Hypertension with Hypokalemia is seen in –
Correct Answer: Liddle's syndrome
Description: Ans. is 'b' i.e., Liddle's syndrome o Liddle's Syndrome is typically associated with Hypokalemia and Hypertension,# Bartter's Syndrome and Gitelman's Syndrome are also associated with hypokalemia but without hypertension.Liddle's syndromeo Liddle's syndrome is a rare autosomal dominant condition in which there is a primary increase in collecting tubule sodium reabsorption and, in most cases, potassium secretion.o Affected patients, typically present with:-# Hypertension# Hypokalemia# Metabolic alkalosisPathogenesiso The genetic abnormality in Liddle's syndrome involves mutations in genes on chromosome 16p l2 that encode the beta and gamma subunits of the collecting tubule sodium channel, which are called SCNN1B and SCNN1G, respectively.o Deletions or substitutions in a short proline-rich segment of the intracytoplasmic C-terminus cause an inability of these subunits to bind with an intracellular ubiquitin protein ligase (Nedd4) that normally removes the luminal sodium channel from the cell surface.o Failure to remove sodium channels results in an inability to reduce their number in response to low levels of aldosterone that result from the volume expansion-mediated suppression of renin secretion.o These mutations result in a "gain-of-function" that mimics the effects of hyper aldosteronism. Diagnosis of Liddle's syndromeConsists of Triad of||||HypertensionHypokalemiaAlkalosisAlong witho Low plasma renin activityo Reduced plasma aldosteroneo Reduced urinary aldosterone
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