Since then, specific mutaion in the epithelial Na+ channel located in the collecting duct of the kidney has been dlucidated as a disease mechanism. Liddle`s syndrome is inherited by an autosomal dominant trait and generally the onset of the syndrome is before the age of 20 with increased risk of premature death due to stroke or heart failure. Recently, however, a few cases of late onset and genetically proven non-familial cases with de novo mutation of β or γ Na+ channel have been reported. We report a case of seventy-one year old woman who had hypertension with hypokalemic metabolic alkalosis and was diagnosed as Liddle`s syndrome. Further evaluation revealed low renin and aldosterone levels. Primary aldosteronism, Cushing`s syndrome, glucocorticoid remediable aldosteronism and deficiency of 11 β-OHase and 17α-OHase were ruled out based on her laboratory data and history. Her hypertension and hypokalemia responded to amiloride treatment but not to spironolactone. (Korean J Nephrol 2003;22(4):464-468)