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Physiology · Renal: Liddle syndrome
Sample itemA 16-year-old has hypertension, hypokalaemia and metabolic alkalosis, with low plasma renin and low aldosterone. Spironolactone does not correct the problem. Which drug is likely to work?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Amiloride
Liddle syndrome is a gain-of-function mutation of the epithelial sodium channel (ENaC) in the collecting duct, so sodium is reabsorbed and potassium lost regardless of aldosterone. Direct ENaC blockers (amiloride, triamterene) correct it, while aldosterone antagonists do not because aldosterone is already low.
Option by option
A. Amiloride (correct)
Correct. Amiloride blocks the overactive channel directly.
B. Dexamethasone
Dexamethasone treats glucocorticoid-remediable aldosteronism, in which aldosterone is high.
C. Furosemide
Furosemide would increase distal sodium delivery and worsen potassium loss.
D. Hydrochlorothiazide
A thiazide would also increase potassium wasting and not block ENaC.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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